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A 22-year-old Caucasian female without any particular medical history suffered from a traumatic fracture of the left humerus great tuberosity in 2002. She underwent a non-operative treatment with a Mayo-Clinic splint and was discharged from the hospital. Radiologic outcome wasunremarkable, though she complained with persistent shoulder pain. Three years later, a painful mass of the shoulder rapidly appeared, impairing shoulder mobility. She was referred to our institution. Clinical examination revealed a hard, bulky mass of the shoulder, with collateral circulation. The tumor involved the whole curving contour of the shoulder, and reached the axilla at its posterior limit. Mobilization of the arm was almost impossible due to both pain and stiffness ().A shoulder MRI revealed a 12 × 6 cm irregular, heterogeneous mass of the posterior part of the shoulder (). The tumor invaded subcutaneous tissue as well as the deltoid and infrasupinatous muscles. Necrotic areas diagnosed as T1-hyposignal were found, as well as other tumor areas that were strongly enhanced by contrast injection. There was no apparent bone destruction. A surgical biopsy was performed, diagnosing aggressive fibromatosis (extraabdominal desmoids tumor).\nDue to the tumor burden, curative surgery could not be performed, and a medical therapy consisting in imatinib mesylate (Glivec) was started at a daily dose of 400 mg. After 4 weeks of treatment, the patient noticed an improvement in the abduction of the arm as well as a slight tumor size decrease. The treatment was continued at the same dosing, with consistent improvement in mobility, tumor measurements and analgesics consumption. There was no significant side-effects. The treatment was finally stopped at month 34, in the setting of tumor regression, complete recovery of arm mobility and function and complete discontinuation of analgesics (). Control MRI of the shoulder confirmed the very good partial response (). At a follow-up of 42 months (1 year off-therapy), the patient remains tumor and symptoms-free, without any tumor re-growth.\nIn the light of such a dramatic response to imatinib, c-kit exon 10 was fully sequenced from the tumor frozen samples. 50 ng of genomic DNA was amplified by PCR in 50 μl reaction volume containing 2,5 U AmpliTaq Gold DNA polymerase (Applied Biosystems, Forster city, CA), 0,2 mM dNTP, 1,5 mM MgCl2, and 0,2 μm of the forward and reverse primers 5′-ATCCCATCCTGCCAAAGTT-3′ and 5′-CTGTGGGGAGAAAGGGAAA-3′, respectively, flanking exon 10. PCR products were verified by electrophoresis, showing a 246 bp amplified fragment, purified by using Microcon-PCR Filter Unit (Millipore, Paris, France) and directly sequenced with the Big Dye Terminator v1.1 Cycle sequencing kit (Applied Biosystems, Forster city, CA). finally the PCR product was analyzed on ABI PRISM 3100 Genetic Analyser (Applied Biosystems, Forster city, CA). The sequences were aligned with the GB sequence of human CKIT (locus HSU63834) using the software Seqscape v2.5 (Applied Biosystems, Forster city, CA). All sequencing reactions were performed in both forward and reverse directions, and the mutation was confirmed by a second sequencing on an independent PCR, revealing a V530I mutation in the transmembrane domain of c-kit (). |
A 69-year-old female presented with a 2-year history of a slowly enlarging right buttock mass. Her past medical history was unremarkable, except for hyperlipidemia. Clinical examination revealed a large, non-tender right buttock mass. The remainder of clinical examination was unremarkable. Computed tomography (CT) scan showed a mass measuring 16 × 15 × 14 cm with imaging features suggestive of a liposarcoma []. Full blood count, biochemical investigations, and tumor markers were all within the normal range. The patient underwent a complete resection of the mass. Histological examination showed a myxoid liposarcoma with clear surgical margins. Staging investigations including CT of the chest and abdomen were unremarkable. Since an excision with clear surgical margins had been performed, the patient was not given any adjuvant treatment.\nEighteen months later, the patient was readmitted complaining of abdominal fullness and dull abdominal pain. Physical examination revealed a large, non-tender mass occupying the left abdomen and a cutaneous mass on the upper inner left thigh. CT scan of the abdomen revealed a large retroperitoneal tumor measuring 22 × 12.2 × 17.5 cm with imaging features suggestive of a liposarcoma. In addition, a smaller tumor measuring 7 × 5 cm was detected in the ileal mesentery close to the ileocecal valve []. At exploratory laparotomy, the retroperitoneal tumor was resected with clear margins. Segmental ileal resection was performed along with sigmoid resection, because a second intraperitoneal tumor was found in the sigmoid mesentery. Wide resection was also performed of the subcutaneous left thigh tumor. Histological examination of all specimens revealed a myxoid liposarcoma with 10-15% round cell component. In addition, the histology of the subcutaneous thigh lesion revealed the presence of tumor emboli within small veins. The patient was given postoperative adjuvant chemotherapy with 6 courses of ifosfamide 5 g/m2 and doxorubicin 50 mg/m2. Follow-up investigations after completion of chemotherapy were negative for recurrent disease. However, 2 years later, on CT scans, multiple intraperitoneal and left retroperitoneal masses were detected despite the excellent physical condition of the patient. At this point, the multidisciplinary team suggested chemotherapy with 3 courses of ifosfamide 6 g/m2, followed by 6 courses of doxorubicin 25 mg/m2. Despite initial stabilization, progression of the disease was noted and the patient was started on new chemotherapy with 6 courses of liposomal doxorubicin with simultaneous administration of granulocyte colony-stimulating factor and erythropoietin. Unfortunately, although the size of the lesions was initially stabilized, the disease progressed again. Finally, on May 2011, a new chemotherapy regimen consisting of trabectedin was started. After the administration of 6 courses, a significant decrease of the lesions’ size was noted, and, thus, it was decided to administer 3 more courses. Presently, the patient remains in a stable partial remission of the disease and is scheduled for new investigations. |
A 43-year-old man with a history of chronic alcoholism presented with abdominal distension. The previous day, the patient had presented to a local hospital with anal bleeding and abdominal pain after an incidental insertion of barbecue skewer per anus in the drunken state; subsequently, he had undergone sigmoid loop colostomy for rectal perforation. However, after the operation, the patient had become hemodynamically unstable. At presentation, his systolic blood pressure was 90 mmHg and the pulse rate was 135 beats/min. Although there was no gross rectal bleeding, the digital rectal examination revealed a penny-sized anterior rectal wall defect 6 cm from the anal verge (AV). Computed tomography (CT) revealed a hematoma (12 × 10 × 15 cm) with active bleeding in the pelvic cavity and a pseudoaneurysm in the anterior wall of the rectum (). Since the patient was hemodynamically unstable, an emergency operation was performed. During the operation, a massive subperitoneal hematoma in the rectovesical pouch and large amount of blood in the peritoneal cavity were found. After evacuation of the hematoma and blood, oozing continued in the rectovesical pouch (). Thus, compression with gauze was performed for 30 min until the oozing stopped. The Hartmann procedure was performed with the suspected bleeding focus included, but the perforation site was not included.\nAlthough the postoperative course was uneventful and there was no evidence of recurrent bleeding on the follow-up CT on the 7th postoperative day (POD), a focal enhancing lesion in the anterior wall of the rectum indicating a residual pseudoaneurysm was noted (). On the 11th day POD, his hemoglobin decreased from 11.6 g/dL to 7.9 g/dL, and the follow-up CT revealed recurrent hematoma (6.0 × 4.2 cm) in the pelvic cavity and the residual pseudoaneurysm (). Following the diagnosis of recurrent bleeding from the residual pseudoaneurysm, an angiography was performed. However, the angiography failed to localize the pseudoaneurysm, and definite signs of extravasation could not be ascertained. Thus, prophylactic gelfoam embolization at the anterior branch of both the internal iliac arteries was performed (). The subsequent hospital course was uneventful, and the patient was discharged on the 25th POD. After 3 months, the previous rectal lesion (AV: 6 cm) healed, and colostomy reversal was performed without morbidity. |
We report the case of a 30-year-old male patient who was treated initially in another facility for SS of the left thigh. He underwent wide excision with free margins. The tumor measured 3 cm in its greatest diameter. Anatomopathological examination showed: a monophasic spindle cell synovial sarcoma in a fascicular arrangement. Immunohistochemistry showed positivity for bcl2, cytokeratin, epithelial membrane antigen (EMA), CK, and negativity for chromogranin A, synaptophysin, and vimentin. About 6 months later, he was admitted to our institute for a local recurrence confirmed by biopsy. A thoracic-abdominopelvic computed tomography (CT) scan was performed and didn’t reveal any secondary locations. The patient underwent a wide excision, with a vascular replacement of the femoral artery and coverage with a flap of the left rectus abdominis muscle. The following operations were straightforward. The Surgical limits were free. The search for the SYT-SXX fusion transcript was not completed. The patient underwent six cycles of ifosfamide-based chemotherapy followed by adjuvant external radiotherapy at a dose of 54 Gy. After 15 months of regular clinical and radiological follow-up, the patient presented a pancreatic mass diagnosed on a CT scan. The mass was located on the tail of the pancreas. It was heterogeneous, solid, enhanced after the injection of the contrast agent. Tumor markers: ACE, CA19-9, and NSE were negative. A biopsy under CT scan was performed and the histologic exam confirmed the metastatic nature of the pancreatic mass. The patient was reluctant to surgical treatment, and he was lost of view for 4 months. Due to the onset of transfixing abdominal pain and the increase in abdominal volume, the patient consulted after 4 months. An abdominal ultrasound showed an increase in the size of the lesion to 15 cm. The thoracic-abdominopelvic CT scan was performed and it showed a pancreatic mass invading the transverse colon without any other metastatic localizations (). The patient underwent a laparotomy. Upon exploration, a mass of the tail and body of the pancreas was discovered invading the transverse colon. There was no ascites or carcinosis. The rest of the abdominal cavity was free of sarcomatosis (). The procedure consisted of a caudal splenopancreatectomy with partial resection of the transverse colon.\nPathological examination showed a pancreatic localization of the previously diagnosed monophasic synovial sarcoma with free surgical margins ( and ). He underwent four cycles of first-line metastatic chemotherapy with high-dose ifosfamide and doxorubicin. The patient developed an endocrine pancreatic insufficiency requiring insulin therapy. The patient died 6 months later due to a diabetic coma. |
A 61-year-old man presented with a one-week history of pain in the right TMJ area, especially during eating, and a restricted range of jaw motion. The patient’s previous medical/dental history was unremarkable. No history of parafunction was present, and the patient had not experienced any TMJ pain prior to the presently reported experience. An examination revealed no deviation in the opening path and no noise in the TMJ.\nThe maximum unassisted opening distance was 40 mm. The patient reported pain upon palpation of the right TMJ (lateral pole, inside ear), but had no extraoral or intraoral muscle tenderness. Twenty-eight teeth were present, and the occlusion was anatomically normal. TMJ tomography showed no evidence of degeneration (Fig. ), and a diagnosis of temporomandibular arthralgia was made based on the Research Diagnostic Criteria for TMD (RDC/TMD) []. Since an anterior flat plane bite plate has been recommended as a provisional appliance to decrease painful symptoms [], an anterior flat plane bite plate covering the upper six anterior teeth and the first premolar teeth on both sides was fabricated for short term use using self-curing acrylic resin (GC, Tokyo, Japan). The occlusal surface of the plate was made flat and perpendicular to the mandibular incisors to allow free movement in all directions (Fig. ). Medication was not prescribed.