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pmc-6080063-1 | A 33-year-old female patient visited the Faculty of Dentistry Clinic of the Pontifical Catholic University of Rio Grande do Sul, in Porto Alegre City, Brazil, because she was dissatisfied with the aesthetics of her smile and felt uncomfortable with the appearance of her two upper central incisors and two upper lateral incisors.
In the anamnesis, the patient reported that she had pain in the cervical region, a feeling of fatigue in the face, and the presence of tooth wear incompatible with her age; there was also an emotional component because she did not like her smile. The patient also reported that she did not use any bruxism night guard. During the clinical examination, moderate dental dimming, the presence of acid erosion on the buccal surfaces of the four upper incisors, and severe wear on the incisal edge region of the same teeth were observed (Fig. ).
After a careful clinical evaluation and radiographic examination, and according to the patient’s main complaint, functional and aesthetic reestablishment were planned as follow: dental bleaching of both arches; gingivoplasty of upper right central incisor, upper left central incisor, upper right canine, and upper left canine (upper right and left lateral incisors did not undergo surgical intervention); ceramic laminates for upper central incisors and upper lateral incisors; and a bruxism night guard.
After the patient agreed with the proposed treatment, the external bleaching was performed using a homemade technique with trays and 7.5% hydrogen peroxide (White Class / FGM Dental Products, Joinville, SC, Brazil). Initially, the tooth color was recorded using the Vita scale (Vita Toothguide, Zahnfabrick, Bad Sackingen, Germany), which was defined as A2 in the incisor region and A3 in the canine region (Fig. ).
The patient was instructed to use the silicone trays made in a thermo-vacuum apparatus with 7.5% hydrogen peroxide gel, one hour per day, for four weeks (Fig. ).
The gel was applied inside of the tray corresponding to the buccal face of the upper and lower teeth. After completion of the bleaching, a color registration was performed and a reduction of the tooth saturation level was observed. The color B1 was defined in the selection phase for the lithium disilicate ceramic restorations (IPS e.max, Ivoclar Vivadent, Schaan, Liechtenstein).
A zirconia bur (Tissue Trimmer, NTI, Kahla, Thuringia, Germany) was used to perform the gingivoplasty on upper right central incisor, upper left central incisor, upper right canine, and upper left canine; its function was to remove excess gum tissue through immediate cauterization and cutting, without requiring any type of suture. The spear-shaped zirconia tip was positioned perpendicularly to the long axis of the tooth, and the gingivoplasty was performed carefully and delicately. The seven-day postoperative period showed well-healed tissue, with no sign of edema, redness, or any inflammatory signal (Fig. ).
After 30 days, two impressions were obtained with irreversible hydrocolloid (Hydrogum, Zhermack, Rome, Italy) to perform diagnostic waxing. In the same session, initial photographs were taken to communicate with the laboratory and determine the final format of the teeth to be restored. The waxing model lets the dentist obtain the final anatomy reference of the teeth to be restored. Still in the preliminary laboratory phase, a guide was prepared with a vinyl polysiloxane impression material (Variotime, Heraeus Kulzer, Hanau, Hesse, Germany), which was used to guide the preparation of both the laminates and the provisionals with bis-acryl resin (Protemp, 3M-ESPE, Saint Paul, MN, USA).
Before beginning preparation for the four upper incisors, # 000 and #0 gingival retraction cords (Ultradent, Oraltech, Indaiatuba, SP, Brazil) were used to protect the gingival sulcus with a hemostatic solution (Hemostop, Dentsply, York, USA). Initially, the teeth wear was performed using a coarse chamfer diamond bur # 4138 (Kg Sorensen, Cotia, SP, Brazil) and completed with the same diameter tips of medium grit. Next,1.5 mm incisal wear at 45˚ was performed and completed with medium-sized aluminum oxide pop-on discs (Sof-lex, 3M-ESPE, Saint Paul, MN, USA) (Fig. ).
In the same session, the simultaneous double impression technique was performed in which the second gingival retraction cord # 0 was removed, enabling the first cord # 000 to maintain the gingival sulcus hemostasis. The impression was performed with a vinyl polysiloxane impression material (Variotime). In the sequence, the provisionals were adjusted and fixed through a silicone guide and bis-acryl resin. The ceramic material chosen was lithium disilicate (IPS e-max, Ivoclar-Vivadent, Schaan, Liechtenstein) (Fig. ).
After the removal of the provisionals, the restorations were placed on the prepared teeth to evaluate the marginal adaptation of the restorations, and after the patient’s approval, the final cementation of the restorations with resin cement shade A1 was performed (RelyX Veneer, 3M-ESPE, Saint Paul, MN, USA).
After the occlusal adjustment, an irreversible hydrocolloid impression was performed to produce a hard acrylic night guard (Fig. ), following the Michigan Protocol. The guard was made as soon as possible to avoid the occurrence of injuries to the restorations, such as cracks, splinters in the incisal edge or even fractures. After the device was made, the occlusal adjustment was performed in left/right laterality, protrusion and Maximum Habitual Intercuspation (MIH).
Periodic maintenance appointments were scheduled every six months, which included prophylaxis and revision of the night guard. The gingival tissue remained intact, with no inflammatory signal at the probe, and the visual examination revealed a light pink and orange peel appearance, typical of healthy gingival tissue (Fig. ). |
pmc-6080074-1 | A 20-year-old Caucasian female, with a past medical history of hypothyroidism
(currently on levothyroxine sodium 50 µg/day) and tobacco use (5 pack-year),
presented in the ED of a university hospital in western Greece with acute dyspnea.
One year before the current presentation, the patient underwent spirometry,
bronchoscopy, chest X-ray, and computed tomography (CT) scan, investigating a
persistent unproductive cough. At that time PLCH was diagnosed based on cytology,
molecular analysis, and immunohistochemical staining of bronchoscopic material.
Initial clinical assessment in the ED revealed respiratory distress with dyspnea,
tachypnea (respiratory rate >24 breaths per minute), hypoxemia (PO2 =
56 mm Hg on room air), and stable hemodynamic status. Physical examination revealed
diminished chest wall movements bilaterally, along with a hyperresonant percussion
note bilaterally in the upper and mid zones. Chest auscultation revealed absence of
air entry bilaterally in the upper-mid zones and substantially reduced in both the
lower zones anteriorly and posteriorly. Furthermore, she had a palpable purpuric
rush on the medial surface of the tibia bilaterally. The remainder of the
examination was unremarkable. Chest X-ray showed bilateral pneumothorax and
intercostal drainage tubes were inserted ( and ). High-resolution computed tomography scan
of the chest showed multiple small, thin-walled, well-defined, rounded cysts evenly
distributed throughout both lungs (), subcutaneous emphysema in the left hemithorax, and
atelectasis in both lower lobes. Blood tests results on admission revealed
leukocytosis, mild anemia, but no major biochemical abnormalities.
The patient underwent bilateral staged thoracoscopic bullectomy followed by
mechanical abrasion of the parietal pleura and chemical pleurodesis with talc. The
postoperative course was uneventful. Five days later, chest X-ray showed complete
lung expansion. Postoperatively, she was treated with paracetamol (500 mg tds [3
times daily] intravenously [IV]), ampicillin/sulbactam (3 g qds [4 times daily] IV),
ranitidine (150 mg bd [twice daily] IV), and levothyroxine sodium (50 µg od [once
daily] po [orally]). The patient recovered sufficiently and was discharged from the
hospital on postoperative day 5 with advice for smoking cessation and referral to
the pulmonology outpatient clinic.
A week later, the patient presented in the ED with mild, right, paravertebral chest
pain and respiratory distress (respiratory rate = 23 breaths per minute,
PO2 = 65 mm Hg on room air). Chest X-ray showed right-sided
pneumothorax. An 18-French chest tube was inserted, and the lung was fully
re-expanded. Three days later chemical pleurodesis, with tetracycline through the
chest tube, was carried out. The chest tube was removed within 24 hours and the lung
remained expanded in follow-up chest X-rays. During hospitalization, the patient’s
hemodynamic and respiratory status remained stable, laboratory testing did not
reveal any abnormalities, and she was discharged to home 4 days later, completely
asymptomatic, with radiologic confirmation of complete lung expansion. |
pmc-6080400-1 | A thirty-year-old male presented with fever and non-productive cough for 2 weeks with exertional dyspnoea. He had hypertension and renal impairment due to mesangioproliferative glomerulonephritis, diagnosed 3 months prior to current presentation, and was on bisoprolol, prazosin and prednisolone (0.5 mg/kg/day). Renal biopsy had not shown evidence of vasculitis.
On examination he was emaciated (BMI 18 kg/m2), febrile (38.2 °C), and had coarse crackles over right upper lung. Other system examinations were unremarkable.
The patient had a pancytopaenia with neutropaenia (neutrophil count 780 / mm3) and dysplastic hypoproliferative bone marrow. Peripheral blood detected cytomegalovirus (29,000 copies per microliter by PCR) and anti-CMV IgM was positive. After 21 days of ganciclovir (100 mg daily IV), CMV viral load became undetectable and pancytopaenia was corrected.
Contrast enhanced CT-chest showed right upper lobe consolidation with cavities (Fig. ). Sputum smear for acid fast bacilli, culture and PCR for mycobacteria (Xpert MTb/RIF) were negative. However, tuberculosis PCR (IS6110 method) performed on his bone marrow aspirate was positive. Therefore, anti tuberculous therapy with isoniazid, rifampicin, ethambutol and pyrazinamide were commenced for disseminated tuberculosis. But, the response was poor.. Aspergillus fumigatus was detected on bronchoalveolar lavage wet smear and culture. Both lavage and serum were positive for galactomannan antigen. Therefore voriconazole 500 mg twice daily (oral) was commenced.
On sixth week of voriconazole, the patient developed acute bilateral lower limb ischaemia. Two emboli occluding bilateral popliteal arteries were extracted during emergency embolectomy (Fig. ). Histology of emboli showed fungal filaments and culture isolated Aspergillus fumigatus. Trans-esophageal echocardiogram and magnetic resonance imaging (MRI) of the aorta were normal. However, MRI abdomen incidentally detected a large splenic abscess (Fig. ).
Despite treatment with voriconazole for further 2 weeks, the splenic abscess enlarged and treatment was switched to liposomal amphotericin 3 mg/kg/day intravenously. Four weeks later he developed acute left upper motor neuron type facial nerve palsy. MRI brain showed multiple small focal lesions in capsular and cortical regions suggestive of fungal embolism (Fig. ). The neurological deficit completely resolved within a week.
Percutaneous aspiration under ultrasound guidance and 6 weeks of amphotericin failed to clear the splenic abscess. Therefore the patient underwent elective splenectomy. Histology of the splenic tissue showed fungal filaments within the abscess as well as caseating granulomas suggestive of tuberculosis of spleen (Fig. ).
Post-operative period was complicated with left Lower lobe pneumonia and parapneumonic effusion, from which he recovered with antibiotics and supportive measures. Subsequently, fever resolved and clinical status improved (see Additional file for the timeline of disease evolution).
Due to the unusual constellation of infections (CMV, tuberculosis and aspergillosis) the patient was screened for an immune deficiency state which revealed B cell, IgM and IgA deficiencies and impaired T cell proliferation with concanavalin A (Table ). Steroids were tapered and stopped 3 months after the current presentation. Six months later above immune defects returned to normal. Autoantibody profile (ANA, ANCA, rheumatoid factor and extracatable nuclear antibody profile) and HIV screening were negative on two occasions 4 months apart. Repeated imaging, bone marrow trephine and tumor screening with tumor markers did not identify any neoplasms. |
pmc-6080506-1 | The grafts came from a 50-year-old man with a history of alcoholic cirrhosis, several episodes of spontaneous bacterial peritonitis requiring antimicrobial treatment, and a recent admission due to acute alcoholic hepatitis that was treated with high-dose corticosteroids. Fifteen days after discharge he was readmitted with acute liver failure that again required corticosteroids and a relapse of C. difficile infection. On day 7 after admission, he presented with fever and an acute neurologic event requiring ICU admission and intubation. A CT scan demonstrated bilateral intraparenchymal hematomas with uncal herniation and new bilateral lung infiltrates.
Bronchoalveolar lavage (BAL) culture revealed extended-spectrum beta-lactamase producing (ESBL) Klebsiella pneumoniae that was treated with meropenem. The patient died on his fifth day at ICU. Death was attributed to a cerebral hemorrhage resulting from severe liver failure with massive bronchoaspiration.
His potential as a kidney donor was based on a renal ultrasound that showed a simple cyst in the cortex of the left kidney (1.3 cm) and a Doppler ultrasound image that revealed adequate vascular flow in both kidneys. Accordingly, the transplant committee accepted his kidneys for transplant. His liver and heart were not used as grafts.
He was a 56-year-old man who had received a liver transplant (LTx) 15 years earlier, for which he took cyclosporine A (CyA) and mycophenolate mofetil (MMF). His general progress was good, and his graft function adequate. Three years after the LTx he experienced hepatitis C relapse and was treated with interferon and ribavirin. Since then, the patient has maintained good liver function.
He developed end-stage renal disease (ESRD) due to hepatitis C associated–membranoproliferative glomerulonephritis. Residual diuresis was 100 mL/24 h, and he had been receiving hemodialysis for the past 36 months. He received a kidney transplant from a deceased donor with 3 HLA mismatches. The pretransplant biopsy graft contained 32 glomeruli, with no involvement of the vascular or tubulointerstitial compartment.
The surgical procedure was uneventful, and the immunosuppression regimen included basiliximab, methylprednisolone, CyA, and MMF. Cold ischemia time was 6 h, and, despite good graft perfusion, he had delayed graft function and required hemodialysis on day 2 after transplantation.
Given the donor's history of respiratory infection, empirical ceftriaxone was started from day 0 to day 3 after surgery and was then changed to meropenem as soon as ESBL K. pneumoniae was identified in a BAL culture.
He was a 56-year-old man with ESRD secondary to nephroangiosclerosis. He had been on hemodialysis for 3 years. Residual diuresis was 1500 mL/24 h, and he underwent deceased donor kidney transplantation with 3 HLA mismatches. The pretransplant biopsy graft contained 39 glomeruli, with no involvement of the vascular or tubulointerstitial compartment.
His immunosuppressive treatment included basiliximab, methylprednisolone, tacrolimus, and MMF. The surgical procedure was uneventful, and cold ischemia time was 10 h. He had delayed graft function despite good graft perfusion. He also received empiric ceftriaxone from day 0–3 post-transplant, and was then switched to meropenem.
At the time of kidney explantation and transplantation, the urologist noticed that one of the kidneys had a cyst. However, kidney biopsy specimens stained using rapid techniques in the pathology and microbiology departments were immediately reported as “normal”, and transplantation was performed.
On day 1 after surgery, a filamentous fungus started to grow in the culture plate of the donors’ respiratory sample (BAL). It was later identified as Aspergillus fumigatus. With this information, analysis of the previous renal biopsies reassessed with specific fungal stains (calcofluor white) revealed filamentous fungi, which were subsequently confirmed by the histopathologist.
Despite the fact that both recipients were in a stable clinical situation and given the potential presence of IA, elective nephrectomy was performed on day + 3 after transplant in both cases. Surgery was uneventful, and the graft and vascular sutures were resected. In patient 1, the gross appearance of the kidney and the transverse section was normal. Samples of the cortex, medulla, hilum, and vascular sutures were sent for culture and histology.
In patient 2, the kidney presented several parenchyma cavitations. Samples of the same areas were sent for culture and histology.
In both cases, A. fumigatus was isolated in samples from the cortex and vascular sutures. Tissue invasion was confirmed, and real-time PCR was positive for Aspergillus spp. in both cases. Chest radiographs were normal in both cases after graft extraction. Serological and urinary detection of , β-D-glucan (BDG) was performed (positive cut-off ≥80 pg/mL). BDG levels in blood and urine were as follows: Patient 1, 411 pg/mL in blood and 1647 pg/mL in urine on the first day after transplant; Patient 2, 433 pg/mL in blood and 1218 pg/mL in urine on the first day after transplant ().
A. fumigatus was genotyped directly from positive PCR samples (BAL sample from the donor and renal biopsies from recipients) using a highly discriminatory microsatellite procedure Short Tandem Repeats Aspergillus fumigatus (STRAf). Clinical isolates were not stored and consequently were not genotyped. All genotypes found were identical and showed the same allele composition for the 9 markers.
Antifungal treatment was initiated in both cases before extraction. In patient 1, liposomal-amphotericin B (3 mg/kg/day) was preferred in order to minimize drug interactions with azole derivatives and the immunosuppressive therapy he received for the LTx. Voriconazole (6 mg/kg/12 h) was chosen for patient 2 on day 1, followed by 4 mg/kg/12 h, with weekly therapeutic drug monitoring and subsequent dosage adjustment if needed.
Both patients were followed for 1 year and remain asymptomatic.
We report 2 cases from the same donor in which the early diagnostic and therapeutic approach prevented potentially fatal IA. |
pmc-6080552-1 | We present the case of a 69-year-old man with type II diabetes mellitus with ocular end-organ dysfunction, on oral hypoglycaemic agents, and with hypertension. He was also affected by an end stage renal failure requiring haemodialysis three times a week. Furthermore, he had other co-morbidities: ischaemic cardiomyopathy treated with oral anticoagulant therapy, mild chronic myelomonocytic leukemia (CMML), dyslipidemia and obesity.
In June 2016, a permanent urinary Foley’s catheter was positioned due to urinary retention.
In August 2017, the patient was seen to the emergency room (ER) of the Montichiari Hospital, Brescia, Italy. On admission, the patient was afebrile and upon physical examination, his vital signs (arterial pressure, heart rate and respiratory rate) were within normal limits. The patient gave a 3-day history of ongoing macroscopic haematuria and reported no lower urinary tract symptoms or other symptoms suggesting an inflammatory response or bleeding tendency. The patient had no history of abdominal or pelvic surgery. The international normalized ratio (INR) was 2.5 and hematologic parameters were within the normal range except red blood cell count, which was decreased (3 × 10 6 /μL), related to kidney failure. Glycated haemoglobin (HbA1c) was 52 mmol/mol. Finally, he was discharged with a hemorrhagic cystitis diagnosis and he was empirically treated with ciprofloxacin at a renally-adjusted dose (250 mg 2/die for 1 week) with the complete resolution of the macroscopic heamaturia.
In September 2017, the patient was seen again to the ER for another episode of macro-hematuria. On admission, he had a temperature of 36.5 °C, the blood pressure and the heart rate were within the normal limits, and there weren’t relevant findings on physical examination; blood cultures were performed but they were negative. Glycated haemoglobin (HbA1c) was 39 mmol/mol.
The patient had already started at home ciprofloxacin (250 mg 2/die) independently, so the clinician suggested that he continued this therapy for 1 week.
In the same month, the patient underwent a full urological investigation of haematuria, to exclude cancer or other abnormalities, a transrectal ultrasound, which identified a benign prostate adenoma, a cystoscopy which was negative for neoplasia, and a urinary cytology screening, which was negative for malignant cells. Despite the antibiotic therapy, the patient had symptoms related to urinary tract infection: bladder tenderness, hematuria and pelvic discomfort.
Therefore, a urine sample for culture was obtained by removing the indwelling catheter and obtaining a midstream specimen analysed by the laboratory of Microbiology and Virology of the Spedali Civili Hospital, Brescia, Italy; then, a new Foley’s catheter has been replaced. Urinalysis showed the presence of nitrites, leukocyte esterase and 6–7 leukocytes per high power field by microscopy.
Patient was discharged with clear instructions given to him for a proper care of the urinary catheter and for a correct hand hygiene to prevent infections, and the prescription of an empirical antibiotic therapy. It comprised levofloxacin at 250 mg for 10 days, switched then to amikacin 500 mg intravenously for the following three dialysis sessions (for a total of one week) for cover against multi-drug resistant Pseudomonas aeruginosa, as guided by local epidemiology.
The urine culture grew a > 105 colony-forming units (cfu)/ml of a gram-negative rod. The bacterium was isolated from Columbia CNA agar (BioMérieux, Florence, Italy) after 24 h of incubation in aerobic conditions. The colonies appeared round, mucoid, yellow pigmented and with a fruity smell. The initial identification as Myroides spp. was performed using a matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) according to the manufacturer’s instructions. The definitive identification was obtained with 16SrRNA gene sequencing. The obtained sequence was compared with the sequences in the GenBank database () and it exhibited a 100% identity homology with Myroides odoratimimus strain BK21.
The antimicrobial susceptibility testing (AST) was first performed by using the standard disc diffusion on Mueller-Hinton agar. Then, the minimum inhibitory concentrations (MICs) were determined by automated microdilution broth test (BD-Phoenix NMIC-502, Becton Dickinson, Milan, Italy). The minimum inhibitory concentrations (MICs) were confirmed by Etest (BioMérieux). Since the breakpoints for Myroides spp. were unavailable, the interpretation of the results was performed according to the EUCAST guidelines for non-species related PK-PD breakpoints. The isolated strain was resistant to all beta-lactams, with and without inhibitors (Piperacillin/Tazobactam, MIC = 64; Ticarcillin/Clavulanate, MIC = 128; Ceftazidime/Avibactam, MIC = 32; Imipenem, MIC = 8; Meropenem, MIC = 4) and it was also resistant to fluoroquinolones, aminoglycosides, fosfomycin, nitrofurantoin and polymyxin. This conferred to the isolated strain a multi-drug resistance pattern. This strain was susceptible only to trimethoprim /sulfamethoxazole with a MIC of 1/19. A test was performed to assess beta-lactamase and carbapenemase production (ROSCO diagnostics, Biolife, Milan, Italy) according to the manufacturer’s instructions. The results showed the absence of synergy between the meropenem disk and the dipicolinic acid, the phenylboronic acid, the EDTA and the cloxacillin.
In October 2017, due to the inadequacy of empiric therapy, the patient suffered from another hemorrhagic cystitis episode. Another urine culture confirmed the presence of a multi-drug resistant Myroides odoratimimus strain. According to the antibiotic susceptibility results, the patient was treated with trimethoprim/sulfamethoxazole at a renally-adjusted dose (160/800 mg daily for 2 weeks) which led to the resolution of macroscopic haematuria. In addition, in the same month, in order to reduce the possibility of recurrent UTIs, the urinary catheter was definitively removed.
Then, we tested its ability to grow in the form of biofilm. A Crystal Violet assay (CV) was performed to evaluate the production of biofilm at different concentrations of glucose and it was measured by spectrophotometry (NanoDrop™ Spectrophotometer, Thermo Fisher). The results indicated that this strain could be classified as a “strong biofilm-producer” [], which is able to produce a high amount of biofilm when it is compared to the reference strains (Pseudomonas aeruginosa PAO1, strain ATCC 15692). The increase of glucose concentration facilitates the production of biofilm by Myroides odoratimimus, contributing to an increase in vivo of its virulence. Therefore, a strong biofilm-producing bacteria, like Myroides odoratimimus, is well protected against antibiotics. A phylogenetic analysis was performed using the Quick Bioinformatics Phylogeny of Prokaryotes web-server and the data were then re-analysed using the Molecular Evolutionary Genetics Analysis software (MEGA 7.0.26) []. Geographical phylogeny was then extrapolated from the Gene-Bank database with a self-written programme. The results showed that our strain clustered with a strain isolated in Jena, Germany (Fig. ). The geographic analysis showed that this pathogen is poorly represented in Western Europe (Fig. ). |
pmc-6080730-1 | A 67-year-old male presented to our care on December 26, 2017, for shortness of breath on exertion and persistent cough for three weeks. Prior to these symptoms, he was completely asymptomatic. Symptoms progressed gradually up to the extent that he was not able to leave his house. Furthermore, the patient experienced severe shortness of breath when he attempted to use the restroom. He described a productive cough with white to yellow sputum. Additionally, he felt feverish on three different occasions since the onset of symptoms. Although he had no chest pain, the patient experienced chest tightness and heaviness. The patient reported orthopnea and waking up in the middle of the night due to shortness of breath. The patient denied palpitations, chills, night sweats, dizziness, recent travel, contact with birds, exposure to tuberculosis, and contact with wild animals. On presentation, his blood pressure ranged from 95/57 to 125/63 mmHg, a heart rate of 98 beats per min, a respiration rate of 19 breaths per min, and temperature of 97.3 F. His oxygen saturation was 94% on room air. Pulmonary function testing revealed a Gold 3 category with a forced expiratory volume in 1 second (FEV-1) of 41%, forced vital capacity (FVC) of 69%, and an FEV-1/FVC ratio of 47%. Lastly, the patient's bicarbonate, electrolytes, platelets, and transaminases were within normal limits. Physical examination findings of a barrel-shaped chest and poor air exchange, combined with the results of pulmonary function testing, supported a diagnosis of chronic obstructive pulmonary disease (COPD).
In addition, a chest x-ray exhibited right-sided apical opacity. The patient underwent computed tomography angiography (CTA), which was negative for pulmonary embolism and illustrated moderate to marked opacity in the right lung apex with cavitation extending up to the pleural surface. Additionally, patchy reticulonodular opacities consistent with infiltrates were present in the right upper, right middle, right lower, and left upper lobe (Figure ). These findings led to a diagnosis of cavitary pneumonia, and blood cultures, along with mycoplasma IgM, were ordered. In addition, the patient tested negative for human immunodeficiency virus (HIV-1 and 2). The infectious disease team was consulted for their expertise and to ensure a thorough workup. Although the patient did not have true exposure to tuberculosis, the QuantiFERON®-TB Gold test (QFT-G) (Qiagen, Hilden, Germany) was ordered and the results for active and latent tuberculosis were negative. Furthermore, acid-fast bacilli (AFB) sputum cultures, fungal stains, serologies, and β-D-glucan were negative as well. Due to inconclusive results, a bronchoscopy with biopsies was performed which yielded a necrotic black specimen. A subsequent culture grew Mycobacterium chimaeraand confirmed the diagnosis.
Although a diagnosis was reached, this patient needed further workup to ensure complete resolution of this condition. The infectious disease team suggested optimizing the patient's respiratory regimen, following up with a chest CT in three months, and treating with a multidrug regimen for atypical mycobacterial infection if the patient clinically worsened. |
pmc-6080731-1 | We describe the case of a 50-year-old woman who presented with adult hip dysplasia of high dislocation (Hartofilakidis Classification, type C) /Crowe grade IV. She is a known smoker and has hypertension. She had been tolerating mild pain for the past few years, but recently it had become debilitating. Additionally, there was a leg length discrepancy making the affected left side 7 cm shorter.
When she initially presented a few years ago, she was started on physical therapy and analgesia as she did not want to consider surgical intervention. However, the pain had become unbearable, and the functionality of the hip was compromised.
We recommended a hip replacement and discussed the potential benefits and risk factors. It was explained to the patient that in dealing with the developmental dislocation we would have to recreate normal hip mechanics which requires positioning of the acetabular component in hemispherical acetabular cavity at the centre of rotation. It would also entail placing a femoral component at a much lower level within a femoral canal which was abnormally narrow. She underwent the left hip replacement which was surgically challenging. A special hip implant was used for the hip replacement. It was a modular implant typically used for difficult cases where there is anatomical distortion. The surgery was completed without any complications. We were able to correct the leg length discrepency by 4cm. Following surgery the patient underwent physical therapy and is now independently mobile without the use of external support and has no functional difficulties in day to day activities, (Figures -). |
pmc-6080733-1 | The patient was a 25-year-old male who had a history of thalassemia minor as well as tonic-clonic seizures following head trauma with identified cystic encephalomalacia in the left parietal lobe. He was diagnosed with seizure disorder three years ago when he experienced episodes of dizziness with lightheadedness and sensation of spinning, with facial flushing. He was initially trialed on Levetiracetam, which seemed to increase the frequency of event rate and eventually was changed to Lamotrigine, which was his regular maintenance medication at the time of this admission.
On initial presentation, he described nine episodes of syncope without prodrome occurring within the span of four weeks. He had loss of consciousness for approximately 20-30 seconds, usually witnessed. He did not experience any lightheadedness, dizziness, presyncope, palpitations, or tachycardia. His vitals were normal. On neurologic exam, cranial nerves were intact, sensation intact to light touch, reflexes intact bilaterally. Gait was normal and Romberg sign was negative. The cardiac exam revealed a regular rate and rhythm with normal S1 and S2 and without S3, S4, gallops, murmur, or rub. An EKG showed normal sinus rhythm with an incomplete right bundle branch block and Epsilon waves in leads V1 and V2 without evidence of Brugada syndrome (Figure ).
Laboratory data showed a hemoglobin level of 11.0 g/dL, white blood cell count 9.1 x 109/L, sodium 140 mEq/L, potassium 4.1 mEq/L, and a point of care urinalysis was negative. A computed tomography (CT) scan of the head showed no intracranial hemorrhage or depressed skull fracture and stable cystic encephalomalacia in the left parietal lobe without infarction or intracranial mass.
Neurology consultation was requested and a continuous 24-hour video electroencephalogram (EEG) was performed, showing normal awake and sleep-prolonged video EEG without event. A lamotrigine level was checked and was therapeutic at 3.9 mcg/mL, and TSH was within normal limits. A urine drug screen was negative.
Cardiology consultation was requested and patient was monitored on telemetry with no episodes of ventricular tachycardia. 2-D ECHO showed normal ventricular function and no valvular heart disease. A cardiac magnetic resonance imaging (MRI) was performed and showed left ventricular ejection fraction normal at 53% with the ventricular septum measuring 9 mm, posterior inferior wall measuring 10 mm and no evidence of abnormal late gadolinium enhancement. There were small focal outpouchings of the right ventricular (RV) free wall raising the question of micro-aneurysms, and with findings consistent with ARVC (Figure ). The pericardium was normal.
A diagnosis of ARVC was achieved, and the patient underwent electrophysioloy (EP) study and successful implantation of a dual-chamber cardioverter defibrillator. |
pmc-6080734-1 | A 75-year-old Caucasian female presented to our emergency department (ED) with a one-week history of progressive dizziness, weakness, early satiety, and chest heaviness. Symptoms started eight days after right knee arthroplasty. Review of systems was negative for abdominal pain, nausea, vomiting, hematochezia, melena, and bone pain. Pertinent past medical history included a remote history of acid reflux disease, history of colonic diverticular disease, and regular use of nonsteroidal anti-inflammatory drugs (NSAIDS).
Physical examination was notable for pallor, tachycardia, and positive fecal occult blood. Examination of the heart, lungs, and abdomen were unremarkable. Examination of the breasts performed in the ED was normal. Laboratory testing revealed a hemoglobin count of 7.6 g/dL, prompting further evaluation for a GI source for the bleeding. Esophagogastroduodenoscopy (EGD) revealed erosive gastritis and duodenitis as well as a non-obstructing Schatzki ring at the gastroesophageal (GE) junction (Figures -).
The pathological evaluation of the gastric and duodenal samples was positive for signet ring carcinoma, favoring a presumptive diagnosis of linitus plastica (Figures -). However, immunohistochemical (IHC) staining of the biopsy revealed positive estrogen receptor/progesterone receptor (ER/PR), GATA3, and mammaglobin with negative human epidermal growth factor receptor 2
(HER2)/neu and a negative E-cadherin (Figures -).
The above findings prompted further investigation for a primary source with high suspicion for the breasts. Detailed examination of the breasts revealed bilateral chest wall nodules concentrated in the inframammary area (Figure ). Notably, the patient had obtained annual mammograms from 2009 to 2017, all of which were normal. Furthermore, magnetic resonance imaging (MRI) of the breasts failed to detect any abnormalities in the breast tissue. Computed tomography (CT) imaging of the chest, abdomen, and pelvis was remarkable only for mild mediastinal lymphadenopathy, an unsuspicious 5 mm pulmonary nodule in the right lower lobe, and hepatomegaly with cirrhotic changes.
Further workup revealed an elevated alkaline phosphatase of 188 U/L (normal range: 30-99 U/L). Liver and kidney functions were otherwise normal. Positron emission tomography (PET) scan was remarkable only for mildly increased uptake in the proximal stomach. Carcinoembryonic antigen (CEA) was noted to be 14.9 ng/ml (normal range: 0.0-3.0 ng/ml); cancer-antigen 15-3 (CA 15-3) was 210 U/ml (normal range: 0-31 U/ml); and CA 27.29 was 234.8 (normal range: 0-40 U/ml). The patient also had persistent anemia as well as non-infectious leukocytosis. Flow cytometry was performed and found to be negative for leukemia.
Given the high suspicion for a breast primary, a full thickness biopsy of the inframammary nodular lesions was attempted. Additionally, the patient underwent abdominal laparoscopy with peritoneal biopsies from the diaphragm, falciform ligament, and lower pelvic peritoneum. Interestingly, pathological results of the biopsies (chest wall and peritoneum) revealed an invasive lobular carcinoma. The patient was emotionally distraught and anxious after receiving the diagnosis. She received appropriate support and counseling from qualified providers.
The patient received one unit of packed red blood cells and symptoms improved. She was subsequently started on hormonal therapy with anastrozole, and later on the cyclin-dependent kinase (CDK) inhibitor, palbociclib.
In spite of extensive hematological work up, the patient continued to have leukocytosis. However, there was no bone pain or evidence of bone marrow involvement on PET scan. Bone marrow biopsy was then pursued. The patient was noted to have metastatic breast carcinoma with marrow replacement. She has since been scheduled for routine oncological follow up. |
pmc-6080736-1 | A 32-year-old female with no previous medical history presented to the emergency department (ED) with weakness and fever, along with diarrhea and vomiting for one day. She had no complaints of chest pain, shortness of breath, chills, headaches, dizziness, or palpitations on arrival. Family history, social history, and past surgical history were all unremarkable. However, she had a history of sudden cardiac death in her family. On arrival in the ED, she had a temperature of 103.1 Fahrenheit, tachycardia at 131 beats per minute (bpm), with a blood pressure of 65/38 mmHg. On physical examination, she was oriented only to self and disoriented to time, place, and person; the physical examination did not show any other significant findings. Laboratory data demonstrated an acute kidney injury with a creatinine of 1.7 mg/dl. Cardiac enzymes were negative. The initial electrocardiogram (ECG) was notable for right bundle branch block (RBBB) with coved Brugada-type ST-T wave changes in V1 and V2 along with diffuse ST depressions (Figure ).
The patient required admission to the intensive care unit (ICU) given her hemodynamic instability and the need for pressor support with norepinephrine. Further investigations revealed a low serum cortisol level (2.1 UG/DL) and a low adrenal corticotropic hormone (ACTH) (<5pg/ml). A cosyntropin stimulation test was performed next, which was consistent with secondary adrenal insufficiency (AI). Steroid supplementation was initiated with a significant improvement in her clinical picture.
Repeat ECG revealed the resolution of the diffuse ST depressions and an incomplete RBBB with mild coving consistent with a type II pattern (Figure ).
No arrhythmias were recorded throughout the admission. A transthoracic echocardiography (TTE) was conducted, which yielded normal results. At discharge, the patient was advised to have an immediate intervention with antipyretics for any future febrile episodes and to avoid certain medications. She was discharged on steroid supplementation and with close follow-up with cardiology. |
pmc-6080739-1 | A 35-year-old gravida four para four female initially presented to urgent care with dysuria and was prescribed antibiotics for a presumed urinary tract infection. She had no significant medical history and was not immunocompromised in any way. Three days later, she was seen by her primary care provider for worsening dysuria, pelvic pressure, and two days of abnormal vaginal bleeding. Prior to this, she denied any history of abnormal vaginal bleeding and reported normal menses. Due to the grossly abnormal appearance of her cervix, she was referred to gynecology oncology for evaluation. The patient’s medical and surgical history was remarkable for a history of loop electrosurgical excision procedure (LEEP) in her late twenties for cervical intraepithelial neoplasia (CIN) grade 2/3, ESSURE (Bayer, Whippany, New Jersey) placement, and chronic tobacco use. She had an inconsistent follow-up after her LEEP, reporting a normal last pap smear “years prior". She was sexually active with a new male partner for the last four months and denied any history of sexually transmitted infections, including HSV. On examination, normal external genitalia were noted without any lesions or abnormalities. Speculum exam revealed grossly bloody vaginal discharge and an approximately 4-cm firm, circumferential cervical mass with possible left parametrial involvement.
An office biopsy of the cervical mass was obtained, and the tissue pathology was found to be negative for malignancy, but remarkable for acute inflammation and cellular changes suggestive of HSV infection. Pap smear results were unremarkable and tested negative for high-risk human papillomavirus (HPV). A magnetic resonance imaging (MRI) scan revealed an elevated T-2 signal concerning for potential neoplasm at the outer surface of the cervix, which encircled the cervical os. Mildly prominent right inguinal lymph nodes were also noted on MRI (Figure ). Due to the ongoing concern for possible cervical malignancy, the patient was taken to the operating room for an exam under anesthesia, additional cervical biopsies, cervical and vulvar cultures, and cystoscopy with bladder cytology. The exam was remarkable for multiple, new external vulvar lesions concerning for HSV, which were cultured. There was a redemonstration of the 4-cm cervical mass with obliteration of the vaginal fornices and without parametrial involvement. The mass was noted to be friable and ulcerated. Normal bladder mucosa without lesions or invasion and a large amount of bladder debris were seen on cystoscopy. Due to suspected HSV, the patient was started on valacyclovir 1000 mg twice daily for 10 days. All cervical biopsies, including deep biopsies, returned negative for malignancy and showed ulceration and inflammation. Cervical, vulvar, and urine cultures all returned positive for HSV.
The patient was seen approximately two weeks later for follow-up, at which time she reported resolution of her symptoms. Pelvic examination showed no active vulvar lesions and a slightly erythematous cervix with near resolution of the cervical mass and normal vaginal fornices. She was counseled regarding her diagnosis of HSV. Follow-up with a general obstetrician-gynecologist was recommended in one month with a repeat pap smear in two to three years, due to concern for inadequate testing secondary to the presence of the cervical inflammation. It was recommended that she undergo human immunodeficiency virus (HIV) testing; however, the patient declined. |
pmc-6080740-1 | A 35-year-old male with an unremarkable past medical history presented with a painful penile erection. He had woken up with a painful penile erection 48 hours ago which had persisted continuously since. He denied penile or perineal trauma, use of recreational drugs or medications, and personal or family history of sickle cell disease or other hematologic diseases. He had two similar episodes in the last six months. The first episode lasted for 24 hours and resolved spontaneously. The second episode lasted for more than 24 hours, and it required decompression with an intracavernous phenylephrine injection. On physical examination, he had an erect penis; however, the rest of the general and systemic examination was unremarkable. Initial lab tests revealed mild leukocytosis of 12 × 103/L, peripheral eosinophilia of 530 cells/L, and a normal hemoglobin level. Peripheral smear and reticulocyte counts were normal. Cavernous blood gas analysis showed paCO2 103 mmHg, and paO2 < 5 mmHg, and pH 6.8. Lactate dehydrogenase (LDH) was mildly elevated at 294 U/L. Therefore, peripheral flow cytometry was obtained which was unremarkable. Urine drug screen was normal. Direct penile aspiration was attempted, which was not successful. The patient received an intracavernous phenylephrine injection, which did not help (Figure ).
The patient was taken to the operation room where penile irrigation was attempted followed by the formation of a distal penile shunt called Winter shunt. Postoperatively, the patient was observed till the next morning; however, his priapism did not resolve completely (Figure ).
Bedside penile irrigation, aspiration, and an intracavernous phenylephrine injection were attempted again but were not helpful. The patient was again taken to the operation room where a surgical shunt was formed between corpora cavernosa and corpus spongiosum bilaterally. Postoperatively, the patient’s erection started resolving. In the next 24 hours, the patient’s priapism had resolved completely (Figure ).