\nThe patient was instructed to wear the plate during the day, except when eating or speaking. This appliance therapy was continued until the symptoms were alleviated; meanwhile, the HOP and BPOP were measured as follows. The HOP (first) and BPOP (second) were recorded over time. The HOP, obtained by voluntary jaw closing while the patient was seated in an upright position with the occlusal plane parallel to the floor, was assumed to be the mandibular position imposed by the motor program of the central nervous system, and was defined as the stable (intercuspal) position. The BPOP, attained during voluntary jaw closing while the patient was seated in an upright position after the patient had worn an anterior flat plane bite plate for five minutes, was assumed to be the muscular position achieved by altering or deprogramming the motor program. This procedure to obtain the muscular position was established in a previous study []. To record the HOP, a vinyl polysiloxane bite registration material (GC, Tokyo, Japan) was applied over the occlusal surfaces with a syringe and the patient was asked to swallow and then close his mouth to maximum intercuspation, then hold that position until the material set (approximately one minute). To standardize the BPOP recording method, the patient was conditioned neuromuscularly using an anterior flat-plane bite plate, against which the patient tapped and slid his anterior lower teeth for a period of five minutes. After conditioning, the bite plate was removed and the registration material was applied over the occlusal surface; the patient was then asked to close his mouth until the upper and lower teeth just came into contact with each other and to hold that position. Three inter-occlusal recordings were made for each of the occlusal positions at each visit. A dentist who was not involved in the recording and who was unaware of the patient’s status performed the following measurements and analysis. The trimmed intraoral records were interposed between the cast on the three-dimensional apparatus of a modified articulator (Fig. ), and occlusal papers of different colors were interposed between the recording surfaces and the recording needles, marking the positions. All registrations were read under a measuring microscope (Pika Seiko, Tokyo Japan) with a resolution of 0.01 mm. The statistical significance of the differences between the HOP and BPOP measurements recorded on each day and between two HOP and two BPOP recordings made on different days were calculated using an analysis of variance (ANOVA) for a two-factor experiment with repeated measurements for both position factors. The statistical significance of the differences in the variations of the two positions recorded on two different days was also calculated using an ANOVA. Significance levels of p<0.05, p<0.01, and p0.005 were established. In addition, the maximum unassisted opening and the pain score on a 10-point Visual Analogue Scale (VAS), where 0 denoted “no pain” and 10 denoted “worst pain” were recorded over time. The changes in the symptoms, the discrepancy between the HOP and BPOP values, and the variations in the HOP and BPOP values are shown in Figs. and . On day one, the difference between the HOP and the BPOP was not statistically significant (Fig. ). On day 3, however, the difference between the HOP and the BPOP at this time-point was significant (p<0.005). The difference between B1 and B2 was also significant (p<0.05), while that between H1 and H2 was not (p>0.1). The mean differences between the HOP and BPOP were 0.18±0.14 mm (mediolateral), 0.71±0.68 mm (anteroposterior) and 0.45±0.34 mm (superoinferior). On day 15, the patient’s maximum unassisted opening increased to 45 mm, the VAS score remained zero, and the difference between H6 and B6 was significant (p<0.01), while that between B5 and B6 was not significant (p>0.1)(Fig. ). If this discrepancy had been left untreated, the symptoms would have recurred. Therefore, for ethical reasons, occlusal equilibration was performed for the BPOP after obtaining the patient’s informed consent. The discrepancy between the HOP and BPOP values was significant on day 25, and the patient reported cheek biting on his left side. However, the discrepancy disappeared by day 33, and the cheek biting ceased. The difference in the BPOP between the visits on day 3 and day 110 was not significant (p>0.1). The variations in the BPOP values measured on days 25, 33, 50 and 110 were significantly smaller than those measured on day 15 (p<0.01) (Fig. ). The HOP shifted anterolaterally to the left after occlusal adjustment. The patient’s symptoms (right TMJ pain while eating and limited jaw opening) did not recur during a two-year follow-up period. |
Patient CS, a single 60-year-old male presenting with a history of generalized anxiety with panic, major depressive disorder, and excessive guilt, was referred from a county hospital to a tertiary psychiatric facility for clarification of diagnosis and a more comprehensive assessment. His sister, and the family physician that had been following the patient for the past 4 years, helped provide collateral history. His family noted that he was born with a large head. He had a history of meningitis at the age of 9 or 10 after which it is thought that he developed a non-communicating hydrocephalus. His past psychiatric diagnoses included major depressive disorder, generalized anxiety disorder with panic, personality disorder, and “borderline intelligence.” He had several admissions to a psychiatric ward over the past 3 years for low mood and had been trialed on numerous psychotropic medications (citalopram, lithium carbonate, risperidone, olanzapine, quetiapine, paliperidone, clomipramine, clonazepam, lorazepam) with little effect or benefit. At the time of admission, he did not smoke, drink alcohol, or take illicit drugs. His past medical history was significant for hypothyroidism corrected with the use of thyroxine, bowel resections secondary to possible malignant changes, fatty liver with lobar resection secondary to liver cancer and nephrolithiasis.\nHe was born and raised in Europe until the age of 5, when he immigrated to Canada, and is bilingual. His family reported that he had always had a large head, micropenis, central obesity and short stature. He had a history of being bullied for “looking like a girl” and being different. At school his peers were physically aggressive, hitting him on his head. Born the youngest of seven siblings, he was raised by his parents and lived under their care into adulthood, until both parents passed away—his father had Diabetes Miletus and his mother had a brain tumor. Thereafter, he was taken care of by his sister. He had an older brother who also passed away secondary to a brain malignancy. One brother has dyslipidemia, and two sisters and one brother are healthy. He had no employment history and as a child had always struggled in school, completing a vocational stream of education until grade 10. Socially, he was active in a band for a few years (plays guitar well) and sang in a church choir. However, he never lived independently, and had no romantic relationships.\nInitial assessment revealed that he was a poor historian unable to give an accurate timeline of events. He often expressed fears that he was going to die. He suffered from delusions of guilt that he had caused the deaths of family members. His conversation was repetitive, he repeatedly asked the same questions and restated his fear of dying despite several reassurances. He had no history of self-harm or suicide attempts. On physical examination, he had a wide stance waddling gait, slow movements, limited arm swing and masked facies. He was noted to have enlarged head circumference (62.5 cm) and limited insight into his illness and the need for treatment. His clinical presentation prompted examination with magnetic resonance imagining (MRI) of the brain and formal neuropsychological testing.\nA sagittal T1, axial T2, axial T2 FLAIR and diffusion-weighted images were acquired throughout the brain. Findings indicated a long-standing overt ventriculomegaly, likely due to aqueductal stenosis, with bilateral gross dilation of the lateral and third ventricles, with a small aqueduct and fourth ventricle, with significant thinning of the corpus callosum and overlying cerebral cortex. Vascular flow-voids at the base of the brain were normal and there were no mass lesions, significant sulcal effacement, downward tonsillar herniation or restricted diffusion observed.\nManual segmentation of gray and white matter and cerebrospinal fluid (CSF; Figure ) of high-resolution T1 weighted MRI images was completed with Freeviewer in FSL (Jenkinson et al., ). Automatic segmentation of a comparison group of sex and age matched healthy controls (HCs; one aged 60, three aged 55 years, Table ) was completed with the FreeSurfer () recon tool. The participant’s volumes were converted to Z scores for comparison. Compared to similarly aged control participants, the patient had extremely large ventricular volume (821,452 mm3, Z = 161), reduced white (333,606 mm3, Z = −2.655) and gray (432,184 mm3, Z = −3.07) matter volume, and within normal range total intracranial volume (1,587,242 mm3, Z = 0.57) see Table and Figure .\nThe patient’s neurological exam was unremarkable.\nThe Wechsler Adult Intelligence Scale (WAIS-III; Wechsler, ) revealed a borderline IQ of 79, with a verbal IQ of 88, non-verbal performance IQ of 74, poor working memory IQ of 71, verbal comprehension IQ of 93, and visual-spatial IQ of 80. The patient had difficulty completing tasks requiring working memory, which was in the 3rd percentile, and processing speed was extremely slow (in the 1st percentile). Hopkins Auditory Verbal Learning Test (Brandt, ) indicated severe memory impairment, with initial memory for only a few items, no significant recall between administrations, and inability to recall any information after a brief delay. Rey-Osterrieth Complex Figure Task (Osterrieth, ; Rey et al., ) performance indicated impaired visual spatial and working memory abilities with more attention to small details, missing elements and less attention to the overall image. The Stroop test (Stroop, ) indicated impaired executive function, scoring below the 1st percentile, with a severe inability to suppress automatic responses. |
A 66-year-old female presented with a history of SCLC of right middle lung lobe (stage 1). She has undergone right middle lobe (RML) lobectomy followed by four cycles of adjuvant carboplatin and etoposide. Fourteen month later, she developed recurrence in her subcarinal/paratracheal nodes confirmed on endobronchial ultrasound (EBUS) biopsy. She then had concurrent chemotherapy with radiation completed followed by prophylactic cranial irradiation. Again she developed recurrent disease in celiac axis nodes confirmed on biopsy for which she was started on nivolumab. Ten months after starting nivolumab therapy, the patient presented to the office complaining of dizziness, imbalance, and double vision. Past medical history included chronic obstructive pulmonary disease (COPD), diabetes mellitus type 2, neuropathy, and myocardial infarction (MI). She was a former smoker with 75 PPD smoking history and had a family history significant of lung cancer.\nPhysical examination was positive for a significant vertical nystagmus. MRI of the brain in November, 2018 showed extensive symmetric linear hyperintensity in T2 Flair throughout medulla, dorsal pons, and midbrain with post contrast enhancement (Figure ).\nThe patient was started on oral steroids with a tapering dose. Nivolumab was held for six weeks and her symptoms improved over this period. Then she was given a re-challenge dose of nivolumab but her vision and balance worsened again. Again, she was started on oral steroids with a tapering dose and nivolumab was held. Blurry vision and imbalance were improved within a few days, but nystagmus gradually improved over five months. The patient remained off treatment with observation alone for nine months when MRI of the brain showed resolution of abnormal linear enhancement of the brain parenchyma (Figure ), however, follow up CT demonstrated focal area of consolidation in superior lobe of right lung and enlarged posterior pancreatic lymph node.\nThe positron emission tomography (PET) scan showed mild activity nodule in right upper lobe (RUL), pleural-based suspicious soft tissue mass, and PET positive nodule in peri-portal region. She was started on pembrolizumab and has had six doses so far. Most recent follow up PET/CT August 2020 showed no fluorodeoxyglucose (FDG) avid uptake. |
A 51-year-old male was diagnosed with muscle-invasive urothelial cell carcinoma in May 2015. Standard neoadjuvant chemotherapy was given followed by radical cystoprostatectomy. Final pathology showed pT3 N0 M0, stage III disease. He remained disease free until April 2016, at which point he developed new exertional dyspnea and a small lung nodule was noted on imaging. During cardiac clearance for a biopsy, a Mobitz 2 heart block with bradycardia was noted. Transthoracic echocardiogram (ECG) demonstrated a mass in the right ventricular outflow tract, which was additionally found to be fluorodeoxyglucose (FDG)-avid on positron emission tomography (PET) computed tomography (CT) (Figure , upper panel).\nThe patient developed progressive dyspnea with minimal exertion and then experienced a syncopal episode prompting hospitalization. Biopsy of the right ventricular mass demonstrated poorly differentiated carcinoma, consistent with urothelial origin (Figure ).\nThe mass was not felt to be respectable and chemotherapy was not felt to offer rapid disease control in the setting of progressive symptoms. He was offered palliative radiotherapy. His bradycardia progressed to a complete heart block, likely due to the growth of the mass, necessitating placement of a dual chamber pacemaker. The right ventricle mass was treated with 45 Gy in 18 fractions (3D conformal photons for 5 fractions, followed by intensity-modulated radiotherapy for the remaining 13 fractions (to reduce dose to the left ventricle). Figure demonstrates the dose distribution.\nProton radiation was considered to spare the uninvolved myocardium. He had substantial tumor thromboembolic disease to his lungs during radiotherapy, requiring medical intensive care unit (MICU) admission for symptom management. He ultimately completed the full course of radiotherapy which improved his functional status.\nFollowing radiotherapy, he began immune checkpoint therapy with the PD-L1 antagonist atezolizumab (1200 mg every three weeks), which was well tolerated. A PET/CT scan seven months after completing radiation demonstrated a complete metabolic response in the right ventricle mass and no other sites of progression (Figure , lower panel). The previously noted pulmonary nodules were stable. He resumed normal activity and returned to work. Eight months after radiotherapy and six months into his immune therapy, he developed progressive shortness of breath. He had heart failure and the ejection fraction reduced to 20%-30%. Cardiac magnetic resonance imaging (MRI) revealed a focal area of subendocardial delayed enhancement, potentially representing myocarditis. Cardiac catheterization revealed extensive coronary artery disease and complete occlusion of the right coronary artery; however, the left to right collaterals were intact. Atezolizumab was held with an initiation of high dose steroids to treat potential immune-mediated myocarditis with minimal effect. Electrophysiology testing demonstrated severe cardiac dyssynchrony. Cardiac resynchronization therapy was recommended and a biventricular pacer was placed with rapid symptom relief. He returned to work with overall minimal symptoms. Atezolizumab was resumed, and a total of 18 cycles (54 weeks) of therapy were completed without issue.\nIn the months that followed the placement of his biventricular pacer, he was evaluated several times for shortness of breath and atypical chest pain, however with no clear etiology. PET/CT imaging in August 2017 demonstrated increased FDG-avidity in his right ventricle concerning for progression. An MRI was ordered and in early September 2017, he was admitted for workup of an exacerbation of chest pain. A contrast-enhanced CT of the chest showed significant disease progression in the area of prior treatment, extension toward the left ventricle and along the heart wall leading to increased right heart pressure (Figure ), and probable encasement of his left anterior descending (LAD) artery by disease progression (Figure ). He had acute progression of chest pain associated with an increased oxygen requirement, shortly after being admitted. His ECG showing ST elevations in the anterior leads, consistent with an LAD infarct (Figure ). The cardiology service was consulted to consider palliative LAD stenting; however, the patient underwent cardiac arrest in the interim and did not wish to be resuscitated. He died 17 months after his disease recurrence. |