The patient received a lupron injection to decrease testosterone levels and to lower the risk of incurring priapism again in the future and was discharged with the recommendation of outpatient follow-up. |
pmc-6080742-1 | A 79-year-old woman with a history of severe aortoiliac occlusive disease requiring a previous aorto-bi-iliac bypass graft presented in 2009 with a two-year history of chronic abdominal pain. Her abdominal pain was diffuse and postprandial in occurrence. She had associated symptoms of sitophobia (fear of food) and a weight loss of 87 pounds from 170 lbs to 83 lbs over a two-year period. At an outside hospital, the patient had workup performed for her symptoms over the previous two years that included an abdominal ultrasound, four esophagogastroduodenoscopies (EGDs), three colonoscopies, upper GI series and a non-IV contrast abdominal computed tomography (CT) scan – all with non-specific results.
After referral, a duplex mesenteric arterial study revealed a peak systolic velocity (PSV) > 350 cm/sec within the celiac trunk indicating severe stenosis. She had reproduction of her abdominal pain after a postprandial challenge. CT angiography of the abdominal vessels revealed a common trunk of the superior mesenteric artery and celiac axis. The study confirmed a 90% stenosis of the celiomesenteric trunk (Figure ). The IMA was also occluded. The aorto-bi-iliac bypass graft limbs were patent to the anastomotic site of the external iliac arteries. Based on the patient’s clinical presentation and diagnostic studies, a diagnosis of CMT ischemic syndrome was made.
The first intervention occurred in 2009 when open bypass graft was the standard of care for mesenteric revascularization. The patient underwent an extra-anatomic right iliac to SMA retrograde bypass graft. The bypass was performed from the right limb of her aorto-bi-iliac bypass graft to the SMA with an 8 mm ringed Gore® Propaten® graft in an end-to-side fashion for both distal and proximal anastomosis (Figure ). An end-to-side anastomosis to the SMA was adopted to enable forward flow to the SMA and retrograde flow to the celiac vessels. She had complete resolution of her symptoms and regained her body weight appropriately over a six-month period. She remained symptom-free for multiple years postoperatively.
The patient returned four years later with symptoms of postprandial pain and dyspepsia. No weight loss had occurred. Duplex arterial abdominal ultrasound showed severe stenosis of the CMT with elevated PSV of 461 cm/sec. Repeat CT angiography of the abdomen revealed dense calcific plaques throughout the native abdominal aorta. A 99% stenosis of the proximal celiomesenteric trunk was noted. The right iliac graft limb to SMA bypass graft was patent, however, the segment retrograde from the SMA anastomosis to the celiac vessels was occluded.
The patient underwent formal mesenteric angiography that confirmed a 99% stenosis of the proximal CMT and a patent right iliac limb to SMA bypass graft (Figure ). She underwent a second surgical intervention with the placement of a 7 mm x 16 mm Atrium® stent in the proximal CMT via a retrograde femoral artery approach. The patient reported complete resolution of her abdominal complaints post-procedure. The patient has been followed at the clinic for the past five years and has remained symptom-free. |
pmc-6081099-1 | A 28-year-old male was transferred to our hospital in November 2006 because of a sudden onset of cramping and abdominal pain and intermittent melena for 4 days. Initially, he exhibited symptoms including generalized weakness, dizziness, and massive bloody stool passage. There was no significant past medical history of recent infection, inflammatory bowel disease, bleeding disorders, changes in bowel habits, significant weight loss, or tuberous sclerosis complex. The patient had no previous surgeries, and the family history was unremarkable. The patient's height was 180.2 cm, weight was 78.5 kg, blood pressure was 74/39 mm Hg, pulse rate was 122 beats/min, and temperature was 36.4°C. The physical examination revealed moderate distention and tenderness in the left lower quadrant with associated defense. On digital rectal examination, there was some gross blood mixed with soft stool and an empty ampulla. Laboratory data showed a white blood cell count of 17,120/mm3, hemoglobin level of 7.4 g/dL, hematocrit of 23.9%, and platelet count of 162,000/mm3. Other biochemical tests were normal. The serum carcinoembryonic antigen level was normal.
The gastroscopic evaluation was normal. The full colonoscopy revealed a greater than 4.0 cm in diameter, large ulceration with an easy bleeding mass of the rectum lying beneath the mucosa but protruding into the lumen 15 cm from the anal verge (Fig. ). This tumor could not be classified by biopsy. However, on the basis of the immunohistopathological features, carcinoma and malignant lymphoma could be excluded. An abdominal enhanced CT scan revealed a heterogeneous mass lesion of approximately 8.9 x 7.2 cm in the pelvic floor at approximately the level of the rectum (Fig. ). A technetium-99m red blood cell scan showed no significant findings. Repeated attempts at endoscopic revaluation resulted in profuse bleeding requiring blood transfusions. A diagnostic biopsy failed before the surgical treatment due to bleeding from a light touch of the mass lesion
Given the difficulty of obtaining a diagnostic specimen, surgical resection and lower anterior resection were performed. On gross examination, the resected specimen was an 8.8 x 5.5 x 4.5 cm, tan, gray-white, soft, and well-circumscribed transmural mass of the rectum, mainly involving the muscularis propria and protruding into the tunica adventitia. The mucosa and submucosa were intact (Fig. ). The tumor protruded into the lumen, and the overlying mucosa showed ulceration. The tumor had a dark, red-grey, solid parenchyma with irregular cystic spaces with colorless serous liquid (Fig. ). The cut surface showed a yellowish-tan to gray-red solid parenchyma with focal irregular cystic spaces containing colorless serous fluid (Fig. ). All surgical margins were macroscopically free of tumor. No separate polyps were identified. Microscopically, the foci of hemorrhage and necrosis were present. The tumor extended through the muscularis propria into the subserosa tissue with lymphatic invasion. The colon mucosal tissue was composed of sheets with atypical glands with spindle-to-epithelioid cells and nuclear abnormalities in a tubular arrangement (Fig. ). Perivascular hyalinization was noted (Fig. ). Most tumor nuclei showed clear to granular, light, eosinophilic cytoplasm, and round to oval nuclei with distant small nucleoli pleomorphism (Fig. ). Less than 50% of the tumor area was necrotic. The mitotic rate was low. One of 27 accompanying serosal lymph nodes contained metastatic tumors that distended the subcapsular sinus. All of the surgical margins were free of tumor. Immunohistochemically, the tumor cells were positive for melanoma-associated antigen (HMB-45) (Fig. ) but negative for cytokeratin, c-kit, synaptophysin, S-100, and actin. A diagnosis of metastatic PEComa was made after examination of the resection material.
Unfortunately, the patient did not complete the follow-up in the outpatient department after surgery because he relocated to another city to seek work. At the 49th month (2010) postoperatively, the patient returned to the clinic upon our scheduled request. An abdominal CT scan showed a 0.6 cm hypodense mass over the liver. He refused further evaluation and treatment. In July 2014, during the course of a required physical health check, he received an abdominal CT that showed that the original mass had increased from 0.6 to 1.5 cm and that the number of tumors had increased from 1 to 3 compared with the previous CT image in 2010. We noted that the distant liver metastasis progressed very slowly during the 4 years of follow-up. The liver function and the other serum tumor markers were normal upon examination. In August 2014, he underwent segmental hepatectomy (S3, S4A, S5, and S6) combined with wedge resection. The pathology report of segmental hepatectomy was metastatic PEComa.
This patient underwent 2 different surgical resections at 2 different times. He underwent the lower anterior resection of the PEComa of the rectum in 2006 and received the segmental hepatectomy in 2014. He is currently undergoing regular surveillance and has remained free of disease 28 months after the second operation. At the follow-up examination, the patient felt well, and the general clinical examination, subsequent colonoscopies, and abdominal CT scan once every 3 months revealed no significant findings after second operation in 2014. Since the primary surgery in 2006, there was also no recurrence of the PEComa of the rectum according to the general clinical examination, subsequent colonoscopies, and abdominal CT scan at the 120-month follow-up of the very first instance back in 2006. |
pmc-6081151-1 | A 44-year-old woman presented to our hospital with symptoms of a headache for 20 days and weakness in the right limbs for 1 day. She had a history of aplastic anemia (AA) and had been taking 150 mg oral cyclosporin A (twice a day) continuously for 18 months. Physical examination was performed and the following parameters were noted: height, 170 cm; weight, 68 kg; body temperature, 36.5°C; and blood pressure, 142/83 mm Hg. No abnormality was observed on cardiopulmonary or abdominal examination. Neurological examination revealed conscious mind and motor aphasia; bilateral eye movement was flexible; size of the bilateral pupils were equal at 3 mm in diameter; light reflex was observed; the right nasolabial sulcus was shallow; tongue was in the middle; gag reflex was noted; neck was soft; according to the manual muscle test, the strength level of the right upper and lower limb muscles was 4; the strength level of the left upper and lower limb muscles was 5 (normal); muscle tension in all limbs was normal and physiological reflex was noted; and the Babinski sign on the right side was positive. The patient had no history of diabetes, hypertension, hyperlipidemia, liver cirrhosis, smoking, use of contraceptive pills, pregnancy, puerperium, and infection. Laboratory examination revealed: white blood cells, 6.19 × 109/L (normal reference value 4–10 × 109/L); neutrophils, 86.9% (normal reference value 50–70%); hemoglobin, 89 g/L (normal reference value for adult female 110–150 g/L); platelets 55 × 109/L (normal reference value 100–300 × 109/L); blood concentration of cyclosporin A (valley concentration), 240.7 μg/L (normal reference value 150–250 μg/L); plasma D dimer, 31.38 mg/L (normal reference value < 0.5 mg/L); and normal levels of blood protein S, protein C, anticardiolipin antibody, immune indexes, indexes of tumor, antithrombin III blood homocysteine, and blood fibrin. Ischemia and hemorrhage were observed in left frontal lobe by computed tomography (CT) scan (Fig. A). There was no abnormity in the cerebral arteries (Fig. B) and sinus thrombosis was observed in the superior sagittal sinus region with computed tomography venography (CTV) (Fig. C). The patient was administered low molecular heparin (enoxaparin, 4000 AXaIU, subcutaneous injection, once every 12 hours) for anticoagulation. After 2 weeks, it was replaced by warfarin, and the dosage of warfarin was adjusted by the international normalized ratio (2.0–2.5). Cyclosporin A was stopped immediately and replaced by Testosterone Undecanoate. After 30 days, the patient had no more thrombosis. But her hemoglobin concentration was declined to 55 g/L. According to the hematology specialists and neurologists’ comments, cyclosporine A was resumed again and warfarin was taken together. The dosage of cyclosporine A was adjusted between 100 to 150 mg (twice a day) according to the blood concentration. The patient developed no recurrence of thrombosis during the 13-month follow-up. |
pmc-6081152-1 | A 70-year-old woman was admitted to the hospital because of fainting and general malaise for 5 years, worse in the prior 6 months. The symptoms were mainly triggered by flexing the neck and changing body position. There was no other discomfort and no significant past history. Her body mass index was 28.52, with blood pressure 144/77 mm Hg, and a radial pulse rate of 80 beats/min and regular. Physical examination showed slight pitting edema in both legs. She had no pathologic cardiac murmur or significant abdominal findings. The echocardiogram revealed a LV mass attached to the posterior wall near the apex, measuring 16.1 mm × 11.1 mm (Fig. ). The mass had a well-defined border and moved when the heart contracted. LV function and outflow were not impaired. There was no associated thrombus or mass in any other chamber or on any valve. Laboratory tests, electrocardiography, coronary angiography, and brain magnetic resonance imaging (MRI) showed no abnormalities.
Surgery was performed through a limited median sternotomy with cardiopulmonary bypass. An intraoperative transesophageal echocardiogram confirmed that the echodense mass was attached by a pedicle to the LV posterior wall adjacent to the apex. As valve motion prevented a clear surgical view because the deeply located mass was near the apex, we inserted a thoracoscope into the ventricle to help visualize the tumor. The mass was found to be a lipoma, based on its well-encapsulated, yellow appearance. The tumor (25 mm × 10 mm) was carefully removed using scissors and suction (Figs. and ). No invasion in the ventricular muscle was observed.
Histopathologic examination was consistent with a lipoma (Fig. ). Postoperative recovery was uneventful and the patient was charged 20 days after surgery. No signs of recurrence were detected on an echocardiogram during a 3-month follow-up period (data not shown). |
pmc-6081158-1 | A 17-year-old male patient presented with a 10-year history of polyarthritis and 4-year history of progressive hip and knee pain and stiffness. His interphalangeal joints were first involved, and then the elbows, knees, and hips. He was misdiagnosed with developmental dysplasia of hips at the age of 9 in another hospital and received no treatment. Physical examination showed swaying gait with fixed flexion deformity of hips and knees (Fig. A). Multiple interphalangeal joints were enlarged (Fig. B). Impaired range of motion of his interphalangeal joints, elbows, and wrists was observed. Hip flexion-extension was 70° to −45° in the left and 80° to −40° in the right. Knee flexion-extension was 120° to −40° in the left and 120° to −45° in the right. No obvious scoliosis or thoracic kyphosis was noticed. Babinski sign was negative and the knee-jerk reflex was normal. Laboratory tests including blood cell count, erythrocyte sedimentation rate, C-reactive protein, rheumatoid factor, antistreptolysin O, and anticyclic cirullinated peptide antibodies were normal. Preoperative visual analog score (VAS) was 7 and the Harris hip score was 32.
Radiograph of pelvis showed broadening width of the pubic symphysis, enlargement of femoral heads, narrowed hip joint space, and osteoarthritis of hips. Radiograph of knees demonstrated enlargement of femoral condylar and narrowed joint space (Fig. A). Spinal X-rays revealed platyspondyly, irregular end plates, and mild scoliosis (Fig. B).
Written informed consent was obtained from the patient and the guardians. Genetic testing for the WISP3 gene was done and showed compound heterozygous mutations: NM_198239.1 (WISP3): c.1064_1065dupGT (p.Gln356ValfsTer33) and NM_198239.1 (WISP3): c.643 + 2T > C. Combined with the patient's history, symptoms, physical signs, and genetic testing, the final definitive diagnosis is PPD.
Deduced from radiographs, the hip and knee joint space was decreased but still had room for remodeling by Ilizarov fixator, since this technique provides with secure fixation against persistent stress and tension, and allows for mild-to-moderate daily activities during treatment. Soft tissue around the contractural knee was stretched gradually is the rational for the Ilizarov techniques. Thus, we carefully worked out preoperative plans according to the condition of the patient and the characteristics of Ilizarov fixator. To deal with severe joint contractures, tenolysis, and soft tissue release needed to be performed, before imposing the fixator, by a 3-cm subinguinal longitudinal incision and another one at gastrocnemius tendon. After that, 4 steel rings should be placed sequentially at the mid-thigh, 5 cm above the knee cap, 5 cm below the knee cap, and above the ankle, and each ring required a piece of K-wire applied by percutaneous corticotomy cuts. Instead of being placed at a parallel pattern, the K-wires should be fixed at different angles, by a rotation of 45° at each plane, to form a stable 3-dimensional structure. We planned to extend the fixator 0.5 to 0.1 cm each day, adjusted by the tolerance of the patient, till a full extension of the lower extremity. Then, the same procedure should be applied to the opposite side, and a cast be used to consolidate the right limb for the same amount of time as dealing with the left limb.
At the first stage, we performed the surgery in his right hip and knee. The patient underwent the surgery under general anesthesia in a supine position. Firstly, the proximal end of rectus femoris muscle was released, and then the hamstring muscle and semitendinosus were released. After that, common peroneal nerve lysis was done. After checking the tension of common peroneal nerve, the Ilizarov external fixation apparatus was placed. After the operation, the Ilizarov external fixation apparatus was lengthened 5 to 10 mm/d (Fig. ). Hip and knee passive movement was also performed every day. Thirteen days after the operation, the flexion deformity of his right hip and knee was greatly improved (Fig. A). There is no possibility of growth potential in this patient which can affect the result for closure of epiphysis.
Thirty-six days after the operation, the Ilizarov technique was performed to his left hip and knee by the same procedures and plaster cast for his right lower extremity was placed at the same time (Fig. B). One month after the stage 2 surgery, external fixation apparatus and plaster cast were removed. The follow-up time period is 1 year. One year after the operation, the improvement of joint deformity was satisfactory (Fig. C). The follow-up results are demonstrated in Table . There was no complication observed in this patient. The patient has provided informed consent for publication of the case. |
pmc-6081190-1 | An 89-year-old Caucasian female with a medical history for AH, ischemic heart disease (coronary artery bypass surgery without prolonged ventilation), heart failure, chronic atrial fibrillation presented to emergency department with dyspnea, difficulty in speaking, hoarseness of voice, and edema of the neck was presented in this study (chronological medical history is provided in Table ). All of the symptoms occurred 2 days ago. Medical records revealed that coughing occurred about a month ago, and gradually became worse. Pneumonia was suspected; therefore, blood tests were collected and chest X-ray was performed. During X-ray, the patient developed airway obstruction requiring intubation. For further treatment, the patient was admitted to the intensive care unit (ICU). Blood tests revealed slight leukocytosis and increased C-reactive protein levels. Empiric antibiotic therapy was initiated. It was revealed from medical records that she was on the following medication: warfarin, metoprolol, amlodipine, torsemide, as well as ACE inhibitor (ramipril) on a daily basis for the past 5 years. No recent changes in medication or dose were performed; also, no history of smoking, seasonal or medication allergies, and no family history of angioedema were reported. After the patient was sedated, ventilated, and monitored for 24 hours in ICU, it was decided to wean her off the ventilator. Ability for spontaneous breathing (SB) was assessed with T-piece test (T). SBT was performed for 60 minutes and was well tolerated: no tachycardia, no tachypnea, and no signs of increased work of breathing presented. The patient was conscious and responsive, therefore extubated. However, 1 hour after extubation, desaturation and partial airway obstruction developed, consequently urgent reintubation was decided. Intubation presented as difficult, due to narrowing of trachea below the vocal cords. Bronchoscopy was performed to evaluate the unknown origin of trachea narrowing; however, bronchoscope could not pass through intubation tube. Further evaluation was performed with contrast-enhanced neck and chest computed tomography (CT) scan. The CT scan revealed soft tissue edema, which involved the base of the tongue and trachea from cricoid cartilage and up to 2.6 cm below. Standard therapy with epinephrine, methylprednisolone, and clemastine was given. However, treatment was highly ineffective because of persistent soft tissue swelling. All of previously used medications were discontinued, including ACE inhibitor. Four days after discontinuation of ACE inhibitor, a sudden drop in oxygen saturation (SpO2) presented, capnography revealed obstructive pattern; therefore, suction with catheter was initiated. Catheter could not pass through intubation tube, due to the obstruction. Manual ventilation was performed; however, oxygenation was inadequate and severe hypoxia resulted in cardiac arrest. Cardiopulmonary resuscitation (CPR) was started immediately after the patient became pulseless. During CPR, the patient received 14 mg of epinephrine and 250 mmol of bicarbonate. Spontaneous circulation returned after 38 minutes. Emergency bronchoscopy revealed blood clot and soft tissue edema fully obstructing the distal end of endotracheal tube. After blood clot was removed, air entry improved and SpO2 increased. Angioedema was suspected; therefore, for further identification of the underlying cause and differentiation of angioedema, diagnostic tests were performed. C3, C4, IgE levels, and C1 esterase inhibitor protein levels were in the normal range; therefore, hereditary angioedema was ruled out and ACE inhibitor induced angioedema diagnosed. Two units of fresh frozen plasma (FFP) were administered; however, no improvement was noted. Neurological examination was performed 24 hours after restoration of spontaneous circulation: eye opening response to pain, no verbal response, and no motor responses to painful stimuli (Glasgow Coma Scale – 4). Patients’ corneal reflex was intact and pupils reactive to light bilaterally. Head CT scan revealed signs of hypoxic ischemic brain damage. Angioedema resolved after 13 days from the discontinuation of ACE inhibitor. Patient did not regain consciousness, despite full clinical resolution of ACE inhibitor induced angioedema. Death due to cardiopulmonary insufficiency occurred 24 days after the admission to ICU. |
pmc-6081229-1 | Our proband is a 39-year-old male who was first seen in our clinic at age 35. In his early medical history, he reports that he was hypermobile (Beighton score of 7/9), small in stature, and had a high-arched palate. He has had several joint dislocations involving the digits and patellae, the first of which occurred around 12–14 years old. Due to family history including cardiomyopathy in three paternal uncles, father, and sister, the proband was investigated by a cardiologist at 16 years old. No heart problems were found, though his other symptoms were suggestive of an underlying connective tissue disorder and he was labeled as having “either Ehlers–Danlos or Loeys–Dietz syndrome.” Years later, the proband was found with a pulmonary artery aneurysm after he was hospitalized for a spontaneous pneumothorax in 2008. The proband was closely followed after this incident due to a worsening of the aneurysm and severe pulmonic regurgitation (PR).
At age 35, the proband's pulmonary artery aneurysm had grown to 5.7 cm. Aside from his severe PR, he was experiencing moderate mitral regurgitation, mild tricuspid regurgitation, severe left atrial enlargement, and biventricular failure with an ejection fraction of 22%. Surgery was now necessary. A pulmonary artery resection with pulmonary homograft valve 27 mm implantation was performed with resultant symptomatic improvement and an improved ejection fraction to 45%. The surgeons had noted myxoid degeneration in the pulmonary valve, supporting the presence of a connective tissue disorder. At age 37 he was found to have gallbladder stones and underwent a laparoscopic cholecystectomy. Days after the surgery, he returned to the hospital with acute respiratory distress, shortness of breath, and fever. Investigations revealed he had contracted Klebsiella pneumonia. He was hospitalized once again, and developed acute liver failure, acute renal failure, and respiratory failure requiring ventilator support, acute-on-chronic systolic heart failure, and pulmonary artery hypertension. The proband returned home after his multiorgan system failure had subsided, though he later experienced dyspnea and exercise intolerance. Pulmonary function tests were performed and found he had reduced left lung volume with restrictive disease of unknown etiology. This finding, along with his existing connective tissue disorder and pulmonary artery aneurysm, led to the proband being referred to medical genetics with suspicion of Loeys–Dietz syndrome.
During a genetics consultation, the proband agreed to multigene panels for thoracic aortic aneurysm and dissection and cardiomyopathy. The tests were negative, finding no pathogenic variants related to Loeys–Dietz, Ehlers–Danlos, or Marfan syndrome. As a next step, whole-exome sequencing (WES) and mtDNA analysis were performed. WES uncovered the likely pathogenic variant c.1039 C>T (p.R347X) in TAB2 in a heterozygous state, which we propose is responsible for the symptoms seen in the patient. This variant inserts a stop codon near the middle of the protein, resulting in TAB2 truncation and dysfunction.
Knowing of the likely pathogenic variant afflicting our proband, his sister was evaluated as well. She is a 36-year-old female who had a ventricular septal defect that was repaired in her infancy, right bundle branch and atrioventricular block, right ventricular dilation and diminished RV function, mild dilation of the aortic root, hypermobility (Beighton 5/9), unilateral sensorineural hearing loss, myopia, and mildly sloped shoulders. The similarity of her phenotype to the proband's was not a coincidence, as she was found to have the same TAB2 variant. Probands mother was negative for the familial variant, suggesting likely paternal inheritance.
In the time since the proband's genetic testing, he has continued to follow with the cardiology clinic. EKG shows severe biventricular enlargement, an LV ejection fraction of 33%, a right bundle block, enlarged atria (the right more severely so), and mild aortic and mitral valve regurgitation. He developed brief episodes of chest pain, neck swelling with underlying lymphadenopathies, and volume overload. His pulmonary valve regurgitation and pulmonary artery aneurysm have worsened since his surgery at age 35, with the aneurysm reaching 5.2 cm. These symptoms prompted his placement on the cardiac transplantation list at age 39. The proband has been considered for a percutaneous pulmonary valve replacement or some form of mechanical circulatory support including a left ventricular assist device (LVAD), biventricular assist device (BIVAD), or total artificial heart while he waits for a transplant. |
pmc-6081489-1 | An 81-year-old man had been diagnosed with early gastric cancer and had undergone gastrectomy with Billroth I construction at 60 years of age. Currently, he underwent upper-gastrointestinal endoscopy for anemia that revealed an irregular lesion in the remnant stomach, for which he was referred to our hospital for further examination. Endoscopy and upper-gastrointestinal tract examination revealed type 3 advanced gastric cancer in the upper body of the stomach and slightly invading the esophagus. (Fig. ). A biopsy specimen confirmed a poorly differentiated adenocarcinoma (Her-2 negative). An abdominal computed tomography (CT) scan showed the thickened gastric wall and two swollen PANs that were 70 mm and 30 mm in diameter, respectively (Fig. ). We diagnosed the patient with unresectable RGC (Borrmann type 3, cT4a, cN1, cH0, cP0, cM1 (LYM), cStage IV according to the 7th UICC guidelines) and administered SOX chemotherapy. We expected that the tumor would be down staged after chemotherapy. S-1 (100 mg/body/day) was orally administered twice daily for the first 2 weeks of a 3-week course. Oxaliplatin was administered as an intravenous infusion of 150 mg/body/day on day 1 of each course. The patient completed three treatment courses without severe adverse effects, although he experienced mild but tolerable weakness and could continue treatment. Upper-gastrointestinal endoscopy after chemotherapy demonstrated that the gastric lesion had disappeared, and a gastric ulcer scar could be observed. Additionally, the abdominal CT revealed a reduction in the size of the PAN to 60% of the original mass. PET-CT was performed and there were no distant metastases. We thought an R0 resection was possible and considered an indication for conversion surgery. Hence, 36 days after the administration of the last dose of chemotherapy, we planned to perform radical surgery. Laparotomy findings showed no peritoneal metastasis, and peritoneal lavage cytology revealed no cancer cells in the abdominal cavity; we performed total remnant gastrectomy and D2 lymphadenectomy as well as PAN dissection with Roux-en-Y reconstruction. The time taken for surgery was 459 min, and the total blood loss was 340 mL. On macroscopic observation, ulcer scars were observed in the remnant stomach (Fig. ). Microscopic examination revealed no tumor cells in the ulcer scar of the resected remnant stomach or in any of the lymph nodes including the PANs harvested from the surgical specimen. The therapeutic effect of SOX chemotherapy was grade 3, i.e., a complete response according to the Japanese guidelines on gastric cancer [].
The patient’s postoperative course was uneventful, and the patient was discharged on postoperative day 24. Adjuvant chemotherapy with S-1 was performed in the outpatient setting, and the patient has remained disease-free for 1 year after surgery. |
pmc-6081512-1 | A 17-year-old African American female with a negative past medical history except intermittent asthma presented to the emergency room complaining of cough and chest pain for the past two weeks in addition to rib pain, back pain, and weakness for about one month. The chest pain had been worsening upon deep inspiration over the past few days. The pain was unlike what the patient had experienced previously associated with acute asthma exacerbations and was refractory to beta-agonist treatment.
One month prior, she had given birth to a 32-week gestation infant after an unremarkable pregnancy and delivery. Two weeks prior to this presentation, the patient had been seen in another emergency room for similar symptoms. A chest X-ray and computed tomography (CT) of the chest was performed then which were read as normal.
Upon examination in the emergency room, the patient was noted to be afebrile and on pulse oximetry was saturating at 98% on room air. An electrocardiogram showed sinus tachycardia at 110 beats per minute with possible left atrial enlargement and no S-T segment or T-wave abnormalities. A chest X-ray revealed a right basilar opacity and bilateral pleural effusion consistent with a diagnosis of pneumonia (). Treatment with intravenous antibiotics was initiated, and the patient was transferred to the pediatric floor.
On the floor, additional history of having experienced swelling of her lower limbs and joint pain in her hands was obtained. Physical examination revealed the following findings: right-sided metacarpophalangeal, wrist, elbow, and knee swelling and erythema, decreased range of motion of both knees, bilateral conjunctival erythema, bilateral nonpitting pedal edema, mild diffuse abdominal tenderness, a confluent erythematous maculopapular rash involving both upper and lower extremities, and a malar rash.
Laboratory investigations were notable for a white blood cell count of 2.4 thousand per cubic millimeter (nl 4.8–10.8 thousand per cubic millimeter) with 84% neutrophils (nl 40–80%), 9% bands (nl 0–6%), and 6% lymphocytes (nl 15–50%), a creatinine level of 1.24 milligrams per deciliter (nl 0.3–0.8 milligrams per deciliter), hemoglobin level of 9.6 grams per deciliter (nl 10.7–17.3 grams per deciliter), an albumin level of 2.9 grams per deciliter (nl 2.7–4.8 grams per deciliter), an erythrocyte sedimentation rate of 72 millimeters per hour (nl 0–20 millimeters per hour), antinuclear antibody titer of 1:2560 with a homogeneous pattern (nl < 1 :80), and an anti-double-stranded DNA antibody level of >1000 iU/ml (nl ≤ 30). Urinalysis was positive for protein of 100 milligrams per deciliter (nl negative) and white blood cells of 12–20 per high power field (nl negative). This was followed by a spot urine protein-to-creatinine ratio which revealed significant proteinuria (871 millligrams per gram creatinine (nl 80–200 milligrams per gram creatinine)) consistent with SLE related kidney disease. All other laboratory values including rheumatoid factor, complement studies, and coagulation studies were normal. Additionally, bilateral lower extremity venous duplex studies showed no evidence of deep vein thromboses.
After approximately forty-eight hours on the pediatric floor, the diagnosis of SLE was confirmed—the antibiotics were discontinued and the patient was started on high-dose intravenous methylprednisolone. Soon after the steroids were started, the patient became hypertensive (180/114 mmHg) and bradycardic with unbearable headaches prompting transfer to the pediatric intensive care unit. Upon transfer, the patient's physical and neurologic examinations were normal except for mildly decreased air entry at the lung bases. An echocardiogram was performed which showed an ejection fraction of 55–60%, mild to moderate mitral regurgitation, peak systolic pulmonary artery pressure of 45 mmHg (moderately increased), normal-sized pulmonary arteries, moderate to severe tricuspid regurgitation, a mildly dilated right atrium, and a less than 50% variation in respirophasic changes in the inferior vena cava and hepatic veins. The patient was started on intravenous hydralazine and oral nifedipine. A second chest X-ray was unchanged. A repeat electrocardiogram showed marked sinus bradycardia at 42 beats per minute with a prolonged QTc interval of 462 milliseconds and flipped T waves in the anterior leads. The patient was then transferred to a tertiary facility for management of her pulmonary hypertension, dysrhythmia, and further investigation and management of her nephrologic and rheumatologic disease. |
pmc-6081519-1 | The procedure discussed in this case report was performed at the Department of Clinical Surgical, Diagnostic and Paediatric Sciences, University of Pavia, Italy, and the procedure was approved by the University Ethics Committee (recorded March 2014).
A 52-year-old woman, with a good health status (ASA score: 0), was enrolled for the study; written informed consent was obtained from the patient to have the case details and any accompanying images anonymously published. She was indicated for a prosthetic implant rehabilitation procedure in the second quadrant after a maxillary sinus lift procedure for atrophy of the maxillary bone at the bicuspid and molar level (1 mm residual bone crest height) in order to collect enough bone to install two endosseous implants (Figures and ).
The patient was prepared for the surgery with scaling and root planning two weeks prior to the sinus floor lift. The surgery was performed under antibiotic prophylaxis: amoxicillin plus clavulanic acid (Augmentin, GlaxoSmithKline S.p.A., Verona, Italy), 2 gr 1 hour before the surgery. For the local anesthesia, articain 4% with 1/200000 epinephrine was used.
A full-thickness flap was lifted via mesial and distal relief incisions. From the palatal flap, a 3 mm periosteal sample was harvested and then washed with a sterile saline solution. Then, it was inserted in the Rigeneracons filter with 1 ml of sterile saline for the disaggregation process (Figures and ).
Tissue graft disaggregation was performed for 2 minutes at 70 rpm and 15 Ncm torque, and the cell suspension was withdrawn with a sterile syringe and added to the PLGA-HA scaffold (Alos®, Allmed srl, Lissone, MB, Italy) in order to be grafted into the new subantral cavity (). In the meantime, the receiving site was prepared according to the standard protocol used for lateral sinus floor augmentation () [, ]. The wall osteotomy was performed with Piezosurgery® (Mectron S.p.A., Carasco, GE, Italy) using an OT5 insert. A resorbable membrane (Bio-Gide®, Geistlich Pharma AG, Wolhusen, Switzerland) was positioned in the newly formed subantral cavity to preserve the sinus membrane, and the space was then filled with the blend of PLGA and micrograft (Figures and ). The bone window was covered with collagen sponges (Gingistat®, Pierre Rolland Pharmaceutical, Merignac, Aquitaine, France) soaked in the cell suspension and a resorbable membrane (Bio-Gide, Geistlich Pharma, Wolhusen, Switzerland) (). The flap was sutured with a 4-0 PTFE suture (Omnia S.p.A, Fidenza, PR, Italy).
During the postoperative period, the patient received antibiotic therapy (1 gr every 12 hours of amoxicillin + clavulanic acid for 7 days) and performed oral rinses with chlorhexidine 0.2% (Curasept®, Curaden Healthcare S.p.A., Saronno, VA, Italy), 3 times/day for 30 days, and she was administered nonsteroidal anti-inflammatory drugs (NSAIDs) if needed.
The healing was uneventful and the sutures were removed after 2 weeks.
At 4 months after the surgery, following a cone-beam CT examination demonstrating a good level of bone regeneration, a mucoperiosteal flap was elevated and two bone tissue carrots 3 mm in diameter were harvested from the implant sites using a trephine bur. Two 3.8 × 9 mm implants were installed (Camlog® Promote® Plus, Camlog Biotechnologies AG, Basel, Switzerland) according to the standard protocol []. The insertion torque was 25 N/cm. The mucosal flap covered the fixtures during the healing phase, and the sutures were removed after 10 days. No adverse events occurred ().
The collected tissues were fixed in a 10% formalin solution and then prepared for microscopic observation in order to determine the ossification grade (). Paraffin-embedded tissue sections were cut into 5 μm-thick slices, following which the paraffin slices were immersed in xylene and then in decreasing grades of ethanol (100% to 75%) and deionized water for deparaffinizing and rehydrating the sections. Subsequently, the slide sections were stained with hematoxylin for 1-2 minutes and rinsed in cold water to remove excess stain. The sections were then stained with eosin for 4-5 minutes and rinsed under running tap water. The tissue sections were then immersed in increasing grades of ethanol (from 50% to 100%), and finally after an immersion in xylene, they were coverslipped with a mounting medium. Histological analyses demonstrated that the combination of micrografts with the PLGA scaffold allowed the ossification process. In fact, at 40x magnification (), lamellar bone formation was observed, as seen by the presence of a typical Haversian system with the deposition of a calcified matrix.
8 weeks after the implant installation, the following standard prosthetic procedures were performed: implant impression, abutment and structure proof, and cemented prosthesis delivery.
After three years, during the follow-up visit [], radiographs were taken, which demonstrated an excellent stability of the graft and of the regenerated bone and the success of the rehabilitation (). |
pmc-6081520-1 | A 73-year-old woman was referred to our department with a complaint of asymptomatic gross hematuria. She had experienced a urinary tract infection and urolithiasis several years ago. On cystoscopy, we identified a gross hematuria from the right ureteral orifice. Noncontrast enhanced CT revealed a right renal stone and a complicated cyst in the upper pole of her right kidney, which was categorized as a Bosniak type IIF cyst on dynamic CT, with a maximum diameter of 58 mm ().
The renal stone was removed using flexible ureteroscopic lithotripsy. The calyceal diverticulum, which had been diagnosed preoperatively as a complicated cyst, was confirmed by ureteroscopy and retrograde pyelogram (). As the diverticulum was filled with a soft protein matrix that was adherent to its wall, it was difficult to remove all the contents of the diverticulum while preserving the inner surface of the calyceal. With many fragments of the soft protein matrix floating in the calyceal diverticulum, renal pelvis, and ureter, obstruction of the ureter and ureteropelvic junction were predicted. Therefore, it was necessary to fully remove the matrix. Considering Bosniak type IIF classification of the cyst and the class II classification of the urine cytology examination, we proceeded with endoscopy combined with intrarenal surgery (ECIRS) to remove the contents completely, without follow-up observation.
We punctured the diverticulum and dilated it using a 24 Fr balloon catheter (X-Force N30 Nephrostomy Balloon Dilation Catheter; Bard, New Providence, NJ, USA), under ultrasound guidance and ureteroscopy, with a working sheath placed at the edge of the diverticular cavity. After the soft protein matrix was completely removed, papillary lesions were observed on the surface of the diverticulum using percutaneous nephroscopy (), and a biopsy was performed. Pathological analysis confirmed the diagnosis of squamous cell carcinoma, with the upper urinary tract stone composed of unspecified protein.
A right radical nephroureterectomy was performed using a laparoscopic approach, with curative intent. Pathological analysis confirmed a high-grade squamous cell carcinoma with renal parenchymal invasion (pT3) (). Local recurrence was confirmed by CT imaging performed 2 months after that surgery, and the patient was treated with adjuvant systemic chemotherapy, using cisplatin and gemcitabine. The patient went into septic shock during the first course of chemotherapy, requiring cessation of systemic therapy, with her general condition worsening thereafter. At that point, the patient opted for palliative care only and she passed away 4 months after the radical nephroureterectomy was performed. |
pmc-6081525-1 | A 60-year-old man with a history of alcohol abuse was found at home with impaired consciousness of unknown origin. The patient's room had numerous unlabeled cans of unknown contents. He was admitted to the hospital's intensive care unit (ICU).
On arrival at the ICU, the patient had a Glasgow Coma Scale score of 3 (3–15). His pupils were equal, round, but nonreactive to light. His skin was pale and cool to touch, and rectal temperature was 32.7°C. The respiration was deep and quiet of 40–50 breaths per minute. The rest of the physical examination was unremarkable. Blood pressure was 144/60 mmHg, heart rate was 77 beats per minute, and the saturation on 3 L of oxygen was 100%. The electrocardiogram showed a supraventricular rhythm with elevated T-waves ().
A point-of-care (POC) blood gas analysis in the ICU using Radiometer ABL800 FLEX blood gas analyzer (Medical Brønshøy, Denmark) showed pH of 6.77, pCO2 1.5 kPa, pO2 23.5 kPa, bicarbonate 2.0 mmol/L, and base excess −30 mmol/L. Serum potassium was 7.4 mmol/L, and lactate was so high that it was not measurable (). To prevent arrhythmia, intravenous calcium chloride was given prophylactically. Infusion of insulin-glucose and bicarbonate was started due to hyperkalemia and severe acidosis. Since no immediate clinical cause could be identified for the unexpected lactic acidosis, extended venous blood analyses and toxicological screening on blood and urine were performed. This revealed an osmolar gap of 106 mOsm/kg H2O, and anion gap was calculated to 41 mmol/L (). The urine sediment showed plenty of calcium oxalate crystals. Ethanol, paracetamol, and salicylate levels were negative. The plasma lactate level measured on the laboratory analyzer Vitros 5.1 (Ortho Clinical Diagnostics, Inc., Raritan NJ) was only 3.8 mmol/L. Repeated analyses confirmed high lactate levels measured on the POC blood gas analyzer and comparatively low lactate levels measured on the laboratory analyzer.
Taken together, the clinical and biochemical presentation was consistent with reports in the literature of a “lactate gap” [, , –]. From a diagnostic conundrum some years earlier [], we had learned that the finding of a lactate gap indicates intoxication from ethylene glycol rather than methanol. A tentative diagnosis of ethylene glycol intoxication was made. Intravenous fomepizole therapy was initiated with a loading dose of 15 mg/kg. Hemodialysis was started two hours after admission due to persistent severe metabolic acidosis and electrolyte imbalance. Given the patient's extremely efficient respiratory compensation, it was decided not to intubate the trachea immediately.
After the patient's metabolic status improved, the trachea was intubated, and the patient mechanically ventilated as the depressed neurological state persisted. Intravenous fomepizole treatment continued during a 6-hour hemodialysis. Osmolar gap decreased to 7 mOsm/kg H2O after 12 hours. The patient regained consciousness the next day. He was transferred to a general ward after extubation of the trachea.
The patient developed anuric acute renal failure with a peak serum creatinine concentration of 872 μmol/L for which he needed additional sessions of hemodialysis for a total of 11 days. On the 16th day, the patient was discharged from the hospital in his habitual condition. Not until the 35th day, creatinine (96 μmol/L) and glomerular filtration rate (>60 mL/min/1.73 m2 based on creatinine, age, and sex) were within the normal reference area.
Our hospital does not have access to analysis of serum ethylene glycol nor methanol. A week after, results of toxicological screening of the blood at admission showed serum ethylene glycol level of 103 mmol/L (normally not in serum). |
pmc-6081529-1 | A 48-year-old man with no apparent past medical history initially presented with abdominal pain associated with nausea and vomiting. Physical examination was unremarkable. Lab results were significant for anemia, with a hemoglobin level of 11.6 g/dL (14–18), hematocrit 32.2% (42–52), and lipase 164 U/L (13–60). His comprehensive metabolic panel was unrevealing. A CT of the abdomen and pelvis with contrast was performed, which revealed an enlarged pancreas without a focal mass, diffuse surrounding mesenteric edema, mild retroperitoneal lymphadenopathy, mild mesenteric lymphadenopathy, rectal wall thickening with perirectal lymphadenopathy, left renal mass measuring 3.7 × 1.4 × 1.4 cm, right renal mass measuring 2.3 × 4.3 × 5.2 cm, marked diffuse urinary bladder wall thickening, and trace pericardial effusion (). The concern at the time was for peritoneal carcinomatosis and/or mesenteric tumor. Urology and oncology services were invited to evaluate the patient, both in agreement for a biopsy of the lymph node; in addition, urine cytology and HIV were tested.
Urine cytology revealed atypical lymphocytes. Renal biopsy revealed diffuse large B-cell lymphoma (DLBCL). Bone marrow biopsy was consistent with involvement of B-cell lymphoma. HTLV-I/II antibody was negative. HIV screening returned with a positive result. Initial CD4 was 440/μl with a percentage of 14%, and HIV RNA viral load by PCR was 61800 copies/mL. Hepatitis C virus screening was negative; hepatitis B screening was positive for the core antibody and surface antibody but negative for surface antigen. His lactate dehydrogenase (LDH) was above the upper limit of the detection for our lab, >2500 U/L (135–225). Cerebrospinal fluid at the time revealed atypical lymphocytes.
Biopsy of the kidney mass revealed atypical lymphocytes positive for CD45, CD20, PAX-5, and CD10, while negative for CD5, CD30, MUM-1, BCL-1 (cyclin D1), BCL-2, BCL-6, c-myc, CD68, CD34, CD117, and MPO, with a high proliferation index (Ki-67) of 80% approximately. The morphology was consistent with diffuse B-cell lymphoma (DLBCL), NOS, and germinal center B-cell subtype involving the kidney. A subsequent bone marrow biopsy revealed involvement by DLBCL ().
His Revised International Prognostic Indicator (R-IPI) was calculated to be 4, NCCN-IPI of 5, and CNS-IPI of 4. Decision was made to offer him dose-adjusted rituximab, etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin (R-EPOCH) to treat his disease. For HIV, he was treated with raltegravir, tenofovir, and emtricitabine, with a subsequent decrease in viral load to our laboratory's threshold for undetectable. This regimen was chosen specifically for the tenofovir to act as prophylaxis to suppress hepatitis B virus reactivation. In addition, he was treated with fluconazole and acyclovir for antimicrobial prophylaxis. He received 3 cycles of chemotherapy and 1 dose of intrathecal methotrexate, with initial improvement in renal lesions. However, after the third cycle, he presented with dysphagia, Bell's palsy, and diffuse bone pain. Neurology service was consulted recommending MRI of the brain without contrast, which was unremarkable. Subsequent CSF flow cytometry was positive for CD10+ B-cells, with a conclusion of DLBCL infiltrating the CNS. Gastroenterology service was consulted, who performed an EGD but found no explanation for dysphagia. ENT service found mucous in the postcricoid region but had no acute interventions to offer, and evaluation by the speech and language pathologist revealed marked pharyngeal dysphagia, with high risk for aspiration.
A repeat CT of the chest without contrast revealed a right pericardial mass with mediastinal lymphadenopathy. At that time, he did not report any chest pain, dyspnea, or cough. His vital signs were remarkable only for sinus tachycardia of 110 beats per minute, and he was normotensive, tolerated room air, and maintained good mentation. A subsequent echocardiogram revealed a large loculated pericardial effusion near the right ventricle, with echocardiographic evidence of tamponade. A large mass could be appreciated in the right atrium in the lateral wall, possibly obstructing the IVC. Cardiac mass size was 2.1 cm × 2.5 cm (). This was concerning for disease recurrence. He was transferred to the coronary care unit for monitoring but did not show any clinical signs of cardiac tamponade. Treatment options were discussed with the patient, who opted for palliative care measures and a transfer to hospice care.
The patient's wishes were followed. A PEG tube was placed for nourishment, and his pain was controlled with opioid analgesics. Plans to transfer him to inpatient hospice care were put in place. Unfortunately, the patient expired before he could be discharged, 5 months following initial diagnosis of DLBCL, and within 3 weeks of pericardial involvement being discovered. His dysphagia never resolved and was likely related to the discovered cardiac mass. |
pmc-6081541-1 | Informed consent for publication was obtained from the patient. A 24-year-old right-handed man fell from the fifth floor balcony and was transported to an emergency hospital. Radiological examinations revealed that he had suffered right traumatic pneumothorax, right humeral shaft fracture, right olecranon fracture, right scapular fracture, and right radial nerve palsy (Figures and ). All fractures were closed. After respiratory system stabilization, the right olecranon and humeral shaft were operated on 11 days after the injury (Figures and ). Three months after the injury, he presented with limited range of motion (ROM) of the elbow and persistent radial nerve palsy.
Physical examination showed grip strengths (measured with a digital dynamometer) of 7.3 kg and 39.0 kg in the right and left hand, respectively. The respective ROM for the right and left extremities (measured with a standard goniometer) was as follows: elbow flexion, 110° and 140°; elbow extension, −75° and 0°; forearm pronation, 85° and 85°; and forearm supination, 65° and 90°. Muscle strength was M4 for the right triceps and M1 for the wrist and finger extensors. Sensory disturbance (3/10 on the ten test) was observed in the area of the radial nerve. The PLRI test result was negative.
Plain radiography and computed tomography at three months after the injury showed that the avulsion fracture of the lateral epicondyle became displaced and malunited, the radiohumeral joint had widened on the anteroposterior view, and the posterior subluxation of the radial head had widened on the lateral view (Figures –). We diagnosed subluxation of the radial head caused by malunion of the lateral humeral epicondyle and incomplete palsy of the radial nerve. Conservative treatment was selected for recovery of the radial nerve palsy.
Surgery was performed under general anesthesia. A lateral approach was used. The avulsed lateral epicondyle was malunited and detached from the original site. The malunited lateral epicondylar fragment was osteotomized by a chisel. The fragment contained the origin of the LCL complex and extensor muscles (). The cartilage of the capitellum and radial head was not injured. Because anatomical reduction of the fragment was impossible, the bony fragment was resected under the periosteum. The fragment size was 27 mm in length (). After the LCL complex was pulled tight, the quality of the LCL was judged as good without cheese cut. The isometric point of the LCL was confirmed during elbow flexion-extension, and the LCL successfully reached this point. The LCL was repaired using two anchors. Extensor muscles were repaired to the surrounding soft tissue. The elbow was immobilized with a splint with an elbow flexion of 90° and full pronation. Two days after surgery, the elbow was immobilized with a long arm cast in the same position. Three weeks after surgery, the elbow was fixed with a hinged brace at the forearm position in pronation. The extension of the elbow was limited to −30°. Six weeks after surgery, extension limitation reduced to 0° and forearm rotation exercises were started. Three months after surgery, the brace was removed. Six months after surgery, all range of activity was permitted.
Because the patient complained of limited elbow ROM (elbow flexion, 110°; extension, −50°) and irritation from the olecranon hardware, a second operation was performed 14 months after surgery. Under general anesthesia, the olecranon hardware was removed, the anterior and posterior capsules were released, and the posterior oblique ligament of the medial collateral ligament of the elbow was resected via a medial approach. Flexion ROM of 135° and extension ROM of −30° were achieved intraoperatively.
At the final follow-up 2 years after surgery, the motor and sensory palsy of the radial nerve recovered to levels of the healthy side and grip strength for the right and left hands were 35.9 kg and 37.3 kg, respectively. The respective ROM for the right extremity was as follows: elbow flexion, 120°; elbow extension, −35°; forearm pronation, 90°; and forearm supination, 70°. The radiographs showed normal congruence of the elbow joint without osteoarthritic change (Figures and ). |
pmc-6081547-1 | A 91-year-old Caucasian female with a history of CLL diagnosed 14 years ago, hypothyroidism, glaucoma, and severe osteoarthritis of spine presented to ER, 24 hrs after receiving first chemotherapy for her CLL with chlorambucil in the setting of recent worsening of lymphocytosis, anemia, and exertional dyspnea, with complaints of nausea and vomiting after chemotherapy. There was no diarrhea, abdomen pain, fever, and chills. She had completed a course of Bactrim for UTI a week ago. There were no recent changes in home medications. Vital signs were within normal limits, and systemic physical examination was unremarkable except for dry mucous membrane in ER. She received a liter of normal saline bolus and was started on maintenance normal saline at 75 ml/hr. She started to feel short of breath and wheezy while in ER. Her oxygen saturation dropped to 85% on room air, which was treated with bronchodilators. Upon initial workup, she was found to have leukocytosis of 210 × 103 cells/μL with 96% lymphocytes, along with chronic anemia with hemoglobin of 8.1 and hematocrit of 28. Her electrolytes and renal function were normal with Na of 137 meq/L, K of 4.6 meq/L, BUN of 15 mg/dL, and Cr of 0.8 mg/dL. CXR was unremarkable. Liver function tests were within normal limits. Uric acid was 2.8 mg/dL, and phosphorus was 2.9 mg/dL. The patient got progressively short of breath overnight after admission and was hypoxic. She was, therefore, evaluated for pulmonary embolism based on her risk profile. CTA chest was negative for pulmonary embolism. This revealed diffuse centrilobular emphysematous changes and bibasilar atelectasis. Patient's care was escalated to intensive care, and noninvasive ventilation was initiated. Repeat basic metabolic panel this time revealed K of 6.6 meq/L. Rest of labs including creatinine were essentially within normal limits. The basic metabolic panel was repeated again in 3 hrs, which revealed K of 8.5. EKG did not demonstrate peaked T waves, prolonged QRS interval, or evidence of heart block. She was treated with intravenous calcium gluconate, albuterol nebulization, IV insulin, IV dextrose, IV Lasix, and sodium polystyrene. Her K remained persistently high in 8 meq/dl most of the day despite medical management. When her K rose to 9.1 overnight, a decision was made to proceed to emergent dialysis. Interestingly, her creatinine remained stable throughout, and she was not oliguric. She received 4 hrs of hemodialysis with 2 K bath. Immediately after dialysis, she had a run of supraventricular tachycardia with a heart rate of 130, which did not improve with adenosine. Her potassium by arterial blood gas was 3.0 meq/dL, and K on basic metabolic panel was 3.1 an hour after hemodialysis. Potassium was supplemented intravenously, and she was also loaded with digoxin following which she converted to sinus rhythm. She sustained a demand ischemia with troponins going up to 10 after this event. No cardiac catheterization or ischemic workup was done, as she was asymptomatic after resolution of SVT. Her plasma K was between 3.5 and 4.7 for next six days prior to discharge home from hospital. |
pmc-6081550-1 | A 62-year-old immunocompetent man with no significant previous medical history was hospitalized for high-grade fever, intractable hiccup, and interscapular pain. On admission, his white blood cell count was 11 × 109/L (normal range 4.50–10.80 103 mmc), his C-reactive protein (CRP) was elevated at 4.30 mg/dl (normal range 0.00–0.75 mg/dl), while his chest radiograph, abdomen ultrasound, and echocardiography were normal. A computed tomography (CT) scan of the brain revealed a diffuse abnormal pattern (presence of aspecific inflammatory material) with hypodense lesions located in the trigonum of lateral ventricle in an underlying condition of demyelination and gliosis, suspicious for chronic ischemic vascular disease. A broad-spectrum antibiotic therapy with vancomycin and ceftriaxone was initiated. The patient became afebrile within a few days. A neurological examination found him to be alert and oriented, and he did not have a stiff neck. However, the patient had persistent hiccups and headache. Magnetic resonance imaging (MRI) showed enhancement of both trigeminal nerves and white spot lesions on the pons, cerebral peduncle, midbrain, and thalamus. He was then transferred to the Neurology Department where a lumbar puncture was carried out. His cerebrospinal fluid (CSF) was clear, WBC count was 50 cells/µl, 100% lymphocytes, normal glucose level (normal range 40–70 mg/dl), 103 mg/dl protein (normal range 15–45 mg/dl), and the CSF culture was negative. As a viral etiology was suspected, antibiotic therapy with vancomycin + ceftriaxone was discontinued and treatment with acyclovir and steroid was initiated. After 72 hours, a progressive deterioration of his clinical-neurological condition occurred: he became hyperpyretic and aphasic and Glasgow Coma Score (GCS) was 9. CT brain imaging showed the involvement of the subcortical left temporoparietal lobe, and he was then transferred to the Infectious Disease Department. Blood cultures were performed, and another lumbar puncture was carried out. A cerebrospinal fluid (CSF) analysis showed cloudy CSF with increased spinal column pressure, granulocytic pleocytosis (180 cells/µl, with PMN 90%), normoglychorrachia, and 145 mg/dl spinal fluid protein. A combination antimicrobial therapy with ampicillin 3 g/6 h + gentamicin 80 mg/8 h was initiated; 72 hours later, fever and other systemic signs and symptoms disappeared resulting in complete recovery (GCS15). Listeria monocytogenes were isolated from the patient's blood and recognized from CSF using the molecular technique (Multiplex Real-Time PCR Meningitis/Encephalitis Filmarray bioMerieux). The patient was treated with intravenous ampicillin for 4 weeks, with combination intravenous gentamicin for the initial 2 weeks and switched to oral trimethoprim/sulfamethoxazole 160/800 mg/8 h for 1 month. An MRI was repeated after 8 weeks of antibiotic therapy due to the persistence of fluent aphasia. MR imaging showed a ring-enhancing lesion in the left fronto-temporoparietal lobe, consistent with a brain abscess with significant perilesional edema (). Surgical excision of the lesion was performed. Molecular identification of the pus using polymerase chain reaction (PCR) identified DNA of Listeria monocytogenes. The patient was represcribed intravenous ampicillin + gentamicin for 4 weeks, and therapy was then switched to oral trimethoprim/sulfamethoxazole 160/800 mg/12 h for further 4 weeks. Patient's condition has improved progressively and with a complete recovery of linguistic abilities. |
pmc-6081550-2 | A 72-year-old man with a history of bullous pemphigoid treated with a monoclonal antibody was admitted to another hospital due to a balance disorder. A neurological examination identified a left hemiplegia with no sensory deficits. An immediate CT brain scan showed a ring-enhancing cortical-subcortical lesion on the right frontal-parietal hemisphere. In view of the CT scan findings, gadolinium MRI of the brain was performed. MRI showed a caudal extension of the lesion with irregular enhancement and a necrotic region (). Blood cultures were collected before initiating antimicrobial therapy. A few days later, his blood cultures grew Listeria monocytogenes. Based on organism sensitivity, intravenous therapy with ampicillin 3 g/6 h + gentamicin 80 mg/8 h + vancomycin 1 g/12 h was initiated. Steroid therapy was also administered due to the associated moderate mass effect. The patient was then transferred to our Infectious Diseases Department for further workup and management. Forty-eight hours after the initiation of target therapy, the patient was afebrile. Twenty days later, he showed progressive clinical and neurologic deterioration characterized by visual hallucinations, frontal symptoms with disinhibition, and persistent hemiplegia. An MRI brain scan showed a substantial increase in lesion size, and new lesions appeared on splenium of corpus callosum and right temporal lobe with a significant mass effect on the right lateral ventricle. Trimethoprim/sulfamethoxazole 160/800 mg/8 h was added. The patient underwent a surgical biopsy of the lesion. Molecular identification of the brain tissue using PCR identified Listeria monocytogenes DNA. At the follow-up appointment five weeks later, additional imaging studies were performed which showed a considerable reduction in the size and enhancement of the lesions. Ampicillin, gentamicin, and vancomycin therapy was stopped while trimethoprim/sulfamethoxazole therapy was continued. The patient's neurological condition improved. An MRI brain scan performed after 8 weeks of antibiotic therapy, showed significant improvement, with noticeable decrease in the amount of vasogenic edema. Trimethoprim/sulfamethoxazole therapy was discontinued, and the patient was discharged. A year after the listeria brain abscess diagnosis, the patient does not show any significant neurologic deficits and is able to carry out all activities of daily living. |
pmc-6081566-1 | A 64-year-old woman was brought to the emergency unit with chief complaints of headache and fever for one week in September 2017. She felt pain over her whole calvarium without a specific trigger point. Her body temperature was 38.7 degrees Celsius. There was no nausea, vomiting, blurred vision, or nuchal stiffness. Cranial and peripheral neurologic function did not have any impairment. Superficial lymphadenopathy, breast nodules, and abdominal tumor mass were not detected on palpation. A Babinski sign was absent. She denied drug or alcohol abuse.
Laboratory examination disclosed a normocytic anemia with hemoglobin level of 7 g/dl, mean corpuscular volume of 89.3 fl, platelet count of 325000/μl, and white blood cell count of 12000/μl comprising neutrophils 61.7%, lymphocytes 26.7%, and monocytes 10.8%. A leucoerythroblastic picture was not present. Blood chemistry tests revealed abnormally elevated serum alkaline phosphatase of 158 iu/l (normal 32~91) and lactate dehydrogenase of 292 iu/l (normal 98~192). There was neither microhematuria nor stool occult blood. Both activated partial thromboplastin time and prothrombin time were normal in value but the D-dimer was extremely high: 6570 ng/ml (normal 0~500).
The chest X-ray routine film showed no active lung lesions. A computerized tomography (CT) scan of head was ordered to rule out intracranial abscess or other central nervous system problems. The brain turned out to be intact but, unexpectedly, multiple osteolytic lesions were detected in the skull, extraordinarily obvious upon comparison with previous films taken six years earlier for other reasons (). She was then admitted to the ward under a suspicious impression of multiple myeloma.
Subsequent immunofixation electrophoresis analysis of serum, however, did not show any evidence of monoclonal gammopathy, and the levels of serum immunoglobulin G, A, and M were all within normal ranges. In contrast, serum tumor markers carcinoembryonic antigen (CEA) and cancer antigen 15-3 (CA 15-3) were meaningfully increased in concentration: CEA 8.1 ng/ml (normal 0~5) and CA 15-3 163.2 iu/ml (normal 0~31.3), respectively. Levels of cancer antigen 19-9 (CA 19-9) and cancer antigen 125 (CA-125) were within normal limits. Afterwards, a CT scan of chest and abdomen revealed osteosclerotic and osteolytic lesions similar to that of skull involving almost the whole skeleton () but not a single clear-cut primary tumor site could be located.
Bone marrow aspiration and biopsy were performed on the right side posterior superior iliac crest. The aspiration smear demonstrated very scanty hematopoietic precursors scattered among crowded groups of oval neoplastic cells with large hyperchromatic nuclei, coarse chromatin, modest granular cytoplasm, and occasionally small nucleoli (). Frequent rosette-like openings were seen in the tumor groups. A metastatic adenocarcinoma was considered as the most likely diagnosis.
Pathologic study of the biopsy specimen after decalcification displayed a picture of metastatic adenocarcinoma composed of round to oval tumor cells arranged in ovoid clusters, small rounded nests, cribriform nests, and focal microacinar pattern stuffed in bone marrow cavity (). The immunohistochemical stains gave positive results for cytokeratin 7 (CK7), cytokeratin 8 (CK8), estrogen receptor (ER) (strong, 99%), progesterone receptor (PR) (strong, 99%) (), Smad4 (DPC4), GATA binding protein 3 (GATA-3), gross cystic disease fluid protein 15 (GCDFP-15), and mammaglobin (). While human epidermal growth factor receptor 2 (HER2/neu), cytokeratin 20 (CK20), thyroid transcription factor-1 (TTF-1), paired box gene 8 (PAX8), and synaptophysin were negative. The positive rate of Ki-67 in tumor cells was 20%. Diffuse bone marrow metastasis from breast carcinoma (invasive ductal carcinoma, not otherwise specified) thus should be considered firstly as the diagnosis based on aforementioned pathological findings and differential diagnosis markers recommended in the literature [, ].
Mammogram brought to light a suspect primary site in the right breast as compared with films taken five years earlier for cancer screening (). The lesion was also detectable in magnetic resonance imaging (MRI) (). Nonetheless, no tumor was found by both physical examination and sonogram of breasts. Additionally, the patient's husband and sons did not want her to know the nature of the disease, so a breast biopsy was pitifully not done at last.
Due to our worry about the risk of fracture associated with aromatase inhibitors [], the patient began to take tamoxifen, 20 mg twice daily, for treatment. The effect seemed very satisfactory. After a period of probable “tamoxifen flare” with elevation of serum CA 15-3 and alkaline phosphatase levels for six weeks [], both of them dropped gradually within four months (). Fortunately, there was no hypercalcemia noted during the whole clinical course. Serum CA15-3 was within normal limits at the last follow-up on March 21, 2018. Positron emission tomography/CT (PET/CT) scans during the treatment course confirmed an impressive improvement of the bone marrow uptake. The mild activity remained in the lumbar spine could be either residual metastasis or, more favored by us, compensatory hyperactive hematopoiesis in its recovery phase (). The patient did not have headache or fever anymore and was enjoying a happy life up to the time of submitting this case report with a hemoglobin level of 12.2 g/dl. |
pmc-6081595-1 | A 23-year-old man presented in 2014 with a white blood cell count of 34 × 109/L with 87% circulating blasts by manual differential count. Flow cytometric analysis on the peripheral blood (PB) revealed 89.6% blasts by CD45/SSC gating. The blasts expressed CD10, CD19, CD34, HLA-DR, and CD20 (dim); partially expressed CD13, CD15, and CD33; and did not express CD2, CD7, CD56, and CD117. A subsequent bone marrow (BM) biopsy was done the next day but was a dry tap; therefore, flow cytometry and other cytogenetic studies were not performed on the BM sample. Immunohistochemistry (IHC) studies were performed on the BM biopsy showing 95% blasts positive for CD79a, PAX-5, and TdT, and negative for CD20 and myeloperoxidase (MPO). The morphology and immunophenotype of the blasts in the peripheral blood and bone marrow biopsy were consistent with B-lineage lymphoblastic leukemia. We did perform FISH analysis with a B-ALL panel on the peripheral blood specimen which revealed CDKN2A (p16 at 9q21) gene deletion on one or both chromosomes 9. Fusion of BCR and ABL1 was not detected. Intensive chemotherapy was initiated according to the CALGB 10403 “Adolescent Young Adult” regimen [], and the patient achieved complete remission. He then proceeded to consolidation without consideration of allogeneic hematopoietic cell transplantation (allo-HCT) based on standard of care for B-cell ALL with favorable cytogenetic and molecular profile.
However, the patient had prolonged cytopenias during consolidation therapy culminating in treatment delay. In early January of 2015 (approximately 24 weeks after initial diagnosis), the patient's complete blood count revealed 27% blasts. Flow cytometry analysis was performed on the PB; the blasts expressed HLA-DR, CD15, CD33, and CD117; partially expressed CD13 and CD56; and did not express CD2, CD3, CD5, CD7, CD10, CD19, CD20, or CD34. A restaging BM biopsy was performed the same day; unfortunately, it was again a dry tap, so flow cytometric analysis could not be performed. The histology examination of the BM biopsy revealed a hypocellular (30%) marrow with 85% recurrent leukemic blasts. IHC showed the blasts were now positive for MPO, while CD10, PAX-5 CD20, CD79a, and TdT were negative. The overall findings on PB and BM biopsy were consistent with a lineage switch to acute myeloid leukemia (AML) (). Although it was a dry tap, a very small amount of aspiration specimen was obtained in the EDTA tube. Despite few marrow spicules on the aspiration smear, cytogenetic analysis and FISH studies were performed. FISH analysis with both B-ALL and AML panels showed persistence of a heterozygous CDKN2A deletion, plus the acquisition of a TP53 deletion, and 7q and 17q duplications. Cytogenetic studies now showed a complex karyotype in 18/20 metaphases, 47,X,-Y,add(1)(p36.1),+18,+add(18)(q23) [12]/47–48,X,−Y,del(1)(p32p36.1),add(11)(p11.2),+18,+18[cp6]/46,XY [].
Intensive salvage chemotherapy was initiated with the MEC regimen [], and nadir BM evaluation was hypocellular without blasts, although FISH demonstrated low-level CDKN2A deletion, consistent with minimal residual disease. Ten days later, a rising PB blast percentage prompted another BM biopsy which showed persistence of AL with the same phenotype and FISH with CDKN2A deletion. Further intensive chemotherapy was initiated, but the patient ultimately died of respiratory failure and refractory AL. |
pmc-6081596-1 | A 59-year-old female initially presented to the emergency department 10 months prior with right arm pain and swelling. A computed tomography of the neck with contrast at the time showed two confluent masses in the right axillary and right supraclavicular regions encasing the right subclavian and axillary vein, the internal mammary artery, and narrowing of the lower internal jugular vein. Patient was subsequently diagnosed with primary mammary carcinoma of the axilla with metastasis. The patient was evaluated by the oncology and radiation oncology services and underwent multiple rounds of chemotherapy and radiation therapy. Her course of chemotherapy was complicated by thrombocytopenia and metastatic disease progression. The patient was determined to be a nonsurgical candidate. Gradually, her pain of the right upper extremity worsened, and the patient was started on opioid therapy. Despite titration of her oral medications to extended release morphine 90 mg two times a day, immediate release morphine 30 mg every 2 to 3 hours, methadone 5 mg daily, and gabapentin 800 mg three times daily, her pain control remained suboptimal. She was referred to our pain clinic for further management of her intractable pain.
On presentation, the patient reported a constant 10/10 pain on the numeric pain rating scale (NRS) of the right proximal humerus, right anterior and posterior shoulder, and right supraclavicular region. The pain was reported as dull, aching, burning, and electric in nature. Her pain was worsened by passive and active range of motion, and the pain at its best was a 7/10 with oral medications. Patient also reported progressive weakness of the entire right upper extremity. Magnetic resonance imaging of the brachial plexus was obtained, and the study revealed a mass encasing the right brachial plexus at the level of the divisions and cords as well as the right brachial artery ().
Diagnostic brachial plexus block was performed in the hospital due to functional decline and acute worsening of pain. The brachial plexus was unable to be visualized using ultrasonography in the classic supraclavicular area due to the tumor effect, thus the block was performed at the level of the trunks using a lower interscalene approach. 18 mL of 0.5% ropivacaine was injected under direct ultrasound guidance. The patient reported complete pain relief lasting approximately 12 hours after the nerve block. Given the success of the diagnostic nerve block, the patient was offered the option of chemical neurolytic brachial plexus block with an extensive discussion of the unique risks, benefits, and alternatives. The patient elected to proceed to a right brachial plexus nerve block with dehydrated ethanol. |
pmc-6081600-1 | A 35-year-old female was diagnosed with type I DM at the age of 9 years. During childhood her DM was poorly controlled and the patient gained significant weight. At the age of 25 years her weight was 105 kg with a body mass index (BMI) of 40 kg/m2 and her renal function started to deteriorate with progression to requiring hemodialysis by age 30. With development of renal failure, secondary hyperparathyroidism was noted. Due to her obesity, she was not eligible for a renal transplant or a SPK. At this point it was decided to offer her bariatric surgery, and, after extensive discussion, it was felt that a RYGBP was the best option for her in terms of weight loss. At the age of 32 years, she underwent uneventful robotic-assisted surgery; the stomach remnant was attached to the abdominal wall for potential future access.
Over the next 2 years she lost 60 kg and underwent SPK during which the donor duodenal segment was diverted to a bowel loop distal to her Roux loop implant site into the common channel. Induction immunosuppression with alemtuzumab was followed by maintenance with tacrolimus (trough levels 5-7 ng/mL), mycophenolate-mofetil (2 g daily), and a steroid taper. She was CMV seronegative and received a graft from a CMV positive donor and received standard prophylaxis with oral ganciclovir (GCV) for 100 days. Within 100 days posttransplant, she was readmitted to the hospital with acute CMV disease, which was successfully treated with intravenous ganciclovir.
Shortly after this episode the patient was found to have skin lesions on her right leg, which were diagnosed as calciphylaxis. Her serum calcium at that time was 14 mg/dl and the diagnosis of tertiary hyperparathyroidism was made. A three-and-a-half-gland resection together with subtotal thymectomy was done without any complications; the left lower parathyroid gland was the only normal appearing and half of it was preserved taking care that blood supply remained intact. Intraoperative parathyroid hormone levels dropped from >1500 to 150. Calcium serum levels within 24 hours postoperatively were 9 mg/dl with ionized calcium of 3.5 mg/dl. She was discharged in good condition within 24 hours postoperatively with daily calcium supplementation of 4.5 g/day divided into three doses.
During the following week her calcium levels started to drop and on day 10 postoperatively at an outside hospital serum calcium was found to be critically low at 5.5 mg/dl with an ionized fraction of 2.1 mg/dl. However, the patient had remained clinically symptom free. She was admitted for intravenous calcium replacement. Pushes of calcium were unable to appropriately raise her calcium levels and, therefore, a calcium drip (85 mg/h) was started. Her calcium levels came up to 7.1 mg/dl. Oral calcium dose was raised to 15 g/day and hydrochlorothiazide was started. The calcium drip was stopped and the patient was discharged home in good condition.
Intense calcium supplementation was continued. Gradually the patient's gastrointestinal tract started to adapt and after two years her calcium levels started to stabilize. She has not experienced any additional complications from her transplant or gastric bypass. She is currently alive with excellent function of both grafts, normal calcium levels, stable weight, and an excellent quality of life almost five years after her last surgery. |
pmc-6081802-1 | A previously healthy 32-year-old man, presented to the outpatient department of our institution with a 3-day history of high fever, sore throat, and mild diarrhea in early September 2016. His chief complaints were severe myalgia in both sides of his cervical and trunk muscles (around the pectoralis major, rectus abdominis, and trapezius areas), in addition to muscles of the upper and lower extremities (both proximal and distal), and orchiodynia. Additionally, he complained of inadequate sleep due to severe leg pain that led him to fear being unable to rise from bed after lying down. He therefore stood by his bed throughout the night.
On physical examination, the patient’s height was 171 cm and body weight was 67 kg (body mass index = 22.9). There was no paresis or muscle tenderness noted, and all deep-tendon reflexes were normal. His pain did not extend to the facial, hand, foot, or joint regions. No tenderness was observed in the testes, despite the complaint of orchiodynia. Rectal examination did not indicate prostatitis. He was fully conscious, and no paresis, speech disturbance, or skin eruptions were observed. The differential diagnoses initially included periodic paralysis, myasthenia gravis, adult-onset Still’s disease, fibromyalgia, and chronic fatigue syndrome.
An antigen-based rapid diagnostic test detecting both influenza virus A and B yielded a negative result. His white blood cell count was 3700/mm3, serum C-reactive protein (CRP) level was 1.41 mg/dL (normal range: < 0.2 mg/dL), serum creatine phosphokinase (CK) level was 48 U/L (normal range: 60–230 U/L), and serum myoglobin level was 63.1 ng/mL (normal range: 20.3–92.3 ng/mL). All liver and thyroid function tests, electrolytes, and serum ferritin level were within normal limits. Two sets of blood cultures both yielded negative findings. Circulating anti-nuclear, anti-acetylcholine receptor, and anti-neutrophil cytoplasmic antibodies were not detected.
At the time of case presentation, the patient’s wife had just delivered a daughter and was temporarily staying at her parents’ house. The patient and his wife also had a 3-year-old son, with whom the patient stayed at their own home following his wife’s parturition. He worked in an office and sent his son to a nursery school during the daytime working hours. Five days before the patient’s initial visit to our hospital, his son developed a fever and mild throat pain, and several infants at the nursery school also developed mild flu-like symptoms and mild diarrhea that improved over 2–3 days. To rule out the possibility of enterovirus infection, serum antibodies were tested for coxsackievirus (type A2, A4, A5, A6, B2, B4, B5, B6) and echovirus (type 13, 30) at the initial visit and 2 weeks later with the neutralization test method. Significant antibody titer changes between acute and convalescent phases were not detected and a serological diagnosis was not established for these enteroviruses. Because adult HPeV-3 infection may occur shortly after an epidemic of pediatric infection, nested polymerase chain reaction (PCR)-based detection tests for HPeV-3 were performed, and the HPeV-3 types were identified by sequencing the VP3/VP1 junction in PCR products amplified directly from the specimens []. We collected blood samples from the patient at the first visit and 2 weeks later, and obtained stool samples from all family members several days later. We also collected specimens from the patient’s parents because the severity of his myalgia restricted his ability to move freely in his house, and he required assistance from his parents in caring for his son and himself.
HPeV-3 was detected in the patient’s initial blood and stool samples and his son’s stool sample (Fig. ); viable HPeV-3 viruses were isolated from stool specimens using a cell culture method. We confirmed that the sequences of the PCR-amplified regions were identical in specimens from the patient and his son, indicating that the son had transmitted HPeV-3 to the patient. The results from the mother and newborn daughter, who had not stayed with the patient and his son during that time, were negative. The results from the patient’s parents were also negative.
He was finally diagnosed with severe epidemic myalgia caused by HPeV-3 infection. Without specific treatment, his severe myalgia, fever, and orchiodynia gradually improved. After a week, he had no difficulty in moving his whole body, and no sequelae remained. Additionally, we measured serum cytokine levels at the initial visit and 2 weeks later. Tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), IL-10, and IL-12 levels were measured by enzyme-linked immunosorbent assays. A cut-off value for each assay was as follows: TNF-α and IL-10, below 2 pg/mL; IL-6 and IL-12, below 4 pg/mL. Although the values of his TNF-α, IL-10, and IL-12 levels were all below each cutoff value, his IL-6 level was elevated to 63.6 pg/mL at the initial visit. Two weeks later, his IL-6 level had decreased to almost half (34.9 pg/mL), but remained higher than normal. |
pmc-6081819-1 | We report a case of a 43-year-old Sri Lankan Sinhalese woman who presented to our Teaching Hospital, Peradeniya, in the morning with a history of fever, arthralgia, myalgia, and headache of 4 days’ duration. She had been previously diagnosed as having type 2 diabetes mellitus and dyslipidemia for which she received treatment with satisfactory control of the medical conditions and she had normal renal functions.
She had experienced postural dizziness since the afternoon of the previous day and she had had nausea, vomiting, and abdominal pain since the morning of the previous day. Soon after admission she collapsed in our emergency care unit. She was severely dehydrated with cold clammy peripheries. Her pulse rate was 130 beats per minute with an unrecordable blood pressure. She had reduced breath sounds in the base of her right lung and marked tenderness over the right hypochondrium with flank dullness with shifting. She was drowsy, but arousable.
Her complete blood count showed hemoglobin of 15.3 g/dL, platelet count of 74 × 103/microL, and white cell count of 3.22 × 106/microL. Her serum creatinine was 277 micromole/L with potassium of 5.8 mmol/L. Alanine transaminase (ALT) was 6542 U/L with aspartate transaminase (AST) of 30,617 U/L. Her serum albumin value was 24 g/L. A bedside ultrasound scan demonstrated bilateral pleural effusions (more in the right) with free fluid in her abdomen. A diagnosis of DHF with decompensated shock complicated with acute liver failure and acute kidney injury was made. DF was confirmed by positive non-structural protein 1 (NS1) antigen and serotype was identified as dengue virus type 2 (DEN-2). Both dengue immunoglobulin M (IgM) and immunoglobulin G (IgG) were positive suggesting a secondary infection with dengue virus (DENV). She was screened for alternative causes for liver necrosis including hepatitis A immunoglobulin A, hepatitis B surface antigen, hepatitis C IgM, leptospirosis serology, and rickettsial serology, which were negative. Her baseline transaminase levels and serum creatinine done 1 month earlier at a medical clinic were within the normal range. She denied taking supra-therapeutic dose of paracetamol or other native medical preparations for her fever. She had been treated with metformin and atorvastatin until the previous night.
She was assumed to be at the peak of the critical phase of DF (that is, 24 hours in the plasma leakage) on admission. On admission her packed cell volume (PCV) was 44%. (Her baseline PCV was 33% for hemoglobin of 10.5 g/dL in her clinic book.) She was given a 10 ml/kg crystalloid bolus (500 ml) over 15 minutes followed by 10 ml/kg bolus over 1 hour. She was given a 10 ml/kg dextran 40% bolus in the next hour. She had heavy per vaginal bleeding and one episode of melena. Her PCV dropped from 44 to 33% without clinical improvement and she had low urine output (< 0.5 ml/kg). She was administered packed cells to maintain the PCV around 40% to a total volume of 1200 ml.
During the latter 24 hours of the critical phase of DF, she had severe metabolic acidosis with lactic acidosis: PH of 7.2, bicarbonate 8 mmol/L, and partial pressure of carbon dioxide 16 mmHg with a lactate level of 12 mmol/L, which was corrected medically with 8.4% sodium bicarbonate 200 ml in divided boluses. Her ionized calcium was persistently low and corrected with multiple boluses of intravenously administered calcium gluconate. Her blood sugar was checked every 2 hours and corrected accordingly.
At the end of presumed critical phase, we gave her 5500 ml of fluid including normal saline, dextran, and packed cells. She was conscious, rational, but drowsy. She had a spiking high temperature. She was icteric but not pale. She was breathless at rest with oxygen saturation of 85% on room air, which increased to 95% with 60% oxygen via mask. Her pulse rate was 120 beats per minute with blood pressure of 140/100 mmHg. She had bilateral pleural effusions up to mid zone. Her liver was 5 cm below the costal margin with normal upper border and markedly tender. She had gross ascites in a horseshoe-shaped distribution. Her serum creatinine was raised to 345 micromol/L with serum potassium of 5.8 mmol/L and during the last 6 hours of the critical phase she was anuric. Her ALT was 8010 U/L and AST 41546 U/L. Her prothrombin time was 22.1 seconds (control 12 seconds) and activated partial thromboplastin time (APTT) was 42 seconds (control 26 seconds). C-reactive protein (CRP) was 240 U/L. Her blood sugars were elevated toward the end of presumed leaking phase of DHF.
At the end of the presumed leaking phase of DHF our patient had: massive liver necrosis; acute kidney injury with acidosis, hyperkalemia, and anuria; deranged clotting with bleeding; and symptomatic volume overload with large plural effusions and gross ascites. Many concerns rose at this point regarding management:The fluid in the third space mainly in the pleural and peritoneal cavities would get reabsorbed and as she probably had an established acute kidney injury with anuria, the reabsorbed fluid would accumulate in her intravascular compartment leading to expansion of intravascular volume and massive volume overload with pulmonary edema and heart failure. Massive liver necrosis with deranged synthetic function would worsen the lactic acidosis which in turn would have a negative effect on the inotropic effect of her heart, clotting derangements might aggravate the bleeding risk, and ongoing hypoxia of the liver might further damage her liver. Sepsis with high fever and elevated inflammatory markers. What is the focus? Management of uncontrolled blood sugar. Is it due to her existing type of diabetes or pancreatitis?
She was started on continuous renal replacement therapy (CRRT) with CVVHD. We decided to keep CVVHD running and titrate the ultrafiltrate according to the volume state. Observations on central venous pressure (CVP) and blood pressure were made hourly. Intermittent measurements of her inferior vena cava (IVC) diameter and internal jugular vein (IJV) diameter were noted. We assumed that reabsorption of the fluid in the third space would increase the CVP, distend the IJV and IVC, and would increase mainly the diastolic pressure. Depending on the above assumptions, observations were made and ultrafiltrate was gradually increased. Surprisingly, fluid reabsorption occurred in an exponential pattern over a period of 5–6 days and came to a halt abruptly (Figs. and ). Maximum ultrafiltrate was 280 ml/hour. Heparin was not used in CVVHD due to high risk of bleeding.