A 70-year-old female with a past medical history of stage IV NSCLC with metastatic disease to the bones and liver presented with lower extremity edema. She reported 2 weeks of swelling that initially began around her eyes and face that then progressed inferiorly to her abdomen and lower extremities. She had gained 12 pounds over a similar time frame and noted new, progressively worsening hematuria with frothy urine. She denied fever or recent illness, although she had endorse dyspnea with dry cough and orthopnea. She also had a new erythematous maculopapular rash on her face over her eyelids, cheeks, and nose. She had received her first cycle of ipilimumab for the treatment of her lung cancer 4 weeks prior.\nShe was diagnosed with NSCLC approximately 2 years prior and completed 6 cycles of carboplatin, pemetrexed, and pembrolizumab with good initial response to therapy, followed by maintenance therapy with pemetrexed and pembrolizumab and progression of disease. Her treatment course was complicated by primary adrenal insufficiency secondary to pembrolizumab use, for which she was initially treated with pulse steroids (1 year prior to current presentation) and remained on oral prednisone (5 mg by mouth daily). She had not received pembrolizumab for 3 months prior to her current presentation. Her other medical history included hypertension, gastroesophageal reflux disease, and osteoporosis. She continued to smoke approximately 1 pack of cigarettes per day.\nOn physical examination, her temperature was 37.1°C, blood pressure was 192/104 mm Hg, heart rate was 73 beats per minute, respiratory rate was 20 breaths per minute, and oxygen saturation was 94% on room air. She appeared to be in no acute distress and did not have evidence of jugular venous distention, though did have faint rales at the bases of the lungs, bilaterally. Her cardiovascular exam was unremarkable. Her abdominal exam did not have a fluid wave. She had 1+ edema of the bilateral lower extremities and a malar-like rash across the face that did not spare the nasolabial folds. She was alert, oriented, and mentating appropriately.\nA complete blood count revealed a white blood cell count of 12.28 × 103/uL, hemoglobin of 11.7 g/dL, and platelets of 301 × 103/uL. A complete metabolic panel revealed a potassium of 4.3 mmol/L, bicarbonate of 25 mmol/L, blood urea nitrogen of 29 mg/dL, and creatinine of 2.5 mg/dL (baseline 0.9 mg/dL prior to the initiation of ipilimumab − shown in Fig. ). Hepatitis B and C serologies, anti-glomerular basement membrane, myeloperoxidase, and proteinase-3 antibodies were all negative. An antinuclear antibody was weakly positive at a titer of 1:160 in a speckled pattern. C3 and C4 complement levels were 163 and 46 mg/dL, respectively. Serum and urine protein electrophoreses did not reveal evidence of an M-spike. Kappa-free light chains were 4.23 mg/dL and lambda-free light chains were 2.02 mg/dL, yielding a kappa/lambda-free light chain ratio of 2.09. A urinalysis obtained was light-brown in color, had 3+ proteinuria, a moderate amount of blood, negative nitrites, small leukocyte esterase, >50 dysmorphic red blood cells per high powered field, 30–50 white blood cells per high powered field, and granular casts (see Table for complete laboratory analysis). A chest x-ray showed interval development of small bilateral pleural effusions and multiple parenchymal opacities consistent with her known lung cancer. A renal ultrasound revealed normally appearing kidneys without evidence of renal vein thrombosis.\nDue to concern for CPI-induced nephritis, she was started on methylprednisolone, 1 mg/kg IV twice daily on day 2 of hospitalization. She underwent a renal biopsy the following day; light microscopy revealed 39 glomeruli, one of which was globally sclerotic and 5 of which had cellular crescents. There was mesangial and endocapillary hypercellularity, the latter including neutrophils. Acute tubular damage, mild lymphocytic tubulitis with a focus of a few eosinophils, and fresh erythrocytic casts were also seen (shown in Fig. ). Immunofluorescence revealed mesangial staining with IgA (3+), C3 (1+), and kappa light chain (2+) (shown in Fig. ). There was no significant glomerular or tubular staining with IgG, IgM, C1q, or lamba light chain. Staining for IgG subunits showed no staining with IgG1, trace segmental staining with IgG2, and no staining with IgG3 or IgG4. Electron microscopy did not reveal evidence of immune complex-type electron dense deposits; however, analysis of the samples was limited by retroprocessing artifact. Overall, the biopsy results were thought to be most consistent with IgA nephropathy.\nThe patient was later discharged on a prolonged prednisone taper, receiving 60 mg by mouth twice daily for 8 weeks. She did not receive further ipilimumab infusions. Unfortunately, her renal function continued to decline, and she was briefly placed on hemodialysis. Once hemodialysis was discontinued, she was subsequently readmitted for bilateral pleural effusions and worsening renal function, then elected not to pursue further dialysis and was discharged on hospice. |
A 70-year-old nonsmoking male presented with a 4-month history of progressive dyspnea, right-sided chest pain, and weight loss. He was initially diagnosed with pericardial effusion, approximately 4 months before the current presentation, and pericardial drainage (2.5 L) was subsequently carried out. Following drainage and a month of ibuprofen, no pericardial effusion was observed on echocardiography. He stated that he had no history of fever or hemoptysis or any other extrapulmonary symptoms or diseases. He was hypertensive and had a history of coronary artery stent. General physical examination was unremarkable. Examination of the respiratory system revealed diminished breath sounds in the right hemithorax, and initial laboratory evaluations were all normal except D-dimer, which was 3640 ng/ml. When he was admitted to the intensive care unit with severe orthopnea, due to his previous massive pleural and pericardial effusion and to exclude pulmonary thromboembolism, a bedside cardiopulmonary ultrasound (US) examination was performed. This surprisingly revealed a large immobile right atrial mass that was obstructing the tricuspid valve orifice and partially obstructing both the superior and inferior vena cava orifices []. The differential diagnoses were thrombus, atrial myxoma, or local invasion of a lung tumor. A chest computed tomography (CT) angiography examination showed a soft tissue mass extending from the right paratracheal area to the subcarinal area. This mass was obstructing the upper and intermediate bronchus, not distinguishable from both pulmonary arteries and invading the right atrium []. To confirm and stage the malignancy, a fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) scan was carried out. This showed that a heterogeneously ill-defined soft tissue lesion of 16 cm was encasing, and causing narrowing of, the right main bronchus and invading pulmonary arteries and the right atrium, with increased FDG uptake (maximum standardized uptake value: 11.1) []. No other pathological FDG uptake was detected. Fiberoptic bronchoscopy (FOB) revealed compression of the right main bronchus. Since insufficient tissue was obtained by repeated FOB and endobronchial US, an interventional radiologist performed a CT-guided tru-cut biopsy. Microscopic examination revealed a well-circumscribed malignant tumor, characterized by spindle cells with hyperchromatic nuclei and areas of markedly increased mitotic activity. The malignant spindle cells tested positive for vimentin, desmin, and smooth muscle actin [] but tested negative for epithelial membrane antigen, pancytokeratin, cytokeratin 7, cytokeratin 20, S-100 antigen, CD31, CD34, MyoD1, and TTF-1. The tumor had no epithelial differentiation. Overall morphological features favored a high-grade sarcoma with evidence of smooth muscle differentiation, making this tumor a leiomyosarcoma. Since a needle biopsy, rather than a surgical biopsy, had been performed, mitoses could not be counted and the tumor origin could not be identified. We retrieved the CT images taken 4 months previously, and the radiological appearance was suggestive of a malignant tumor arising from the right pulmonary artery []. Since the mass was rapidly growing and was not suitable for surgery, the patient was instructed to follow up with an oncologist. |
Patient B was a 37-year old male of Turkish descent referred for immunological investigation by the treating nephrologists because of IgM deficiency. Asymptomatic renal insufficiency was detected at the age of 28 years when a cirrhosis of the left kidney and mild hydronephrosis of the right kidney were found. Serum creatinine was 3.2 mg/dl (normal range 0.6–1.2 mg/dl), proteinuria was 2.5 g/d. He reported no increased susceptibility to infections, and his chronic renal insufficiency caused only mild clinical symptoms (development of fatigue and tachycardia upon physical strain).\nThe mRNAs encoding the membrane-bound and secreted immunoglobulin heavy chains are produced from identical primary transcripts, which are differently processed at their 3′ ends. Regulation of membrane-bound vs. secreted forms of the immunoglobulin heavy chains depends on the competition of 2 mutual cleavage polyadenylation sites (pAs/pAm) (). In mice targeted deletion of the mu heavy chain cleavage polyadenylation site pAs leads to deficiency of secreted IgM with intact expression of surface IgM and normal secretion of other immunoglobulin isotypes (). Therefore, we sequenced mu heavy chain gene including the polyadenylation sites in both patients with sIgMD and found no alterations (data not shown). Both patients' B cells were able to express precursor, secreted and membrane IgM mRNA (Figure ). Furthermore protein expression of monomeric and native pentameric IgM (Figure ) and surface expression of IgM on the B cell membrane (data not shown) was comparable to healthy controls.\nTo elucidate the genetic basis of the patients' selective IgM deficiency we used a targeted resequencing approach to sequence potential candidate genes. In both patients, we identified defects within the intrinsic B-cell receptor signaling pathway. Patient A harbored a c615G > T missense mutation in exon 8 in the tyrosine kinase BTK. The G > T transition resulted in a glutamic acid to aspartic acid substitution at position 205 within the highly conserved proline-rich (PRR) region located at the C-terminus of the TEC homology (TH) domain (Figures ). Proline rich regions are involved in protein-protein interactions, including interactions with G proteins and intramolecular association with the SH3 domain (). Mutations within the proline rich regions have been shown to abolish SH3 domain binding and result in functional impairment of BTK, pointing toward a potential biologic relevance of the BTK mutation found in patient A (). |
A 17-year-old male patient had a chief complaint of proclination and spacing of upper and lower incisors. He had angle Class II malocclusion with anterior open bite []. He had an unpleasant smile. The presence of thick cortical bone in the anterior region was needed to intrude upper anterior, and the decision was made to perform PAOO. After the initiation of orthodontic treatment till both the maxillary and mandibular arches reach 17 × 25 stainless steel (ss) wire.\nA modified corticotomy procedure was carried out under local anesthesia. A mucoperiosteal flap was elevated labially beyond the apices of the upper incisors.[] The vertical cuts were performed from the distal of the right upper lateral incisor leaving 2 mm from the alveolar bone tip to the distal of the left upper lateral incisor and the cortical bone was removed by tungsten carbide bur with continuous saline irrigation []. These incisions preserve the interdental papilla on the buccal sides of the maxillary anteriors, and no flap elevation or corticotomy was performed on the lingual or the palatal side in this case. Care was taken not to damage neurovascular bundles.[]\nPlatelet-rich fibrin was placed on apical region of anteriors. The horizontal envelope mucoperiosteal flap was extended to the apical region of the anteriors. The mucoperiosteal flaps were replaced and sutured with 4–0 silk sutures. The patient was given amoxicillin, 500 mg tid for 3 days. The sutures were removed after a week and advised to use chlorhexidine mouthrinse 0.12% bid for 2 weeks.\nFull maxillary and mandibular braces (17 × 25 ss) with conventional brackets were placed before periodontal surgery. Soon after a week of periodontal surgery, continuous burst one intrusive arch was given which was made in 17 × 25 TMA wire []. Elastic thread was placed from molar hook to helices of the intrusive arch for retraction with intrusion. After 15 days of recall, the deep bite got reduced from 6 to 2 mm.\nAfter 2 months of periodontal surgery, the continuous intrusive arch was removed. Eight months after surgery detailing of the occlusion was completed [].\nThe following orthodontic results were achieved:\nIdeal occlusion was obtained Open bite correction was made Anterior deep bite was corrected from 6 mm to 2 mm Straight profile Pleasant smile. |
A 54-year-old Caucasian female without relevant medical history noticed acute paresthesias and mild weakness of the left leg in the evening before admission. When she awakened at night she noticed that the weakness had progressed and that she had lost control of bladder function. On admission, she also complained of acute severe pain on the thorax, paresthesias and weakness of the right leg, and she reported diffuse pain in the left leg. In the weeks previous to presentation she had been experiencing a different sensation on the left side of her thorax. Clinical exam revealed an atactic paresis of the left leg graded 4/5 on the MRC scale. Tendon reflexes were pathologically increased in the lower limbs, and Babinski’s response was absent. There was a loss of superficial sensation below the T6 dermatome, most pronounced on the left side, and there was diminished sense of vibration, touch and position in the left leg. Her sphincter pressure was decreased, and there was 150 mL residue after micturition.\nSpinal MRI showed a calcified Th8–9disc herniation which slightly indented the anterior cord without cord abnormalities (). The paresis progressed to grade 1 of the left leg and grade 2 of the right leg within two hours. Emergency laminectomy of the eighth thoracic vertebra was performed that same night. Due to rapid deterioration no further (time-consuming) diagnostic procedures (eg additional imaging) were carried out prior to surgery. The partly calcified disc was removed through a transpedicular approach until there was no more spinal cord compression. Surgery was uneventful. Of note, because of the emergency setting, intra-operative neuromonitoring was not available. The day after surgery the paresis improved slightly, and there was no further deterioration of neurological function. Repeat MRI performed the day after surgery demonstrated multiple hyperintense intramedullary lesions (). Therefore, acute myelitis (possibly neuromyelitis optica spectrum disease, NMOSD) was considered as a differential diagnosis, and treatment with high-dose intravenous methylprednisolone was initiated, followed by oral maintenance dose. Repeat MRI 10 days after symptom onset revealed a clearly demarcated T2 hyperintense lesion in the posterior cord extending from the fifth to the tenth thoracic vertebrae, with partial contrast enhancement (). Cranial MRI was normal.\nThe results of additional laboratory exams including erythrocyte sedimentation rate, C-reactive protein, vitamin B12, angiotensin-converting enzyme, and thyroid-stimulating hormone. Serology for CMV, EBV, Lyme, and Treponema pallidum and thyroperoxidase, thyroglobulin, antinuclear, anti-extractable nuclear antigen, anti-neutrophil cytoplasmic, lupus anticoagulant, aquaporin-4 (AQP4), myelin oligodendrocyte glycoprotein (MOG) and paraneoplastic antibodies were within normal limits or negative. Visual evoked potentials showed delayed latencies (p100: left 120 ms, right 122 ms). The last-mentioned finding led to a possible diagnosis of neuromyelitis optica (NMO), and the patient was started on azathioprine for long-term immunosuppression. However, repeat ophthalmologic exam revealed no abnormalities, and cerebrospinal fluid (CSF) analysis showed normal cell count, mild elevation of protein levels and no oligoclonal bands. Therefore the azathioprine was discontinued. At this point, the patient was referred for a second opinion, and spinal cord ischemia was established as the most likely diagnosis.\nThe patient was transferred to the division of rehabilitation. After three months the patient was able to walk short distances with a walker. MRI showed a marked decrease in cord abnormalities, and contrast enhancement was no longer present (). Written informed consent was obtained from the patient for publication of this case report. Institutional approval was not required to publish the case details. |
We report the case of a 60-year-old man with CSCC, and a past medical history of HIV diagnosed ten years prior to cancer diagnosis and controlled with HAART.\nHe first presented in early 2018 with a right-sided neck mass. At the time, he was diagnosed with cutaneous squamous cell carcinoma of the neck and was treated with Mohs surgery with subsequent reconstruction in February 2018. A few months later, the patient noticed swelling in his right neck at the median surgical margin. As the swelling progressed, the patient underwent a CT scan of the head and neck in June 2018, which revealed a right-sided cervical heterogeneous mass measuring approximately 4 × 2.5 × 2 cm.\nOn June 30, 2018, the patient underwent wide local excision of the lesion, modified radical neck dissection, right superficial parotidectomy, and left anterolateral thigh-free flap reconstruction. Pathology demonstrated seven out of 34 lymph nodes were positive for metastatic SCC. The largest node was at level 2b and measured 10 mm with extracapsular extension. Due to the extracapsular extension, at this juncture the patient went on to receive treatment with chemoradiation.\nThe patient started one cycle of cisplatin on August 14, 2018, with concurrent radiotherapy (CRT) at a dose of 6600cGy in 33 fractions. Due to acute kidney injury, cisplatin was replaced with carboplatin. He received a total of 3 cycles of platinum-based CRT, which concluded on September 24, 2018. In May 2019, the patient presented with a mid-neck subcutaneous mass. FNA biopsy showed SCC, consist with dermal metastasis of CSCC. In light of these findings, he was started on cemiplimab on July 2, 2019.\nThe patient has since received all 33 total planned cycles of cemiplimab. Cycle 14 of cemiplimab was delayed due to the start of the COVID-19 pandemic, but immunotherapy was resumed promptly thereafter. The final cycle of cemiplimab was administered in June 2021. Clinically, he has had a complete response (CR) with no radiologic evidence of disease first reported on neck MRI and CT scans in late August 2019 (NI-RADS 1). An MRI of the neck and a CT scan of the thorax from June 2021 both revealed no evidence of recurrent CSCC or suspicious lymph nodes, along with expected postsurgical and post-radiation changes. |
An 18-year-old male patient was referred to Department of Oral and Maxillofacial Surgery, Faculty of Dentistry Istanbul University for the investigation and treatment of a unilateral TMJ ankylosis. The chief complaint was a limitation of severe mouth opening []. The patient claimed that he had been operated before from both sides. Radiographic assessment was carried out using CT scan and panoramic radiography. CT scan and panoramic radiograph revealed the massive heterotopic bone development and bony ankylosis surrounding the right TMJ. Panoramic radiograph also demonstrated the area of previous surgery on the left side [Figures and ]. A preoperative model which was maintained from the CBCT of the patient was also used to observe properly the extension of the ankylotic mass [].\nOn clinical examination, 11 mm mouth opening with facial asymmetry and bird face-like appearance was observed. On the left side, a scar at preauricular region was seen, but on the right side, there was no sign of a previous operation. Clinical findings demonstrated slight mandibular retrognathia and deviation of the mandible to the affected (right) side.\nGeneral anesthesia with nasotracheal intubation and total muscle relaxation was administered. A single intravenous dose of steroid was given at the beginning of the case. The TMJ is approached through a modified preauricular incision with temporal extension to expose the temporalis fascia and muscle, zygomatic arch, ankylotic mass, and sigmoid notch. The ankylosed TMJ was palpable, and an incision was made directly onto the bone, exposing the ankylosed TMJ. After determining the anterior and posterior limits of the ankylosed condyle, the bony segment was resected using a piezoelectric osteotome. The irregular edges of the segments were smoothed by a bur, and the ramus was completely disconnected from the upper bony block. Ipsilateral coronoidectomy was also performed to maintain adequate mouth opening. A manual-guided mouth opening was done to observe condylar movements. The abdomen was prepared from above the umbilicus to the pubic region. A 4–5 cm transverse incision was made in the midline through skin and subcutaneous tissue and then deepened to obtain fat graft. The fat graft was trimmed, and the dermis with fat graft was passively inserted between the segments and secured with 3-0 vicryl [Figures and ]. Layer-wise closure was performed. Maxillomandibular fixation was maintained to 3 days, and the patient was discharged from hospital 2 days after surgery.\nPostoperative medication including antibiotics and nonsteroid anti-inflammatory drugs was prescribed for approximately 2 weeks after the operation. The patient was started on a soft diet. Jaw-opening exercises, active chewing movements, and intensive physiotherapy were started within 7 days of the operation. In first 3 weeks of the physiotherapy, daily active and passive range of motion (ROM) exercises were made in hospital setting with the aid of physiotherapist, and a home exercise program was given to the patient. Before ROM exercises, 10 min hot-pack and 25 min transcutaneous electrical nerve stimulation in burst mode and 4 min therapeutic ultrasound (0.5 watt/cm2, pulsed) were applied to the TMJ region in every session. The patient continued home exercise program after 3-week hospital setting physiotherapy program. Thirty-five millimeters of mouth opening was maintained at the end of the physiotherapy []. The follow-up of the patient was made also by the physiotherapist during 1 year after the operation. |
The deceased donor was a 67-year-old man with a kidney Doppler ultrasound (DUS) that was negative for any nodular lesion. As part of the routine postoperative follow-up management, the recipient underwent DUS to assess the patency of the graft on postoperative day 1. The DUS finding was suspicious for an acute arterial thrombosis but did not reveal any focal irregularities. Consequently, a computed tomography (CT) scan was urgently obtained but it did not show any arterial complications. However, it serendipitously revealed a 2.4-cm lesion on the upper pole of the renal allograft which was not detected during the back-table or ultrasonography monitoring. A biopsy of the lesion was performed, and its histology revealed an epithelial proliferation of large cells with finely granular cytoplasm and medium round nucleus vesicular acidophilus, arranged tubules, and alveoli and cords immersed in a connective tissue stroma. This picture was consistent with oncocytoma. However, because the eosinophilic variant of chromophobe renal cell carcinoma (RCC) may morphologically resemble renal oncocytoma, immunohistochemical staining was performed using Ki-67 antibodies and RCC antigens. The results were negative, ruling out chromophobe RCC. The therapeutic options and potential related outcomes were clearly discussed with the patient. Given the low risk of malignant transformation in an oncocytoma [], we found no reason for resection of the lesion or an allograft nephrectomy. Consequently, we opted for active surveillance of the benign tumor with ultrasonography, every 2 months, for the first year and, then, with magnetic resonance imaging (MRI), every year (Fig. ). The patient received mycophenolate-mofetil, tacrolimus, and prednisone throughout the 5-year follow-up period and the regimen for immunosuppression was not changed despite the presence of the renal mass. After 60 months of active surveillance, we report that radiological studies have shown no growth, regression, or any other interim morphological changes to the lesion, and the patient is alive and well (Fig. ). |
The patient, a 67-years-old woman, who came to the first clinical consultation with a history of hypothyroidism and allergy to aspirin and penicillin, with painful symptoms in the submental and mental right side of mandibular bone region, with a duration of 3 months. The patient was attended by professionals of the Department of Dentistry, who proposed a procedure of surgical extraction of the mandibular teeth involved in the anterior section. Orthopantomography was performed, in which a large radiolucent area was observed in the chin region, so for this reason, she was referred to the Oral and Maxillofacial Surgery Department ().\nAfter medical and dental details described above, an intraoral clinical assessment of the area was made, in which an increase in volume of the vestibular bone table//plate?/ was observed, tooth mobility between grade 1–2 found on inspection of 4.1–4.2. On the other hand, teeth 3.3 and 4.3 showed no pathological mobility. In addition, the surrounding mucosa was erythematous, especially in the region of teeth 3.1 and 3.3, in which the tissue was fluctuating due to possible perforation of the cortical vestibular bone as seen in .\nComputed tomography (CT) assessment showed the presence of a delimited, unilocular, hypodense zone along its entire perimeter, limited by a thin bone cortex on the one hand, from the mesial-apical region of teeth 33–46, below the mandibular basilar edge. There was a short conservation of the basilar border, especially in the midline with greater resorption on both sides, so that greater involvement was shown in the upper part of the alveolar ridge, involving the teeth present, in the mesial and apical regions of tooth 33, and teeth 41, 42, 43.\nIn the region itself of these teeth, an accentuated loss of bone support was found, at the level of the middle and apical third, only the cervical third of the roots was preserved. On the other hand, a smaller amount of resorption of the lingual bone and greater resorption of the vestibular cortical bone were observed throughout the extent of the lesion, measuring a thickness of only 1–1.5 mm, as shown in .\nAs the clinical and radiographic examination of the lesion was performed and having made an initial diagnosis compatible with odontogenic keratocyst, it was decided to perform a fine needle aspiration puncture of the content for subsequent pathological analysis. Thereby, a milky-yellow liquid content of approximately 10cc was obtained. In addition, the lesion showed a thick cystic capsule of intense red wine color, which showed some degree of resistance to surgical excision. Both surgical pieces were analyzed histopathologically confirming the initial diagnosis of moderate-grade unilocular Parakeratinized Odontogenic Keratocyst (former classification of odontogenic keratocyst that was reclassified as a keratocyst odontogenic tumor) as shown in .