She was started on intravenous NAC 100 mg/hour infusion which was continued for 5 days. She was given orally administered metronidazole 400 mg 8 hourly and syrup lactulose to maintain bowel motion 2–3 times per day. She was started on an intravenous infusion of proton pump inhibitors, intravenously administered tranexamic acid, and orally administered norethisterone. She was given intravenously administered vitamin K 10 mg daily for 3 days. She was given 4 units of fresh frozen plasma and 10 units of cryoprecipitate, and 6 units of platelets to correct the coagulopathy. She was transfused with packed cells to maintain PCV around 40% in order to maintain adequate oxygenation of hepatocytes. CVVHD was continued and her lactate level was noted to decline gradually.
She had spiking high fevers on day 3 of hospital stay with high CRP. Septic screening was done with blood culture and urine culture and intravenously administered ceftriaxone was changed over to renal-adjusted dose of intravenously administered meropenem and teicoplanin. Later cultures were negative after 72 hours of incubation. However, gradually her fever settled by lysis of fever over the days.
Although during the presumed critical phase her blood sugar was rather low, her blood sugar started to rise over the days. She was a type 2 diabetic with good control with metformin. Her blood sugar was checked hourly and insulin infusion was continued and titrated according to her blood sugar. Her amylase was 450 U/L (normal range 1–37 U/L).
Over a period of 6 days she was closely monitored. Gradually her transaminases declined, her lactate level normalized, and serum creatinine reduced and normalized (Table ). Her urine output gradually increased. CVVHD was terminated after 138 hours of dialysis. She was discharged on ninth day of admission after restoring her full physiology. She was discharged on Mixtard insulin (biphasic isophane insulin injection) for diabetic control. She was reviewed in the ward after 3 days, 7 days, and at 1 month after discharge. Her renal functions and liver functions were within the normal range. She was started again on metformin and atorvastatin 1 week after discharge and insulin was discontinued. |
pmc-6081828-1 | A 68-year-old man presented to our institution. He previously underwent laparoscopic intersphincteric resection of the rectum with diverting loop ileostomy for lower rectal cancer at the age of 56. The immediate postoperative course was uneventful. A pathological examination revealed that the patient had T1, N1a, M0, Stage IIIA rectal adenocarcinoma. He did not receive adjuvant chemotherapy, and there was no recurrence of cancer during the follow-up period. However, soon after surgery, he developed an anastomotic stenosis requiring repeated endoscopic balloon dilatation.
His loop stoma was not reversed because these treatments were unsuccessful. Therefore, the surgeon proposed surgical resection of the anastomosis, but the patient did not agree to undergo permanent colostomy. Consequently, he had lived with loop ileostomy since undergoing surgery.
Twelve years postoperatively, he felt swelling and pain in the scrotum. He was seen by a local physician and diagnosed with a perineal abscess. He was referred to a urologist at our hospital for treatment. Computed tomography (CT) scans revealed a low-density area and air-fluid level in the perianal region (Fig. ), which was consistent with the signs of a perianal abscess. Drainage was performed, and the abscess was resolved. The urologist was concerned about the cause of the perineal abscess, and anastomotic complications were considered to be associated with the abscess. The patient was then referred to the department of surgery for further evaluation and treatment.
During examinations, we found that the patient did not have a significant medical history, including inflammatory bowel disease. He denied having any allergies. He had undergone surgery for an inguinal hernia and duodenal ulcer. He quit smoking after undergoing rectal cancer surgery. He took oral antibiotics after the perineal abscess was drained; otherwise, he denied taking any routine medications.
He was 161 cm in height and 50 kg in weight, and his body mass index was 19 kg/m2. A physical examination revealed a well-healed scar on his abdomen and a loop stoma in the right lower quadrant. A digital examination revealed severe stenosis at 2 cm from the anal verge. The patient was afebrile and did not have pain in the scrotum or anus. Given his stable medical condition, we judged that urgent surgical intervention was unnecessary. Close observation with a radiological evaluation was planned.
One month later, the patient developed skin ulcers in the right ankle (Fig. ) and around the stoma (Fig. ), with a fever over 39 °C. He also presented with bloody discharge per anus. Laboratory data showed there was an elevated C-reactive protein level (11.1 mg/dL) and white blood cell count (7710/mL), while the hemoglobin and albumin levels were decreased (9.4 and 2.6 g/dL, respectively). Regarding the skin ulcers, the culture studies were negative for bacteria, whereas a pathological examination of the biopsy specimen revealed pyoderma gangrenosum. These findings suggested that the patient’s acute illness may have reflected an inflammatory response rather than infectious disease.
Colonoscopy revealed that the bowel lumen had narrowed at 2 cm from the anal verge and extended to 10 cm from the anal verge, where a fistula was incidentally found (Fig. ). Moreover, edematous and easily hemorrhagic mucosa was observed throughout the proximal colon (presumably the transverse colon). The endoscopic examination was terminated soon after we detected a mucosal laceration in the transverse colon that was possibly caused by air insufflation (Fig. ). A pathological examination of a biopsy specimen of the colon revealed inflammatory mucosa with infiltration of neutrophils, eosinophils, and lymphocytes. These findings were interpreted as nonspecific colitis and did not indicate a definitive diagnosis of a condition such as UC, DC, or infectious colitis. Contrast-enhanced abdominal CT did not show signs of cancer recurrence or bowel ischemia.
A contrast enema confirmed that the distal bowel was narrowed with a fistula, whereas the remnant colon appeared to be consistent with the “lead pipe” phenomenon (Fig. ). We observed active and diffuse mucosal inflammation that indicated UC; pyoderma gangrenosum of the skin is well-known to be an extra-intestinal feature of UC. Given the findings of the physical, laboratory, and imaging studies, we hypothesized that the pathogenesis of the disease was either UC in the diverted colon or DC mimicking UC.
After reviewing the patient’s clinical episodes and extensively discussing the case with gastroenterologists, surgeons, stoma nurses, and the patient, we proposed total colectomy with end ileostomy. Then, the patient finally agreed to undergo this procedure.
With the patient under general anesthesia in the lithotomy position, we performed total colectomy with open laparotomy and transperineal resection of the rectum, in accordance with the abdominoperineal resection. The loop ileostomy was taken down and the end ileostomy was reconstructed at the same stoma site. The operative time was 267 min and the estimated blood loss was 210 mL.
The postoperative course was uneventful, and he was discharged home on the 11th postoperative day.
A macroscopic view of the resected specimen is shown in Fig. . The coloanal anastomosis had significant stenosis. There was a fistula proximal to the anastomosis. There was atrophic mucosa, and haustra had disappeared from the distal colon. In contrast, the mucosa appeared to be reddened, edematous, and rather dilated in the proximal colon. The pathological findings included erosion and ulceration in the edematous wall, a crypt abscess, and inflammatory infiltration into the mucosa throughout the colon (Fig. –), without any evidence of dysplasia or carcinoma. The pathological diagnosis was consistent with UC. Given that he developed these inflammatory mucosal alterations after the surgery requiring fecal diversion, we concluded that DC mimicking UC was appropriate for the final diagnosis.
The skin ulcers in the right ankle and around the stoma healed over time. Six months after the last surgery, the ulcer areas were significantly reduced (Fig. –). Three years after the last surgery, the skin ulcers were completely healed with some scar tissue (Fig. –).
We reported the rare case of a man with acute and severe DC mimicking UC with extra-intestinal manifestations, and he was successfully managed with surgical treatment. His clinical symptoms and histological findings were consistent with the findings of UC, suggesting there is a possible pathogenetic link between DC and UC.
DC is a frequently seen consequence of the interrupted fecal stream and is characterized by nonspecific mucosal inflammation. In patients who undergo fecal diversion surgery, 70–91% have the endoscopic diagnosis of DC, and 70–100% of those have some histological changes [, , , ]. Typically, the altered bacterial flora decreases luminal SCFA in the diverted intestine, and this may play a role in the development of DC [, ]. Despite the high incidence of DC, most patients are asymptomatic [, ]. Surgical re-anastomosis is the most favorable treatment, whereas some medications are also available [–]. However, acute and severe colitis may occur [, ], and a careful assessment with a prompt decision about the treatment is required in clinical settings.
In our case, the decision-making process was as follows. Initially, we considered nonsurgical, conservative treatment targeting UC. However, an enema or oral agents could not reach the diseased colon directly, and steroids or immunomodulators may have provoked a secondary infection and/or recurrent perineal abscess. The presence of a fistula also prohibited the use of these agents. There does not appear to be sufficient evidence supporting cytapheresis for the treatment of DC.
Second, the friability of the colonic mucosa represented by insufflation-induced lacerations required urgent and effective treatment. Although the patient underwent diversion, he was nearly at risk of experiencing colonic perforation. Moreover, imaging studies had already shown the presence of a mucosal fistula.
Third, pyoderma gangrenosum of the skin had worsened rapidly. These features were the patient’s chief complaints, and delayed or prolonged treatment would have diminished his quality of life. Finally, we considered reversing the fecal stream with loop ileostomy closure. However, we deemed it impossible because of the anastomotic stenosis and colonic fistula. Thereafter, we decided to perform surgical resection of the residual colon, including the coloanal anastomosis.
To diagnose DC, it is necessary to exclude other bowel disorders such as IBD, radiation colitis, infectious colitis, and nonsteroidal anti-inflammatory drug-associated colitis. A histological examination is paramount for the diagnosis; however, a comprehensive assessment of the patient’s disease, social, and medical histories is also required to determine the treatment strategy.
DC is regarded as a multifactorial disease []; the causative factors include alterations in intestinal bacterial flora [, , , ], overproduction of oxygen free radicals, impairment of butyrate oxidation [, ], defects in the transport of SCFA, and immunological factors []. Regarding immunological factors, some intestinal mucosal changes or disruption may produce inflammatory or immunoregulatory cytokines, and they induce predominant T helper cell type 1 (Th1). A previous report demonstrated that the Th1 phenotype might be the pathogenesis of DC []. Others indicated that tumor necrosis factor alpha, interleukin 1 beta, interleukin 6, and transforming growth factor beta are important in the treatment of DC [–].
The endoscopic features of DC include erythema, friability, and edema, and in severe cases, ulcers, filiform and inflammatory polyps, and strictures are found [–]. The most common pathological features include expanded lymphoid aggregates and inflammation in the lamina propria with lymphocytes and plasma cells [, ].
In some cases, follicular lymphoid hyperplasia with crypt distortion and basal lymphoplasmacytosis were observed in DC [, , ]. In such cases, the crypts may appear atrophic and short and are displaced by lymphoid infiltrates. Lymphoid hyperplasia, in which the germinal centers of B and T cell lymphocytes are enlarged, is found in pediatric patients but not in all adult patients with DC [, , , , ]. The microscopic abnormalities found in those with DC may also occur in those with UC, and these diseases cannot be distinguished from one another []. One study hypothesized that the histological resemblance of DC to active UC suggests there is a pathogenetic link between the two diseases [].
Immunological factors could play important roles in the pathogenesis of both DC and UC. In our case, the differential diagnoses of DC and UC remain unclear. The acute illness of this patient was represented by a perineal abscess, high-grade fever, bloody discharge per anus, and skin ulcers, which could be interpreted as UC with extra-intestinal manifestations. The colonic fistula may indicate chronic inflammation in the diverted colon. These findings suggest that DC may mimic UC, and acute progression of the disease can eventuate in a long-term period in patients without a pre-existing IBD.
This report has some limitations. There is still controversy about how to distinguish DC and UC, and there are concerns about total colectomy in patients with acute and severe diseases. Larger case series are required to compare symptomatic and asymptomatic patients with DC and should include periodic histological examinations of the diverted colon. This will help to elucidate the pathogenesis of DC. |
pmc-6081829-1 | A 72-year-old male visited Gangneung-Wonju Dental Hospital due to pain and induration of the left pre-auricular area. He had no specific medical history and discomfort and crepitus during mouth opening that had persisted for a couple of years. Recently, he suffered from pain upon palpation of the left pre-auricular area. The patient had a mild limitation of mouth opening that was 30 mm. A radiologically, well-defined calcified mass was observed surrounding the left mandibular condyle in cone beam computed tomography (CBCT) images (Fig. a). The mass was 49 × 35 × 25 mm in size and encompassed the mandibular condyle as a round shape. It occupied the infratemporal fossa and parotid gland area (Fig. b) and medially extended to the area near the pterygoid plate of the sphenoid bone. Due to the extension of the mass, the mandible condyle was laterally displaced. The margin of the mass was clear, and small calcified materials were distributed throughout the mass. An infiltrative sign to the surrounding tissue was not observed. This case report was approved by the institutional review board (IRB) of Gangneung-Wonju National University Dental Hospital (2017-018).
This mass was assumed to be a benign calcifying lesion, such as pseudogout or synovial chondromatosis. We decided to surgically excise the mass and perform a biopsy under general anesthesia. We planned a surgical excision of the mass on the lateral aspect of the condyle using a pre-auricular approach. In addition, for complete excision of the mass, the medial aspect of the condyle and infratemporal fossa area was accessed by resecting the condyle after vertical ramus osteotomy of the mandible. After complete excision of the mass, the separated condyle segment was repositioned to its original location and fixed with a titanium plate (Fig. ).
The pre-auricular incision was performed and slightly extended to the left retromandibular area to approach the left mandible angle area, similar to an S shape. The skin flap was raised to expose the superior, anterior, and inferior border of the mass. The mass was bluntly dissected from adjacent soft tissue to prevent facial nerve injury. One half of the mass was first removed by excision, and then, the lateral surface of the condylar process was exposed. Before vertical osteotomy of mandible ramus, two titanium mini-plates were pre-drilled and adapted to the lateral surface of the ramus for accurate repositioning of the condyle segment. In addition, all of the titanium mini-plates and screws were removed. L-shaped ramus osteotomy was performed from the sigmoid notch to the posterior border of the mandible with a reciprocating saw. After extracorporeal removal of the condyle segment, the residual half of the mass on the medial aspect of the condyle and infratemporal fossa was carefully dissected with the pterygoid muscles and excised without damaging the surrounding vessel and nerve tissue. Next, the condyle segments were repositioned and fixed to the remaining mandible body with two titanium plates and mini screws (Fig. ). The defect was filled with a gelatin sponge, and then, a layered suture was performed. After surgery, the patient showed transient weakness of the facial nerve. Otherwise, the 3-month post-operative follow-up was uneventful and he shows normal jaw movement.
The excised specimen was stained with hematoxylin and eosin (H&E) for a histopathological examination and was observed using a polarized microscope under polarized light. This amorphous tissue showed abundant basophilic crystal deposit material in the connective tissue stroma. The material of this crystal deposit had a rod and rhomboid shape and was mostly surrounded by histiocyte, fibroblast, mononuclear inflammatory, and multinucleated giant cells (Fig. a). Phagocytosis by multi-nucleated giant cells was frequently observed around the crystal material. The weak strained crystal materials formed tophi and exhibited birefringence in polarized light. The rhomboidal shape of the crystal materials was prominently observed in a polarized microscope view (Fig. b). The length of the crystal was approximately 10–20 μm, and its diameter was 1–2 μm. The histological features strongly suggested CPDD in the joint space. Furthermore, the histological diagnosis was suggested as CPDD on the left TMJ.
SEM/EDS microanalysis was performed to analyze the element composition of the crystal deposit in the specimen. The paraffin block-embedded specimen was sectioned into 5-μm sections and de-paraffinized. Unstained sections were coated with 0.7 nm of OsO4 (HPC-1SW; Vacuum Device, Inc., Mito city, Japan). The specimen was observed using a scanning electron microscope (Quanta FEG 250, FEI, Oregon, USA), and its chemical composition was analyzed using an energy-dispersive spectrometer (EDS) (Octane Elite EDS, EDAX, New Jersey, USA) attached to the SEM.
The result of the SEM/EDS microanalysis was shown in Fig. c. The EDS spectrum showed peaks of calcium (Ca) and phosphorus (P) in the crystal deposit. The first Ca peak has a similar energy position with oxygen. And the high intensity of the first peak would be attributed to the oxygen peak. Oxygen concentration cannot analyze quantitatively by EDS method; thus, it was excluded in the chemical concentration result. The EDS data was adjusted by the ZAF correction, which considers atomic number, self-absorption, and fluorescence effect to eliminate the atomic number effect on the quantitative analysis of chemical composition. The Ca and P weights in the crystal deposit were 69.84 and 30.16%, respectively (Fig. c). The EDS spectrum indicated that the crystal deposit consisted of calcium pyrophosphate dihydrate. The EDS mapping signal showed the distribution of Ca and P elements in the surface of the crystal deposit (data not shown). The high proportion of Ca and P in the crystal deposits further supported the histological diagnosis of CPPD in the left TMJ.
CPDD is a rare pathology in the articular tissue and forms a calcified crystal mass in the synovial membrane []. CPDD has been found in a local or generalized form []. Local CPDD can be observed in degenerative or necrotic tissue, resulting from a secondary trauma []. Generalized CPDD is associated with metabolic disorders, such as hyperparathyroidism, hypothyroidism, hypomagnesemia, and hyperphosphatemia []. In addition, diabetes mellitus increases the incidence of CPDD []. The site that is predominantly involved is a relatively large joint, such as the knee, shoulder, hip, and wrist of the hand, and sites that are less commonly involved are small joints, such as the TMJ []. In CPDD of the TMJ, the calcified crystal mass occupies the TMJ space and induces pre-auricular swelling, pain, tenderness, and limitation of the joint movement []. In our case, the patient experienced discomfort and crepitus of the left TMJ a couple of years ago. Recently, he suffered from pain in the left pre-auricular area and limitation of mouth opening.
CPDD in the TMJ should be differentiated from other neoplastic disorders []. Radiographically, CPDD in the TMJ appears with small, multiple, and radio-opacity nodules around the TMJ []. Occasionally, the calcified radio-opaque mass is extended into adjacent areas, such as the skull base or middle cranial fossa []. The radiological findings of CPDD in the TMJ are non-specific and are difficult to differentiate from other diseases by clinical and radiographic findings [, ]. The radiological feature of CPDD mimics other neoplasms such as synovial chondromatosis, osteochondroma, and chondroblastoma or malignant tumors [, ]. The calcified mass observed in our case occupied the left TMJ space and extended into the infratemporal fossa. The mass was well-defined and consisted of small calcified particles (Fig. ). The mass was a suspicious, benign calcifying lesion that included CPDD or synovial chondromatosis. A biopsy and histological examination were recommended to confirm the diagnosis.
Microscopically, CPDD is characterized by the deposition of a basophilic calcium pyrophosphate dihydrate crystal in the joint space []. The crystal in CPDD has a rhomboid structure and is birefringent under polarized light []. The birefringence is a key differential diagnostic tool between gout and CPDD. The gout crystal demonstrates negative birefringence []. The specimen in our case had numerous rod- and rhomboid-shaped crystals that were surrounded by histiocyte and giant cells (Fig. ). In addition, the crystals had a strongly positive birefringence under a polarized microscope. Consistent with these findings, it was strongly suggested that CPDD occurred in the TMJ. However, other crystal materials, such as calcium oxalate, synthetic steroids, and ethylenediaminetetraacetic acid (EDTA), can be positive birefringent under polarized light []. For a definitive diagnosis of CPDD, other quantitative and chemical analyses were performed []. Electron probe microanalysis can be used to analyze the composition of the crystal. In addition, this technique has been used to detect Ca and P in specimens and for differential diagnosis of CPDD [].
In our case, SEM/EDS microanalysis was used to analyze the elemental composition of the crystal. The SEM/EDS results showed that the crystal mass completely consisted of calcium pyrophosphate dihydrate. The weight composition of the crystal was 69.84% of Ca and 30.16% of P (Fig. c). In a previous study, CPDD occurring in the TMJ showed numerous crystalline deposits and consisted of 100% calcium pyrophosphate dihydrate in infrared spectrophotometry []. In addition, the CPDD crystal in the left TMJ showed a high concentration ratio of Ca and P in the SEM/EDS microanalysis []. The composition of Ca and P in our specimen confirmed the diagnosis of CPDD. Diagnosis of CPDD should be based on quantitative and chemical analyses. Using SEM/EDS microanalysis, we analyzed the composition of the crystal mass and performed a differential diagnosis of CPDD with other diseases.
The infratemporal fossa is a highly complex anatomical region that has various nerves and vessels exiting from the foramina of the skull base []. The inferior boundary of infratemporal fossa is the medial pterygoid muscle and the temporal bone locates in the superior and posterior region of the infratemporal fossa. Superiorly, it is bounded by a greater wing of sphenoid bone and, anteriorly, by the posterior border of the maxillary sinus []. The medial border consists of the pterygopalatine fossa and pterygoid plate []. Laterally, the zygomatic arch, temporalis muscle, condyle, parotid gland, and facial nerve are surrounded []. The surgical approach to the infratemporal fossa has been performed by the pre-auricular and temporal approach []. Osteotomy of the zygomatic arch, mandible condyle, and coronoid process has been performed for surgical access to the middle cranial fossa [, ]. The mandibular condylar process is temporarily resected to the access of the infratemporal fossa and excised the chondrosarcoma []. After osteotomy of the mandible condyle, the internal maxillary artery, pterygoid muscle, and mandibular nerve can be accessed [].
The crystal deposit mass in our case extended to the infratemporal fossa and lateral pterygoid plate area in the CBCT (Fig. ). Condylar resection was required to access the infratemporal fossa and for complete excision of the mass. Before vertical osteotomy of the mandibular condylar process, plates and screws were pre-drilled on the condylar neck and mandible ramus area. Next, osteotomy was performed from the sigmoid notch to the posterior border of the mandible. After resection of the condyle segment, the crystal mass was carefully dissected from the surrounding structure including the internal maxillary artery, pterygoid muscles, and mandibular nerve (Fig. ). After excision of the mass, the segmented condyle was anatomically repositioned with the pre-drilled plate and screws (Fig. ). After the operation, the condyle segment was successfully repositioned and the patient showed normal jaw movement and mouth opening. The patient showed transient weakness of the facial nerve. Otherwise, the post-operative follow-up was uneventful. |
pmc-6081846-1 | A 66 year-old woman presented with gas and FI for twenty years. She has one daughter, born in 1970 by cesarean section after a long labor without pelvic lesions or lacerations. She takes the following home therapy:Telmisartan 40 mg, 1 tablet at 12 h and 1 tablet at 20 h; Levotiroxine 75 mcg, 1 tablet at 8 h; Bromazepam 1,5 mg, 1 tablet at 8 h and 1 tablet at 20 h; Clomipramine 10 mg, 1 tablet at 8 h; Nebivol 5 mg, 1 tablet at 8 h.
She refers FI of liquid or solid stool and gas incontinence two-three times per day, which had a marked negative impact on her social life. She is suffering from anxiety-depressive syndrome that worsened because of incontinence. She has changed her lifestyle, her behavior and she is very embarrassed of her incontinence.
Physical examination: nothing to report.
Rectal exploration: anal sphincter hypotonia.
Anorectal manometry was performed with detection of:Low median basal pressure: 20 mmHg (normal range 40–60 mmHg), The lower limit of normal pressure after maximal voluntary contraction: 93 mmHg, Duration of maximal voluntary contraction 15 s (normally more than 1 min), Sensitivity threshold to 30 ml (normally 40–60 ml), Threshold of subjective reflection to 40 ml (normal value 50–70 ml), Normal threshold of the inhibitory objective reflex: 40 ml (normal value 30–50 ml) (Fig. ).
She did not perform any medical therapy for incontinence but she has performed 3 cycles of rehabilitation with anorectal biofeedback with poor benefit.
We proposed to the patient to undergo SECCA procedure.
Lithotomy position, general anesthesia was performed. A dose of 500 mg of metronidazole was administered intravenously to induction of anesthesia. Then, 20 applications of RF through the 4 nickel needles of the device were performed from the dentate line and proceeding cranially every 5 mm to 2.5 cm total. The same procedure was performed on the 4 quadrants of the internal anal sphincter, including the recto-vaginal wall (which is often the thinnest area and for this reason not always surgically treatable). The entire procedure lasted 40 min.
The day after surgery she was discharged in good health. After 10 days she presented intermittent hyperpyrexia, leak of purulent material through the anus and anal pain. We performed general physical and proctologic examination with anoscope and found evidence of abscess of the right posterior-lateral anal wall at 2 cm from the anal verge. We have sent the purulent material for bacterial culture and antibiogram: “Escherichia coli multi resistant”. The patient has performed blood tests without indices of inflammation replaying (Figs. and ). The authors administered antibiotic therapy with metronidazole and ciprofloxacin without satisfactory improvement of the symptoms.
Surgery has been organized to remove the abscess after 20 days from SECCA procedure.
The patient was in lithotomy position. Metronidazole 500 mg was administered intravenously. The authors explored the anal canal finding about 2 cm from the anal verge, a recess of about 2–3 cm in diameter, undermined for about 1 cm in cranial direction. Opening and deroofing with curettage of the fundus treated the abscess (Fig. ).
The day after surgery she was discharged in good health. Four days after the procedure, the patient was in good conditions. At 6 months follow up the patient was in good health and during the anal exploration it was possible to feel a rectal depression in the wall with a smooth consistence. In spite of the complication and subsequent surgical treatment, the procedure has been able to ameliorate the patient incontinence. |
pmc-6081948-1 | In 2015, a 47-year old man presented with pain in the TMJ (temporomandibular joint) and whose aesthetic concern was having a chipped maxillary central incisor veneer, as seen in Fig. , and . After a clinical and radiographic analysis, as seen in Fig. , a loss of the vertical dimension and tooth ware, caused by bruxism, was diagnosed.
Digital intraoral photographs were taken from a retracted frontal view, occlusal view and lateral view and extra oral photos (frontal, lateral and 45º) with a digital single lens reflex (DSLR) camera. A diagnostic impression of both arches was made with an intraoral scanner (Carestream 3500) as seen in Fig. . A maximum intercuspation position (MIP) was registered intra orally with the intraoral scanner (Carestream 3500), and the new vertical dimension of occlusion (VDO) was obtained by opening the appropriate amount on the virtual articulator in the CAD/CAM (computer-aided design/computer-aided manufacturing) software. The digital smile design (DSD) dynamic documentation protocol was applied: four videos were taken with a smart phone (iphone 6) from various calculated angles to achieve an ideal development of the facially smile frame. A facial frontal video with and without retractor smiling, a profile video, a 12 o’clock video and an anterior occlusal video perpendicular to the occlusal plan without mirror were recorded. Four more complementary videos, a facial interview, a 180º phonetics video, an intraoral functional and structural videos using a retractor, as seen in Fig. , were taken for functional, facial and structural analysis. The information was sent to the DSD Lab. The main goal of the DSD technique is to reconcile the photos of the three views (occlusal, frontal and 12 o’clock) with a digital ruler to create a smile frame supported by video analysis. Then a facially guided smile frame was created following these steps: digital facebow, smile curveshape and position, width determination using the recurring aesthetic dental (RED) proportion, length proportion, gingival curve, papillae curve, vermilion curve and arch curve. The 2D smile frame was turned into a 3D digital wax-up on CAD software. The final 3D file STL format was exported to a printer which generated the model with the new design. It was then used to fabricate a matrix for the motivational mock-up, made with bisacryl (Structur; VOCO), as seen in Fig. and Fig. . In the new model, vertical dimension was augmented, so the patient spent two weeks with the provisional mock up to test the adaptation to the new vertical dimension (VD). After this bite was test driven, and since the patient was comfortable and stable, there was no need for further deprogramming the bite and defining a new centric relation (CR). With this new VD, the patient felt more comfortable and had no pain on the TMJ. The treatment plan was presented but, due to economic reasons, the patient did not want to continue the treatment.
In 2017 the patient returned to restart the treatment, as seen in Fig. , and a new intra oral scan (Carestream 3600) was made, as seen in Fig. . A new mock up for tooth preparation was made with bisacryl (Structur; VOCO) using a vaccum formed matrix (V-print Ortho clear; VOCO) printed by a 3D printer (Soflex; VOCO). Guided by the mock up, the abutment teeth were minimally prepared, as seen in Fig. . The old preparations from the second sextant teeth were maintained, no preparation in the posterior upper (14–17, 24–27) and lower teeth (34, 35, 36, 37, 44, 45, 46, 47), and a minimal preparation on the anterior lower teeth (31–33, 41–43) was made. A new intra oral scan (Carestream 3600) was made, as seen in Fig. . The information was sent to the DSD lab, as seen in Fig. , which then produced a STL file with virtual models that were sent and fabricated in the lab (Anatomic Lab). These 3D models (V-Print model; VOCO) were printed in a 3D printing machine (Solflex 650; VOCO). The definitive veneers and crowns were prepared digitally, using prosthetic software (Ceramill mind, Amann Girrbach), and fabricated in a milling machine (Ceramill Motion 2, Amann Girrbach) with machinable lithium disilicate ceramic blocks (VITABLOCS TriLuxe forte for Ceramill Motion 2, Amann Girrbach), as seen in Fig. .
After confirming marginal fit and optical properties in a trial insertion, isolation with a lip retractor (OptraGate, Ivoclar Vivadent) was applied. Following the manufacturer’s recommendations, the abutment teeth and ceramic crowns and veneers were prepared: the ceramic surface was prepared with aluminium oxide 50 μm, hydrofluoric acid 5% 20 s and rinsed 20 s, phosphoric acid 37% (Total etch, Ivoclar Vivadent) and alcohol 96% for cleaning, and silane 20 s (Monobond plus, Ivoclar Vivadent). The crowns (11–13, 21–23) and veneers (14, 15, 16, 17, 24, 25, 26, 27, 31, 32, 33, 34, 35, 36, 37, 41, 42, 43, 44, 45, 46, 47) were adhesively luted to the abutments using a light polymerizing resin luting agent (Futurbond U and Bifix QM; VOCO) polimerized by a high-power LED curing light device (Celalux 3; VOCO), as seen in Fig. and Fig. . All the excess of the luting agent was removed and occlusal adjustments were done and confirmed using T-scan technology (T-scan; TeK-scan) as seen in Fig. , , , and , Fig. and Fig. . A removable appliance in acrylic was made for protection of the final restorations.
After 6 months, final restorations were evaluated and they remained stable, without any fracture trait. Patient also referred that with the new vertical dimension he had no headaches. |
pmc-6081951-1 | A 22-year-old man presented with a 70-day history of bilateral lower extremities weakness, followed by fever, productive cough and jaundice. On March 20th 2017, the patient suddenly developed lower limb weakness without any other notable symptoms. After the patient’s admission to the local hospital, brain computed tomography (CT) scan found no abnormalities while the contrast magnetic resonance imaging (MRI) detected multiple lesions in the brain and abnormal signals in spinal cord on T2 (Fig. ). Chest CT scan revealed multiple nodules in both lungs and a large lesion in lower left lung combined with cavity formation and gas-fluid levels inside (Fig. ). Abdomen CT scan showed hepatosplenomegaly and multiple retroperitoneal lymph node enlargement. Laboratory tests reported a leucocyte count of 12.7 × 103 cells/μL, a alanine aminotransferase activity (ALT) of 116 U/L, a total bilirubin concentration (TBil) of 37 μmol/L (direct bilirubin (DBil): 31.8 μmol/L), an serum albumin level of 25 g/L and moderate anemia. HIV serology test, HBV/HCV serology test, serum T-SPOT.TB test, blood culture and cryptococcal latex agglutination test (CLAT) were all negative. Lumbar puncture revealed a decreased glucose concentration, an increased protein concentration and a normal leucocyte count of the cerebrospinal fluid (CSF). The results of bacterial and fungal cultures, CLAT, indian ink staining and acid-fast staining of CSF were all negative. The local hospital suspected disseminated tuberculosis infection and empirical anti-tuberculosis treatment with isoniazid, rifampin, ethambutol and pyrazinamide was prescribed.
After the treatment, no remission of the previous symptoms was observed and the patient developed new discomforts including fever (Tmax 39 °C), productive cough and jaundice in the early April. Repeated blood, sputum and CSF cultures all came back negative. During the next two months, the patient continuously to have intermittent low grade fever, weakness of lower extremities and jaundice and by the end of May, rashes started to appear over his face and back. The local medical facility then transferred the patient to our hospital on June 7th, 2017.
A careful history was taken and the patient reported no significant past medical history and denied any history of addiction, drug abuse, and exposure to toxic matter. During examination, physician identified multiple papules on the patient’s face and backside, which were 0.5 cm to 2 cm in diameter with crusted center (Fig. ). His skin and conjunctiva were yellow and both legs’ muscle strength were rated grade 0. He had slightly increased muscle tone and positive Babinski signs in both lower extremities. Laboratory testing revealed a C-reactive protein concentration of 29 mg/L, a ferritin concentration over 2000 ng/ml, an ALT of 36 U/L, an aspartate transaminase activity (AST) of 119 U/L, and a Tbil of 302.6. HIV RNA test were negative, and the lymphocyte subsets were within normal range. Blood gas analysis suggested a type II respiratory failure and other laboratory results were all insignificant. Contrast MRI scans revealed multiple lesions in the brain with ring enhancement, and abnormal enhanced lesions in spinal cord, pia mater spinals and vertebrae similar to previous imaging results. Contrasted CT scan further discovered suspicious lesions in liver, spleen, left adrenal gland, prostate, intra-abdominal lymph nodes and positron emission tomography/computed tomography scan found abnormal uptakes in these regions as well. After obtaining written consents from the patient, bone marrow biopsy and skin biopsy of the facial lesions were conducted on June 8th followed by lumbar puncture and fibro bronchoscope in later days. All samples were sent for culture and NGS (See Additional file for NGS method) and during the following days, only supportive treatments were given.
On July 11th, NGS reported detecting 18,987 of 28,648,375 T.marneffei nucleotide sequences (Cover rate: 0.066%) in bone marrow and 144,780 of 28,648,375 T.marneffei nucleotide sequences (Cover rate: 0.51%) in DNA extracted from the skin lesions (Fig. ). Considering the notably high sequencing reads compared to the negative control (in which no T.marneffei nucleotide sequence was detected) and the patient’s clinical manifestation, physicians reached the diagnosis of T.marneffei infection and intravenous anti-fungi therapy consisting of 25 mg amphotericin B deoxycholate and 250 mg itraconazole per day was initiated. On July 14th, culture of the skin lesion tissue reported T.marneffei (Fig. ) and 7 days later, repeated blood and bone marrow culture for bacteria, fungi and tuberculosis all came back negative. On July 19th, NGS reported detecting 25,906 of 28,648,375 T.marneffei nucleotide sequences (0.09%) in the BALF sample and 17,877 of 28,648,375 T.marneffei nucleotide sequences (0.062%) in the CSF sample. The cultures of both CSF and BALF for fungi and bacteria came back negative (Both Myco/F lytic system from BD company and Sabouraud’s agar medium were used in fungal culture).
We then conducted sequencing of the isolated strain from the skin lesion culture, and a 97% coverage of T.marneffei was identified (Fig. ). Close relationship between our strain and other T.marneffei strains was revealed in phylogenetic analysis (Fig. ). Confirmatory nested PCR [] was done in patient’s blood, CSF, BALF and bone marrow samples and all PCR results were positive.
In consideration of all the facts, the physicians diagnosed the patient with disseminated T.marneffei infection, which invaded lung, bone marrow, central nervous system and skin. The anti-fungi therapy consisting of amphotericin B deoxycholate and itraconazole was continuously prescribed and the patient’s fever resolved three days after the initiation of the treatment, while other clinical symptoms such as cough, jaundice, skin lesions and lower limb weakness resolved within a month of the therapy (Fig. ). Until this article was submitted, the total amount of amphotericin B deoxycholate reached 1170 mg, and the patient was discharged from the hospital with itraconazole oral solution. |
pmc-6081952-1 | Our patient is a 60-year-old female with severe emphysema who underwent a double-lung transplant in 2008, donated from a 64-year-old female with a 25-pack-year smoking history, stopping in 1982. The explanted lungs showed signs of severe emphysema but no malignant features. Post-transplant imaging demonstrated a mild to moderate degree of emphysema in the donor lungs. The patient received triple-drug immunosuppression with cyclosporine A, azathioprine, and prednisone and remained well until 2014, when she developed post-transplant lymphoproliferative disorder (PTLD), requiring chemotherapy with cyclophosphamide/doxorubicin/vincristine/prednisone-rituximab and discontinuation of azathioprine. While her PTLD is currently in remission, she subsequently developed chronic lung allograft dysfunction (CLAD) in 2015, with a marked decline in forced expiratory volume in 1 s (FEV1) from the post-transplant baseline of 3.1 L to 0.9 L. Her FEV1 has been stable at 0.9 L since 2015.
In 2016, the patient presented with a solitary right upper lobe pulmonary nodule on routine computed tomography (CT) scan. Following serial growth (12 mm) on CT, the lung nodule was biopsied, revealing primary lung adenocarcinoma. The pathologic sample was negative for both the ALK fusion oncogene and EGFR mutation. Staging whole-body positron-emission tomography-computerized tomography (PET-CT) scan and magnetic resonance (MR) scan of the brain did not reveal any evidence of distant metastases.
The patient was not a surgical candidate due to her poor pulmonary function (FEV1 = 0.9 L, FEV1/forced vital capacity [FVC] = 39%). She consented to undergo SABR. 4D-CT simulation was performed with vacuum cushion immobilization and the gross tumour volume (GTV) was contoured on the end-inspiratory and end-expiratory phases. No margin for microscopic disease extension was used. An internal target volume (ITV) was generated by merging the end-inspiratory and end-expiratory GTVs and a margin of 0.5 cm around the ITV was used to generate the planning target volume (PTV). Free-breathing treatment delivery was chosen due to minimal tumour respiratory motion. A risk-adapted schedule of 60 Gy in 8 fractions (biologically effective dose = 105 Gy10) was delivered every other day via a flattening filter-free volumetric modulated arc therapy technique using two 225-degree 6MV arcs on a linear accelerator. The dose was prescribed to the 80% isodose line, which encompassed 97.5% of the planning target volume. Planning was performed on Pinnacle [] version 9.10 with heterogeneity correction, using published dose constraints for normal tissues []. The mean lung dose was 3.3 Gy, and the volume of lung receiving ≥ 20 Gy or more (V20) was 4.3%. She completed radiotherapy in February 2017. Figure shows selected images from the patient’s radiotherapy plan.
Two months following completion of SABR, the patient was admitted to a community hospital with dyspnea, hypoxia, tachypnea and increased production of yellow sputum. She was afebrile, with no leukocytosis. A CT pulmonary angiogram did not demonstrate pulmonary embolism, though bilateral inflammatory opacities were suspicious for an infectious etiology. There was no evidence of an inflammatory process geometrically associated with the radiotherapy field suspicious for radiation pneumonitis. Sputum cultures did not identify a causative organism, though the patient was started on antibiotics empirically prior to obtaining a sputum sample and a viral cause was also possible. After discussion with the Respirology and Radiation Oncology services, she was started on cefuroxime and prednisone 50 mg daily to cover all potential etiologies. Her respiratory function returned to baseline and she was discharged after 12 days of admission with a tapering schedule of prednisone. A follow-up CT 1 month later showed resolution of the bilateral opacities.