\nAfter 30 days of initial decompression of the lesion, surgical resection of the lesion was performed under general anesthesia. A supracrestal incision was made in the mandibular residual edentulous alveolar ridge, covering the teeth present and extending bilaterally to the first molar regions. After this, the mucoperiosteal flap was lifted, exposing the vestibular cortical bone to obtain a complete and improved direct view of the lesion.\nSubsequently, a peripheral osteotomy was performed to enable direct visualization of the odontogenic keratocyst, using tungsten carbide surgical drills and a gouge clamp. In addition to the surgical procedure of excision of the lesion, enucleation and vigorous curettage were performed. The remaining teeth were removed and Carnoy’s solution was applied as an agent for chelating the remaining bone tissue, embedded in a gauze for 3 min. Finally, a reconstruction plate of the unlock 2.0 system was positioned to cover the middle portion of the mandibular body and extending from the region of the right first right molar to the left first molar. This was stabilized with 7 mm long mono-cortical screws to prevent a possible inter- or postsurgical mandibular fracture. The anatomical layers were repositioned using simple stitches with Vicryl 4.0 ().\nFurthermore, post-surgical control was performed for the removal of the stitches, observing adequate healing of the postoperative wound, without signs of dehiscence and surgical bed infection. In addition, plans were made for follow-up consultations a month later, at three months, and at one year, with control panoramic radiography, in which satisfactory functional results were observed and without signs of recurrence, as shown in .\nClinical and radiographic control was performed after 6 months of follow-up, and a favorable healing process was observed without signs of recurrence of the lesion, as may be seen in .\nAfter two years of follow-up; a satisfactory bone remodeling of the region that underwent surgery was found, showing bone characteristics similar to those of the surrounding normal bone tissue. Correct positioning of the osteosynthesis material was also verified, with the absence of resorption areas around it ().\nIn an accurate CT assessment after the two year follow-up, isolated radiolucent areas, approximately 1 cm in diameter were identified in the region of teeth 4.1 and 4.2, which was compatible with recurrence of lesion, and therefore it was decided to undertake a second surgical procedure in order to obtain the diagnosis of recurrence. The osteosynthesis material was removed, as shown in .\nTherefore, the histopathological assessment performed the second time confirmed a recurrence of the initial odontogenic keratocyst - parakeratotic subtype as shown in . A new conservative surgical approach was performed to remove the recurrent lesion and patient continues with control follow-up to ensure absence of new local recurrence. In the last Orthopantomography performed, a local area with new bone formation was noted instead of a local lesion appearance as may be visualized in . |
A 56-year-old male patient sustained a total amputation at the level of the right upper arm after a cable was wrapped around it in an occupational injury. The patient had complete disruption of the muscles, vessels and nervous structures, as well as a fracture of the humerus in the distal third.\nThe patient had no other injuries and was otherwise fit and healthy. Immediate preparation of the stump and the amputated arm was performed by two separate teams. After debridement, bone resection of the humerus and identification of the vascular and nervous structures was performed, and a stable osteosynthesis with a 4.5-mm plate was executed. Subsequently, an anastomosis of the brachial artery was completed. Successful revascularization was achieved 4.5 h post trauma. Revascularization was followed by anastomosis of the veins, immediately after which a veno-venous hemodiafiltration was initiated. Then, an epineural suture of the radial, ulnar and median nerves was performed, and the muscles were readapted. A fasciotomy of the lower arm and carpal tunnel release were also performed. The skin was left open. The procedure took 5.5 h. Post-operatively, the patient showed good re-capillarization of the fingers and physiotherapy was initiated to mobilize the fingers. On the 4th post-operative day, a skin graft was used to close the wound. The patient developed neuropathic pain of the ulnar and median nerves with a positive Hoffmann-Tinel sign, which was treated by opioid therapy. The patient was transferred to rehab and showed good progress in elbow movement; however, bending of the thumb and fingers was not possible, although intensive therapy had been performed. This was the indication for the functional transfer of the gracilis muscle with adaption to the deep antebrachial flexor tendons and nerval anastomosis to the ulnar nerve.\nSix years after the trauma, the sensitivity of the fingers was almost normal, and prehensility had been preserved. Although the patient was unable to work after the injury, he was otherwise relatively satisfied and able to independently perform daily activities. He displayed a good capacity for shoulder and elbow motion. Wrist extension was possible, but both wrist flexion and finger bending were reduced. If needed, he would undergo replantation again (Fig. ). |
A 17-year-old female was admitted to the hospital due to severe suicidality. At the time of admission she complained about an irritating feeling in her nose, which made her constantly grimace in the area around the nose. She was excessively worried about having a serious illness of her nose (secondary hypochondriacal delusions) and was suicidal as a consequence. Her belief persisted even after any underlying medical condition of the nose has been ruled out by extensive medical examinations. She also presented with disorganized behavior, stereotypical movements, emotional instability and lability, and a below average level of intelligence during hospitalization. On the PANSS, her symptoms scored 29/23/70 (for the Psychotic, Negative and General Psychopathology Scale, respectively). Brief neurological examination revealed no abnormal neurological signs. As ascertained by the history taken from the patient and her mother, she had a history of school phobia that began at the age of 12 years, emotional disorders, normal cognitive and physical development, and a three-year history of chronic headache. She managed to complete primary and secondary education with the help of school counseling services given to her on account of school phobia. She had not received any psychiatric care before the described admission. A diagnostic evaluation for chronic headache at the University Children’s Hospital was undertaken a year before admission.\nCalcium, phosphate and parathyroid hormone blood levels were normal. Vitamin D levels were decreased with decreased calcium levels in the urine. No signs of calcium depositions in organs other than the described brain regions were determined by ultrasound. Ophthalmological, ear-nose-and-throat examination and electroencephalography were also normal.\nDetailed neurological examination revealed dysfunction of pursuit eye movement, dystonic positioning of both arms when stretched ahead, discrete ataxia of the arms and legs, and a pathological extensor response of the left big toe.\nBilateral symmetrical calcification in head, body and tail of the caudate nucleus and ventral part of the thalamus were determined by computerized tomography (Figs. and ). MRI was preformed twice over a two-year period using the same protocol. Corresponding MR T1 sequences showed hyperintense calcifications in the same regions as those found on CT examination (Figs. and ). Both MR examinations showed a high signal of calcified areas on T1 weighted sequences due to the surface area of calcium crystals. The same areas had an isointense signal on T2 weighted sequences. No other abnormalities of the brain were detected on the MRI.\nDuring psychometric evaluation, the patient’s cognitive abilities were assessed with RPM, TOL-II, d2, CTMT and Stroop tests. The patient performed significantly worse than her normative age group in terms of general cognitive abilities, coming in below the 10th percentile. She was unable to perform problem-solving operations that require abstract thinking. Assessment of her attention performance showed below-average results in scanning and alternating attention. She also showed below-average performance in her sustained and divided attention, with her concentration performance in the 8th percentile. Her planning abilities were significantly worse in comparison with her normative group, where she was unable to construct a problem-solving strategy. Her approach was a trial-and-error strategy and she failed to solve the problem within the time limit. The patient had no significant difficulties with inhibition of dominant response, reaching a borderline average result. We performed a retest after a year of treatment and the results showed no significant changes, although she was slightly better, but unfortunately without significant improvements, in her planning abilities and in her sustained attention.\nA whole blood EDTA sample was used for extraction of genomic DNA according to established laboratory protocols using the FlexiGene DNA isolation kit (Qiagen, Germany). Whole exome sequencing a trio (index patient and her parents) was performed in collaboration with NovoGene Corp. Inc. (Davis, CA, USA) using an Agilent Sure Select Human All Exon V6, 5191–4004 kit for whole exome enrichment preparation together with an Illumina Platform PE150 (Illumina, San Diego, USA) to perform the whole exome sequencing. Genetic variants with coverage >15x were analyzed using Variant Studio 3.0 software (Illumina). Evaluation of variants was firstly restricted to those located in eight genes related to Fahr’s disease (SLC20A2, PDGFRB, PDGFB, XPR1, KRIT1, SLC19A3, TREX1, MYORG). We reached 99.9% with at least 10X coverage for the patient. A search tool for the retrieval of interacting genes/proteins (STRING, ) was used to construct the protein-protein interactions that are involved downstream and upstream in Fahr’s syndrome (GNAS, ERCC8, PDGFB, CYP2U1, GNA11, SLC20A2, IFIH1, PSMB8, PDGFRB, CA2, ERCC6, SAMHD1, TREX1, CASR, TREM2, TYROBP, GJA1, ERCC3, FAM111A, RNASEH2B, SLC46A1, SLC7A7, ATP13A2, PARK7, HMBS, KRIT1). No causative mutations were found in the selected genes in the patient.\nComputerized tomography scans of the heads of the patient’s parents were normal.\nThe patient was treated symptomatically with quetiapine sustained release (initially 200 mg and gradually increasing to 900 mg daily) and sertraline (150 mg daily, gradually increasing to 200 mg daily). We did not observe any side effects with the use of quetiapine, although special attention was given to the possible exacerbation of extrapyramidal symptoms. Psychotic (PANSS scores at discharge were 8/13/27, PANSS scores after two years were 9/16/34 for the Psychotic, Negative and General Psychopathology Scale, respectively), affective and behavioral symptoms were improved; she was no longer suicidal and remained stable on gradually increasing doses of antipsychotic medication within two years of treatment, however, her intellectual abilities were not improved. Even though the patient completed secondary professional education and intense professional rehabilitation efforts were made, she has not been able to start working, mainly due to emotional instability. The patient was transferred to adult psychiatric services at the age of 21. |
During a dissection of the right upper limb of the cadaver of a 70-year-old male fixed in 10% formalin that was conducted in an anatomy department, we observed an anomalously high origin from the brachial artery of the radial and ulnar arteries, which also had variant courses. The brachial artery bifurcated into the radial artery medially and the ulnar artery laterally at 21 cm cranial of the cubital fossa (), whereas normally it divides at the cubital fossa itself with a different disposition. After descending 5.5 cm below the lower border of the teres major muscle, and at 21 cm above the cubital fossa, the brachial artery terminated anomalously, giving rise to the radial artery medially and the ulnar artery laterally. The radial artery remained medial to the median nerve in the arm (). At the elbow, the radial artery continued along the medial aspect and then crossed to the lateral side at the level of the upper third of the forearm, superficial to the flexor muscles (). The radial artery was found to be tortuous from the beginning to around the medial epicondyle. Thereafter it ran straight along its oblique course, before once again becoming highly tortuous. The total length of the radial artery was 55 cm. The ulnar artery remained on the lateral side of the arm, then entered the forearm by passing between two heads of the flexor carpi ulnaris muscle and remained on the medial aspect of the forearm. These anomalies were associated with abnormal formation and course of the median nerve. The lateral root crossed the axillary artery anteriorly and fused with the medial root to form the median nerve on the medial aspect (). It remained on the medial aspect and in the cubital fossa it traversed between the two heads of the pronator teres, entered the forearm and followed the normal course. |
A 40-year-old male patient, a known case of NF-1, reported to our hospital with the complaint of a swelling on the right maxillary region in relation to upper back tooth. The swelling, initially noticed 3 weeks back, was small in size, but grew rapidly to reach the present size. He also gave the history of dull aching pain associated with the swelling. His past medical history revealed that multiple cutaneous nodules seen on the entire body [Figures and ] were present since he was 13 years old. He was evaluated for the complaint of pain in the right chest 4 months back, following which chest X-ray, computed tomography (CT) and magnetic resonance imaging (MRI) scan were taken. The imaging studies revealed the presence of a mediastinal tumor which was later diagnosed as MPNST [Figures –]. Since the lesion was inoperable, he was subjected to radiotherapy. His family history was noncontributory.\nOn general examination, the patient was poorly built and nourished. No pallor, icterus, cyanosis, clubbing, pedal edema or lymph node enlargement were noticed. Multiple cutaneous nodules of varying size were seen distributed on the entire body. Multiple café au lait pigmentation was noticed on the axillary region and on the arms. A large pigmented macule was present on the right side chest [].\nOn intraoral examination, an exophytic soft tissue mass measuring around 3 cm × 4 cm × 5 cm was present on the right alveolus in relation to 16 and 17. The lesion presented as a lobulated dumbbell shaped mass extending buccally and palatally. The buccal mass was found to be extending into the buccal vestibule, and the palatal mass involved the entire half of the posterior palate. The swelling was sessile, irregular in shape and normal in color. The surface was smooth with superficial candidal infection in some areas []. On palpation, the swelling was nontender, firm in consistency and was found to be fixed to the underlying tissue. Slight bleeding was noticed. Grade II mobility was present in 16 and 17.\nIntraoral periapical radiograph and orthopantamograph showed severe bone loss in relation to 16 and 17 with periapical radiolucency in relation to 16 [].\nBased on the history and clinical examination, a provisional diagnosis of intraoral neurofibroma was given. Other diagnosis considered included MPNST, other mesenchymal neoplasms and odontogenic neoplasms.\nIncisional biopsy was done from the palatal aspect of the tumor. Microscopically, the lesion showed alternating fascicles of hypercellular and hypocellular areas arranged in a streaming pattern []. The cellular component comprised predominantly atypical spindle cells with hyperchromatic, wavy nuclei, which were pleomorphic, and indistinct cytoplasm []. Short fusiform cells with large hyperchromatic nuclei and a thin rim of cytoplasm were also seen. Increased mitosis (four to six per high power field) was noticed. The vascular changes like sub-endothelial proliferation of tumor cells and herniation of tumor cells into the vessels were appreciable in the sections []. The walls of some of the large vessels showed small vascular proliferations []. Neurofibromatous areas and areas of necrosis were also present [Figures and ]. Immunohistochemical staining by the tumor marker S-100 was found to be negative. Based on the histopathologic appearance and its clinical association with NF-1, the lesion was diagnosed as MPNST.\nThe patient was given radiotherapy for the oral lesion and was treated with 3000 cGy in 10 fractions. One month later, the patient developed similar lesions on the scalp and the right side of the upper back region and finally succumbed to death after 2 months. |
A 35-year-old man presented with severe headache and subsequent loss of consciousness and was admitted to our hospital. No focal neurological abnormalities were noted. Initial computed tomography (CT) showed findings typical of subarachnoid hemorrhage in the basal cistern. Three-dimensional CT angiography revealed an anomalous artery originating from the posterior surface of the ACoA and a saccular aneurysm arising from the trifurcation of the anomalous artery, the branching point of the ACoA with fenestration, and the right A1or A2 segment of the ACA []. The aneurysmal fundus projected superolaterally to the right. The anomalous artery arose from the posterior surface of the ACoA and ran around the genu of the corpus callosum, parallel to bilateral pericallosal arteries, which distributed branches to the m edial frontal lobes []. The anomalous artery was interpreted as an accessory ACA.\nRight frontotemporal craniotomy was performed. Following dissection of the Sylvian fissure and interhemispheric fissure, the ACoA complex including bilateral A1 and A2 segments of the ACA, and the anterior surface of the ACoA were exposed []. After partial resection of the ipsilateral gyrus rectus, the aneurysmal fundus was found behind the ipsilateral A2 segment of the ACA, which hid the accessory ACA. After gently pushing the aneurysm forward by spatula to the corner of the ACoA, the distal artery of the accessory ACA and its origin were confirmed, working between the right frontal lobe and the right A2 segment of the ACA []. A straight fenestrated Yasargil titanium clip (No. FT598T; blade length 4 mm, fenestration diameter 3.5 mm) was applied across the ipsilateral A2 segment of the ACA and toward the accessory ACA from the ACoA origin, parallel to the ACoA []. The aneurysm was successfully obliterated with clipping. Complete aneurysm occlusion and patency of the ipsilateral A2 segment of the ACA and the accessory ACA were confirmed intraoper atively under indocyanine green angiography []. Postoperative three-dimensional CT angiography revealed patency of both the right A2 segment of the ACA and the accessory ACA, resulting in disappearance of the aneurysm [].\nThe postoperative course was uneventful, and the patient was discharged with no neurological deficits. |
A 49-year-old male patient (body mass index, 23.8 kg/cm2) presented with the chief complaint of right abdominal pain persisting for >1 month. Pelvic computed tomography examination revealed part of the unevenly thickening ascending colon wall. Colonoscopy and endoscopic biopsy were performed and revealed adenocarcinoma of the ascending colon. Tumor markers and routine laboratory blood tests were within the normal reference ranges.\nThe surgery was performed in accordance with the procedure of totally laparoscopic right hemicolectomy. The resected specimen was deposited in the collection bag and placed in the pelvis. Before the specimen extraction, the anus was fully dilated and the rectum was repeatedly rinsed with antiseptic (1% povidone-iodine). Povidone gauze was inserted into the rectum to support the intestinal wall and the full-thickness anterior wall of the upper rectum was cut open 3–4 cm lengthwise using an electrocautery device (). Oval forceps were inserted into the rectal incision to hold one end of the collection bag and the whole specimen was gently removed through the anus (). Continuous barbed suture was used to close the rectal-wall incision from the distal to the proximal end and the seromuscular layer was sutured to reinforce the incision (). Further, 3,000 mL of hyperthermic distilled water was used to repeatedly rinse the abdominal and pelvic cavities. The trocar holes were closed and the operation was completed ().\nThe patient’s vital signs were stable during the surgery; total operating time was 184 minutes and intraoperative blood loss was ∼50 ml. The patient could sit up on the bed unaided post-operatively and resumed normal daily living activities without pain within 18 hours. The first flatus occurred within 48 hours. He reported a Numeric Rating Scale for pain score of 3 and 1 on the post-operative first and third days, respectively. The patient was started on a liquid diet 3 days after surgery and discharged on the post-operative ninth day. Pathology of the surgical specimen showed moderately differentiated colonic adenocarcinoma. The number of lymph nodes retrieved was 50. A follow-up colonoscopy was performed 1 month later and no stenosis or scar contracture was observed in the intestinal cavity (). The patient recovered well with accurate anal sensation and smooth defecation. |
A 28-year-old male patient () reported to our unit with chief compliant of difficulty in speech, disfigurement of face, and difficulty in mastication of food since his childhood. Patient underwent surgery for closure of facial clefts at the age of 1 month under General anaesthesia and at the age of 16 years. On extraoral clinical examination gross facial asymmetry noticed with mandibular midline deviation towards right side, upper, and lower lips was incompetent and previously operated extraoral scars were noticed extending from right side of the corner of mouth, cheek, and auricle and scar on midline of lower lip () and noticed nonoperated clefts on left ear ().\nOn intraorally soft tissue examination scar was noticed on right side of the hard palate showing with a residual cleft on right soft palate (). On hard tissue examination mandibular symphysis cleft with ankyloglossia () was noticed and on bimanual palpation 2 halves of mandibular segments were freely movable. Midline shift towards right side and crowding in the lower anteriors, supernumerary teeth distal to 18 and between 44 and 45 region, retained deciduous teeth of 83 were noticed and missing 43. On Radiographic examination () mandible symphysis cleft was noticed between 31 and 41 and impacted 3rd molars of 28 and 38.\nUnder general anaesthesia, routine preparation of patient was done and throat pack was placed; IMF done from 6/6 to 6/6 with lower segmental arch bar was placed guiding the occlusion. The tongue was released, and a frenectomy of the lingual frenulum was performed after the frenectomy; an incision was made in the mandibular buccal sulcus between the cuspids; and a mucoperiosteal flap was raised; the cleft bony margins were exposed on either sides till the lower border of mandible, genioglossus, and hyoid muscles were released from mucosal part of lower lip region. Iliac crest bone graft was taken from right iliac region and contoured to the symphysis of the mandible using 2 mm 6 hole; with gap and without gap plates were placed in both superior and inferior borders. Donor site was sutured with 3-0 vicryl and dynplaster dressing was given to reduce hematoma formation. Recipient site that is intraoral symphysis region was sutured after undermining the margins for removal of fibrous tissue and was sutured with 3-0 and 2-0 vicryl. Postoperative radiograph () was taken immediately and after 6 months of surgery, the bone graft was well integrated with full union of the mandible. The tongue presents with normal movements. Occlusion is satisfactory. (Figures and ). |
A 14-year-old female presented to our service following an incidental finding of a gastric diverticulum on magnetic resonance urography (Figure ) while being followed for complex renal cysts. Review of images from a CT scan of the abdomen and pelvis performed seven months earlier for abdominal pain at an outside institution also showed the gastric diverticulum (Figure ). However, this finding was not mentioned in the outside report for that CT scan. The patient had a yearlong history of intermittent abdominal pain and nausea without emesis. She denied any changes to her bowel habits during this time. Her father had a history of diabetes. Physical exam revealed a healthy, alert adolescent female in no acute distress. On palpation, her abdomen was soft, non-tender, and non-distended. A very small fascial defect was noted at the umbilicus, but no other masses or organomegaly were evident. She had normoactive bowel sounds on auscultation. A fluoroscopic upper gastrointestinal (UGI) exam showed a wide-mouth diverticulum arising from the posteromedial aspect of the gastric fundus (Figure ). No extension of the diverticulum into the thoracic cavity was noted.\nThe patient began omeprazole for her intermittent abdominal pain prior to the procedure and was instructed to continue the medication after surgery. In the operating room, a 2 cm infraumbilical incision was made, and electrocautery was used to progress through the subcutaneous tissue. The stalk of the umbilical hernia was isolated, opened, and a 5 mm port was inserted in the fascial defect. Carbon dioxide was infused into the abdominal cavity to a pressure of 15 mmHg. Three additional 5 mm ports were placed in the left and right upper quadrants and right mid-abdomen. The anterior surface of the stomach was free of abnormalities. The lesser sac was opened, and the short gastric vessels were divided using the LigaSure Maryland jaw device (Medtronic, Minneapolis, MN, USA). At the gastric fundus, approximately 2 cm distal from the gastroesophageal junction, the wide-mouth GD was identified (Figure ). Of note, an endoscope was on standby in the operating room during the surgery to aid in its identification if necessary. The attachments to the GD were taken down using the LigaSure Maryland jaw device. The 5 mm umbilical port was replaced with a 12 mm port. The GD was divided away from the stomach using an Endo GIA reinforced stapler (Medtronic), placed in an Endo Catch bag (Medtronic), and removed from the abdomen through the umbilical port. The resected specimen was sent to pathology and found to have normally folded gastric mucosa with mild chronic inflammation. The patient was tolerating clear liquids on postoperative day one and was discharged after tolerating a regular diet on postoperative day two. |
A 14-year-old boy visited the ear, nose, and throat (ENT) outpatient department of our center, with a history of left-sided nasal obstruction gradually progressive over a period of 1 year, with recurrent history of associated rhinorrhea. There was no history of epistaxis or any other ENT complaints, and the patient’s past medical history and family history were insignificant.\nOn anterior rhinoscopy, a single polypoidal mass was seen filling the left nasal cavity extending up to the vestibule. The mass was firm in consistency, insensitive to touch and pain, and did not bleed upon touch. General and systemic examinations and an examination of the rest of the ENT were all within normal limits. A contrast-enhanced computed tomography (CT) scan showed a homogenous soft tissue lesion measuring 44×68×12 mm in the left nasal cavity, extending posteriorly into the nasopharynx ().\nThe mass extended superiorly into the left ethmoid air cells up to the cribriform plate with its thinning and breach. The rest of the sinuses was clear. On contrast study, there was heterogeneous enhancement. Based on the CT findings of the anterior skull base breach, gadolinium-enhanced magnetic resonance imaging (MRI) was performed to rule out intracranial extension, and revealed small sub-centimeter focus posteriorly without any significant intracranial extension ().\nRigid endoscopy was carried out and the mass was seen attached to the anterior part of the nasal septum. A punch biopsy was taken, which presented as an inflammatory polyp.\nThe patient was referred for standard endoscopic sinus surgery under general anesthetic after providing informed consent. The mass was removed endoscopically, and was seen to be extending intracranially but extradurally. Complete resection of the tumor was achieved and while managing the tumor in the region of the cribriform plate, there was an obvious CSF leak. The site of leakage was identified and closed using an underlay technique with septal cartilage, fascia lata, and tissue glue. The intra-operative blood loss was minimal.The post-operative recovery period was uneventful. Histopathology of the excised specimen showed the tumor mass lined by pseudostratified columnar epithelium with squamous metaplasia. The subepithelial tissue was composed of loose myxoid areas with mature adipose tissues and foci of cartilage. Foci of neuroepithelial cells occupying more than four low-power fields were seen. Grade 3 immature teratoma was given as final diagnosis (-).\nAdjuvant chemotherapy was started based on the histological grading, with four cycles of bleomycin, etoposide, and cisplatin. A regular follow-up for a period of 2 years showed no signs of recurrence. |