Since discharge, our patient’s pulmonary function and subjective dyspnea have returned to pre-treatment levels and remained stable. Her most recent FEV1 was 0.9 L, with FEV1/FVC of 37% in March of 2018. Surveillance CTs at regular intervals demonstrated good local control, and post-treatment fibrotic pulmonary changes that are common following SABR (Fig. ). To date, the radiographic fibrotic changes do not harbor adverse features [] that would be suggestive of recurrence and/or warrant PET-CT scan or repeat biopsy. The patient is alive and well as of May 2018. |
pmc-6081983-1 | A 73-year-old woman presented with dyspnea on exertion. Chest radiography showed an enlarged mediastinal silhouette. Preoperative computed tomography (CT) and echocardiography revealed a giant cyst (12 × 10 cm in diameter) occupying a large area around the left atrium and ventricle, and it was present behind the pulmonary artery. Based on the preoperative CT findings, a pericardial or an epicardial cyst was suspected. Additionally, attachment of the cyst to the left atrium, left ventricle, pulmonary artery, and pulmonary vein was suspected (Fig. ). Compression of the left atrium and ventricle was considered to be the cause of her symptoms. Her hemodynamic condition was stable. Percutaneous cystocentesis was performed, and 800 ml of serous liquid was aspirated. However, 1 week after cystocentesis, the cyst recurred, and its size was the same as that before the procedure. Therefore, resection was planned.
If the cyst was an epicardial cyst, tight adhesion of the cyst to the left atrium, left ventricle, pulmonary artery, and pulmonary vein was considered possible. However, we believed that the cyst was more likely a pericardial cyst because a pericardial cyst is more common than an epicardial cyst. Therefore, resection involving video-assisted thoracoscopic surgery (VATS) was planned initially. However, the pericardium was intact and an epicardial cyst was diagnosed intraoperatively. As preoperative CT showed compression of the left atrium, ventricle, pulmonary artery, and pulmonary vein, adhesion was suspected. Additionally, the possibility of cardiopulmonary bypass (CPB) was considered. Therefore, open surgery through median sternotomy was performed. The cyst was found to be attached to the visceral pericardium involving the left atrium and pulmonary artery. Contrary to our expectation, the left ventricle was not involved. Additionally, coronary vessels were not involved. The cyst was most tightly attached to the main pulmonary artery. Thus, it was thought to have originated from the pulmonary artery (Fig. ). The content fluid was aspirated via direct puncture, and a heart positioner was used to provide sufficient traction to the right side, with several sutures placed on the cyst wall, so that the back of the heart and the pulmonary artery could be observed. The cyst wall was thick and hypervascular, and it was completely dissected with an ultrasonic scalpel. The surgery was successfully completed without CPB.
The postoperative course was uneventful. CT confirmed absence of cyst recurrence or pericardial effusion. There has been no recurrence of the cyst since discharge (2 years).
Histopathological examination revealed a single layer of mesothelial cells. In addition to blood and lymphatic vessels, calretinin-positive cells lining the cystic wall, alpha-smooth muscle actin-positive cells, smooth muscle negative for myoglobin, neuron-specific enolase positive cells, lymphocyte infiltration, and fibrosis were observed (Fig. ). |
pmc-6081987-1 | A 20-year-old African American woman with no other known prior medical history, presented to our institution January 2018 with fevers of 3 weeks’ duration. The fevers were predominantly in the late afternoon hours, associated with night sweats, frontal headache, tender cervical lymphadenopathy, anorexia and malaise.
Two weeks prior she saw her primary care physician who diagnosed her with viral illness and recommended supportive care. She also visited the emergency department and was diagnosed with lymphadenitis; a course of amoxicillin/clavulanic acid was prescribed of unrecalled dose and she wasn’t able to finish the whole course. Symptoms however persisted, and the patient also developed bilateral periorbital swelling and non-bloody diarrhea prompting her presentation at our institution. The patient indicated they had no cough, chest pain, dysuria, abdominal pain, arthralgia, rash, recent travel or sick contacts.
The patient was not in distress, with blood pressure of 120/70 mm Hg, febrile to 39.6 C and tachycardic with heart rate of 110 bpm. Physical exam was notable for bilateral periorbital swelling with violet discoloration of the eyelids, conjunctival pallor and painless bilateral cervical lymphadenopathy. No rash or joint swelling was noted.
Complete blood count revealed leukopenia with a white cell count of 2.9 × 10
3 /mcL (65% neutrophils, 13% lymphocytes, 13% bands), microcytic anemia with a hemoglobin of 8.5 gr/dL (mean corpuscular volume 65 fL) and 181 × 10
3 /mcL platelets. C-reactive protein (CPR) and erythrocyte sedimentation rate (ESR) level were markedly elevated at 51 and 84 respectively. Lactate dehydrogenase (LDH), ferritin and haptoglobin were also elevated. The patient tested negative for β-human chorionic gonadotropin (hCG), HIV, hepatitis B and C, angiotensin converting enzyme (ACE), antinuclear antibodies (ANA) and rheumatoid factor (RF).
Computed Tomography of the neck revealed bilateral cervical lymphadenopathy, enhancement and mild enlargement of the parotid and lacrimal glands and diffuse swelling of the pharyngeal mucosa and marked enhancement of bilateral cervical soft tissue planes [
and
].
She was observed off antibiotics. Blood cultures, serology for Epstein Barr virus (EBV) and cytomegalovirus (CMV), bone marrow biopsy and flow cytometry were all negative. Excisional biopsy of the left cervical lymph node revealed characteristic findings of Kikuchi-Fujimoto disease which showed geographic necrosis with fibrinoid deposits and apoptotic cells surrounded by a mononuclear infiltrate characteristically without neutrophils and eosinophils [
]. The patient was started on prednisone 40mg per day with rapid resolution of symptoms. Steroids were tapered after one week of treatment. Upon follow up in Rheumatology clinic 4 months later, patient was noted to be completely symptom free. |
pmc-6082045-1 | A 51-year-old man presented in mid-May (early winter) 2006, eight days after onset of swollen groin lymph nodes followed three days later by fevers, rigors, headache, dry cough and subsequent non-pruritic rash. He had been hiking alone along a popular hiking trail, the Bibbulmun track, in the southwest of WA and had received numerous tick bites. A tick removed from the skin of his back after biting near Walpole () was identified as Ixodes australiensis, a tick indigenous to WA and Tasmania.
On examination, he did not appear acutely unwell but was febrile. There were multiple tick bites on the trunk and limbs, a sparsely distributed papulovesicular rash on the trunk and extremities, an eschar overlying the left tibia () and pronounced inguinal lymphadenitis. Full blood count was normal but for moderate lymphopenia; there was moderate elevation of alanine transaminase (ALT) to 177 U/L, increasing to 430 U/L one week later, and C-reactive protein (CRP) was 40 mg/L. Chest X-ray was normal. He was prescribed doxycycline 100 mg bd for seven days. Within 24 h there was significant symptomatic improvement and two weeks later he was completely well with healing of the eschar and normalisation of CRP.
Citrate synthase gene PCR specific for rickettsiae [] was performed at the Australian Rickettsial Reference Laboratory (ARRL) on a biopsy taken from the edge of the eschar the day after the commencement of the doxycycline. This confirmed the presence of the Rickettsia genus, but there was insufficient product for DNA sequencing and species identification. Histopathological examination revealed florid perivascular granulomatous inflammation (). PCR on the whole blood was negative. Serological testing in this and subsequent cases was performed by immunofluorescence assay with commercial slide preparations (Scimedx Corp, Dover, NJ, USA) using R. conorii (Moroccan), R. typhi, and O. tsutsugamushi (Karp strain) to represent the spotted fever group, typhus group, and scrub typhus group respectively, using a polyvalent conjugate to detect total antibody (IgG and IgM). Serum tested in parallel showed evidence of acute infection with SFG rickettsia (). Rickettsial organisms were unable to be isolated from the tick retrieved from the patient’s skin and PCR for rickettsial DNA was also negative. |
pmc-6082045-2 | A 36-year-old female spent four days during November (early summer) 2014 on a field trip to Salisbury Island, situated in the Recherche Archipelago approximately 150 km east of Esperance (), trapping black-flanked rock wallabies and carrying out botanical inventory work. On return to the mainland she discovered a tick, which she promptly removed. Three days after her return she complained of fatigue and headache, followed three days later by fevers with rigors and general malaise. A maculo-papular rash () over the limbs, trunk and face started to emerge the following evening accompanied by myalgia and arthralgia. Four days into her febrile illness she was prescribed doxycycline 100 mg bd. Her fevers and arthritis abated within 48 h. Malaise, fatigue and myalgias took more than three weeks to resolve. During the acute phase she developed mild thrombocytopenia (104 × 109 platelets/L). The white cell count remained normal while the CRP rose to 256 mg/L and she had a mild hepatitis (ALT 87 U/L). Seroconversion to SFG rickettsia was demonstrated ().
DNA was extracted from her acute phase serum and the rickettsial 17 kD antigen gene was amplified and sequenced using primers MTO1 and MTO2 []. A 429 base pair (bp) sequence was generated (GenBank sequence ID: KU521358.1) which showed 100% match to the R. honei strain RB (411/411 bp; AF060704.1) with a 4 bp difference from the R. honei marmionii strain (421/429 bp; KT032120.1) and a single bp mismatch with “R. gravesii” (393/394 bp; DQ269436.1). Attempts to amplify and sequence the 16S rRNA gene from the limited volume of residual serum were unsuccessful. |
pmc-6082432-1 | A 46 year old male was an alcohol-intoxicated restrained passenger in a head-on motor vehicle collision, suffering blunt force abdominal trauma. At the collision site EMS recorded a Glasgow Coma Score of 15 with stable vital signs. He was transferred to Cabell Huntington Hospital where he was intubated and found to be tachycardic and profoundly academic to an arterial pH of 7.19 with accompanying base deficit of -11. Hemoglobin was 12.9, hematocrit 37.1, PT 11.5 sec, and APTT 23.5 sec. CT showed acute thoracic aortic rupture with a large volume of blood inferiorly along the aorta, right middle and lower lobe lung contusions, and a 4 cm laceration along the inferior lobe of the liver with blood in the renal hilum suggesting right renovascular injury and free fluid within the pelvis (a). He was transferred to Saint Mary’s Medical Center for emergent management of aortic rupture.
Emergent surgical management of the ruptured thoracic aorta was completed by the cardiothoracic team. Upon completion, initiation of exploratory laparotomy was begun, noting blood in the peritoneum and bile staining in the right upper quadrant associated with the transverse and hepatic flexures of the colon. The gallbladder was visibly distended, firm to palpation, and discolored but showed no signs of rupture. Subcapsular hematoma was appreciated along the right posterior lobe of the liver but showed no signs of actively expanding or gross laceration. Open cholescystectomy with intraoperative cholangiogram was performed on a thick, distended and grossly-discolored gallbladder consistent with hemorrhage into the gallbladder lumen (b). Conray contrast was used to demonstrate a completely intact biliary tree with extravasation noted from the common bile duct. Duodenal injury was ruled out by passing methylene blue through the orogastric tube with no extravasation appreciated and colon was assessed via direct visualization by Kocher maneuver and mobilization of the hepatic flexure. Due to the duration of the thoracic aorta repair and the exploratory laparotomy, the decision was made to place a 16-French T-tube in the common bile duct in addition to two large #24 Jackson-Pratt tubes in the vicinity of the bile duct (). The patient was maintained on ventilator in the neurotrauma intensive care unit. In the postoperative period the T-tube drained golden brown bile and was well-tolerated.
Urine collection via Foley catheter showed blood-tinged urine that persisted until POD 10 but then spontaneously resolved. During this time on POD 6 the patient underwent gastrostomy with placement of percutaneous gastrostomy and tracheostomy tubes. Due to the PEG becoming disloged on POD 8 the patient underwent exploratory laparotomy and gastrostomy with jejunostomy tube placement and removal of foreign body. Evaluation on postoperative day 10 showed successful drainage of the operative site via T-tube (c). The patient continued to improve and was discharged on POD 28 with T-tube and J tubes in place.
Recovery was complicated during the outpatient course as frank blood began appearing in the patient’s urine on POD 39 and the patient began complaining of pain around tube sites. CT urogram showed no residual renal injury and was presumed to be due to residual posttraumatic blood accumulation. Of note, a small amount of fluid was appreciated in the anterior right middle and upper abdomen along the inferior right lobe of the liver not seen previously but consistent with postoperative seroma. A urinary tract infection was diagnosed and managed medically. T tube cholangiogram revealed no abnormalities and the the T-tube was removed along with J-tube on POD 54. The remainder of the patient’s recovery period was uneventful with regards to hepatobiliary procedures, however frank blood in the urine persisted for an additional 6 months due complications of subclinical traumatic kidney injury sustained during the motor vehicle collision. |
pmc-6082433-1 | A previously well 15 year old male presented to the Emergency department at 1600 h, 4 hours after a seemingly minor blunt abdominal injury during a weekend soccer match. Initially following the impact of another player's knee to the abdomen, the adolescent rested out of the game for half an hour before returning to play the second half. Following the game, he returned home, tolerated an afternoon meal before the gradual onset of constant, dull, severe, non-radiating, generalised abdominal pain that was not exacerbated by movement. Due to family concerns, the patient presented to our Level I Tertiary referral trauma centre by own means. He was normotensive and not tachycardic. The patient had no significant medical history. Physical examination revealed a guarded abdomen. Focused Assessment with Sonography for Trauma (FAST) suggested a trace of free fluid in the sagittal pelvic image. The initial blood pathology was normal except for an elevation of amylase 410 units/l, lipase 771 units/l and white cell count 12.2 × 109/l. He was admitted for observation and serial clinical and FAST assessments.
The patient's symptoms failed to improve after 24 h observation, during which time he developed shoulder tip and central back pain. A repeat FAST 24 h post-admission again revealed possible trace free fluid in the sagittal pelvic images with new concerns regarding trace free fluid to the spleno-renal angle. A computed-tomography (CT) scan was performed with intravenous contrast. This revealed a transverse laceration through the neck of the pancreas with normal enhancement of the pancreas and no pancreatic duct dilatation () consistent with American Association for the Surgery of Trauma (AAST) Organ Injury Scale (OIS) grade IV injury. Additionally there was a small volume, low density intraperitoneal fluid, retroperitoneal stranding, omental contusion and a small linear laceration of the posterior left kidney without peri-nephric collection (AAST-OIS Grade II injury). There was no injury to the other solid organs or the duodenum.
The patient was kept nil by mouth with intravenous antibiotics, TPN and octreotide 100 mg three times a day via a percutaneous inserted central catheter (PICC). Ongoing central abdominal pain consistent with acute pancreatitis prompted further imaging on day 2 by magnetic resonance cholangiopancreatography (MRCP). This confirmed the CT findings and the patient was booked for ERCP the following day. At ERCP, the pancreatic duct was selectively cannulated and contrast extravasation was seen at the pancreatic neck (). A 5 cm 5Fr pigtail plastic stent was inserted across the defect and the patient remained on TPN and octreotide for 10 days. Repeat MRCP 10 days later showed a contiguous main pancreatic duct of normal calibre and appearance with no evidence of transection and improvement of the peri-pancreatic oedema. The patient was allowed to resume a low fat diet and TPN was weaned and ceased. The patient returned home on pantoprazole 40 mg daily, he was electively brought back to hospital 12 days later for ERCP and stent removal. At ERCP the pigtail stent was not seen, having likely passed out, and there was no evidence of extravasation of contrast, consistent with resolution of injury (). Follow-up as an outpatient was without incident over a three-month period. The patient has been advised not to partake in contact sports for six months. |
pmc-6082499-1 | A 16-year-old male unrestrained backseat passenger was brought to our trauma bay after a high-speed motor vehicle collision with a rollover. He suffered multiple injuries, including traumatic brain injury; complex pelvic fractures; spleen, liver, and renal injuries; and left diaphragmatic rupture. His initial chest X-ray (CXR) revealed his stomach herniating inside his left chest. He underwent immediate laparotomy and repair of his left diaphragm. Intraoperatively, the surgeon examined the right diaphragm by palpation and noted no abnormality. Postoperatively, the patient had a prolonged ventilator-dependence. His daily CXR showed right-sided diaphragmatic abnormality () but was nonspecific. We performed a dynamic ultrasound to evaluate the right diaphragm; the result suggested that the right diaphragm was paralysed, probably secondary to right phrenic nerve injury. On 3 occasions (post-injury day 0, 4, and 15), the patient underwent CT of the chest; none of those 3 scans reported right-sided diaphragmatic injury, despite the presence of “hepatothorax”, as well as “hump”, “band”, and “collar” signs (). Eventually, on post-injury day 17, the patient underwent a diagnostic laparoscopy; we found the liver herniating into the right chest. We reduced the herniated liver and performed an open repair of his right-sided diaphragmatic rupture |
pmc-6082581-1 | Patient is a 79 year-old man with history of hypertension and coronary artery disease initially presented for evaluation of a palpable right neck mass with associated symptoms of dyspnea on exertion and 36lb weight loss. Workup revealed stage 4 adenocarcinoma with negative anaplastic lymphoma kinase (ALK), c-ros oncogene 1 (ROS1), epidermal growth factor mutation (EGFR) and positive for programmed death-ligand 1 (PD-L1). Positron emission tomography/computed tomography (PET/CT) revealed disease mostly restricted to the thoracic area, but there was also dense hypermetabolic activity with mural thickening in the sigmoid colon of unclear significance. Patient refused nivolumab systemic therapy and was treated with palliative radiation therapy to his right neck mass, right hilum and mediastinum with good palliative response.
Two weeks after radiation therapy, patient presented to the hospital with dysuria, fecaluria and abdominal pain which did not improve after taking a course of antibiotics. CT of the abdomen and pelvis revealed a colovesical fistula between the sigmoid colon and bladder, large mesenteric lymph node just above the sigmoid colon mass and wall thickening in the sigmoid colon (Figure ).
Cystoscopy revealed edematous mucosa and exudation of feculent material from the fistula site. Sigmoidoscopy revealed a fistula in the distal to mid sigmoid colon about 25 cm from dentate line and a 3 cm area of extrinsic compression.
Patient was taken to surgery for resection of colovesical fistula mass, sigmoidectomy with colostomy, drainage of the intraabdominal abscess and repair of a complicated bladder fistula. Pathology of the tumor revealed metastatic poorly differentiated lung adenocarcinoma with an associated colovesical fistula and extensive lymphovascular invasion. Comparison of resected colovesical fistula mass to primary lung cancer biopsy showed similar morphology by Haemotoxylin and Eosin staining (H&E) and lung cancer specific thyroid transcription factor-1 (TTF1) marker consistent with the colovesical fistula mass being metastatic lung cancer (Figures -D). |
pmc-6082582-1 | We describe two siblings from a Pakistani consanguineous family, 18-year-old female (ABC) and 15-year-old male (XYZ) diagnosed as having IDDM since the age of eight years, with progressive flexion contracture of the small joints of both hands and feet for seven to eight years. One female child of the family expired at the age of eight years with a similar complaint of IDDM and joint contracture.
On examination, ABC had short stature with standard deviations (SDS) of -2.48 and a hyperpigmented skin lesion at the posterior medial aspect of both thighs without hypertrichosis. She had a Tanner stage of B3P3A2 M0. She also had a contracture of the proximal metacarpophalangeal joint of both hands and a contracture with plantar flexion of the metatarsophalangeal joints of the bilateral feet with restriction of both active and passive movement (Figure ).
On examination, XYZ also had short stature with SDS of -3.3. He had bilateral cervical lymphadenopathy and a Tanner stage of prepuberty. He had a similar contracture of the proximal metacarpophalangeal joints of both hands and a contracture with plantar flexion of the metatarsophalangeal joints of bilateral feet with restriction of both active and passive movement (Figure ).
In both siblings, there were no signs of inflammation and no large joint involvement. The laboratory workup of both siblings, including antinuclear antibodies (ANA), rheumatoid factor (RA factor), erythrocyte sedimentation rate (ESR), thyroid profile, and Celiac serology, were negative. A genetic study for monogenic diabetes revealed a homozygous nonsense mutation in the SCL29A3 gene in both. A genetic study of both parents revealed a heterozygous mutation in the SCL29A3 gene. This result confirmed a genetic diagnosis of histiocytosis lymph adenopathy plus syndrome (also known as H syndrome). The patient left against medical advice before further workup and treatment. |
pmc-6082584-1 | A 60-year-old man was referred to our center in October 2015 due to ascites and increased liver enzymes ongoing since six months (Table ).
He had no previous history of liver disease and he complained of fatigue and weight loss (16 Kg) over eight months. The patient referred alcohol consumption of about 50 g/day in the last 30 years, which he stopped on the month before admission when ascites was noted. He had no history of chronic diseases except a known allergy to eggs and soy proteins.
On the examination at our center, the patient featured sarcopenia and ascites. Liver ultrasound showed a large hepato-splenomegaly with an irregular surface, a hyperechoic liver parenchyma, and signs of intrahepatic portal hypertension (patent paraumbilical vein; reversed portal venous flow) as well as ascites (Figure ). No focal liver lesions were observed.
Liver stiffness measurement (LSM) was performed using two different ultrasound elastography techniques: transient elastography (TE); M probe (FibroScan, Echosens, Paris, France), and 2-dimensional shear wave elastography (2D-SWE); SC6–1 probe (Aixplorer ultrasound system, Supersonic Imagine, Aix-en-Provence, France). Both showed very high values, clearly above normality (Table ) [].
Spleen stiffness was measured by TE and demonstrated values compatible with portal hypertension (Table ) []. A computed tomography (CT) scan confirmed the morphological imaging findings shown on ultrasound; CT-based volumetry of the liver and spleen reached high values of 3298 ml and 621 ml, respectively (Figure ).
Esophagogastroduodenoscopy demonstrated small esophageal varices. A diagnostic paracentesis showed a high serum-ascites albumin gradient (2.1 g/dL), confirming a portal hypertension-related cause. Urinalysis showed a ++ proteinuria. All common causes of chronic liver disease (viral, autoimmune, metabolic) were excluded and a decision to perform liver biopsy was taken. Given the presence of portal hypertension, a transjugular liver biopsy was preferred in order to simultaneously measure the hepatic venous pressure gradient (HVPG) and to minimize the risk of intraperitoneal bleeding.
The procedure was performed by accessing the right jugular vein through Seldinger’s technique. The HVPG was 24 mmHg (wedged hepatic venous pressure 33 mmHg; free hepatic venous pressure nine mmHg), which confirmed a sinusoidal cause of portal hypertension. The liver biopsy was uneventful.
Liver histology showed massive amyloid deposition predominantly in the Dissé space with obliteration of sinusoids and trabecular atrophy, with only minimal portal and perisinusoidal fibrosis (Figures -).
A bone marrow biopsy demonstrated mature cells plasmacellular myeloma with kappa immunophenotype and 20%-30% diffuse interstitial infiltration. Congo Red stain showed no amyloid infiltration.
Multiple myeloma with systemic primary amyloidosis (AL) with liver involvement was diagnosed and the patient was referred to the oncology department. Cardiac magnetic resonance imaging (MRI) and electrocardiogram (EKG) did not show features of cardiac involvement. Amyloidosis-related neuropathy affected the autonomous nervous system (relevant orthostatic hypotension) and the peripheral sensory nervous system (hypo- and dysesthesia in the distal upper and lower limbs). Due to the severe malnutrition and sarcopenia associated with amyloidosis and portal hypertension, the patient was put on parenteral nutrition.
From November 26, 2015 until February 23, 2016, the patient was treated with four cycles of a first line regimen according to the CyBorD scheme, comprising cyclophosphamide 350 mg/m2 intravenous (iv) on days one, eight, and fifteen; 1.0 mg/m2 bortezomib subcutaneous (sc) on days one, four, eight, and eleven; and dexamethasone 20 mg per os (po) on days one, four, eight, and eleven; within a 21 days schedule.
The treatment was haematologically well tolerated, and orthostatic hypotension resolved completely in the fourth cycle, whereas the patient developed fatigue (CTCAE grade two), loss of appetite, and aggravation of peripheral sensory neuropathy (PNP CTCAE grade two, pain); the latter was assumed as bortezomib-triggered. During the course of chemotherapy, he also developed severe complications of portal hypertension including refractory ascites requiring multiple large-volume paracentesis, bacterial infections, and hepato-renal syndrome.
After the four therapy cycles, the remission status indicated a very good partial response (VGPR), with a difference between involved and uninvolved serum free light chains (DFLC) of 21 mg/L, compared to 258.6 mg/L at diagnosis. Abdominal MRI in February 2016 showed stable hepato- (3574 ml) and splenomegaly (613 ml) with minimal heterogeneity of the liver parenchyma in the T1w phase suggesting amyloid content.
Few weeks after completion of first line chemotherapy, fatigue and loss of appetite resolved completely; parenteral nutrition could be stopped. A few months later, the drug-induced PNP also improved to CTCAE grade one. The patient refused further consolidation of first-line treatment with high dose chemotherapy (HDCT) and autologous stem cell transplantation (ASCT) because of the side effects related to the induction therapy with CyBorD he previously experienced. No further episodes of clinical decompensation of liver disease were observed.
One year later (eighteen months after the diagnosis), the patient was in very good clinical condition, asymptomatic, without ascites and his liver tests improved (Table ). On ultrasound, the liver was significantly smaller, with slightly heterogenous aspect of the parenchyma as seen in Figure . No signs of portal hypertension were observed; notably, the previously noted paraumbilical vein had disappeared, and the direction of blood flow in the portal vein was now normal (Figure ). No ascites was present. Both liver and spleen stiffness decreased markedly (Table ). The MRI based volumetry of liver showed a marked improvement of hepatomegaly (2631 ml, decrease of 26% vs. baseline) as seen in Figure and a slight improvement of splenomegaly (554 ml, decrease of 9.6% vs. baseline) correlating with the ultrasound and clinical findings. Another year later, his clinical conditions are fully stable. |
pmc-6082585-1 | A 69-year-old African American female presented with complaints of epigastric pain and 10-pound unintentional weight loss over three months. Abdominal pain was dull, non-radiating, localized in epigastric region and was not associated with food intake. She also had associated occasional non-bloody, non-bilious vomiting and early satiety. Three weeks ago, upper gastrointestinal (GI) endoscopy showed mild antral gastritis for which she was started on proton pump inhibitor (PPI). Her past medical history was significant for chronic kidney disease stage IV (baseline creatinine 1.6–2 mg/dl), anemia of chronic disease, chronic obstructive pulmonary disease (COPD), two ischemic strokes, right internal capsule infarct three years ago, left lacunar infarct six months prior with no significant residual neurological deficits, and esophageal carcinoma treated 14 years prior with chemotherapy and radiotherapy. She also used to consume hard liquor on daily basis for most of her life and smoked three packs per day for 40 years. There was no family history of any bleeding disorders. Her medications prior to this admission included aspirin, clopidogrel, omeprazole, amlodipine and atorvastatin.
On presentation, the patient was hemodynamically stable. Abdomen was soft with some epigastric tenderness, but without any palpable masses. Neurological examination was non-focal. Hematological investigations from admission was suggestive for hemoglobin 8.2 g/dl, total leukocytes 6300 cells/mm3, peripheral blood eosinophilia (12%), normal anion gap metabolic acidosis with serum bicarbonate 13 mEq/L and potassium 4.1 mg/dl, serum lipase 94 IU/L, albumin 5.0 g/dl, total protein 8.9 g/dl. Iron studies obtained for evaluation of anemia showed normal iron level and iron saturation with ferritin elevated to 738 ng/ml. Urinalysis showed 30 mg/dl protein, pH 5.5, small blood, too-numerous-to-count white cells, 0 to 5 red cells per high-power field. Creatinine at admission was elevated at 6.94 mg/dl with blood urea nitrogen (BUN) 83 mg/dl. Her bloodwork done three months prior to admission showed hemoglobin of 10.1 g/dl. At that time, renal ultrasound had demonstrated both kidneys to be atrophic with bilateral renal cysts. The kidneys were echogenic and atrophic. There was a 0.8 x 0.9 x 0.9 cm cystic lesion in mid-pole of the right kidney (Figure ). There was a 1.2 x 0.9 x 1.0 cm cyst noted in the mid-pole of the left kidney (Figure ).
The patient was admitted for acute kidney injury and was started on intravenous fluids with bicarbonate. Intravenous ceftriaxone was given for possible urinary tract infection. Urine was also found positive for presence of eosinophils raising concern for acute interstitial nephritis (AIN) from PPI, for which omeprazole was changed to famotidine. Computed tomography (CT) scan of chest, abdomen and pelvis was pursued to rule out any malignancy but was limited study in absence of intravenous contrast. Underlying renal dysfunction limited the use of contrast. It confirmed the presence of previously known emphysematous changes in lungs and diverticulosis coli. No omental or abdominal masses were demonstrated. With conservative management, creatinine improved to about 5.1 mg/dl and plateaued.
On fourth day of admission, the patient developed new left lower quadrant pain and swelling. She was receiving subcutaneous injections of heparin 5000 IU every 8 hours at that site for venous thromboembolism (VTE) prophylaxis. She has received a total of 10 doses of heparin prior to this event.
Subcutaneous hematoma was suspected and aspirin, clopidogrel, and heparin products were stopped. Ultrasound of abdomen identified heterogenous hypoechoic mass measuring 10.2 x 6.4 x 4.8 cm (craniocaudad x width x depth) in subcutaneous tissues of the left lower quadrant, extending inferiorly from the periumbilical area concerning for large rectus sheath hematoma (Figure ).
Her hemoglobin dropped down to 6.6 g/dl requiring 3 unit packed red cell transfusion. Her platelet count never dropped down and coagulation studies were normal.
Oral prednisone was also started at 40 mg daily for possible AIN from PPI use, and was continued for four weeks. Patient’s renal function did not improve with conservative management for which the decision was made to start her on renal replacement therapy. Right internal jugular vein Perm-A-cath was placed to initiate hemodialysis. Clopidogrel was resumed after one week and aspirin was resumed after two weeks of developing rectus sheath hematoma.
Patient’s hemoglobin at discharge was 10.3 g/dl and remained stable on follow-up two months later. Her renal function did not improve despite corticosteroid therapy, and she was eventually transitioned to peritoneal dialysis six months later. |
pmc-6082586-1 | A 55-year-old previously healthy man presented with a two-hour history of abrupt, severe pain and paresthesia of the right lower extremity (RLE) after playing basketball. This is the first time he had experienced this, but he had experienced intermittent episodes of dizziness and palpitations over the past two months. He denied any history of trauma, smoking, alcohol abuse, and illicit drug use.
Vitals were as follows: blood pressure (BP) from the right arm - 151/83 mmHg; heart rate - 102 beats/min; respiratory rate - 16 breaths/min; temperature - 36.3° C, and oxygen saturation - 100% on room air. On examination, his RLE was cold, pale, and insensate. There were no palpable femoral, popliteal, or pedal pulses in the RLE. Distal pulses were also negative by Doppler ultrasound. He had conserved 5/5 motor strength in both feet. Examination of the left lower extremity (LLE) was normal. Cardiopulmonary exam was unremarkable, with no murmurs or audible extra heart sounds. All other physical exam and laboratory findings were unremarkable. Computed tomography angiography (CTA) of the RLE revealed an occlusion of the right common femoral artery (CFA). There was also a second occlusion at the proximal popliteal artery at the level of the knee joint with no opacification of the leg vasculature distal to the popliteal artery (Figures -). Electrocardiogram did not reveal any atrial fibrillation.
The patient was diagnosed with ALI secondary to an arterial embolus of unknown origin initially, started on heparin, and was taken emergently to the operating room for right groin exploration and a femoral thromboembolectomy by groin cut-down with a four-compartment fasciotomy. An intraoperative angiogram of the RLE showed good runoff and no additional occlusion distally. A formal arteriogram of the aorta was not performed. The artery was closed, and a four-compartment fasciotomy was performed on the right lower leg. The leg showed evidence of reperfusion ischemia. In the recovery room, the patient had palpable pedal pulses in both legs and heparin anticoagulation was continued.
On postoperative day one (POD 1), the patient’s troponin and creatinine kinase levels were elevated. A CTA of the chest revealed an irregular mass in the left atrium (Figures -), which was also observed on a subsequent echocardiogram which also showed that the mass was attached to the interatrial septum and was bulging through the mitral valve (Figure ). The patient’s fasciotomies were closed on POD 6, and he was discharged from the hospital on warfarin without further complication. Histological evaluation of the embolus revealed spindle-shaped cells set within a collagenous myxoid stroma, which definitively confirmed the diagnosis. The patient was sent to a tertiary center for further managment by cardiothoracic surgery. |
pmc-6082587-1 | A 31-year-old Guyanese female presented to the emergency department (ED) complaining of odynophagia and neck pain for few days. She also reported an increased appetite over the past few weeks. She was otherwise healthy in the weeks leading up to her visit to the ED. She denied any history of palpitation, heat intolerance or muscle weakness. On physical examination, her blood pressure was 136/72 and pulse was 112/min with a sinus rhythm. Her skin was warm and dry. There was no exophthalmos or lid lag. The thyroid gland was diffusely enlarged and tender on palpation. A bruit was heard on bilateral lobes of the thyroid gland. She had fine tremors on outstretching of hands. Thyroid-stimulating hormone (TSH) was suppressed 0.03 mI U/ml (0.34- 5.6), while free thyroxine (free T4) 5.37 ng/dl (0.58-1.64), free triiodothyronine (free T3) 767.5 ng/dl (6.09-12.2), and total thyroxine (T4) 27.88 mcg/dl (6.09-12.2) were elevated. The suppressed TSH and elevated thyroid hormones were consistent with hyperthyroidism.
Further workup also demonstrated elevated levels of thyroid stimulating antibody (TSI) 221 (<125). Thyroid peroxidase antibody (TPO Ab) >1000 IU/mL (<35) and thyroglobulin antibody (TgAb) 279.0 IU/ml (<20) were also positive. Erythrocyte sedimentation rate (ESR)and C-reactive protein (CRP) were 10mm/hr (10-20) and <0.01 mg/dl (0-0.8), respectively. The radioiodine thyroid uptake scan (RAIU) was not done, as the patient was given iodinated contrast in ED for computed tomography (CT) of the neck. The presence of an enlarged thyroid gland and elevated thyroid hormones with positive TSI suggested that the patient had Graves' disease. As ESR and CRP were within normal limit, the diagnosis of subacute thyroiditis was effectively ruled out.
The patient was discharged from the hospital on methimazole 30 mg daily and propranolol 20 mcg three times a day. During the third month follow up visit, she had no pain in the thyroid and was clinically euthyroid on antithyroid medication. This was a rare case of Graves' disease presenting as a painful and tender thyroid gland without the evidence of subacute thyroiditis. The cause of pain, in this case, remains uncertain. |
pmc-6082661-1 | Following an elective open pyeloplasty for pelvic ureteric junction obstruction, an 11-month-old female presented with low grade fever and irritability on the first postoperative (PO) day. The birth and developmental history were unremarkable, except for two episodes of pyelonephritis and a right pyeloureteral stenosis, which led to a programmed open pyeloplasty. She had been on fosfomycin antibiotic prophylaxis for the last months, and a single dose of amoxiclavulanate was given intravenously during surgery. Examination on the first 12 hours following surgery revealed a febrile irritable infant, with pulse of 120 and normal blood pressure. Neutrophil count (19.8 × 10
9
/L) and C-reactive protein were elevated (9.1 U/L), and her hemoglobin was decreased following surgery (from 11.5 to 8.3 mg/dL). Her surgical wound was by then only slightly erythematous. She was started on cefotaxime therapy (150 mg/kg/day e.v.), and a erythrocyte transfusion was ordered. Nevertheless, the local examination at the next day revealed a dark bluish erythema of the entire surgical wound measuring 9 × 6 cm, plus a satellite lesion of the same characteristics 5 cm away (
). These lesions were tender and warm in palpation. As a ring retractor was used during surgery, to enhance surgical field view, and its blades lay directly on peri-incisional skin (
), a surgical fomite or contact vehicle mechanism was suspected to explain the satellite lesion. Apart from these, all her right flank appeared also erythematous and warm. C-reactive protein and leukocyte count were raised to 23.5 × 10
9
/ L and 23.4 U/L, respectively. Although still febrile, and tachycardic, she needed neither further homodynamic nor ventilatory support. A diagnosis of necrotizing soft-tissue infection (NSTI) with sepsis was determined, and meropenem (60 mg/kg/day) plus vancomycin (40 mg/kg/day) were started. Urine and blood cultures yielded no bacteria. Although fever disappeared the next day (PO 2nd day), the wound worsened, showing blisters on a well demarcated necrotic apparent patch of 9 × 5 cm. The satellite lesion (of 2 × 2 cm) developed in the same manner. On PO day 3, a surgical incision and debridement of both lesions were performed. Necrosis reached the external oblique muscle fascia, and a small fasciotomy was required. Tissue culture grew
Pseudomonas aeruginosa
sensitive to meropenem, and no anaerobic bacteria grew on the corresponding culture. Apart from the above-mentioned antibiotic regimen, daily wound care with silver-containing foam was continued following debridement. At PO 6th day, both lesions still appeared considerably exudative and poorly perfused (
), so vacuum-assisted therapy (VAT) treatment was started (
). From then on, exudate control, perfusion, and granulation improved in the consecutive days (
and
), which permitted direct closure (no grafting needed) at PO day 12. Antibiotics were discontinued and the patient discharged at PO day 15. |
pmc-6082747-1 | A 75-year-old man was admitted to our hospital because of left chest pain and dyspnea. A chest X-ray showed a left pneumothorax (Fig. ), and a previous chest computed tomography (CT) scan revealed a subpleural pulmonary nodule with cavitation in the left upper lobe (Fig. ). The patient had been on prednisolone and methotrexate for rheumatoid arthritis. He also had a past history of acute coronary syndrome 5 years previously; this was treated with coronary stent implantation followed by anticoagulant therapy. He additionally had a past history of a right pneumothorax 3 years previously that was presumptively caused by rupture of a subpleural rheumatoid nodule and successfully treated with conservative thoracic drainage. Regarding his present admission, the left pneumothorax did not resolve after 1 week of chest tube drainage. There were no bacterial pathogens present in the pleural effusion. We decided to perform pleurodesis because his left lung expanded well after chest drainage and he had some risks for pneumothorax surgery, as previously mentioned. The air leakage finally subsided after six times pleurodesis procedures (with a blood patch once, a talc slurry three times, and OK-432 (streptococcal preparation) two times) prior to discharge (Fig. b). However, he presented to our hospital 1 week after being discharged because the pneumothorax had asymptomatically relapsed (Fig. c). Thus, we decided to perform thoracoscopic surgery to resect the subpleural lesion that caused intractable pneumothorax 2 months after the first admission. The surgery was performed through the fourth intercostal space along the midaxillary line, sixth intercostal space along the anteroaxillary line, and seventh intercostal space along the posteroaxillary line. There were rigid adhesions on the apex, mediastinum, and diaphragm due to repetitive pleurodesis. A sealing test revealed a pleural fistula with air leakage of the left upper lobe where the previous chest CT scan had demonstrated a small subpleural nodule with cavitation (Fig. a). The visceral pleura was so thick and hard that we had to resect the lesion with surgical scissors instead of with automatic staplers. The resected lung parenchyma was continuously sutured with 4-0 Prolene® (Ethicon endo-surgery) and covered with a polyglycolic acid sheet (Fig. b). There was no air leakage at the end of surgery. The lung gradually expanded (Fig. ) for a few days. The chest tube was successfully removed on the third postoperative day, and the patient was discharged on the seventh postoperative day. The final pathological diagnosis was a pulmonary rheumatoid nodule on the pleura (Fig. ). The patient has had no evidence of recurrence of pneumothorax during 1 year of follow-up.
Pulmonary rheumatoid nodules occur in less than 1% of patients with RA []. Rheumatoid nodules can rupture into the pleural cavity and cause pneumothorax, empyema, and bronchopleural fistula (BPF). Some reports also indicate that rapid growth of rheumatoid nodules may be associated with azathioprine treatment [] or biological therapies [] of RA. Kim et al. also reported a case of recurrent pneumothorax after etanercept therapy induced by the progression of interstitial lung disease []. Pneumothorax caused by noncardiac intrathoracic complications of RA can be resistant to standard care because RA patients are immunosuppressed, which predisposes them to infection and poor healing. Some authors have reported difficulties in performing surgical intervention for pneumothorax induced by RA. Rueth et al. reported that some patients with RA-induced pneumothorax and BPF required long-term care and had pneumothoraces that were resistant to repeat talc pleurodesis []. The success rate for pleurodesis via thoracoscopic surgery for the management of pneumothorax in patients without RA is 97%, and the long-term recurrence rates range from 5 to 9% []. Regarding pneumothorax in patients with RA, we hypothesize that the control rate for pneumothorax is lower than in those without RA because RA patients have poor wound healing and chronic local inflammation in the causative lesions. Due to the intrathoracic rigid adhesions that form surrounding the causative fistula after repetitive pleurodesis, pleurodesis can be useful for treating patients with multiple rheumatoid nodules because this procedure can prevent recurrent pneumothorax resulting from subsequent rupture of other nodules. On the other hand, surgery to treat any residual air leakage is very difficult because of adhesions that have formed at other locations not contributing to the air leakage and due to the thick visceral pleura. A wide pleural covering technique with an oxidized regenerated cellulose mesh [] or a polyglycolic acid sheet can be used to treat intractable pneumothorax rather than fistula resection and suturing in patients in whom the cause of pneumothorax are still unknown at the time of surgery. The use of a latissimus dorsi muscle flap placed into the pleural cavity can also be effective because this flap is made with a large muscle with a reliable blood supply and is relatively easy to harvest. Bronchial occlusion with a spigot is also one of the most effective techniques for patients with poor conditions for surgery []. In our patient, the causative lesion was suspected to be solitary, and it was difficult to treat the air leakage with bronchial occlusion because the lesion was located in the peripheral lung parenchyma. Additionally, we selected surgical resection to pathologically demonstrate that the definitive etiology of the pneumothorax was rupture of a rheumatoid nodule. In RA patients, it is important to obtain a pathological diagnosis of the underlying cause of pneumothorax because these patients have higher risks of lung malignancies than the general population []. |
pmc-6082884-1 | An 85-year-old Caucasian female presented to our institution complaining of a one-week history of progressive sharp right flank and scapular pain with mild shortness of breath, dry cough, and pleuritic chest pain exacerbated with deep inspiration. The patient denied constitutional symptoms, nausea, vomiting, diarrhea, or constipation. The past medical history was significant for essential hypertension, hypercholesterolemia, rheumatoid arthritis, and asthma; and surgical history significant for uterine benign tumor removal and unilateral oophorectomy 40 years prior. The patient was allergic to iodinated contrast media. The social history included 15 years of occupational asbestos exposure, southeastern European descent, and nonsmoker. Vital signs were significant for uncontrolled arterial hypertension and oxygen saturation of 93% on room air. Physical examination showed no acute respiratory distress, mild bibasilar crackles greater on the right side, diffuse abdominal tenderness and right costovertebal angle tenderness. Initial laboratory studies revealed normocytic normochromic anemia and arterial blood gas analysis consistent with acute respiratory alkalosis. The comprehensive metabolic panel, lipase, troponins and EKG were unremarkable. The chest X-ray showed a dense peripheral right lung pleural-based opacity and blunting of the right costophrenic angle and multiple nodular opacities in the left midlung. Findings were new compared to previous imaging test done two months prior to presentation (Figure ). The patient had multiple previous visits to the emergency department with similar complaints and negative workups.
CT chest without contrast showed a large loculated right pleural effusion with adjacent pleural thickening. In addition, there were multiple masses throughout both lung fields, greater on the right (Figure ). CT abdomen and pelvis were negative for other neoplastic findings. A flexible bronchoscopy was performed and showed only tracheal and right lung bronchial edema. Bronchial washing and bronchial biopsies were negative for neoplastic or infectious processes. A CT-guided core biopsy of the right pleural-based mass was performed. At the same time, 300 mL of red fluid was obtained from an ultrasound-guided thoracentesis from the right pleural effusion. Analysis showed an exudative fluid with 60,000 RBC/UL with lymphocytic predominance. The cytologic analysis of the pleural fluid was negative for malignancy.
The initial pathologic report from the pleural mass was positive for malignancy, exhibiting a spindle to plasmacytoid small blue cell tumor. The cellular smear showed numerous non-cohesive to loosely cohesive plasmacytoid cells. Immunohistochemical stains done in our institution were diffusely positive for Vimentin and focally positive for CD138. Given the CD138 focal positivity, the possibility of a hematolymphoid malignancy or plasma cell disorder was considered; however, workup was essentially negative. The samples were sent to Mayo Clinic Medical Laboratory, Rochester, MN for confirmation. The external pathology report revealed neoplastic cells that were diffusely positive for Vimentin, focally positive for OSCAR keratin, WT-1, CD138 and CAM5.2, and rarely positive for ALK. All other markers tested were essentially negative (Table ).
In-situ hybridization studies for kappa and lambda showed polytypic plasma cells. The submitted pathology slides revealed a hypercellular lesion with areas of epithelioid cytology; in other areas, they were rather spindled resembling sarcoma-like cells. A high nuclear to cytoplasmic ratio was noted with focal necrosis. A concern was raised for a biphasic tumor such as a metastatic carcinoma, malignant melanoma or mesothelioma but given the focal OSCAR keratin expression together with the morphology and the focal WT-1 expression in the absence of carcinoma markers, a malignant mesothelioma, biphasic type was diagnosed (Figure ).
Further workup with PET-CT scan was advised to evaluate the extent of the illness and cytotoxic chemotherapy combined with immunotherapy or tyrosine kinase inhibitors was recommended by oncology. The patient refused further imaging and treatment, and palliative care was consulted. |
pmc-6083208-1 | A 37-year-old Caucasian male patient was referred to our Department from the National Organ Procurement and Transplant Network waiting list for KT.
The recipient was an unmarried office worker with hypertension under pharmacological treatment, no previous abdominal surgery and ESRD due to ADPKD currently not requiring hemodialysis (preemptive). Pretransplant estimated glomerular filtration rate (eGFR) was 13.7 mL/minute per 1.73 m2. Body mass index was 21.9 kg/m2. At physical examination, an asymptomatic bilateral flank mass was palpable.
Abdominal MRI scan confirmed the presence of multiple cysts of different diameter within significantly enlarged native kidneys, whose sagittal diameter was >25 cm bilaterally (). No space constraints were anticipated before RAKT at the putative transplantation site (right iliac fossa) (). As such, no indication was placed for NKN.
The brain-dead donor was a 50-year-old Asiatic male, without significant comorbidities, deceased in a motor vehicle accident. At CT scan, a single renal artery and vein were noticed. Surgical technique of abdominal organ procurement followed established surgical principles. For KT, the right kidney was assigned to our Institution.
After multidisciplinary board discussion and obtaining the informed consent, the patient was scheduled for RAKT.
A step-by-step overview of surgical technique of RAKT used in our case is described in , as well as in accompanying the article.
RAKT was performed using the da Vinci Xi Robot® (Intuitive Surgical, Sunnyvale, CA) in a four-arm configuration using a 0° lens with the patient in a 30° Trendelenburg position. Surgical technique followed the principles of the Vattikuti–Medanta technique with specific technical modifications to tailor the surgical strategy to the specific patient's anatomy ().
At the beginning of the procedure, the abdominal cavity was inspected to ensure the adequacy of the working space for subsequent transplantation at the right iliac fossa ().
After exposure of iliac vessels and creation of an extraperitoneal pouch, the bladder was detached and prepared for subsequent ureteroneocystostomy. At this point, the graft within the gauze jacket was introduced into the abdominal cavity through the GelPOINT device. Regional hypothermia was achieved by cooling the graft with 200–250 cc of ice slush introduced through the GelPOINT.
After a venotomy was made using a curved cold scissors, the graft renal vein was anastomosed in an end-to-side continuous fashion to the external iliac vein using a 6/0 Gore-Tex suture on a CV-6 TTc-9 needle (). After completion of the venous anastomosis, additional ice slush was introduced to ensure graft cooling.
Subsequently, after clamping of the external iliac artery, a linear arteriotomy was made using robotic curved cold scissors. Then, the arteriotomy was modeled as a circular arteriotomy and the lumen was flushed with heparinized saline. Then, the renal artery was anastomosed in an end-to-side continuous fashion to the external iliac artery using a 6/0 Gore-Tex suture on a CV-6 TTc-9 needle (). After testing the integrity of the anastomosis, the kidney was revascularized. The graft was inspected for color and turgor and pneumoperitoneum was reduced to 8 mm Hg to check for any bleeding.
Firefly® fluorescence imaging technology was used to check kidney and ureteral vascularization in conjunction with intraoperative duplex ultrasound using the da Vinci TilePro™ feature ().
The kidney was then allocated into the extraperitoneal pouch. Finally, the ureterovesical anastomosis was performed using a modified Lich-Gregoir technique using two semicontinuous sutures with 4-0 Monocryl sutures (Ethicon, Inc, Cincinnati, OH) over the preplaced 6F, 14 cm Double-J stent. The detrusor muscle was closed with a continuous suture creating an antirefluxing mechanism ().
Intraoperative and perioperative outcomes are shown in .
Duplex ultrasound of the graft performed on postoperative day 1 showed normal graft perfusion.
At 6-month follow-up, the patient was free of symptoms, with regular voiding function, absence of lymphocele at abdominal ultrasound and optimal renal function (eGFR 76.8 mL/minute per 1.73 m2). |
pmc-6083471-1 | A 10-month-old girl was referred to our endocrine unit for evaluation of excessive and rapid weight gain. She was born at full term by normal vaginal delivery and weighed 3.0 kg [-0.52 standard deviation score (SDS)] at birth. She is the second child of healthy, non-obese parents with third degree consanguinity. There was no history to suggest gestational diabetes, hypertension, hypothyroidism or excess weight gain by mother during pregnancy. Parents noticed increased appetite at about two months of age. She started demanding feeds at half to one hourly intervals. Subsequently, there was a rapid gain in her weight to 9.5 kg (+3.14 SDS) at four months and 15 kg (+8.17 SDS) at six months of age. There was no history of lethargy, dryness of skin, constipation, excessive hair growth, seizures, visual or sleep disturbances. There was a history of EOO in a paternal uncle and a male cousin.
Physical examination revealed generalized body fat distribution, a rounded face and deep skinfolds (). There were no stigmata of a syndrome or underlying endocrinopathy, except acanthosis nigricans in axillae and neck folds (). The vital parameters were normal. Her weight was 19 kg (+7.38 SDS), length 71.0 cm (-0.24 SDS) and body mass index 37.7 kg/m2 (+10.94 SDS). Anthropometric calculations were done using the World Health Organizetion (WHO) Anthroplus software (version 1.0.4 WHO, Geneva, Switzerland). Ophthalmological evaluation did not show retinitis pigmentosa. Systemic examination was unremarkable.
Laboratory investigations revealed normal routine hematological and biochemical parameters, except for serum liver aminotransferases. The results of other laboratory evaluations are shown in . Abdominal ultrasound showed normal morphology of kidneys, a liver span of 12 cm (normal 6.3-9.6 cm) and features of hepatic steatosis. Magnetic resonance imaging of the brain, with fine cuts through the pituitary and hypothalamus, showed no abnormality. In view of intense hyperphagia followed by rapid weight gain, early age of onset, family history of EOO and low level of circulating leptin, a diagnosis of monogenic obesity due to LEP gene mutation was considered. Written informed consent was obtained from the parents for conducting all laboratory studies and for publishing clinical information and photographs.
For genetic studies, genomic DNA extracted from blood was used to perform targeted gene capture using a custom capture kit on Illumina HiSeq 2000 sequencing platform (Illumina, California, USA). Sequencing identified a homozygous missense mutation in exon 3 of the LEP gene (chr7:127894610;c.298G>A) resulting in the amino acid substitution of asparagine for aspartic acid at codon 100 (p.Asp100Asn). Validation of the identified mutation was done by Sanger sequencing to exclude false positivity (). The Asp100Asn variant lies in the functional domain of the leptin protein and has not been reported in the 1000 Genomes database. It has a minor allele frequency of 0.0008% in the Exome Aggregation Consortium (ExAC) database. The in silico predictions of the effect of the mutation are “probably pathogenic” by Polyphen-2 (HumDiv) and “pathogenic” by Sorting Intolerant From Tolerant, Log ratio test and MutationTaster2. The reference codon is conserved across species.
Sequencing also revealed a homozygous missense variation in exon 11 of Bardet-Biedl syndrome-1 (BBS1) gene (chr11:66291279; G>A; Depth 168x) resulting in amino acid substitution of isoleucine for valine at codon 346 (p.Val346Ile). This variant has a minor allele frequency of 0.16% and 0.1% in the 1000 Genomes and ExAC databases respectively. The in silico prediction of the effect of the mutation is “pathogenic” by only MutationTaster2. The reference codon is conserved across mammals. This BBS1 mutation is classified as a variant of uncertain significance based on the above evidence. Sanger sequencing of exon 3 of the LEP gene and exon 11 of BBS1 gene of the unaffected parents identified the same variations as in the index patient but with heterozygous inheritance. |
pmc-6083476-1 | A 13-month old girl was admitted to our department due to failure to thrive. She was the second child of healthy, unrelated parents, whose heights were 190 cm (father) and 175 cm (mother). An ethical review board approval and informed consent from both parents of the proband presented here were obtained, in accordance with national laws.
The patient was the product of a 37 weeks gestation. During the 4th-8th gestational week, the mother experienced vaginal bleeding. Intrauterine growth retardation was diagnosed in the 8th gestational week due to placental insufficiency. Additionally, the mother admitted she was smoking during the entire pregnancy period. The newborn was asymmetrical and small for gestational age (SGA), with a birth weight of 2420 g (<3rd percentile, z-score: -1.93), and a length of 44 cm (<3rd percentile, z-score: -2.76) (). Head circumference was 34.5 cm (70th percentile, z-score: 0.52). She was partially breast-fed during the first 30 days of life. Due to the infant’s unwillingness to take formula milk, she was transferred to the pediatric gastroenterology department where a 24-hour nasogastric tube was placed at the age of nine months and hypercaloric oral supplements were administered, without significant effect on body weight gain ().
On physical examination, at 13 months of age, the infant was small and skinny, not resembling obese GH deficient neonates. Her length was 60 cm (<3rd percentile, z-score: -6.03) and her weight 5470 g (<3rd percentile, z-score: -4.35). Head circumference was 45 cm (40th percentile, z-score: -0.27) and head shape was triangular with open fontanelles. Hair was very sparse and ears were low set. Nasal bridge was hypoplastic and dental development was significantly retarded (one tooth). Motor milestones were delayed; she was able to sit but could not stand. Systematic clinical examination of the heart, lungs and abdomen did not reveal abnormal findings.
Complete blood count, haemoglobin levels and glucose concentrations as well as renal function were within the normal range for her age. Karyotype analysis showed a normal female genotype of 46 XX. Thyroid and adrenal hormone levels were normal. Serological indices for celiac disease or food allergy were negative. Serum GH response to clonidine, glucagon and arginine stimulation tests revealed very poor response, with a peak GH value of 4.77 ng/mL, demonstrating IGHD (). An IGF-1 generation test after administering GH at a dose of 33 µg/kg for four consecutive days showed low IGF-1 levels with a modest response (). After 12 months of GH treatment, serum IGF-1 level rose to 23 ng/mL. Bone age was two months at the chronological age of 13 months. Magnetic resonance imaging of the brain revealed a normal pituitary gland and normal hypothalamus.
At the chronological age of 19 months the patient was administered GH at a starting dose of 0.28 mg/kg/week, subcutaneously. After ten months, GH dose was increased to 0.35 mg/kg/week. At the age of 22 months she started to walk. At the chronological age of 24 months she presented a 12 month phalangeal and a nine month carpal bone age. After ten months of medication she gained 7 cm in length (8.14 cm/year), 300 g in weight and her head circumference had increased by 2.2 cm. After one year of treatment (at chronological age of 31 months) the patient had achieved a length of 73.5 cm (<3rd percentile, z-score: -5.2), a weight of 6100 g (<3rd percentile, z-score: -5.65) and a head circumference of 48 cms (50th percentile, z-score: -0.02) ().
Due to the facial features of the patient, Silver-Russell syndrome has been suspected. The absence of the clinical criteria of Price et al (7) along with a deletion/duplication analysis with array genomic hybridization, Silver-Russell syndrome was excluded. Additionally, presence of intrauterine growth retardation, along with facial characteristics and delayed eruption of teeth, could suggest a possible diagnosis of 3M syndrome. Triple whole exome sequencing (WES) of the affected girl and parents (CentoXome GOLD®) using Illumina technology was performed. No mutation on CUL7, OBSL1 or CCDC8 genes, the mutations leading to 3M syndrome were found. A novel homozygous nonsense variant in the GHRH-R gene, the c.97C>T (p.Gln33*) was detected. The observed variant creates a premature stop codon and is classified as likely pathogenic-class 2 variant. Parental genotyping detected the novel variant in the mother in a heterozygous form, but it was not found in the father. It is suspected that a large deletion not detectable by WES in the paternal allele is present. The detected c.97C>T variant of the GHRH-R gene has never been reported before and not listed, in the CentoMD. |
pmc-6083477-1 | We present a 9-year-old girl with newly diagnosed DM1, DKA, brain edema, multifocal vasogenic brain lesions and lower limb paresis.
The patient was reported to have polyuria and polydipsia over the past week and a weight loss of 3 kg over the previous month. The patient was admitted to the district hospital in a serious clinical condition with severe dehydration. Initial intravenous fluid therapy included infusion of 15 mL/kg of 0.9% sodium chloride during the first 90 minutes. The total volume of fluids administered during the first 12 hours and consisting of 1250 mL 0.9% sodium chloride (NaCI) and 1000 mL of 5% dextrose with 0.9% NaCI (2:1 proportion, sodium concentration-51.34 mEq/L) was 65 mL/kg (patient’s weight-34.6 kg). Intravenous insulin therapy was introduced in an initial dose of 0.05 units/kg/hour in order to prevent a rapid decrease of glycaemia. After three hours, the patient’s medical state and neurological condition was reported to deteriorate. She experienced motor restlessness and agitation followed by upper limb spasms. At the end of the first day of treatment the patient was transferred to the Intensive Care Unit (ICU) of the Children’s Memorial Health Institute in Warsaw with a Glasgow Coma Scale (GCS) score of 13 points. Results of laboratory tests are shown in .
Six hours after admission to the ICU, her clinical state was deteriorating rapidly and the GCS score had decreased to 7 points. Computed tomography scan revealed brain edema and a 13-mm hypodense lesion in the left temporal region ().
The patient was sedated and intubated. Insulin infusion was continued and intravenous fluid administration was diminished. Anti-edematous treatment was introduced (Mannitol 0.3 g/kg/dose, three times per day). The patient’s state showed a gradual improvement. After four days, she was extubated. Subsequently the patient was transferred to the Department of Endocrinology and Diabetology. Despite improvement in her clinical condition, the patient was found to have developed symmetric lower limbs paresis. Brain magnetic resonance imaging (MRI) revealed numerous, diffuse lesions (, ). Presence of infection and neoplasm of CNS were ruled out.
Lower limb nerve conduction studies (NCS) revealed damage to the motor neuron in both lower extremities with dysfunction in both peroneal nerves and in the right tibial nerve. Neurological opinion was that the etiology of the multifocal brain lesions was vasogenic. However, the cause of neuropathy was not fully clear. Presence of DKA and peripheral ischemia were given as the probable factors leading to development of the neuropathy.
Alpha lipoic acid and vitamins B1, B6 and B12 were introduced to the therapeutic regimen and the patient underwent intensive physiotherapy, which led to improvement of left lower limb motor function.
Brain MRI was performed three months later in which no progression in size and number of the brain lesions was observed. NCS revealed normalization of the left peroneal nerve parameters. However, findings indicative of deep motor neuropathy of the right lower limb was found to persist. An informed consent form for publication was given by the parents. |
pmc-6083480-1 | A 70-year-old male patient presented in our department with a painful swelling over the left lateral malleolus, which rapidly emerged over the past 3 months. The patient had no history of trauma. His medical history was completely negative regarding any comorbidities, especially no rheumatic disease, psoriasis, or gout.
At initial presentation, a large swelling over the left lateral malleolus was evident with no redness. A local tenderness was present over the peroneal tendons but not the lateral malleolus itself. The range of motion of the left ankle joint was extension/flexion 5/0/30°. Anterioposterior and lateral X-rays of the ankle joint did not show any signs of osteoarthritis or other osseous destruction (). For further differential diagnosis, a magnetic resonance imaging (MRI) tomography was planned.
MRI revealed a lesion of size of 4.3 × 2.7 × 3.7 cm in close relation to the peroneal tendons with hypo- and hyperintense central areals, especially in the inframalleolar area, and an inhomogenous, diffuse uptake of the contrast agent, highly suspicious for a synovial sarcoma (). C-reactive protein concentration was 5 mg/dl, and the white blood cell count was 8800 × 106/l. Based on these findings and under the suspicion of a malignant soft tissue tumor, an open incisional biopsy of the region was carried out. Tissue samples of the biopsy were sent for microbiological and histopathological examination.
The microbiological examination showed no bacteria growth. The histopathological examination revealed a chronic granulomatous inflammation with debris and deposition of urate crystals indicating tophaceous gout, with no signs of any malignancy.
The histopathological report was then discussed with the patient. At that point, gout was unknown for him and he has never had any gout-specific complaints prior to the onset of these symptoms. Laboratory examination showed a serum uric acid concentration of 7.0 mg/dl (normal values: 3.4–7.0 mg/dl).
Due to the mechanical obstruction of the swelling, especially for shoes wearing, the patient wished for excision of the tophus. 14 days after the open biopsy, revision surgery was performed. The peroneal tendons were prepared along their entire course from the musculotendinous transition to the insertion of the peroneus brevis tendon to the fifth metatarsal bone. After opening of the tendinous sheath of both peroneal tendons, the tophaceous lesion was exposed (). The lesion was then excised from proximal to distal (). Both tendons of the peroneus brevis and longus were then prepared along their entire course and debrided from any remaining tophaceous infiltration (Figures and ).
Postoperatively, the operated leg was immobilized in a Vacoped® shoe (Fa. Omed, Germany) for the first 6 weeks. The patient was allowed to put weight bearing as tolerated. The further postoperative course was uneventful, and the patient was dismissed 4 days after surgery. The patient was advised to follow gout nutrition therapy along with allopurinol medication. |
pmc-6083481-1 | The patient is a 65 year-old male with a past medical history significant for coronary artery disease (status post a drug-eluting stent to his right coronary artery in 2011, with occluded left circumflex), paroxysmal atrial fibrillation (status post ablation in 2015, rhythm controlled, on apixaban), abdominal aortic aneurysm (status post endovascular repair), diabetes mellitus (A1C of 6%), polymyalgia rheumatica on chronic prednisone, and colon cancer (status post resection), who was admitted to the General Medicine Service for lumbar 5-sacral 1 discitis. Cardiology was consulted for chest pain. The patient normally had stable angina pectoris but now had been experiencing unstable angina and nocturnal angina. His electrocardiogram at the time demonstrated sinus rhythm with anterolateral ST segment depressions and T wave inversions and isolated ST segment elevation in aVR. He was treated with sublingual nitroglycerin, after which the chest pain and electrocardiographic changes were resolved (EKG at rest showed sinus bradycardia, occasional premature atrial contractions (PAC), and upright T waves, with no ischemic changes).
About five months before the current episode, he had a nuclear stress test which demonstrated a small reversible perfusion defect of moderate severity in the basal, lateral region, consistent with mild ischemia. His ejection fraction was 53%.
His cardiac medications include carvedilol, aspirin, isosorbide, and lovastatin.
His physical exam was not remarkable for any physical exam findings.
His echocardiogram here showed normal ejection fraction, with inferior wall hypokinesis with a pseudonormal filling pattern.
A cardiac heart catheterization was recommended given the significant symptoms and significant electrocardiographic changes as part of his preoperative risk assessment.
The catheterization showed that the left main bifurcates into the left anterior descending (LAD) and a small left circumflex (LCx). There was also mild disease of the mid and distal left main.
The LAD wraps around the left ventricle apex, with diffuse mild-moderate disease. It gives off a large diagonal branch with mild diffuse disease ().
The LCx was described as a small vessel with proximal occlusion at the takeoff of a large marginal branch that has mild diffuse disease (Figures and ).
The right coronary artery (RCA) is a dominant vessel with a focal area of moderate eccentric disease in the mid vessel. Otherwise, it has mild diffuse disease. It gives off a large posterior descending artery (PDA) and posterolateral branch (PLB) that directly provide retrograde collateral supply to the distal atrioventricular (A-V) circumflex. The A-V circumflex turns at an acute angle away from the left main artery () (video ).
The patient underwent uncomplicated back surgery. He continued to have exertional angina (Canadian Cardiovascular Society (CCS) class III-IV) with substernal chest tightness and radiation to the shoulder with minimal activity and at rest at night. Symptoms were relieved by sublingual nitroglycerin. He was subsequently referred for hemodynamic assessment of the left main and left anterior descending with planned CTO PCI of the occluded circumflex.
During CTO PCI, initial dual angiogram demonstrated that the RCA collateral to the circumflex was in a different plane than the left main coronary artery ().
This raised the suspicion that the circumflex artery was probably anomalous in origin ().
Further probing of the right coronary cusp demonstrated an anomalous origin of the circumflex artery. The patient did not have CTO of the circumflex but instead had severe stenosis of his anomalous circumflex ().
The patient was found to have an anomalous left circumflex artery, the most common coronary anomaly. Fractional flow reserve of the left main and the left anterior descending were 0.91 at rest and 0.82 at hyperaemia.
The patient underwent successful PCI of the anomalous circumflex with drug-eluting stents () (video ). |
pmc-6083489-1 | We present a 79-year-old male with history of polymyalgia rheumatica on chronic prednisone who presented with a two-week history of progressively worsening dyspnea, cough, and a 10 pound weight loss. He initially presented to an urgent care and had been prescribed antibiotics without improvement in his symptoms. He returned to the urgent care and underwent a chest X-ray, which was remarkable for cardiomegaly. Given this finding in conjunction with his respiratory symptoms, he was referred to the emergency department (ED).
In the ED, a CT of the chest showed a large mediastinal mass with invasion of the pericardium (); a soft-tissue mass within the right atrium; compression of the left atrial appendage; encapsulation of the thoracic aorta and pulmonary artery; and extensive mediastinal, hilar, and subcarinal lymphadenopathy—the largest of which measured 3 cm in diameter. CT of the abdomen and pelvis showed numerous intra-abdominal and retroperitoneal soft-tissue masses.
The patient was admitted to the intensive care unit (ICU) where a TTE showed a left ventricular ejection fracture of 55% along with a large, homogenous adherent mass infiltrating the right atrium and ventricle, abnormal thickening of the interatrial and interventricular septum of the right heart, severe right ventricular dysfunction, severe basal to apical hypokinesis to akinesis, and a pulmonary artery pressure of 21.8 mmHg (Figures and ).
Initial differential included primary lymphoma, cardiac sarcoma, or metastatic involvement of the heart. The patient underwent a biopsy of an abdominal soft-tissue mass. Pathology showed diffuse large B-cell lymphoma (DLBCL), nongerminal center type, with BCL2 and MYC.
The patient received intravenous (IV) methylprednisolone 250 mg daily for five days for debulking. He was initially treated with two cycles of R-CHOP; however, given his persistently reduced ejection fraction, the patient was changed to liposomal doxorubicin (R-CDOP) for the third and fourth cycle. Furthermore, his first cycle was also complicated by new onset first-degree atrioventricular block and a right bundle branch block. A positron emission tomography (PET)/CT scan and TTE were scheduled prior to the next cycle of R-CDOP. In addition to his systemic chemotherapy, the patient received three cycles of central nervous system prophylaxis with high-dose methotrexate. |
pmc-6083508-1 | A 58-year-old woman presented with a 2-year history of a non-productive cough and progressive shortness of breath. She had a history of renal insufficiency and persistent proteinuria, without any extra-renal involvement. She was diagnosed with IgA nephropathy for 15 years and had received immunosuppressive therapy for 6 years.
Her vital signs were stable at initial examination; the patient was afebrile and oxygen saturation was 95% in ambient air. On physical examination, auscultation of the lungs detected slight coarse crackles at the bilateral bases. The remainder of the examination was unremarkable.
Her hemoglobin was 95 g/L but renal function and calcium level were normal. The patient was negative for antinuclear and antineutrophil cytoplasmic antibodies in immunofluorescence assays. Repeated exams of sputum smear did not yield any pathogenic micro-organisms. Serum free light chain analysis showed measuring lambda light chain of 2.59 g/L. Serum protein electrophoresis revealed low IgG, IgA, and IgM levels, with reported immunoelectrophoresis (IEP) showing monoclonal lambda light chain peak with the monoclonal protein. A 24-h urine contained 5521 mg of protein. High-resolution chest CT revealed disclosed ground-glass opacities (GGOs) with interlobular septal thickening in bilateral lungs (Fig. and ). Chest and abdominal CT shows soft tissue infiltration of the subcutaneous fat layer with asymmetric bulging of the chest and abdominal wall (Fig. and ). Pulmonary function tests revealed a moderately restrictive ventilation disorder, with a forced vital capacity (FVC) of 1.76 L (69.0% of predicted) and severe reduction of diffusion capacity (DLCO SB 20.8% of predicted). Cardiac biomarkers, such as natriuretic peptides, particularly B-type natriuretic peptide (BNP) and cardiac troponin-T were normal. Echocardiogram showed normal left ventricular ejection fraction of 61% and there were no features of cardiac amyloidosis. A VATLB was performed, which showed marked thickening of the alveolar wall deposition of amorphous eosinophilic amyloid at the bronchial mucosa, pulmonary vessel wall and interstitium. Congo red staining display apple-green birefringence in polarised light (Fig. and ). The immunohistochemical stain for protein A was negative for secondary amyloid. Following the VATLB results, bone marrow examination was performed. Bone marrow examination showed 11.5% plasma cells with lambda light chain restriction. Bone marrow cells by flow cytometry exhibited a typical phenotype for plasma cells, expressing monoclonal cytoplasmatic CD138, CD38, and cLambda instead of CD19, CD56 and cKappa, which are characteristic of the typical immunophenotype of MM. Fluorescence in situ hybridization (FISH) was carried out to check on the bone marrow aspirate. The most frequent abnormality in the patients was 1q21 amplification (25%), followed by 14q32 (IGH) translocations (29.5%), without 13q14 (RB1) deletion, 13q14.3 (D13S319) deletion and p53 deletion. No lytic bone lesions were detected with positron emission tomography/computed tomography (PET/CT). This confirmed the diagnosis of MM, λ light chain type, stage I. Surgical biopsy of abdominal wall skin and subcutaneous fat was also performed, which showed the apple-green birefringence with polarized light on Congo red stain was demonstrated in dermis (Fig. and ).
She was therefore diagnosed with diffuse parenchymal pulmonary amyloidosis accompanied by MM. The patient was referred to the hematology department, where the patient started chemotherapy with bortezomib and dexamethasone, and her symptoms relieved 2 months after the initial presentation. No adverse effects were observed and the laboratory results were stable. |
pmc-6083511-1 | An 86-year-old multiparous Japanese woman with an unremarkable medical history was transferred to our hospital after a traffic accident (Table ). She was hit by a car while walking at a crosswalk. On presentation to our emergency department, she complained of pain in her buttock. Her Glasgow Coma Scale score was 15/15. Her blood pressure was 100/53 mmHg, heart rate was 93 beats/minute, respiratory rate was 15 breaths/minute, and oxygen saturation was 100% while breathing 2 L/minute of oxygen. A whole-body computed tomography scan revealed fractures of her left pubic bone and sacrum and a hematoma with contrast extravasation in front of the sacrum (Fig. ). Her blood pressure then suddenly dropped to 67/38 mmHg secondary to hemorrhagic shock. Rapid resuscitation with fluids and blood was performed. We attempted to perform transcatheter arterial embolization (TAE). Based on angiographic findings (Fig. ), bilateral internal iliac artery embolization was performed with gelatin sponge particles. She received 560 ml of packed red cells, 480 ml of fresh frozen plasma, and 200 ml of platelets, and she became hemodynamically stable.
After admission to our intensive care unit, she developed shaking chills and a high fever. She was hemodynamically stable the following day. However, reddish urine was observed. Her serum lactate dehydrogenase level was extremely high, and fragmented red blood cells were present on peripheral blood smears. On the third day of hospitalization, despite fluid challenges and the use of diuretics, she became anuric and thus underwent hemodialysis. However, she developed severe delirium and was intubated under sedation. She was not diagnosed as having TTP at this point because her platelet count was not reduced despite the worsening of her hemolysis. Her prothrombin time and activated partial thromboplastin time were normal, and her fibrin degradation products were returning toward the normal concentration within 3 days of admission; therefore, disseminated intravascular coagulation (DIC) was excluded.
On the fifth day of hospitalization, her platelet count, measured by a different hemocytometer, was very low. Her fragmented red blood cell concentration measured by visual judgment based on the International Council for Standardization in Haematology (ICSH) reference method was 28.8% (Fig. ). We finally confirmed the diagnosis of TTP based on the classic pentad of TTP and began plasma exchange.
After retrospectively checking her platelet count, we found that it had decreased to 55 × 109/L on the second day of hospitalization (Fig. ). The presence of many fragmented red cells is often associated with a spurious increase in the platelet count because the fragmented red cells are erroneously measured as platelets by automated blood cell counters.
Plasma exchange was continued for 5 consecutive days. Her clinical course dramatically improved in just a few days, and her platelet count increased. She was weaned from hemodialysis on the 15th day of hospitalization. She recovered fully and was discharged from our hospital on the 31st day of hospitalization. The ADAMTS13 activity measured by an enzyme immunoassay on the third hospital day was not reduced (65%), but a direct Coombs test was negative and the complement factor level was normal. |
pmc-6083513-1 | A 53-year-old female presented with an asymptomatic gastric tumor found incidentally during screening upper gastrointestinal endoscopy. The lesion appeared as a flat protrusion in the lesser curvature of the lower third of the stomach (Fig. ). Endoscopic ultrasound (EUS) showed a 23 × 12-mm, hypovascular, heterogeneous lesion thickening the second and third gastric layers. The findings strongly suggested scirrhous gastric cancer (Fig. ). Although the lesion biopsy revealed no evidence of malignancy, our suspicion of scirrhous gastric carcinoma persisted due to the configuration of the tumor and our EUS findings. We performed an endoscopic open biopsy to make an accurate diagnosis. However, evaluation of the specimen revealed only slight chronic inflammatory cell invasion. Computed tomography (CT) scanning showed only gastric wall thickening at the angle of the stomach (Fig. ). Positron emission tomography-CT showed no evidence of metastasis or abnormal uptake in the tumor (Fig. ). The laboratory findings were normal as were levels of tumor markers (CEA, 1.6 ng/ml; CA19-9, 12 U/ml).
In spite of these findings, given our high level of suspicion, we decided to perform surgery to obtain a definitive diagnosis and treat the tumor. We chose gastric wedge resection using a combined laparoscopic and endoscopic method for several reasons. Firstly, the biopsy did not show cancer, but the tumor configuration strongly suggested malignancy; therefore, we selected a non-exposed method to prevent interoperative dissemination of tumor cells. Secondly, the tumor appeared to be a submucosal tumor (SMT), and gastric wedge resection using a combined laparoscopic and endoscopic method is among the safest procedures for resection of gastric SMTs [, ]. Lastly, we chose a wedge resection because if the tumor was not malignant, a distal gastrectomy could be considered excessive. We obtained an interoperative pathological diagnosis, and we planned to perform partial gastric resection in the absence of malignancy and laparoscopic distal gastrectomy with lymph node dissection if cancer was identified.
The first port was inserted through the umbilicus using an open technique. Four additional ports were inserted: the second in the subcoastal arch, the third at the mid-point between the camera port and the second port, and the fourth port and fifth port symmetrically. The first, second, and third ports were 12 mm. The fourth and fifth ports were 5 mm.
The lesser curvature gastric tumor was easily recognized laparoscopically. Our intraabdominal examination revealed a scar-like tumor with twitch. After preparation of lesser curvature vessels, the tumor periphery was viewed endoscopically. The entire circumference of the tumor was marked to ensure an approximately 0.5-cm margin from the tumor edge (Fig. ). We then injected indigo carmine into the gastric submucosal layer with an endoscopic needle. The seromuscular dissection was performed using a laparoscopic electrocautery scalpel (Fig. ). The specimen was pulled up along with the surrounding mucosa. A full-layer resection including the specimen was achieved using a laparoscopic stapling device (Fig. ). We used a hand-sewn technique for seromuscular suturing so the staple line would not be exposed (Fig. ). Finally, we inserted the endoscope into the duodenum to ensure that there was no gastric stenosis.
The patient had gastric hypoperistalsis on postoperative day (POD) 1. We inserted a gastric tube; however, we planned for an extended period with no oral intake in case of persistent hypoperistalsis. On POD 4, a W-elemental diet (W-ED) tube was inserted to drain the stomach and provide nutrition. We started the patient on continuous low-dose erythromycin, mosapride citrate, and Rikkunshi-Tou to treat her hypoperistalsis. The W-ED tube was removed on POD 11 after an oral contrast study confirmed gastric motility. On the same day, oral intake was initiated. The patient was discharged from the hospital on POD 18.
The submucosa tumor could be seen in the resected specimen. The tumor had 20 mm × 20 mm × 13 mm size, including spindle cells with myxoid changes and collagen fibers. There was no evidence of adenocarcinoma. In this findings, fibromatosis can be a differential diagnosis.
Spindle tumor cells were identified in the gastric wall from the submucosa to the serosa accompanied by myxoid changes and collagen fibers in the stroma (Fig. and ). The spindle cells were strongly immunopositive for alpha-smooth muscle actin, and anaplastic lymphoma kinase (ALK), but immunonegative for c-kit, desmin, and S-100. CD34, bcl-2, beta-catenin, CD31, pankeratin, platelet-derived growth factor-A, and DOG1 were almost immunonegative (Fig. and ). Based on these findings, the tumor was diagnosed as gastric IMT. |
pmc-6083529-1 | A 57-year-old female with no previous history of liver disease presented with abdominal pain and vomiting for one day. The abdominal pain was described as 7/10 in severity, nonradiating, and diffuse, but most intense in the right upper quadrant. She denied previously experiencing any similar pain. She denied fever or chills but reported 3 episodes of nonbloody, nonbilious emesis after the pain started. There was a history of heart failure with preserved ejection fraction. She had been taking vitamins A and D and an herbal supplement for weight loss but she denied the use of any prescription weight loss medications. She denied using alcohol, acetaminophen, or any illicit drugs. Her vital signs were normal. Physical examination was significant for diffuse abdominal tenderness without any rigidity or guarding. There was no hepatosplenomegaly or scleral icterus. Laboratory evaluation revealed an alanine aminotransferase (ALT) of 738 U/L [normal: 7-55 U/L], aspartate aminotransferase (AST) of 856 U/L [normal: 8-48 U/L], and an alkaline phosphatase of 80 U/L [normal: 45-115 U/L]. Her total bilirubin was 2.4 mg/dL [normal: 0.1-1.2 mg/dL] and direct bilirubin was 1.4 mg/dL [normal: 0-0.4 mg/dL]. International normalized ratio (INR) was 1.19 [normal: 0.8-1.1] and prothrombin time (PT) was 12.7 seconds [normal 11-13.5 seconds]. Testing for hepatitis A, hepatitis B, hepatitis C, hepatitis E, Herpes-Simplex virus, Ebstein-Barr virus, Parvovirus, and Cytomegalovirus was negative. She had normal vitamins A and D levels ruling out hypervitaminosis as the cause of hepatitis. She tested negative for alcohol and acetaminophen. Anti-smooth muscle antibody, anti-mitochondrial antibody, antinuclear antibody, and anti-liver kidney microsomal antibody were negative. An iron profile was normal. Abdominal ultrasound showed a normal liver with normal echotexture and no biliary ductal dilatation. When the herbal supplement was scrutinized, it was found to be 100% pure GC fruit rind extract. She had consumed two capsules daily as recommended for about one month. Each capsule had 1400 mg of GC extract. She was asked to stop taking the supplement. Her liver enzymes decreased significantly with an ALT of 396 μ/L and AST of 138 μ/L within three days with resolution of her abdominal symptoms. Her total and direct bilirubin came down to 0.6 mg/dL and 0.4 mg/dL, respectively. Her INR and PT also normalized. She had a normal ALT of 25 and AST of 12 in the one-month follow-up visit. Six months later, patient was found to have an elevated ALT of 301 and AST of 69. On interrogation, it was found that she had started taking the same supplement again in the desperate need to lose weight. The CIOMS/RUCAM scale, a scoring system () that is used to establish the etiology of liver damage when drug induced liver damage is suspected, gave a score of 11 for our patient ()[, ]. The score classifies the drug as highly probable (score ≥ 9), probable (score 6-8), possible (score 3-5), unlikely (score 1-2), and excluded (score 0) as the cause of liver injury [, ]. This lead us to conclude that the etiology of the patient's hepatitis was GC extract. |
pmc-6083534-1 | A 41-year-old HIV-1-infected male farmer presented with a five-day history of right hypochondriac abdominal pain associated with decreased appetite, nausea, vomiting, and two days of yellowish discolouration of sclera, with no history of fever, diarrhoea, blurring of vision, and odynophagia or dysphagia.