A 43-year-old male with prior history of pulmonary sarcoidosis, obstructive hypertrophic cardiomyopathy, and paroxysmal atrial fibrillation presented with haemoptysis. The patient was diagnosed with obstructive hypertrophic cardiomyopathy 15 years prior, had a single-chamber ICD placed for primary prevention, and underwent PV isolation 2 years prior to presentation. He had recurrent syncopal episodes from left ventricular outflow tract obstruction and underwent septal myectomy, mitral valvuloplasty, left atrial appendage clipping, and epicardial radiofrequency ablation 8 months prior to presentation. Myectomy pathology revealed myocyte hypertrophy and granulomas consistent with cardiac sarcoidosis.\nThe patient first began coughing up blood-tinged sputum 1 week prior to admission but was thought to have bronchitis at two urgent care encounters. Over the course of the week prior to admission, he developed progressive shortness of breath and increased frequency and quantity of blood in his sputum. On presentation, he was afebrile, normotensive, with normal work of breathing. In the emergency room, he had several episodes of massive haemoptysis, each over 100 mL, and was emergently intubated for airway protection. Immediate bronchoscopy demonstrated active bleeding with large clots in the left lower lobe. Prothrombin complex concentrate was administered to mitigate the effect of rivaroxaban, which he had been taking prior to admission for atrial fibrillation (CHA2DS2VASc Score 3, HAS-BLED Score 2). Initial non-contrast CT and repeated bronchoscopies suggested primarily left lung involvement without a clear source of bleeding.\nThe patient was transferred to our institution after several days with limited control of the haemoptysis. Repeat contrast-enhanced CT angiography did not reveal active bleeding, but the left upper and left lower PVs were poorly visualized, increasing clinical suspicion for PVS as the underlying cause of haemoptysis (). Cardiac catheterization revealed complete obstruction of the left upper PV, with evidence of collateralization to left lower PV circulation, suggesting chronicity. Balloon angioplasty and stenting of the left lower PV was performed with a 9 mm diameter by 26 mm long bare-metal stent, reducing the PV mean pressure difference from 15 mmHg to 4 mmHg via cardiac catheterization measurements. The left upper PV was inaccessible. He was started on heparin post-procedure to maintain stent patency.\nHe was eventually extubated 9 days post-procedure and safely transitioned to apixaban prior to discharge. Repeated chest X-rays have shown re-inflation of the left lung. Thirty-six weeks after discharge, he is doing well without recurrence of haemoptysis. |
A 46-year-old and 60 kg weight male patient was admitted in kidney transplant unit with a history of end-stage renal disease (ESRD) and on maintenance hemodialysis twice weekly since 21 months. He was having type 2 diabetes mellitus since 7 years and was on oral hypoglycemics. He was also hypertensive since 18 months and was on nifedipine retard 20 mg TID, clonidine 100 μg TID, metoprolol 50 mg OD, and furosemide 40 mg BID. During preoperative evaluation, the patient was New York Heart Association Class III having a history of breathlessness and dyspnea on exertion. Jugular venous pressure was raised. Mild ascites was present without evidence of organomegaly. His daily urine output was around 500 ml. His 12-lead electrocardiogram (ECG) showed left ventricular hypertrophy with strain pattern. Two-dimensional echocardiography revealed moderate mitral regurgitation, moderate tricuspid regurgitation, dilated left ventricle with global hypokinesia, and LVEF of 15% with moderate pulmonary artery hypertension (right ventricular systolic pressure = right atrial pressure + 44). Coronary angiography was normal. His routine blood investigations were within normal limits except hemoglobin of 8.7 g% and a serum creatinine of 6.9 mg/dl on preoperative visit. His creatinine value before starting of dialysis 21 months earlier was 9.4 mg/dl. His chest radiograph showed cardiomegaly with cardiothoracic ratio of 0.7. On airway examination, his mouth opening was 3 cm with normal dentition. He was Mallampati Class 2 with a full range of neck movements and a thyromental distance of 7 cm. All antihypertensive medications were continued as prescribed. Heparin-free hemodialysis was done 24 h before surgery. A written informed consent for surgery and anesthesia was obtained along with high risk of cardiovascular events and postoperative mechanical ventilation.\nIn the operating room, an 18-gauge intravenous (IV) cannula was secured in the right upper limb and 5-lead ECG, noninvasive blood pressure (NIBP), and pulse oximetry were attached, and baseline hemodynamic parameters were noted. A 20-gauge arterial cannula was secured in right radial artery under local anesthesia (LA), and FloTrac™ monitor for cardiac output monitoring was attached. A functioning brachiocephalic arteriovenous (AV) fistula was present in the left upper limb. Right internal jugular vein was cannulated under ultrasound guidance, after adequate LA infiltration for continuous central venous pressure (CVP) monitoring and vasopressor administration if required. His baseline IBP was 155/92 mmHg, heart rate (HR) of 64/min, oxygen saturation of 98% on room air, and CVP was 23 mmHg. Dobutamine and epinephrine infusions were kept ready for use. Under strict aseptic precaution, epidural was placed in left lateral position at T10–T11 interspace after adequate LA infiltration, with a 16-gauge Tuohy epidural needle using the loss of resistance technique. Correct placement of epidural catheter was confirmed by a negative test dose of 3 ml lignocaine 2% with 15 μg of epinephrine. A total of 15 ml of bupivacaine 0.5% with 50 μg fentanyl was given in epidural space in “graded” doses of 5 ml every 10 min while assessing the pulse, blood pressure changes, respiration, and keeping constant verbal command to the patient. IV dobutamine was started at the rate of 5 μg/kg/min after giving the first bolus of epidural and titrated according to IBP response. After 30 min of bolus doses, sensory level was T8 with adequate motor paralysis. A continuous epidural infusion at rate of 6 ml/h of 0.5% bupivacaine with 2 μg/ml was started for maintenance. Surgery was allowed to start, and IV infusion of propofol was started at the rate of 50 mg/h for sedation. Supplemental oxygen was given by Venturi mask (FiO2 0.5). Goal-directed fluid therapy was given based on pulse pressure variation. Cardiac output, cardiac index, and stroke volume variation were also considered during fluid therapy and inotropes titration. A total of 2 L of crystalloid and 200 ml of 20% albumin were given intraoperatively. One unit of packed red blood cell and 2 units of fresh frozen plasma were also given. Surgery lasted for 6 h and total loss was around 800 ml including 500 ml of ascites and 350 ml blood. At the time of release of cross-clamp, his IBP was 157/94 mmHg, HR was 67/min, and CVP was 12 mmHg. Urine output was adequate after release of cross-clamp. All vital parameters, blood gasses, blood sugar, and electrolytes remained within normal range throughout surgery. Epidural infusion was stopped on starting on skin closure. Renal transplant was completed uneventfully. Patient was shifted to postanesthesia care unit with vital near baseline and urine output at 250–300 ml/h. Postoperative analgesia was maintained by continuous epidural infusion of 0.125% bupivacaine with 2 μg/ml fentanyl at the rate of 6 ml/h along with IV paracetamol 1 g TID. |
A 43-year-old female with a past medical history of severe allergy-induced asthma and chronic nasal drip presented to the clinic with worsening cough and chest tightness for 10 days and was treated for bronchitis with steroids and antibiotics. She then presented to the emergency department with severe and acutely worsening shortness of breath, sudden onset pleuritic chest pain, and sharp radiating pain between both shoulder blades with deep inspiration. At time of presentation, the patient was afebrile with vital signs within the normal limits. Lab work was insignificant with negative leukocyte count and negative cardiac enzymes. A chest X-ray demonstrated an abnormal right cardiomediastinal silhouette with large opacity over the right mediastinum adjacent to the right atrial border. A follow-up CT scan revealed a large right-sided mass adjacent to the right atrium and extending into the right chest measuring 5.1 cm × 9 cm × 4.3 cm (). Her last imaging study was a fluoroscopy study 10 years ago that showed no indications for a mediastinal mass. An echocardiogram revealed a normal ejection fraction (55–59%), no wall motion abnormalities, and a cyst near the right atrium. The patient had continued pleuritic chest pain and difficulty breathing and the decision was made to perform video-assisted thoracoscopic surgery (VATS) for pericardial cyst removal. The patient underwent general anesthesia with a 37 French left-sided double-lumen tube. Standard ASA monitors were applied. Two large-bore peripheral IVs and an arterial line were placed for continuous blood pressure monitoring. Intraoperatively, a large cystic lesion was adherent to the pericardium (). There was no solid component and no obvious communication with the pericardium. Part of the cyst wall was left on the phrenic nerve to preserve it. The patient tolerated the procedure well, had no postoperative complications, and was discharged home on postoperative day number two. The final pathology report revealed benign, acute inflammatory pericardial cyst. |
A 22-year-old man who was involved in a car accident sustained a hemorrhagic cerebrovascular accident. His hospital stay extended for several months and was remarkable for prolonged intubation (2.5 months),tracheostomy tube placement, jejunostomy tube placement, and recurrent chest infections. During his hospital stay, the patient did not receive any lower limb physical therapy. Upon discharge, he had right-sided spastic hemiparesis with aphasia and was started on private physiotherapy and rehabilitation. He presented to our outpatient clinic after 1.5 years of physiotherapy with significant resistance and progressive limitation in the range of movement around the right hip joint. At this stage, the patient was bed ridden with the inability to ambulate independently. On physical examination, the patient was continent, the right hip joint was absolutely stiff with flexion deformity and fixed external rotation, and power was 4/5 all over the right lower extremity with intact sensation. His left hip joint had a completely normal range of motion and the rest of the physical examination was unremarkable.\nRadiographs of the right hip joint revealed evident ossification(). On 3D-CT, HO surrounding the right hip joint was seen associated with atrophied muscles and partial fusion of the proximal femur and dorsal acetabulum/ischium, causing fixed external rotation of the right femur (). SAP level at presentation was within normal limit. The bone scan initially demonstrated increased blood flow and hyperemia at the involved region, suggesting that the observed HO was immature; however, when repeated after 6 months, the bone scan was normal. The patient was consented for excision and arthrolysis. Intraoperatively, the patient sustained a stable intertrochanteric fracture and a dynamic hip screw (DHS) was inserted ().Postoperatively, there was a significant improvement in the range of motion with physiotherapy and oral indomethacin as the first two cases. Sadly, however, the follow-up was only for 1 month as the patient died from a pulmonary embolism. |
A 35-year-old male presented with left-sided exophytic scrotal mass [] that had been steadily enlarging over last 6 months. It was nontender, firm, nodular and subcutaneous in location, with ulceration of overlying skin. The mass was mobile, and not adherent to the underlying testis, epididymis and spermatic cord. There was no inguinal or abdominal lymphadenopathy. The testis and epididymis on both sides were normal. There were no local or systemic signs and symptoms suggestive of any sexually transmitted disease. There were no other complaints suggestive of any other local or systemic illness. Past medical history of the patient was unremarkable. There was no history of previous local irradiation or long term anabolic steroid abuse. There was no history of high-risk sexual behavior. General and systemic examination was within normal limits. The clinical differentials considered included warty carcinoma/sarcoma, nodular stage of Kaposi sarcoma, leiomyoma with secondary ulceration of the overlying skin and unusually large mass formed by coalesced benign warts.\nHematological examination and routine biochemical investigations were within normal range. He was seronegative for human immunodeficiency virus and venereal disease research laboratory test. Culture of urine and urethral swab was negative. Ultrasound (USG) of the mass revealed a well-circumscribed hypoechoic lesion 7 cm × 6 cm × 3 cm in the subcutaneous plane without any calcification. The underlying and contralateral testes were normal.\nWide excision of mass was done under general anesthesia, and the specimen was subjected to histopathological examination. On gross examination, the specimen revealed an ulcerated exophytic growth measuring 7 cm × 6.5 cm × 3 cm []. Cut section of the mass was grey white []. Microscopic examination revealed a spindle cell tumor in the subcutaneous location []. The tumor cells were arranged in fascicles containing eosinophilic cytoplasm and cigar-shaped nuclei revealing nuclear pleomorphism, prominent nucleoli and 3–5 mitotic figures per high power field. A few bizarre pleomorphic giant tumor cells were also present []. Immunostaining showed the presence of vimentin, desmin and smooth muscle actin []. Calretinin, CD34, S 100 and desmin [] were negative excluding the diagnosis of malignant mesothelioma, fibromatosis, liposarcoma and rhabdomyosarcoma respectively. A diagnosis of leiomyosarcoma was made. The macroscopic margins taken were free from tumor infiltration.\nHis postoperative period was unremarkable. He was started on six cycles of vincristine, doxorubicin and cyclophosphamide, given three weekly. Computerized tomography (CT scan) of chest and abdomen was performed to rule out lung and liver involvement, which did not reveal any evidence of metastasis. Whole body bone scan was also within normal limits. Radiological investigations (local USG, CT chest, CT abdomen and bone scan) were repeated at an interval of six months. There were no signs of local recurrence or distant metastasis up to one year of follow up. |