He presented to us for the first time in September 2016, when he was diagnosed with pneumocystis jiroveci pneumonitis (PJP) on transbronchial lung biopsy and successfully treated with co-trimoxazole and prednisolone. This biopsy did not reveal CMV inclusion bodies. His CD4 count was 109/μL and HIV-1 viral load was 4.89 log copies/ml.
He was diagnosed with HIV-1 infection in 2000 and was treated by another physician, for which he was initiated on zidovudine (ZDV)/lamivudine (3TC)/nevirapine (NVP) and switched to tenofovir disproxil fumarate (TDF)/3TC/atazanavir/ritonavir (ATV/r) in 2011 due to virological failure. After control of PJP, we switched his HAART to raltegravir (RAL)/darunavir (DRV)/r based on the genotypic resistance report. Within 4 weeks of initiating HAART, he presented with the above mentioned symptoms.
Physical examination was unremarkable except for icterus and tender hepatomegaly. Fundoscopy did not reveal CMV retinitis. He had normocytic normochromic anaemia (Hb: 10.7 g/dl), normal total leucocyte, and differential counts. Liver enzymes were total bilirubin: 9.2 mg/dl (reference value: 0-1 mg/dl), aspartate transaminase (AST): 109 U/L [15–37 U/L], alanine transaminase (ALT): 41 U/L (14–59 U/L), and alkaline phosphatase (ALP): 906 U/L (46–116 U/L). Tests for hepatitis B and hepatitis C were negative.
Hepatomegaly (liver span-18 cm) with grade I-II intrahepatic biliary dilatation with focal parenchymal lesion was seen on abdominal ultrasound. The common bile duct (CBD) was dilated, measuring 14 mm suggestive of possible obstruction at the level of ampulla with no definitive mass or obstructive lesion.
An endoscopic retrograde cholangio-pancreatography (ERCP) was performed, which revealed oedematous papilla with stenosis. Cholangiogram showed a dilated CBD with prepapillary CBD stricture. All these features were suggestive of AIDS cholangiopathy ().
Wide papillotomy was performed, and a 7 Fr CBD stent was placed for biliary drainage. Bile aspirate was negative for Cryptosporidium, Microsporidium, Cyclospora, and Mycobacterium. Brush cytology of the stricture revealed inflammatory cells, pigment-laden macrophages, and several red blood cells with no acid-fast organisms.
Histopathological examination of ampullary biopsy showed focal ulcerations with superficial mucosal necrosis and acute and chronic inflammation in the mucosa and lamina propria. Cells of submucosal Brunner's glands showed nucleomegaly with eosinophilic intranuclear and basophilic cytoplasmic CMV inclusions ().
After intervention, the patient had immediate symptomatic relief and did not receive any anti-CMV treatment. Liver enzymes normalised 2 weeks later.
After 6 months, CD4 improved to 363 cells/μL with HIV viral load of 423 copies/ml. Repeat ERCP revealed normal cholangiogram with no residual stricture in lower CBD, with normal papilla (). Previously placed stent had migrated out. Cells from ampullary biopsies did not reveal intranuclear/cytoplasmic CMV inclusions ().
A repeat viral load after 8 months was <50 copies/ml. ART was switched to dolutegravir (DTG)/DRV/r. |
pmc-6083539-1 | A 58-year-old Thai previously healthy male farmer, living in Roi Et Province (Northeast Thailand), was referred to our hospital, King Chulalongkorn Memorial Hospital, due to problems with total ophthalmoparesis and productive cough for 2 months. Two months prior to admission (PTA), he developed productive cough and low-grade fever with no response to many courses of antibiotic treatment. One month PTA, he noted binocular horizontal diplopia of the right eye, before progression to visual loss and ptosis despite antibiotic and steroid treatment. His past medical history was unremarkable except heavy alcoholic drinking.
The right eye examination revealed eyelid swelling, proptosis, marked chemosis, and complete ptosis; light perception of visual acuity; absent afferent pupillary reflex; and optic disk swelling and retinal hemorrhage. Neurological examination revealed right eye total ophthalmoparesis and decreased pinprick sensation of right cranial VI nerve area. Multiple dental caries were noted. Pulmonary examination revealed fine crackles at both the lungs. Other examination was unremarkable.
Complete blood count showed hemoglobin of 9.3 g/dL, white blood cells of 25,540 cells/mm3 (85.1% neutrophil, 9.8% lymphocyte, and 4.8% monocyte), and platelets of 697,000 cells/mm3. Blood chemistry was normal. Anti-HIV test was negative. Chest X-ray revealed bilateral diffuse reticulonodular and alveolar infiltrations. Orbital computed tomography revealed a 1.5 × 2.9 × 1.5 cm rim-enhancing hypodense lesion at the right orbital apex abutting to the optic nerve sheath complex and right medial rectus muscle ().
A diagnosis of orbital and pulmonary infections was made, and emergent anterior orbitotomy was performed and revealed purulent discharge from the orbit. The pus and sputum Gram stain exhibited many Gram-positive filamentous bacilli, but non-acid-fast, on both standard and modified acid-fast bacilli stains. Hence, Actinomyces species was suspected, and Nocardia species could be excluded due to negative results on modified AFB stain. The pus cultures finally grew Actinomyces israelii (Figures and ). Further bacterial identification by base sequencing of 16S ribosomal DNA was A. israelii with 99% identity (NCBI BLAST Search).
He was given penicillin G sodium of 24 million units per day for 6 weeks and amoxicillin of 2 g per day for another 12 months. After 10 days of treatment, there was a much improvement of lung infiltrates on his chest X-ray. The patient was doing well when last seen 1 year after discharge with mild residual impaired ophthalmoparesis and ptosis but still moderate visual impairment. |
pmc-6083543-1 | A 14-year-old female presented to an outpatient psychiatrist with severe depression and suicidal ideation. She was admitted to the pediatric psychiatric unit for evaluation and treatment.
Her medical history was significant for anxiety, depression with multiple suicide attempts, postconcussive syndrome, chronic migraines, and previous diagnosis of complex regional pain syndrome of the lower extremities. While being admitted for depression and suicidality, she also complained of exacerbation of her bilateral leg pain, which significantly limited her mobility and worsened her mood.
She was admitted two months prior for bilateral generalized neuropathic leg pain which limited her mobility. A lumbar magnetic resonance image (MRI) was unremarkable. She tried multiple classes of pain medications including acetaminophen, nonsteroidal anti-inflammatory agents, tricyclic antidepressants, gabapentinoids, antiepileptics, and opioids. Additional interventions included acupuncture, physical therapy, occupational therapy, guided imagery therapy, and epidural steroid injection.
Upon readmission for suicidality, the pain management team was consulted. On her psychiatric evaluation, patient had a depressed, flat affect endorsing suicidality. The patient reported despair regarding her chronic pain as well as flashbacks to a previous concussion after a fall several years prior with subsequent development of her neuropathic type pain. She had also reported several instances of self-injurious behaviors including cutting and two suicide attempts with a shoelace and pillowcase. She was placed on continuous observation. The patient's chronic outpatient psychiatric medications include fluoxetine and aripiprazole. While she was an inpatient, the patient was also trialed on bupropion but was discontinued due to increased agitation and irritability. She also reported severe burning bilateral leg pain. In addition, she described her pain in terms of “shooting”, reporting painful paresthesias, dysesthesias, and hyperalgesia as well as a “numbness” characterization to her bilateral leg pain, which followed a neuropathic pattern.
Her lumbar spine was mildly tender to palpation, but her neurological exam was otherwise intact. While her characterization of pain was neuropathic in nature, she did not meet the criteria for complex regional pain syndrome. There were no allodynic features, vasomotor, pseudomotor, or tropic changes on exam. Despite her prescribed regimen of pregabalin, ibuprofen, acetaminophen, lidocaine 5% patch, tizanidine, and subsequently oxycodone as needed, her pain continued. She had also been tried previously on morphine with also minimal efficacy. The patient also underwent a ten-day trial of duloxetine with minimal improvement and reported worsening severe depressive symptoms, suicidal ideations, and neuropathic pain. It was then recommended to start an intravenous ketamine infusion which was theorized to have a more rapid clinical onset and effect.
Patient was transferred to the pediatric intensive care unit for intravenous ketamine infusion and monitoring. Prior to starting intravenous ketamine infusion, her leg pain was rated 7/10 on the numerical rating scale (NRS, 0-10 with 10 being the worst pain) of burning quality. There was no weakness or changes in sensation, though there was limited mobility secondary to pain. Intravenous ketamine infusion was started at 7 micrograms/kilogram/minute (mcg/kg/min). The ketamine infusion was used 24 hours per day for the entire duration of the treatment with ketamine infusion. The total dose was titrated based on the specific mcg/kg/min. The patient's weight (72 kg) was used for the dosing calculation.
The patient remained hemodynamically stable with no dysphoria or hallucinations. On day one of intravenous ketamine infusion, the patient had significant improvement of her depressive symptoms, as noted by psychiatry and pain management teams, and the maximum NRS throughout the day was 6/10. On day two, she reported less pain in her legs with a maximum NRS of 5/10, and significant improvement in her mood with no further suicidal ideations. On day three, intravenous ketamine infusion was increased to 8 micrograms/kilogram/minute; however physical exam revealed nystagmus and visual changes and was decreased to 7 micrograms/kilogram/minute. Patient's pain maximum NRS score remained at 5/10. On day four, maximum NRS score reduced to 4/10 and self-reported 70% pain relief since initiation of intravenous ketamine infusion. She also had functional improvement in her legs and was able to ambulate freely. On day five, dosage was titrated down to 4 micrograms/kilogram/minute with maximum NRS score of 0/10 and sustained improvement in mood. Intravenous ketamine infusion was further reduced to 2 micrograms/kilogram/minute and discontinued that same day. She was able to tolerate physical therapy and maintain analgesia. Psychiatric reassessment determined her to be no longer at suicide risk, and she was discharged with no immediate sequelae and was placed on chronic aripiprazole, topiramate, and lithium by her outpatient psychiatrist.
The patient had five months of symptom relief after her first intravenous ketamine infusion. She was readmitted five months later with repeated suicide attempts, depression, and worsening bilateral upper and lower extremity neuropathic pain, though with decreased baseline NRS. Due to her dramatic improvement and sustained relief with intravenous ketamine infusion during her last admission, as well as the lack of other successful treatment options, she was again admitted to the pediatric intensive care unit for five days of intravenous ketamine infusion with resolution of her neuropathic pain and suicidal/depressive symptoms allowing for discharge home without any immediate sequelae. |
pmc-6083553-1 | A 63-year-old woman visited our outpatient clinic because of general malaise that lasted 6 days. She developed RA at the age of 60 years and had been treated with 400 mg monthly intravenous tocilizumab for the past 10 months and 3 mg/day prednisolone. She had no history of blood transfusion, alcohol use, travel abroad, or raw meat intake, and her joints were not tender or swollen. Disease Activity Score 28-joint count C reactive protein was 1.13. Laboratory data revealed elevated liver enzyme levels: AST, 338 IU/L; ALT, 523 IU/L; ALP, 377 IU/L; and γ-GTP, 68 IU/L. Blood counts, total protein, albumin, total bilirubin, electrolytes, renal tests, C reactive protein, and coagulation test results were almost within normal ranges. Her serum HBV nucleic acid levels were monitored regularly to detect HBV reactivation because she tested positive for antibodies to HBV surface and core antigens without HBs antigen before the initiation of tocilizumab. At admission, HBV DNA levels were within normal range. Tests to detect antibodies to hepatitis A and C were negative. Tests to detect antibodies to Epstein–Barr virus and cytomegalovirus were both negative for immunoglobulin M (IgM) but positive for immunoglobulin G (IgG). Abdominal ultrasound revealed normal liver morphology.
The patient was diagnosed with HEV infection (genotype 3) because tests to detect anti-HEV immunoglobulin A (IgA) antibody and HEV RNA in her sera were both positive. Tocilizumab, pregabalin, eldecalcitol, and teriparatide were discontinued, and stronger neo-minophagen C and ursodeoxycholic acid were administered. Liver enzyme levels decreased and returned to normal 3 weeks after admission, and she was discharged from our hospital. Results of HEV RNA tests were negative 6 weeks after admission. Tocilizumab and eldecalcitol were reinitiated 4 weeks after liver enzyme normalization. RA remained in remission, and liver enzymes remained stable for the subsequent 5 years under tocilizumab therapy.
Because she had been a participant in a prospective clinical study to investigate the incidence of HBV reactivation in patients receiving immunosuppressive and/or anticancer therapies [], her sera that was collected prior to hepatitis E onset had been stored. The use of serially stocked sera for HEV detection was approved by the Ethics Committee of Gunma University Hospital (#15-61). We examined her serum for anti-HEV antibodies and HEV RNA before and after admission (). Neither anti-HEV antibodies nor HEV RNA was detected in the preadmission samples. In contrast, all of them were positive at admission. Anti-HEV IgM and IgA antibody levels peaked 1 week after admission and declined thereafter. Anti-HEV IgG antibody levels remained elevated until the final observation at 57 months. HEV RNA was detected at 0, 2, and 3 weeks after admission and was undetectable thereafter. |
pmc-6083556-1 | A Folliculotropic Mycosis Fungoides was diagnosed in a 58-year-old male patient in 1997 and treated with local chlormethine between 1998 and 2006.
In 2006, MF progressed toward a tumoral form with infiltrating plaques and nodules all over his body, the most important being an exophytic one arising from the nasal region. No Sezary cell was noted in the blood smear. A biopsy of cutaneous tumor was performed and the pathologist confirmed a localization of tumoral nontransformed MF.
Between 2006 and 2014 the patient received several systemic treatment lines including methotrexate, PUVA therapy, pegylated liposomal doxorubicin, polychemotherapy, histone deacetylase inhibitors, and anti-CCR4 monoclonal antibody. All these drugs were without long-lasting effect and tumoral lesions progressed including the tumoral lesion of the nose (Figures and ).
The extent, progression, and resistance of his skin lesions gave a palliative intent to his treatment project. The patient reported that the aspect of his nose refrained him from interacting with people, which led him progressively to get socially isolated. He reported difficulties in interacting with his family members especially with his young grandchildren. Histology from the nasal lesion was obtained and showed classical Mycosis Fungoides of granulomatous type without transformation.
He was then referred to our radiotherapy unit in August 2014. We opted for a conventional radiotherapy with 12 MeV electrons and 6 MV and 18 MV photons. The patient received 36 Gy in 18 fractions (2 Gy per fraction, 5 fractions per week).
Lesions disappeared completely within a few weeks (Figures and ). The patient presented acute grade I radiodermatitis (NCI CTCAE Version 4.03) which resolved spontaneously. No clinical relapse had been noted 3 years after the treatment.
As the physical appearance of the irradiated nose got better the patient reported a psychological relief. The recovery of the normal aspect of his nose helped him resume some of his social activities, use public transportation, and better interact with friends and family members. |
pmc-6083593-1 | An 80-year-old man in good health was admitted to our hospital for severe pain in the left hip associated with functional impairment after a fall at home. X-rays and computed tomography (CT) of the painful hip showed a femoral trochanteric fracture with an Evans classification of 1c (). Two days after injury, the patient underwent internal fixation with an intramedullary nail via the lateral approach with gentle traction, internal rotation, and adduction using a traction table (). The intramedullary nail was fixed with a lag-screw and 2 cortical screws at the distal site (Zimmer® Natural Nail™ System-Cephalomedullary Femoral Nail-Asia short; Zimmer, Warsaw, IN, USA). The operation took 60 minutes, and there was minimal blood loss. The surgical procedure was performed without any intraoperative complications, and there were no changes in his vital signs during the operation. Postoperatively, the patient had no significant clinical problems. However, 1 day after the surgery, his hemoglobin (Hb) value dropped from 12.0 to 6.0 g/dL without any noticeable signs of bleeding, so we transfused 4 units of packed red blood cells to the patient. Four days after the surgery, his Hb value had improved to 8.5 g/dl, but after that, the Hb value continued falling. Twelve days after the surgery, the Hb value dropped to 5.9, and another 4 units of packed red blood cells were transfused. However, the Hb value did not improve. We noticed warmth and subcutaneous bleeding in the left femoral region and suspected an arterial injury due to the surgery (). On magnetic resonance imaging (MRI), a huge hematoma was detected in the left inner femoral region (Figures and ). Since pseudoaneurysm due to the surgery was suspected, we performed CT angiography with 3D reconstruction. On CT angiography, a pseudoaneurysm was detected near the tip of the cortical screws at the distal site (). After conducting angiography, vascular leakage from a deep femoral artery branch was confirmed (). The vascular lesion was immediately treated by transcatheter embolization, and the vascular leakage was completely resolved with catheter embolization (). After embolization, the patient's clinical state improved rapidly, and the Hb and C-reactive protein (CRP) values improved to normal after several weeks (). One month later, the patient finally left the hospital using a crutch to aid in walking. |
pmc-6083596-1 | Patient n. 1 was a 66-year old male, suffering from diabetes type I, requiring insulin therapy since 2006. The patient also presented associated diabetic retinopathy, and a primary open angle glaucoma (POAG) had been diagnosed in January 2014. Values of IOP were successfully maintained within normal range with hypotensive topical drugs. In January 2016, the patient had referred to increasing irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand in his eyes, scored as OSDI (Ocular Surface Disease Index) [] of 65 out of 100, with a VAS (Visual Analogue Score) [] score of pain of 74 mm out of 100 and no difference between eyes. Slit lamp evaluation had not shown epithelial damage, also with the aid of fluorescein vital stain observed with the blue cobalt filter, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 7 seconds in both eyes had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.
At a subsequent visit in April 2016, the patient reported no relief from severe symptoms (OSDI: score 62 out of 100, VAS: 80 out of 100 mm) which remained severe, despite the regular administration of HA, increased from 4 to multiple times each day. As recorded from the history of the patient's charts, previous therapy with anti-inflammatory drugs had turned to be unsuccessful, and in May 2016 the patient was proposed to receive a treatment with topical CBS, as a compassionate unconventional therapy. The rationale for this therapy was to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful.
The patient signed the informed consent, specifically designed for this purpose, and started administration of the CBS eye drops in June 2016, with the posology of 0.4 ml (8 drops) in each eye, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month of treatment.
In September 2016, the patient reported a significant relief from subjective symptoms of discomfort. OSDI score was reduced: 24 out of 100 and VAS was also reduced: 35 mm out of 100. During this visit, the IOP resulted in the normal range, and the analyses of the visual field tests performed since 2014 were carried out.
In the mean deviation (MD) values recorded in January 2014, June 2016, and September 2016 were graphed. A significant lowering in MD values was observed which was followed by an amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. In the central 30-2 visual field tests before ((a) right eye RE; (b) left eye LE) and after (c, d) the CBS treatment were shown. An amelioration of the defect was observed in all the four quadrants in both eyes, as it is demonstrated by the unvaried Pattern Standard Deviation (PSD) values. |
pmc-6083596-2 | Patient n. 2 was a 60-year old female, diagnosed with suffering from POAG for ten years, under treatment with hypotensive drugs and regularly controlled IOP twice a year which appeared maintained within normal ranges. During a control visit in January 2016, the patient had reported irritating symptoms of eye discomfort, described as burning, itchiness, and feeling sand, mainly in her right eye (RE), over the last six months. The situation had been managed with the use of several types of tear substitutes, none of them successful in symptom relief. Symptoms were scored in the RE as OSDI of 74 out of 100 and a VAS score of pain of 85 mm out of 100. In the left eye (LE) the symptoms were defined by the patients as light and acceptable: OSDI score was 22 out of 100 and VAS 21 mm out of 100. Slit lamp evaluation had not shown epithelial damage, and there were no signs of inflammation, but only a reduced Tear Film Break-Up Time (TFBUT) of 4 seconds in RE and 8 seconds in LE had been recorded. A therapy with hyaluronic acid (HA) based tear substitute to be administered 4 times/day in both eyes was prescribed.
In March 2016, the patient was proposed to receive in her RE a treatment with topical CBS, as a compassionate unconventional therapy, with the aim to reduce the pain symptoms for which the previous therapeutic attempts had turned to be unsuccessful. The therapy for the contralateral LE was maintained with HA based tear substitutes.
The patient signed the specifically designed informed consent and started administration of the CBS eye drops in April 2016, with the posology of 0.4 ml (8 drops) in RE, each day for a total of two months. In the GF dosages determined for the two CBS lots were administered during the first and second month.
On September 2016, the patient reported a significant relief from subjective symptoms of discomfort; in RE the OSDI was 28 out of 100, with VAS: 32 mm out of 100. Also in LE a reduction was observed, with OSDI score determined as 16 out of 100 and VAS 15 mm out of 100. During this visit, also the IOP was measured, which resulted in the normal range in both eyes and the analyses of the visual field tests performed since 2006.
In the mean deviation (MD) values recorded over several visits performed from December 2006 through September 2016 were graphed. A significant progressive lowering in MD values was observed which was followed by an important amelioration in correspondence with the period of treatment with CBS, in the figure highlighted with the arrow. It has to be noted that the increase in MD values was recorded either in the treated right eye or in the untreated left eye. Moreover, the MD values recorded in September 2016, four months after the end of the CBS eye drop administration, showed in both eyes a further amelioration.
The improvement is also demonstrated by the analysis of the PSD shown in . A progressive worsening in PSD values had been observed over ten years, whereas a rapid change was recorded in correspondence with the CBS eye drop treatment, either in the treated right eye or in the untreated left eye.
In the central 30-2 visual field tests before ((a): right eye RE; (b): left eye LE), at the end (c, d) and after four months (e, f) from the end of the CBS eye drop treatment were shown. An amelioration of the defect was observed in all the four quadrants in both the treated RE and the untreated LE.
Taking together these observations, a positive effect also in the left untreated eye could be recorded, which suggests a neural cross-talk mechanism between the eyes. |
pmc-6083606-1 | A 51-year-old man with no past medical history presented to the emergency room with pressure-like chest pain of two-day duration. He had multiple episodes of pain, and each lasted for around 20 minutes. It was associated with palpitations and exacerbated by physical exertion. He was a former smoker and reportedly quit smoking 20 years back. His blood pressure was 153/95 mmHg, temperature 98.1°F, heart rate 73/min regular, and respiratory rate 18/min. Electrocardiogram (ECG) showed Type 1 Wellens' Biphasic pattern in leads V2 and V3. ().
Three sets of cardiac troponins were normal. Wellens' pattern on ECG is highly specific (89%) for critical left anterior descending artery (LAD) stenosis [, ]. Therefore, the patient was sent for emergent cardiac catheterization. Coronary angiography revealed normal left main, left circumflex, and right coronary artery. However, LAD artery showed moderate myocardial bridging. |
pmc-6083641-1 | A 41-year-old Hispanic male (BMI: 44.6) presented to the emergency department after seven days of severe, novel, left-sided rectal pain. The patient denied fever, nausea, vomiting, bowel changes, or signs of blood in his stool. Though a proper rectal exam could not be performed due to pain, a 6 mm mass with surrounding erythema was noticed adjacent to the rectum in the 4 o'clock position. A diagnosis of perirectal abscess was made and incision and drainage were performed. One week after the procedure, the patient described a “ripping” sensation during a large bowel movement that led to worsening of his rectal pain. He was referred to a colorectal surgeon for presumed anal fissure, but confirmatory rectal exam was not possible due to physical discomfort. A subsequently scheduled rectal exam under general anesthesia was cancelled by the patient, and he was lost to follow-up.
During an unrelated consult for weight-loss surgery three months later, the bariatric surgeon discovered that the patient had new onset pruritus ani for nearly one month. Evidence of anorectal pain, hemorrhoids, fissures, or fistulas were absent at this time. Patient was prescribed lidocaine 5% topical ointment for two weeks PRN. Four months later, at the patient's request, the bariatric surgeon rechecked for the possibility of an anal fissure. Between these office visits, patient continued to have rectal pain (though of diminishing severity), bright red blood on toilet paper, pruritus ani, blood in his semen, loose stools, and the onset of outright FI. Patient attempted self-treatment of his FI with stool-bulking agents, fiber, psyllium, lidocaine cream, and Sween Cream, but with no relief. As these conservative measures failed, it was determined that anorectal manometry was warranted, but this test showed no abnormalities. Both the physician and patient decided that DxHA injections were the next best option. One week later, 4 X 1 mL injections of DxHA were administered approximately 5 mm above the dentate line at the posterior, anterior, left lateral, and right lateral positions without complications.
One month following DxHA injections, the patient underwent a previously scheduled screening colonoscopy because of a significant family history of colon cancer in his mother (diagnosed at 58 years; died at 62 years). A 5 mm sessile serrated polyp in the ascending colon and a 3 mm hyperplastic polyp in the sigmoid colon were removed during this procedure, but it was also noted by the gastroenterologist that a 15 mm benign-appearing submucosal lesion was present in the distal rectum (~5 cm from the anus). Unsure of what this lesion could be, a flexible sigmoidoscopy and endoscopic ultrasound were ordered to be performed two weeks later. On flexible sigmoidoscopy, the bulging submucosal lesion was again noted ~6-7 cm from the anal verge () and endoscopic ultrasound showed this mass to be a homogenous, hypoechoic lesion (0.79 cm X 2.98 cm) that was contiguous with the muscularis propria. Fine-needle aspiration of the rectal lesion was performed and sent to pathology.
Under microscopy, the hospital pathologist described the rectal FNA samples as having clusters of reactive macrophages, acute inflammation, giant cells, mucin, and multiple, spherically shaped dark microparticles (Figures –). Though noted as clearly foreign by the pathologist, the presence and etiology of these particles were perplexing. After calling the gastroenterologist to describe these findings, additional history attained by the gastroenterologist from the patient revealed the recent history of DxHA injections. Thus, it was surmised that the rectal lesion and corresponding histopathology were both a result of the patient's FI treatment.
Unfortunately, though there was initial improvement with the DxHA injections, at the two-month follow-up appointment the patient described worsened FI (several episodes daily, especially after bowel movements). As of this report, supportive measures, biofeedback training, and topical ointments were being used to treat the patient's incontinence. |
pmc-6083644-1 | A 29-year-old secundigravida Caucasian woman at 19+5 weeks of gestation was referred to our Fetal Medicine Centre to perform a level II ultrasound scan because of positive serology for syphilis in the first trimester. No maternal clinical manifestations of disease was found on examination. She had had a history of a feverish erythematosus maculopapular rush localized to trunk, limbs, palms, and soles two years before. The diagnosis of syphilis was performed only during antenatal screening in the first trimester by a positive venereal disease research laboratory (VDRL) and a treponema pallidum hemagglutination assay (TPHA) title of 1:2560. The HIV status of the woman was negative. Antibiotic therapy was started immediately with benzathine penicillin G 7.2 million units total, administered as 3 doses of 2.4 million units IM each at 1-week intervals according to the stage of syphilis, in line with CDC guidelines. At the end of the treatment the serology tests were stable with VDRL positive, a TPHA title of 1:2560.
The serology for syphilis also resulted positive in her husband (VDRL positive with TPHA title of 1:320) who was treated with a recommended regimen.
The scan performed at 19+5 weeks of gestation in our centre revealed no abnormalities, the amniotic fluid was found regular, and fetal growth parameters were normal.
At 23+5 weeks of gestation a follow-up scan was performed and a massive hydrocephalus (Figures and ), severe hydrothorax, ascites, and hepatomegaly were found. The measurement of the middle cerebral artery (MCA) was performed and an increased peak systolic velocity for gestational age was found. Careful counseling with the couple was performed after the ultrasound finding of the fetal anomaly. Maternal blood tests for cytomegalovirus, coxsackievirus, parvovirus, toxoplasma, and herpes virus types 1 and 2 were found negative while syphilis serologic tests were confirmed positive with RPR title 1:4 and TPHA title 1:640. Fetal karyotype was normal. Further counseling was made and the woman decided to pursue the termination of pregnancy. Fetal autopsy showed a cutaneous erythematous papular disseminated rash, widespread edema (stronger in the limbs), ascites, hydrothorax, internal obstructed hydrocephalus, ischemic-haemorrhagic brain injury with disseminated lesions, productive pachymeningitis with dystrophic calcification, hepatomegaly, acute hepatic and splenic hematopoiesis, productive bilateral peripyelitis, pronounced glomerulus-poiesis, and involution of the thymus. The research of Treponema Pallidum with coloring of Warthin-Starry identified images compatible with the morphological appearance of the germ. The diagnostic conclusion deposed for congenital syphilis. |
pmc-6083646-1 | A 46-year-old nulliparous woman presented after having experienced pain in the right side of her groin and labia majora for four months, as well as a lump that was increasing in size. She had previously undergone three laparotomies for ovarian cystectomy at 20, 10, and 8 years prior to this visit due to her severe progressive pelvic pain. The pathological examinations of surgical specimens obtained from all three operations indicated ovarian endometriosis.
Three years prior to this visit, she experienced progressive pelvic pain and she also noticed a growing mass at the right labia majora. She underwent total abdominal hysterectomy (TAH) with bilateral salpingooophorectomy (BSO) and excision of a 3.7 × 2.5 cm labial mass. Pathological examination revealed adenomyosis and multiple leiomyomas in the uterus as well as endometriotic cysts in both ovaries. The labial mass contained focal atypical endometriosis on a background of benign endometriosis (Figures and ). The patient had an uneventful recovery. However, she did not return to follow-up after this operation. She had no history of hormonal replacement therapy (HRT).
One month before this visit, she had undergone an incision biopsy of her labial mass at the provincial hospital, and the pathological report indicted only apocrine hidrocystoma. Nevertheless, the mass had increased in the size with accompanying progressive pain.
Upon presentation at our hospital, there was a 7x4-cm hard mass at the right labia majora extending to the right groin area. The mass seemed to be in continuity with the pubic symphysis that would require pubic bone excision and reconstruction with flap surgery to achieve complete resection. However, the patient refused such extensive surgery. Based on the previous pathological diagnosis of endometriosis of the vulva in this patient, she was then initially treated with two doses of GnRH agonist followed by one 150 mg dose of depot medroxyprogesterone acetate. These medications were administered with the aim of shrinking the bulk of the mass. However, the mass developed into an ulcer and rapidly increased in size (). A second biopsy of the mass was then performed. The pathologic examination showed a papillary growth structure lined by round tumor cells with clear cytoplasm and pleomorphic nucleoli (). shows residual atypical endometriosis that had merged with the tumor. The patient's tumor exhibited focal cytoplasmic staining for napsin A. A diagnosis of clear cell adenocarcinoma of the vulva with coexisting atypical endometriosis was made based on these pathological findings.
Computed tomography of the abdominopelvic region showed an ulcerative mass at the right labia majora and nodal metastasis at the bilateral external iliac and superficial inguinal regions that varied in size from 2.0 to 3.5 cm. Other CT findings were unremarkable.
As this case was considered inoperable, systemic chemotherapy consisting of paclitaxel and carboplatin was administered. The growth of the tumors stabilized during the first two cycles of chemotherapy but rapidly progressed thereafter. Palliative treatment under multidisciplinary care was subsequently initiated. Pelvic radiation was administered to slow down the progression of disease and to alleviate pelvic pain. At 17 months after her initial presentation, the patient passed away due to the progression of the disease and severe sepsis. |
pmc-6083649-1 | A 77-year-old female with hypertension, hyperlipidemia, and paroxysmal atrial fibrillation (AF) presented with palpitations. She has neither prior cardiovascular procedure nor cardiac surgery. AF was diagnosed 4 years ago, and she was started on oral anticoagulation with warfarin. She was active and independent. She was very symptomatic with frequent palpitations. Dofetilide was started for rhythm control. She was successfully cardioverted in January 2017 while on dofetilide and warfarin. However, she went back to AF in March 2017. Dofetilide dose was adjusted. Then, she converted to sinus rhythm and always reported to be compliant with the medication. In May 2017, she had palpitations and was found to be in AF again. Given the fact that symptomatic AF is affecting her daily life and she failed rhythm control therapy, ablation was offered. She underwent cryoablation and pulmonary vein isolation procedure.
Computed tomography angiogram of the pulmonary veins (Figures –) and transesophageal echocardiogram (Figures and ) were ordered prior to the procedure as evaluations for the cardiac anatomy and to rule out intracardiac thrombi, which revealed she had no left atrial appendage. Anticoagulation therapy with warfarin was continued as per current guidelines since we have no data on anticoagulation management in CALAA. |
pmc-6083738-1 | A 74-year-old male patient visited the emergency department of our hospital for tenderness and swelling of the left knee. He had a past history of hypertension, spinal stenosis, benign prostate hypertrophy, and unruptured aneurysm of the right carotid artery. He also had visited an orthopedic surgery clinic due to an old fracture of the right tibial tuberosity six weeks previously. Initial physical examination found tenderness and crepitus of the left knee. Radiographs of both knee joints confirmed the diagnosis of supracondylar fracture of the left distal femur. His initial vital signs were as follows: blood pressure 164/81 mmHg; heart rate: 66 beats per minute; respiratory rate: 18/min; and body temperature: 36.0°C.
The results of an initial complete blood cell count were within the normal range: white blood cell count 8.66x109/L with 70.3% neutrophils, hemoglobin 14.1 g/dL, hematocrit 40.7%, and platelet count 173x109/L. Serum blood urea nitrogen and creatinine were 15.2 mg/dL and 1.07 mg/dL, respectively. In addition, serum calcium and alkaline phosphatase levels were elevated at 13.9 mg/dL and 152 U/L, respectively; inorganic phosphorus and serum albumin levels were within the normal ranges (4.34 mg/dL and 3.9 g/dL, respectively). Thyroid function test was also normal. The patient underwent a successful closed reduction and retrograde intramedullary nailing of the fractured joint the following day. However, he soon began to complain of general weakness, nausea, vomiting, and anorexia. His serum creatinine level was increased to 2.37 mg/dL on postoperative day 7, while the hemoglobin level decreased to 8.2 g/dL on postoperative day 9. On postoperative day 10, the patient's right distal femur fractured when rising from a wheelchair. Whole-body radionuclide bone scan with technetium-99 found multiple focal activities at bilateral femoral shafts, both knee joints, and left tibia (data not shown).
He was referred to our endocrinology department for evaluation of hypercalcemia. A serum assay of intact parathyroid hormone (iPTH) was 5.24 pg/mL (range: 15-65), and the serum 25(OH)D level was 22.33 ng/mL. In addition, a workup for anemia was concurrently carried out: serum iron of 77 μg/dL (range: 65-175), total iron binding capacity of 296 μg/dL (range: 250-425), ferritin of 761.91 ng/mL (range: 22-322), and serum lactate dehydrogenase of 258 U/L (range: <290 U/L). The peripheral blood smear was unremarkable, except for moderate toxic changes in neutrophils. His reticulocyte index was 0.6%, which implied inadequate red blood cell production despite the presence of anemia. Also, β2-microglobulin was elevated to 7.33 mg/L (range: 0.81-2.19). Due to the combination of persistent hypercalcemia, renal failure, anemia, and bone lesions, serum and urine electrophoresis were performed to rule out the possibility of multiple myeloma, both of which were negative. However, serum PTH-related peptide (PTHrP) assay showed a level elevated to 4.0 pmol/L (range: <1.1).
The patient underwent a complete diagnostic workup to find any hidden malignancy. Serum tumor biomarkers were all within normal ranges (carcinoembryonic antigen: < 2 ng/mL, cancer antigen 19-9: 7.3 U/mL, alpha-fetoprotein: 3.71 ng/mL, and prostate specific antigen: 0.17 ng/mL). A thin-slice (1 mm) chest CT and abdominopelvic CT scans were unremarkable (data not shown). A torso positron emission tomography-computed tomography (PET-CT) scan also showed no abnormal FDG uptake (). To exclude hematologic malignancy, a bone marrow biopsy at the left iliac crest was performed. The bone marrow smear revealed several large atypical lymphocytes with a high nucleus-to-cytoplasm ratio and prominent nucleoli. Initial chromosomal karyotyping revealed both aneuploidy and multiple deletions and translocations across a number of chromosomes, including 73 chromosomes, XXY, del(1)(p22), and (q21;q26.2). Pathology of the bone marrow confirmed the presence of atypical lymphocytes that stained positive for the CD20 marker, which is consistent with B-cell lymphoma (BCL) involving the bone marrow. Immunophenotyping of bone marrow cells further supported the diagnosis of BCL, with the population testing positive for CD45, CD19, CD20, CD79a, and HLA-DR (Figures and ); however, terminal deoxynucleotidyl transferase expression in bone marrow cells was negative. This case was confirmed to be a rare case of primary bone marrow DLBCL. |
pmc-6084490-1 | Mr. BH, 39 years old, right-handed, presented with a Bennett fracture of the first right metacarpal following a work accident. The fracture was initially treated in another institution by reduction and percutaneous pinning using Iselin’s technique. Three weeks later, the patient developed an inflammatory reaction around the entry point of the distal pin, at the second metacarpal. A purulent discharge led to early removal of the distal pin. The isolated organism was a methicillin-sensitive Staphylococcus aureus. Regular topical treatment was given, and antibiotic therapy was initiated for five weeks based on laboratory results. Bone healing of the Bennett fracture was obtained in the sixth week after the accident, and the proximal pin was removed.
Three months later, the patient was referred to us by his doctor because of persistent pain at the second right metacarpal, accompanied by attacks of inflammation and intermittent purulent discharge. Standard hand X-ray demonstrated the presence of an area of osteolysis surrounded by osteocondensation at the second metacarpal, indicating a focus of osteomyelitis along the path of the distal pin (). An MRI was performed on the hand. It confirmed the diagnosis, revealing the extent of the focus of osteomyelitis, the path of the fistula, and the inflammatory extension into neighboring soft tissues ().
Surgical treatment called for the induced membrane technique.
The first stage included a segmental resection of the area of osteomyelitis through a dorsal approach with excision of the reformed tissue while preserving the index finger extensor tendon.
The second metacarpal was stabilized with a mini external fixator, and a cement spacer was interposed ().
Antibiotic therapy was initiated for six weeks based on sensitivity testing. The external fixator was then removed, and the hand temporarily immobilized with a splint until there was healing along the path of the inserts.
The second stage was performed in the eighth week. The cement spacer was removed and the biological chamber was filled with a spongy bone graft taken from the ipsilateral iliac crest. Internal fixation was performed with a mini plate while preserving the induced membrane. The postoperative period was uneventful. Bone healing was observed three months later () with complete disappearance of pain. The patient has regained normal use of the hand with a Quick DASH score of 11 and complete mobility (. We are currently at the 36-month follow-up. X-ray of the hand shows incorporation of the graft and evidence of corticalization (). |
pmc-6084498-1 | Case 1: A 58-year-old right-hand dominant female underwent the first carpometacarpal arthrodesis using two cannulated compression headless screws. Postoperatively, strong pinch movement was prohibited for 4 weeks, and range of motion exercises were undertaken. There was persistent pain in the fixed joint from 6 weeks postoperatively; hence, a thumb spica cast was applied for immobilization. However, the pain was still present at 6 months postoperatively, and radiography showed nonunion and loosening around the screws. The patient was treated with a vascularized bone graft from the second metacarpal base and plate fixation ( and ). The length of surgery was about 90 minutes. Radiographs showed bone union at 6 weeks postoperatively, and the Kapandji score was 7. Tip pinch and side pinch were restored to 92% of the healthy side, and the VAS and DASH scores were improved (). The patient returned to unrestricted daily activity at 3 months after the second surgery. |
pmc-6084498-2 | Case 2: A 78-year-old right-hand dominant female had a 7-year history of left thumb pain at night. Physical examination and radiography revealed osteoarthritis of the TMC joint. Radiographs showed degenerative arthritis (Eaton classification stage III). Following failure of conservative treatment, the patient underwent arthrodesis using a vascularized second metacarpal base bone graft and two crossed cannulated compression screws (). The length of surgery was about 60 minutes. Radiography showed successful bone union at 6 weeks postoperatively, and the Kapandji score was 7. Tip pinch and side pinch were restored to 92% of the healthy side, and the VAS and DASH scores were improved (). At 6 weeks postoperatively, the patient could perform all activities of daily living without restriction. |
pmc-6084507-1 | A 76-year-old woman presented to our clinic due to fluctuating diplopia, worse at the end of the day while watching television, which gradually developed for 8 months prior to presentation. She complained about binocular vision in primary gaze position, in vertical and right lateral gaze directions. Her medical history included breast cancer surgically resected without recurrence, smoldering multiple myeloma, bilateral cataract and osteoporosis.
Before presenting to us, she was evaluated as an outpatient.
Neurological examination showed right lateral rectus palsy causing an esotropia with gaze paretic nystagmus, moderate not fatigable ptosis and slight adduction deficit in the left eye. Pupils were round, equal and reactive to light. Vision was preserved on gross examination (the patient did not complain any vision loss) and confrontation visual field test resulted normal. Ophthalmological examination showed bilateral visual acuity deficit due to cataracts (20/63 in the right eye and 20/50 in the left eye), normal ocular pressure and normal fundus examination. The orthoptic measurements revealed an abduction deficit in right eye, a slight adduction deficit in left eye and a bilateral elevation deficit, greater in the left eye. The patient presented a 35 prism diopter esotropia in primary gaze. The strength of facial muscles, including orbicular oculi, was normal and Cogan's lid twitch sign was negative. The rest of physical examination was unremarkable.
Lab test included a complete cell blood count, liver, renal and thyroid function test, and returned normal.
Due to the clinical history of fluctuating symptoms, a screening for myasthenia gravis was undertaken: serum assays for acetylcholine receptor (AChR) antibodies and muscle-specific tyrosine kinase (MuSK) antibodies tested negative, electromyography (EMG) of the facial nerves with repetitive supramaximal stimulation and single-fiber EMG gave normal results.
When she presented to our medical attention she was admitted in our department and a brain MRI was undertaken. Axial T1 and T2 weighted images and MRA demonstrated large bilateral CCAs, with a larger multilobulated aneurysmal sac on the right, along with fetal origin of posterior cerebral arteries and small basilar artery (Figure ). A CT angiography of brain vessels confirmed the presence of bilateral large CCAs, measuring approximately 21 × 17 × 16 mm on the right and 18 × 15 × 16 mm on the left (Figure ).
Surgical resection and endovascular occlusion were held at this time due to high procedural risk in an elderly frail patient.
Based on the hypothesis that cranial nerve involvement in this patient was probably due, at least in part, to thrombosis-related edema and inflammation, an empirical steroid therapy with oral prednisone 1 mg/kg/day was started, followed by slow tapering over the course of 2 months.
MR and CT angiographies performed 4 months later showed no change in the bilateral CCAs compared to previous scans (Figures ). A follow-up examination showed a severe evolution of the neurological involvement: the patient had complete sixth nerve palsy in the right eye causing convergent strabismus with compensatory head rotation toward the right side, severe ptosis and complete ophthalmoplegia in the left eye. In addition, her left pupil was mydriatic, without light response. Although the size of the aneurysms was unchanged, the patient also started experiencing left periorbital headache.
Because of this progressive deterioration of the clinical conditions, intervention could no longer be delayed. Therefore, the patient underwent endovascular embolization of the left carotid aneurysm with flow diversion. She was treated with dual antiplatelet therapy for 1 month, followed by single antiplatelet drug for other 3 months. At discharge, ptosis in the left eye improved, but complete palsy of oculomotor muscles was still present. |
pmc-6084563-1 | A 56-year-old non-smoker male, engineer for a gas company, presented in early January 2017 with right chest pain and a 1.56 L (29.4%) loss in forced vital capacity (FVC) over the previous 3 years. The anamnestic investigation for professional exposure including asbestos resulted negative. The physical examination revealed the reduction of lung sounds at the right lung basis while no clinical sign of autoimmune disease was found. A chest X-ray (CXR) was performed and showed a considerable reduction in the right lung volume with associated right pleural effusion (Fig. ). All serological, autoimmune and microbiological tests resulted negative, so he underwent plain and contrast enhanced computed tomography (CT) that revealed a slight reduction in right lung volume with associated right pleural effusion and pleural thickening (Fig. ). A supplemental investigation was conducted excluding history of trauma, tuberculosis and pneumothorax. Echocardiography excluded pulmonary hypertension. Diaphragm dysfunction was also investigated through phrenic nerve stimulation, but with a negative result. On March 2017 a total body positron emission tomography–computed tomography (PET-CT) with 18-FluoroDeoxyGlucose (18-FDG) was performed and revealed a low intensity hyper-accumulation of 18-FDG in the right pleura with increased concentration in the basal, middle and posterior pleural surface. Thus, a surgical lung biopsy (SLB) was carried, identifying a non-specific fibrinous pleurisy (Fig. ). The search for neoplastic or infectious cells resulted negative. PFTs performed 1 month later revealed further important drop in FVC (2.5 L loss). The patient developed shortness of breath on exertion, therefore, a prednisolone course (0.25 mg/kg daily) was started. However, PFTs persistently declined with the onset of restrictive respiratory failure. The patient died 2 weeks later for cardiac ischemic attack. Autopsy excluded mesothelioma or other pleural neoplastic diseases, but reported the presence of diffuse fibrinous pleurisy with collagen deposition. |
pmc-6084687-1 | A 73-year-old male with a past medical history of diabetes, hypertension, and dyslipidemia presented with a chief complaint of visual change for one day in his left eye. The patient reported that he was at home the previous night when he suddenly experienced loss of vision in the left eye. The patient also reported sharp, 5/10, non-radiating left eye pain during the onset of vision loss which has now resolved. He denies trauma to the eye. He also denies fever, headache, eye discharge, ear pain, nasal congestion, nausea, vomiting, diarrhea, paresthesias, or focal weakness. There is no past medical history of contact lens use, eye surgery, or glaucoma.
An examination of extraocular motility revealed full motility in the left and right eyes. The pupils were equal, round, and reactive bilaterally. The visual field testing was normal in the right eye whereas the left eye showed decreased vision in the nasal visual field. Visual acuity was 20/50 in the left eye and 20/20 in the right eye. Intraocular pressure, measured with a Tono-Pen, was 19 mmHg in the left eye and 23 mmHg in the right eye. There was no fluorescein uptake in either eye. A fundoscopic examination was performed after the application of two drops of tropicamide (0.5%) in both eyes. A funduscopic examination of the left eye revealed a black spot at 5 o’clock.
An ultrasound examination of the eyes was performed using a linear array 13-6 MHz ultrasound transducer. An occlusive dressing (Tegaderm) was placed on top of the eye to shield it from the gel. Ultrasound gel was applied to the transducer. The probe was placed in a transverse orientation to scan the axial anatomic plane. A scan of the left eye showed a hyperechoic smooth folded membrane within the vitreous, consistent with a retinal detachment (Figure ).
. The membrane can be seen "floating" on video imaging (Video ).
The ophthalmologist on call was consulted, and we were instructed to send the patient to the clinic for follow-up. The patient was discharged with follow-up instructions. The patient saw the ophthalmologist, who referred him to a retinal specialist. Retinal repair surgery was performed on the left eye two days after the emergency department (ED) visit, and the patient reports that he is healing well. |
pmc-6084690-1 | The patient is a 33-year-old Caucasian female, who was brought to the local emergency room by the police. The police were repeatedly called by the patient about rapes and shootings in her community. On the day she was brought to the hospital, the patient called the police under a fake name and complained that her husband was raping another individual. She was making nonsensical comments, including being ritualistically haunted by her father and sister. The patient was found to be unmarried and lived alone but was adamant about being married to a celebrity. She had no significant psychiatric history prior to this incident. After acknowledging that she was a certified shaman and practices healing through the utilization of the kambô ritual, she claimed that she uses the kambô toxin to alleviate her chronic pain. Her frequency of performing the ritual changed from once per month to up to nine times per month. She presented with characteristics of paranoia, anxiety, bizarre delusions, labile mood, and panic attacks. On physical examination, scars were noted on the patient’s legs from the burns and administration of the toxin. She subsequently had an unremarkable extensive medical workup. As part of her treatment plan, the patient was started on risperidone and she gradually improved after nine days in the hospital psychiatry unit. |
pmc-6084697-1 | A 20-year-old male patient, with type 1 diabetes mellitus of 15 years duration, on twice daily premixed insulin and poor glycemic control (glycated hemoglobin of 10.8%), presented to us with evidence of advanced microvascular disease. He had bilateral proliferative diabetic retinopathy (PDR), distal symmetrical sensorimotor polyneuropathy (DSSN), autonomic neuropathy and nephrotic range proteinuria with new onset hypertension without azotemia. He had high-risk bilateral foot with the presence of hammer toes and hallux valgus but without any active foot ulceration. Hand examination revealed fixed flexion deformity at proximal interphalangeal joints with associated tightening of the skin and cord-like induration on palms at the level of metacarpophalangeal joints (Figures -).
Tinel’s sign and Phalen’s test (for carpal tunnel syndrome) were negative and there was no evidence of trigger finger. Furthermore, there was no finding suggestive of adhesive capsulitis of the shoulders. He denied any history of joint pain, swelling, early morning stiffness, Raynaud’s phenomenon or local trauma. The patient, however, did not have any functional limitation associated with the hand deformities as he could inject insulin, use computer keyboard and carry out all his routine work without any difficulty. Radiographs of hands and feet were negative for any neuroarthropathy or inflammatory arthropathy; immunological markers were also negative. In the background of chronic longstanding diabetes with poor glycemic control and advanced microvascular complication, diagnosis of diabetic hand syndrome was entertained.
The patient was shifted to multiple subcutaneous insulin injection (basal and bolus regimen) for better glycemic control and blood pressure control achieved using renin-angiotensin-aldosterone (RAAS) blockade combined with calcium channel blocker. For PDR, the patient underwent the first session of pan-retinal photocoagulation (PRP). He was provided with customized footwear and educated regarding foot care for the high-risk feet. For hand deformity, hand surgeon was consulted, who advised for conservative management in the form of stretching exercises and braces in view of minimal functional disability and guarded results of the surgery. |
pmc-6085214-1 | A 65-year-old woman presented with abdominal pain and hypophagia for 2 weeks. She had a medical history of alcoholic pancreatitis. A computed tomography (CT) scan of her abdomen and pelvis revealed thickening of the transverse colon wall. Colonoscopy showed an ulcerated tumor in the transverse colon, and histological analysis indicated moderately differentiated adenocarcinoma. Serum carcinoembryonic antigen (CEA) levels were normal, and no sites of distant metastasis were reported on preoperative examination. She underwent a laparoscopic extended right hemicolectomy using the non-touch isolation technique. Histopathological examination of the specimen revealed a moderately differentiated adenocarcinoma invading the subserosal layer with low venous invasion (v1) and no lymphatic invasion (ly0). The surgical margins were negative. A total of 92 lymph nodes were removed, of which none showed metastases. The tumor was diagnosed as stage IIA (T3, N0, M0) according to the International Union Against Cancer tumor, node, and metastasis (TNM) classification (7th edition) []. The patient had an uneventful recovery and was on regular follow-up every 3 months without adjuvant chemotherapy.
Fifteen months after her colectomy, a CT scan demonstrated a tumor in the antero-lateral rectal wall that was 20 mm in diameter, and a right ovarian tumor that was 25 mm in diameter (Fig. ). The ovarian tumor was a cystic mass with a solid component on magnetic resonance imaging (Fig. ). Colonoscopy revealed a superficial elevated lesion in the middle rectum that was shown by histological examination to be moderately differentiated adenocarcinoma (Fig. ). Serum CEA levels were normal. These findings were indicative of rectal cancer with ovarian metastasis, or double primary cancer of the rectum and ovary. A diagnostic and therapeutic laparoscopy detected no disseminated peritoneal metastases or liver metastases. Intraoperative cytological examination of the peritoneal lavage was negative for carcinoma. An operative rapid pathological diagnosis of the resected right ovarian tumor indicated metastasis of the colorectal cancer. Thus, we performed laparoscopic low anterior resection preserving the left colic artery with partial resection of the vagina, and bilateral adnexectomy.
The resected rectal tumor measured 20 × 18 mm (Fig. ). Histological examination demonstrated a moderately differentiated adenocarcinoma that had invaded the vagina and formed a metastasis in one of 32 lymph nodes. High venous invasion (v3) and low lymphatic invasion (ly1) were observed. The adenocarcinoma was mainly present in the submucosa and muscularis propria with a small range of invasion to the vagina (Fig. ), while the carcinoma-involved region of the mucosal layer had mucosal colonization representing the spread of metastatic tumor cells along the basement membrane of preexisting crypts and/or villi (Fig. ) []. There was no adenomatous precursor at the edge of the tumor, and surgical margins were negative. The right ovarian tumor was moderately differentiated adenocarcinoma that was positive for cytokeratin (CK) 20 and negative for CK7 immunohistochemical staining, indicating metastasis of colorectal cancer. The rectal and ovarian tumors shared high similarities with transverse colon cancer in architectural and cytological atypia (Fig. ). Both adenocarcinomas of the transverse colon and rectum were negative for p53 immunohistochemical staining and RAS wild type in genetic assessment (Fig. ). These findings support a diagnosis of rectal and ovarian metastases from primary transverse colon cancer.
The patient recovered well after surgery, and adjuvant chemotherapy was decided after a multidisciplinary meeting. She was treated with eight cycles of oxaliplatin and capecitabine, and neither relapse nor metastasis has been observed 18 months after surgery.
The incidence of colorectal metastasis from primary colorectal cancer is rare, and distinguishing primary from metastatic colorectal cancer can be challenging. To our knowledge, only three reports in English and six in Japanese have described suspected cases of colorectal metastasis of colorectal cancer [–]. We reviewed a total of eight patients, including seven out of the abovementioned nine patients with detailed clinical information as well as our own (Table ). The patients were three males and five females, with a median age of 66.5 years (range 52–88 years). Ascending colon cancer was the most common primary tumor (n = 3), followed by tumors of the sigmoid (n = 2), transverse colon (n = 2), and cecum (n = 1). Metastatic colorectal lesions were located in the rectum in all cases. In terms of other metastatic sites, seven patients had other metastases (lung, liver, spleen, abdominal wall, axilla, and ovary) and one had no other metastases.
A previous study of 278,208 malignancies in a nationwide Japanese pathologic autopsy database from 1990 to 2003 identified 18,252 case with metastatic colorectal cancer. Of these, 1302 (7.1%) were from primary colorectal cancer []. The database does not include information about the suspected metastatic pathway (hematogenous, lymphogenous, direct invasion, or dissemination), but most metastatic tumors in the database are attributed to direct invasion or disseminated metastasis, reflecting late-stage disease. However, the number of reported clinical cases might not represent the actual incidence of colorectal metastasis from colorectal cancer. One possible reason for the rarity of reported colorectal metastasis cases is that most cases occur as part of systemic advanced disease, for which surgical resection will not be performed []. The other reason is that diagnosing metastasis is difficult [, ]. Although gastrointestinal metastatic carcinoma usually represents a submucosal tumor, differential diagnosis (as primary disease or metastasis) becomes difficult if the tumor invades the mucosal layer. Estrella et al. argued that metastatic carcinomas involving the mucosal surface frequently mimic second primaries, so histologic features cannot reliably distinguish metastatic from primary carcinoma []. Additionally, primary colorectal cancers resembling submucosal tumors have been reported in previous studies [, ]. This explains why colorectal metastases may be misdiagnosed and treated as primaries.
Distinguishing primary from metastatic colorectal cancer can be challenging, but a comprehensive evaluation of histological features, clinical history, and tumor distribution enables making the correct diagnosis and implementing the optimal treatments []. In this case, we suspected a metachronous metastasis for the following reasons. First, histological findings demonstrated that the adenocarcinoma was mainly present in the submucosa and muscularis propria with mucosal colonization, which is a supportive finding for metastatic carcinoma, as this indicates a low possibility of primary rectal cancer and implantation of the transverse colon cancer. Second, the rectal and ovarian tumors were very similar to the transverse colon cancer in terms of architectural and cytological atypia, further suggesting metastasis of primary transverse colon cancer. Third, there was no adenomatous precursor at the tumor edge, indicating metastatic cancer. Fourth, both adenocarcinomas of the transverse colon and rectum were negative for p53 in immunohistochemical staining and RAS wild type in genetic assessment, indicating metastatic rectal cancer from primary transverse colon cancer.
In reviewed cases, the exact mechanism of colorectal metastasis from primary colorectal cancer has not been fully elucidated; thus, the possibility of hematogenous or lymphogenous spread remains. We suspected hematogenous or lymphogenous metastatic pathways to the rectum from the primary transverse colon cancer because the carcinoma was mainly located in submucosa and muscularis propria; however, there was no additional evidence than tumor localization. In this case, the rectal tumor penetrated into a small range of the subserosal layer with invasion to the vagina; additionally, there was a metastatic ovarian carcinoma. Peritoneal metastatic invasion primarily toward the deep layer of the rectal wall cannot be ruled out. The metastatic colorectal lesions were located in the rectum in all reviewed cases, and this suggests that peritoneal spread to Douglas’s pouch or the rectovesical pouch is a possible pathway for colorectal metastasis from primary colorectal cancer.
Considering that the majority of reported cases had multiple metastases, the existence of other metastatic lesions might be a risk factor for colorectal metastasis. Therefore, more attention should be paid to colorectal lesions when other metastatic sites have been identified. Colorectal metastases usually represent late-stage disease and have poor prognoses; however, prolonged survival after surgery and complementary therapy can be achieved in some patients []. In this case, neither relapse nor metastasis has been observed 18 months after surgery. The follow-up periods varied too widely to evaluate patient prognosis in reviewed cases. Therefore, additional studies are needed to better understand this rare metastasis and to determine the optimal therapeutic strategies. |
pmc-6085532-1 | A 36-year-old woman, gravida 1, para 1, with a history of laparoscopic fulguration of pelvic endometriosis 3 years previously was referred to our institution because of dysmenorrhea and monthly right-sided shoulder pain associated with menses. She was using oral contraceptive pills (OCP) without pain relief. Deep pelvic endometriosis with endometriotic involvement of the diaphragm was suspected and abdominopelvic and diaphragmatic magnetic resonance imaging (MRI) was performed. MRI showed deep pelvic endometriosis involving the bladder and the uterosacral ligaments, and multiple posterior subphrenic lesions suggestive of diaphragmatic endometriosis. We performed a multidisciplinary team laparoscopy (gynecologic and thoracic surgeons) and found deep pelvic endometriosis and extensive endometriosis involving the right posterior hemidiaphragm (only visible with the 30º optic and after liver mobilization). Laparoscopic partial cystectomy, uterosacral ligament resection and a full-thickness partial diaphragmatic resection were performed without complications; a chest drain was left in place for 2 days and she was discharged on postoperative day 5. Histopathology confirmed endometriosis in all specimens (). To date, she is using OCP and remains asymptomatic after 38 months’ follow-up. |
pmc-6085532-2 | A 42-year-old nulliparous woman with a history of infertility and recurrent catamenial pneumothorax (2 previous episodes, the last one 6 months earlier) was referred to our emergency department due to right-sided chest pain and mild dyspnea, which started within 48 hours of onset of menses. She had no previous history of endometriosis and never had dysmenorrhea or dyspareunia. The initial examination included chest X-ray, which revealed a right pneumothorax. A chest computed tomography (CT) scan confirmed the diagnosis and VATS was performed. During VATS, we found several diaphragmatic fenestrations that communicated with the abdominal cavity, through which the liver had herniated. The involved area was resected and the diaphragm was repaired using a nonabsorbable interrupted suture (). Pathologic report confirmed diaphragmatic endometriosis. The patient underwent in vitro fertilization (IVF) 4 months after surgery and became pregnant. She is now at 20-weeks of a normal pregnancy and remains asymptomatic. |
pmc-6085532-3 | A nulliparous, 26-year-old woman, with a known diagnosis of pelvic endometriosis and medical treatment with continuous OCP for 9 years was referred to our institution due to recurrent pelvic pain, severe dysmenorrhea, and dyspareunia. Moreover, she presented with chronic right shoulder pain, which was exacerbated during menstruation. She had a history of one previous laparoscopy for endometriosis in which both pelvic and diaphragmatic endometriosis were discovered, but the latter was not treated. The biopsy confirmed endometriosis in all pelvic samples. After the surgery she was treated with gonadotrophin-releasing hormone (GnRH) analogues for six months with transitory improvement, but she could not receive more OCP due to de discovery of a hepatic adenoma. Due to persistent and incapacitating catamenial right shoulder pain accompanied by severe dyspnea, a chest CT was performed with only nonspecific findings. She underwent an exploratory VATS and we found several endometriotic foci in the central tendon of the diaphragm and right hemidiaphragm, which were fulgurated and resected. The pathology report was consistent with fibrosis but not with endometriosis. However, it also reported a marked thermal effect on the tissue. Four months after surgery she conceived spontaneously and delivered a healthy newborn at 38 weeks of gestation. She is currently under treatment with an implantable contraceptive and reports great improvement after a 7-year follow-up. |
pmc-6085532-4 | A 35-year-old nulliparous woman, was referred to our unit with a long history of infertility and chronic pelvic pain. She also had severe dysmenorrhea and dyspareunia, but reported no thoracic symptoms. She underwent gynecologic laparoscopy, and deep pelvic endometriosis in the uterosacral ligaments was resected; several endometriotic lesions in the right hemidiaphragm were left behind and not treated, due the lack of symptoms. The pathology report confirmed endometriosis in all pelvic specimens. Fourteen months after surgery, she underwent three cycles of intrauterine insemination and became pregnant. She underwent emergency cesarean section at 30 weeks of gestation due to placental abruption with good perinatal outcome. She remains asymptomatic after 55 months’ follow-up. |
pmc-6085532-5 | A 40-year-old nulliparous woman, with history of four previous surgeries for endometriosis, persistent dysmenorrhea, and infertility was evaluated in our emergency department due to epigastric and left flank pain, dyspepsia, and nausea. Abdominopelvic CT revealed a left diaphragmatic hernia, with the splenic flexure of the colon herniated into the chest and signs of severe pelvic endometriosis. Chest CT confirmed the diagnosis and the absence of pneumothorax. She had no history of diaphragmatic surgery, trauma or any pulmonary disease. Moreover, she had undergone chest CT one year earlier due to a deep venous thrombosis, which revealed no diaphragmatic defects. VATS was performed; the edges of the diaphragmatic hernia were resected and the diaphragm was repaired using a direct suture. A pathologic examination of the resected tissue confirmed endometriosis. She remains asymptomatic after 26 months’ follow-up. |
pmc-6085604-1 | A 51-year-old male was referred to the Department of Periodontics, College of Dentistry, Qassim University (Buraydah, Saudi Arabia) to extract the non-restorable tooth #45 and to evaluate the site #45 and #46 for the placement of implants.
The patient had hypercholesterolemia and was taking 20 mg Lipitor (atorvastatin) tablets once daily. Dental history revealed that his lower right first molar was extracted 11 years ago due to caries.
A cone beam CT scan was taken to evaluate the ridge width and height and the location of vital structures (
and
). The radiographic examination revealed deformity of the ridge at site #46 (Siebert class 1). After a discussion with his referring dentist, it was decided to extract tooth #45. A free gingival graft was planned to increase the width of keratinized tissue at site #46 which was followed by ridge augmentation after waiting at least 6 weeks to allow soft tissue healing. The treatment plan was explained to the patient, and written informed consent was acquired.
Tooth #45 was extracted and soft tissue healing was completed about 6 weeks later.
6 weeks after the extraction of tooth #45, a free gingival graft was performed to increase the width of keratinized tissue prior to ridge augmentation.
At 8 weeks after the free gingival graft procedure (
), ridge augmentation was performed using a titanium-reinforced non-resorbable polytetrafluoroethylene PTFE membrane and FDBA. Local anesthesia with 2% lidocaine and 1:100,000 epinephrine was used to anesthetize the surgical area. A full-thickness mid-crestal incision was made on the edentulous area using a sulcular extension to the distal aspect of tooth #47 and to the distal aspect of tooth #42. A vertical incision was made at the disto-buccal line angle of tooth #42. The flaps were elevated to expose the atrophic ridge (
). Flap advancement adjacent to the mental foramen area was conducted after a full-thickness mucoperiosteal flap was elevated beyond the mucogingival junction by pushing back the flap using wet gauze until the mental nerve was located (
). An evident horizontal bone defect was found.
Periosteal scoring was performed to release the buccal flap, allowing for coronal advancement of the flap. Decortication was performed in the buccal bone with a round bur (
).
A titanium-reinforced non-resorbable PTFE membrane (Cytoplast™ Barrier Membranes Ti-250) was stabilized to the buccal plate at the apical end using membrane tacks (Salvin, USA) then FDBA (OraGRAFT®, USA) was placed beneath the membrane and packed gently (
), then the membrane coronal part was stabilized with two tacks. Flaps were sutured with 4-0 non-resorbable PTFE sutures (Cytoplast™ Sutures, Osteogenics Biomedical) (
) Postoperative instructions (about diet, pain, bleeding and healing) and medications, including 600 mg ibuprofen three times a day for 5 days, 875 mg amoxicillin twice daily for one week and 0.12% chlorohexidine mouth wash twice a day for 2 weeks, were given to the patient.
At the 2-week follow up point, the surgical site was healing well, with no sign of infection (
).
At the 4-week follow up point, clinical examination revealed that there was post-operative exposure of the membrane. The size of the exposure was approximately 4 × 8 mm (
and
). The sutures were removed and it was decided to manage the exposure surgically by making two small vertical incisions and positioning the tissue coronally to cover the membrane. Non-resorbable sutures 4-0 (Cytoplast™ PTFE) were used (
). Patient was instructed to use chlorhexidine mouthwash and weekly recall to monitor the surgical site.
At the 5-week follow up point, 1 week after our surgical attempt to cover the membrane exposure, the size of exposure had increased (
). Sutures were removed and the outer surface of the membrane was cleaned with cotton swap dipped in chlorohexidine. The patient was instructed to use cotton swab dipped in chlorohexidine to clean the exposed membrane.
At the 6-week follow-up point, 2 weeks after the membrane covering procedure, intraoral examination revealed pus discharge between the membrane and the tissue (
). The membrane had to be removed owing to the infection. An incision was made to split them membrane from the tissue then the membrane was removed (
). After removing the membrane, the underlying tissue had a red, jelly-like appearance, with no bone -graft remnants observed in the surgical site. The flap was sutured using resorbable sutures (
). The patient was instructed to take 1 g Augmentin twice daily for 1 week and to use 0.12% chlorhexidine mouth wash twice a day for 2 weeks.
At 8 weeks after the horizontal ridge augmentation, the surgical site was healing well with no sign of infection (
).
At 5 months after the guided bone regeneration (GBR) procedure, a cone-beam CT scan was performed to evaluate the bone width. The bone width was 9 mm, meaning that the bone width gain after ridge augmentation was 6 mm. two implants were successfully placed at site #46 and #45 (
and
). |
pmc-6085645-1 | The patient is a 69-year-old woman who presented to her primary care physician with worsening cough and pain wrapping around her chest to her back. The chest pain was initially thought to be pleuritic in nature and related to an exacerbation of her chronic obstructive pulmonary disease (COPD). When there was no improvement in her symptoms with treatment of her COPD, and considering the confounding radicular distribution of her pain, a plan was made to have an MRI of the spine performed to explore other possible etiologies of her pain.
Prior to the scheduled MRI, the patient was seen in the emergency department and diagnosed with a myocardial infarction due to hypertensive emergency. During her admission to the hospital, the patient was noted to be experiencing neck pain, bilateral upper extremity pain in a C8-T1 dermatomal distribution and a loss of urinary and fecal urges. On examination, the patient’s force of flexion and abduction in her upper extremities were mildly reduced. The strength in her lower extremities was decreased. Her deep tendon reflexes were 3+ in her bilateral upper extremities and 3+ in her bilateral lower extremities with clonus at the ankles.
She had bilateral extensor plantar responses. In light of these progressive myelopathic symptoms a spinal MRI was done.
The initial pre and post -contrast MRI of the entire spine showed extensive, mostly brightly enhancing intradural extramedullary nodules extending from C5–6 to T11 (Fig. ). Some nodules at the T6 level were only minimally enhancing, with pre-contrast T1 hyperintensity apparent. The lesions were causing multilevel severe central canal stenosis with multilevel cervical and thoracic spinal cord compressions. The largest mass conglomeration extended from C6 to T4, measuring up to 1.2 cm AP × 1.9 cm transverse × 10.0 cm CC. Despite the extensive mass effect with flattening of the spinal cord, the spinal cord demonstrated no intramedullary T2 hyperintensity except for a small focus of enhancing T2 hyperintensity at the posterior columns at the T7-T8 level that could have represented a small area of invasive change or perhaps focal indentation by a focal nodular component of the large extramedullary mass.
The multifocal spinal masses were suspicious for leptomeningeal carcinomatosis and imaging was ordered to look for a primary tumor source. An MRI of the brain showed no tumor although there was a small nonspecific non-enhancing extra-axial nodule in the left side prepontine cistern at the level of the upper pons. The patient’s computed tomography (CT) of the chest, abdomen and pelvis showed no tumor, and two CSF specimens were also negative for neoplastic cells. A follow-up MRI of the spine was performed ten days later to determine the patient’s response to steroid therapy and this showed no significant change. Multiple spinal meningiomas were also considered due to the patient’s age, the intradural extramedullary location and the homogeneous enhancement seen on MRI.
Of note, the patient did not have a history of neurofibromatosis or any other neurocutaneous disorders. The patient also did not have a past medical history of tumors and was never previously treated with chemotherapy or radiation. The patient does have a history of polio which she recalled contracting at about two years of age. The patient’s mother was diagnosed with a fast-growing brain tumor in her 80s but otherwise the patient’s family history was unremarkable.
An initial T7-T8 laminectomy for partial excision of the tumor was done for tissue diagnosis. Once the lesion was determined to be a pilocytic astrocytoma, a decision was made to proceed with an extensive resection of the tumor. The residual tumor was removed in two stages. In the first surgery a multilevel laminectomy was done from T4-T10. After a midline dural opening was made, tumor was identified which was light silver in color and attached to the spinal cord (Fig. ). A careful microsurgical dissection was done to separate the tumor from the spinal cord. There was an identifiable plane between tumor and the cord which facilitated safe removal of the tumor, with the exception of certain areas where the tumor seemed to be more adherent and possibly invaded the pial covering of the spinal cord. At these locations the tumor was shaved off the spinal cord leaving only a very thin layer of tumor. Later a C4-T3 laminectomy was done for the second resection and removal of the remaining tumor.
Routine hematoxylin and eosin–stained sections of the tumor samples showed multiple well-circumscribed masses made up predominantly of interlacing fascicles of spindle cells with thin wavy nuclei. The degree of cellularity varied greatly with some nodules exhibiting highly cellular compact areas and others showing more loosely arranged cells. Perivascular pseudorosettes were prominent in some areas, giving an ependymoma-like appearance. Foci of dense calcifications and microcystic degeneration were identified. Mitoses were elevated (up to 8 per 10 high power fields in the most cellular areas), nuclei were hyperchromatic, and the proliferation index was high in those areas signifying the presence of anaplastic features. The tumor cells were strongly and diffusely immunoreactive for S100, GFAP, and Olig2, confirming their glial nature. They were negative for EMA and progesterone receptors (meningioma markers) as well as CK18 (usually positive in ependymomas). SOX10, a marker of peripheral nerve sheath tumors, was also negative. The diagnosis of pilocytic astrocytoma with focal anaplastic features was rendered (Fig. ).
The patient presented for a physician visit a month after the final surgical procedure. She was experiencing paresthesias in all four extremities, in addition to thoracic radicular type numbness bilaterally at T5. The patient was participating in physical therapy and had persistent bilateral lower extremity weakness.
Radiotherapy with concurrent temozolomide chemotherapy was given to treat the residual tumor. The patient was treated with large field radiation to the spinal cord, receiving a total dose of 36 Gy in 20 fractions with daily dose concurrent temozolomide. Maintenance temozolomide was started, with a plan to treat the patient for six additional cycles. At the time of this writing it has been 13 months since her symptoms first began and six months since her final surgery. |
pmc-6085650-1 | A 39-month-old Sri Lankan Sinhalese boy from a poor socio-economic background presented to the pediatric ward with circulatory collapse. He did not have fever, history of infection, or other identifiable focus of sepsis. He was born to non-consanguineous parents at 38 weeks of gestation with a birth weight of 2.5 kg and had an uncomplicated perinatal period. In early infancy he had problems of poor feeding, episodes of loose stools, and failure to thrive despite nutritional supplementation and had chronic constipation during the past 2 years. On further inquiry his mother described several episodes of unexplained drowsiness at times of minor infections which settled without interventions except for an episode 6 months previously which was associated with hypoglycemia that required a dextrose infusion.
Anthropometric measurements revealed: weight 6.9 kg (well below third percentile), height 76 cm (below third percentile), and occipitofrontal circumference 42 cm (below third percentile). He had dysmorphic features which included microcephaly, bitemporal narrowing, upward slanting eyes, epicanthal folds, partial ptosis, broad nasal bridge, low set posteriorly rotated ears, high arched palate, and short neck. Anterior fontanelle was still open. Marked hyperpigmentation was noted in his perioral, buccal, and palmar areas (Fig. ). His fingers and toes did not show any abnormalities and his genitalia were normal. He had tachycardia, low volume pulse, and his systolic blood pressure was recorded as 50 mmHg. There were no abdominal masses or genital abnormalities. Hypotonia with reduced power (4/5) was noted in all muscle groups. Tendon reflexes and examination of eyes were normal. A development assessment by examining his developmental milestones revealed global developmental delay with a developmental age between 15 and 18 months.
His blood glucose (30 mg/dL) and serum bicarbonate (20 mmol/L) levels were low and serum electrolytes revealed hyponatremia (sodium 120 mmol/L) with hyperkalemia (potassium 7.1 mmol/L). Serum osmolality was 275 mosm/L. Renal and liver function tests and serum calcium, magnesium, and phosphate levels were normal. Blood and urine cultures were sterile and C-reactive protein level was within normal range. Serum spot cortisol level on admission was 133 nmol/L (normal 120–626) and 17-hydroxy progesterone level was 1.171 ng/dL (reference 3–90) suggesting failure of initial steps of steroid hormone synthesis. Serum cholesterol was normal (153 mg/dL). His condition did not permit us to perform an ACTH stimulation test before commencement of treatment. Ultrasound scans of his abdomen and brain, echocardiography, and electroencephalography were normal. His karyotype was 46XY. Genetic studies and 7-dehydrocholesterol (7DHC) levels were not performed due to unavailability.
Acute adrenal crisis was diagnosed based on characteristic biochemical abnormalities and SLOS was considered as the possible etiology based on dysmorphic features. The child was resuscitated with two 0.9% sodium chloride 20 ml/kg fluid boluses and hypoglycemia was corrected using 10% dextrose 3 ml/kg bolus. This was followed by intravenous 5% dextrose in 0.9% sodium chloride fluid infusion. Intravenously administered hydrocortisone 50 mg single dose was followed by 12.5 mg dose every 6 hours for the next 24 hours and he was started on intravenously administered cefotaxime. He showed a marked response to hydrocortisone with normalization of hyponatremia and hyperkalemia which did not require other specific treatment. After 24 hours, orally administered hydrocortisone 10 mg/m2 per 24 hours in three divided doses and orally administered fludrocortisone 0.1 mg daily were commenced. His parents were asked to give foods high in cholesterol (for example, eggs). Follow-up was arranged in the pediatric clinic with monthly measurements of weight, height, blood pressure, and serum electrolyte levels. |
pmc-6085819-1 | A 24-year-old man (height: 169 cm, weight: 44 kg, blood pressure: 168/75 mmHg) presented with paroxysmal disturbance of consciousness accompanied by muscle spasms. He was diagnosed with cerebral haemorrhage. He had experienced two previous cerebral haemorrhages between the ages of 23 and 24 years. He was an only child, and his parents did not have any history of haemorrhage or abnormal skin appearance or other medical histories. He was referred to the endocrinology department with multiple intracranial calcifications, acute intracerebral haemorrhage, and several other unusual features. His features were as follows: beaked nose (Figure ), “bird” face, light, and sparse scalp and body hair, no obvious armpit hair or eyebrows, exophthalmos (Figure ), hoarse voice, lipoatrophy, skin pigmentation (Figures ), severe abdominal aortic, and peripheral artery disease, cerebral haemorrhage (Figures ), and erectile dysfunction.
An initial brain computed tomography (CT) scan showed that the right occipital lobe was haemorrhagic with approximately 1.5 ml (Figure ). CT angiography revealed plaque formation in, and vascular calcification of, the aortic arch, bilateral subclavian artery, brachiocephalic trunk, proximal internal carotid artery, aorta abdominalis, and arteria iliaca communis (Figures ). Intracranial calcification was also revealed on CT (Figure ). Vascular ultrasonography showed atherosclerosis and plaque formation in the intracranial vessels and bilateral carotid and posterior tibial arteries. Doppler ultrasonography showed mitral calcification (Figure ). Bone density scans revealed osteopenia (T level−1.8SD); plain skull x-ray imaging also showed decreased bone density. The patient's blood count results were as follows: white blood cells, 15.77 × 10∧9/L; neutrophils, 73.9%;triglycerides (TG) 1.76 mmol/L; cholesterol (CHOL), 6.24 mmol/L; low density lipoprotein cholesterol (LDLc), 5.61 mmol/L; high-sensitivity C-reactive protein (hsCRP), 87.26 mg/L; myocardial enzyme lactate dehydrogenase (LDH), 267.12 U/L; creatine kinase (CK), 899.52 U/L; MB isoenzyme of creatine kinase (CK-MB), 24.34 U/L.
Once-daily atorvastatin (20 mg) was prescribed; however, another large cerebral haemorrhage developed 8 months post-discharge (Figures ). Although the patient accepted minimally invasive intracranial haematoma surgery, paralysis persisted.
After obtaining informed consent from both the patient and his parents, genetic analysis was carried out. We sequenced the WRN and LMNA (GenBank reference sequence NM_170707.2) coding sequences in the patient and his parents. The results showed a missense mutation within exon 5 of LMNA (c.898G>C) that caused a substitution of aspartate 300 by histidine (p.Asp300His). There were no WRN mutations (Figure ). The patient was the only child of his parents. Both parents had a normal appearance, and both carried the wild-type LMNA sequence without any symptoms of AWS, cardiovascular disease, or abnormal medical history. This disease is closely linked to mutations in the lamin A/C, or LMNA, gene, which confirmed the diagnosis of AWS. |
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