A 72-year-old woman with a history of persistent atrial fibrillation was treated with radiofrequency catheter ablation at an outside center. Her procedure included extensive substrate ablation that resulted in electrical isolation of the LAA. Despite maintenance of sinus rhythm and compliance with anticoagulation, the patient suffered a thromboembolic stroke 2 years after her ablation. A year later, she experienced a hemorrhagic stroke in a separate vascular territory. Watchman LAA occlusion was attempted but was aborted owing to complex appendage anatomy (). The patient was then referred to our institution for percutaneous LAA ligation.\nAppendage ligation was performed with the LARIAT device (AtriCure, Mason, OH). Epicardial access was first obtained using a micropuncture technique. After transseptal puncture with an SL0 sheath, we were unable to navigate a pigtail catheter into the LAA. Hand injection of contrast through the SL0 sheath failed to sufficiently opacify the appendage. Following standard LARIAT protocol, a magnet wire was placed within a balloon-tipped catheter and advanced into the left atrium through the SL0 sheath. A slight curve placed at the end of the magnet wire allowed the magnet to be maneuvered into the LAA. Owing to suboptimal TEE image quality, it was difficult to confirm that the magnet was in the anterior/superior lobe of the LAA (A). Injection of contrast through the balloon-tipped catheter suggested that the magnet was positioned in the distal appendage (B).\nA 6F angled pigtail catheter was inserted into the epicardial space and guided toward the appendage. Hand injection of 5 mL of iodixanol injectable contrast medium mixed with 5 mL of normal saline resulted in clear definition of the LAA border (C, ) and indicated that more distal positioning of the magnet was required. After the magnet was advanced to the appendage apex, the endocardial and epicardial magnets were then connected and the LAA was ligated (D). Complete LAA closure was confirmed by endocardial contrast injection against the closed appendage ostium through the SL0 sheath. After closure, 20 mL of saline was introduced into the epicardial space and then withdrawn to clear residual contrast. Fluoroscopy time was 11.0 minutes and total procedural time was 112 minutes. |
Case 1 was a 60-year-old woman. She was raised in a coastal area in a small town, in what is described as a well-functioning family, and has been trained as a healthcare assistant. Prior to moving into the nursing home, she had been married and had two adult children, with whom she had no contact. She had gradually increased her consumption of alcohol over the years, and in spite of several contacts with outpatient services for alcohol dependence, her drinking had steadily increased. After losing her job, she became increasingly socially isolated. Prior to moving into the nursing home, her home nurses visited several times per day, and they often found her in severe withdrawal, occasionally convulsing. She was underweight and incontinent. Her apartment was untidy and rarely cleaned, smelled of urine and feces and evinced her lack of personal hygiene. She was depressed and talked about suicide.\nAfter moving into the home, she gradually became stable, and was able to manage her personal hygiene with minimal assistance. She ate at meals and began to look better. She was still drinking, but at a level that did not cause problems with other residents. Occasionally, she drank heavily for 1–2 weeks. Her contact with other residents and staff stabilized, and she participated in simple practical activities. She seemed less anxious, and did not go through serious withdrawal.\nDuring the 18-month period before moving into the nursing home, she had been hospitalized nine times for periods ranging from 1 to 19 days; in total, she spent 43 days in hospital, had one outpatient visit and several ER visits. The total cost of her hospital-based care was estimated to be 154,649 DKK (20,798.74 Euros).\nAfter moving into the nursing home, she was admitted to inpatient treatment on two occasions for a total of 4 days. The total cost was 25,226 DKK (3392.64 Euros). She also had two visits at an emergency room (ER) and four outpatient visits. |
An outborn male neonate was delivered vaginally at 39 weeks and 2 days GA to a 34-year old gravida 2 para 2 mother. A neonatologist attended birth due to intrauterine diagnosed polyhydramnios and dextrocardia with normal four-chamber-view in fetal echocardiography. The infant developed severe dyspnea immediately after birth requiring intubation. Correct position of the endotracheal tube was confirmed by positive capnography. Apgar scores were 1/3/5 at 1, 5, 10 min, respectively. The birth weight was 3500 g. Our neonatal transport team was informed due to persistent ventilation problems and varying oxygenation resulting in severe combined acidosis. Suspicion of cyanotic congenital heart defect was raised and prostaglandine infusion was started. Upon arrival, we encountered a severely depressed neonate with oxygen saturation of 30% on mechanical ventilation with high inspiratory pressures and fraction of inspired oxygen (FiO2) of 1.0. To verify correct endotracheal intubation laryngoscopy was performed revealing oesophageal intubation, while the larynx appeared atretic. Indeed, endotracheal intubation could not be achieved. Eventually, hypoxemia led to bradycardia requiring two courses of CPR and high doses of catecholamine support. After placement of a laryngeal mask adequate oxygenation was achieved while ventilation only improved slightly and severe acidosis persisted. Anal atresia without fistula and cryptorchidism was diagnosed clinically. Upon arrival in our tertiary neonatal center, endoscopy revealed laryngeal atresia with an oesophageal fistula to the respiratory system. The CT scan (Fig. ) confirmed laryngeal atresia and showed long-segment agenesis of the trachea with a fistula from the oesophagus to a distal tracheal pouch (blind proximal ending) at the level of thoracic vertebrae 4–5 and tracheal bifurcation at thoracic vertebra 6, known as Type I tracheal agenesis according to the Floyd classification. Echocardiography confirmed dextrocardia with otherwise normal cardiac structures. Chest and abdominal X-ray showed fusion vertebrae, only 11 pairs of ribs, bony changes of the sacrum and pelvis. Cerebral ultrasound showed cerebral edema. The neonate met the criteria of hypoxic ischemic encephalopathy regarding the resuscitation details and the neurological assessment with a Thompson score of 10. Due to both the absence of a non-experimental therapeutic option to establish a functional airway and severe hypoxic ischemic encephalopathy we redirected care.\nIn this case, autopsy was performed and confirmed tracheal agenesis. Laryngeal atresia (Fig. ) was found with total loss of cartilage and connective tissue for the whole segment between larynx and tracheal bifurcation. Both principal bronchi emerged from the distal trachea with a proximal closed pouch (Floyd Type I; Fig. ). The tracheal pouch itself showed a fistula to the esophagus which was capable of delivering small amounts of oxygen to the lungs during ventilation through a laryngeal mask. Histologically, a blind ending of the larynx at the thyroid level was verified (Fig. ). A cut section through the fistula showed a proximally blind-ending laryngeal pouch with overlying respiratory mucosa passing over into a normally structured system of main bronchi (Fig. ). We confirmed anal atresia and additionally found an incomplete segmentation of the right lung. Because of the severe hypoxia acute neuronal and myocardial necrosis as well as petechial bleeding in serous membranes showed at the autopsy. In the end, cardiorespiratory failure occurred due to acute severe hypoxic injury of heart and brain. |
The patient was a 36-year-old nullipara referred to our fertility unit by a gynaecologist on account of 6-year history of inability to achieve pregnancy. Details of assessment prior to referral were not made available but summary revealed primary infertility due to male factor infertility. She had ovulatory cycles; tubal factor was not investigated because of no apparent risk factor. She had undergone four failed intrauterine inseminations with husband semen.\nAssessment revealed that uterus was retroverted, and day 3 FSH value is affirmed to be 9.9 I.U/L. Semen volume was 2.2 mLs, highly viscous with sperm concentration of 5 million/mL, total sperm number per ejaculate being 11 million, progressive motile sperm of 28%, and normal sperm forms of 11%. Based on duration of infertility with severe male factor couple was counseled for IVF-ICSI (in vitro fertilization with intracytoplasmic sperm injection cycle). She had a long protocol agonist cycle using daily buserelin injection and highly purified FSH/LH (Menopur). Four grade 1 embryos (three 6-cell and one 4-cell) were transferred into the uterus on day 2 of embryo life using Wallace soft embryo transfer catheter. Introducer was used and embryo transfer time was approximately 4 minutes.\nUrine pregnancy test was positive on day 20 of embryo life. TVS done on day 33 of embryo life revealed uterus devoid of gestational sac with thickness of 20 mm endometrial lining () with double gestational sacs with viable fetal poles () in right adnexum and normal right ovary. Couple was counseled on possible modes of treatment. The woman initially refused any modality that would threaten continuity of pregnancy though ectopic in location despite possible risk to her life. It was difficult to accept losing these pregnancies which she had waited 6 years to achieve. After 48 hours of diagnosis she gave consent to laparatomy when one of the two sacs started to leak blood and she developed colicky abdominal pains. She requested for transfer of unruptured gestation into her uterus at laparatomy if it was possible. At operation, findings were hemoperitoneum of 250 mLs, uterus was bulky and bound down by adhesions, left tube was not visualized at all, and left ovary was seen with difficulty. Right tube looked deformed with ectopic gestation in ampullary region and leaking spot was 5 cm short of fimbria. The patient had partial right salpingectomy because adhesions prevented total salpingectomy. She was discharged home on postoperative day 3 in stable condition.\nShe represented for another cycle ten months following the salpingectomy. Using the same protocol above, she had three embryos (four-cell grade 1, two-cell grade 1, and one-cell embryos) transferred on day two of embryo life. She became pregnant and ultrasound diagnosis of triplet gestation was made. One of the fetuses was noticed to have died at gestational age of twenty-four weeks. She subsequently had elective abdominal delivery of living twins (female 2.7 kg and male 2.4 kg) and a fetal papyraceous at 37 weeks of gestation. |
A 63 year old man was referred from a primary health center with a penile lesion of 8 months duration. The lesion started as a small, pruritic, slightly thickened brown discoloration on glans penis, for which a topical corticosteroid cream was prescribed with a clinical suspicion as lichen planus. But it continued to grow to reach the present size with recent surface ulceration.\nThere was no history of any systemic disease except he has been on diet for glucose intolerance.\nNo history of exposure to risk factors of sexually transmitted diseases obtained. There was history of mild intermittent voiding and post void dribbling. No history of fever, weight loss or any other skin diseases. Local examination revealed an ulcerated firm, reddish brown, slightly tender nodule of 8 mm. on glans penis near corona on the ventral aspect of the penis (Fig. a).\nNo other similar lesions seen in genitalia. No inguinal lymphadenopathy noted. Dermatologic examination also did not reveal any skin or oral lesions.\nThe laboratory investigations for CBC, serum creatinine, serum PSA and complete urine analysis including urine culture did not show abnormal result. Swab culture from ulcer was negative. The blood HbA1c was 5.8%. The serological tests for HIV, HBsAg and RPR were negative. The pelvic ultrasound showed moderately enlarged prostate (size of 45 g.), but otherwise normal study.\nAfter a signed consent, the patient underwent an excisional biopsy with the clinical impression of pyogenic granuloma. The initial histopathology report revealed atypical spindle cell proliferation with clear resection margins and advised immunohistochemical studies for final diagnosis. The immunostains revealed the spindle cells were immunoreactive for CD31, CD34 &HHV8 LANA-1 and non-reactive for SMA. (Fig. b, c, d); consistent with Kaposi sarcoma.\nOn consultation, uro-oncologist advised close clinical follow - up for any recurrence. No local recurrence or systemic lesions observed during last three years of follow up. |
A 31-year-old woman, gravida 2 para 1, presented for a prenatal ultrasonographic examination at 36 gestational weeks owing to a suspicion of a fetal thoracic wall defect. Her personal history revealed a spontaneous abortion and no consanguinity. She underwent routine ultrasonographic examinations at 13, 22, and 30 gestational weeks at a regional hospital; however, at 35 gestational weeks, ultrasonography revealed an abnormal fetal thoracic wall.\nPrenatal ultrasonography revealed a fetal thoracoabdominal wall defect with partial displacement of the left ventricle and the liver associated with rotation and elongation of the heart and a high index of clinical suspicion for intracardiac malformations such as tricuspid atresia, a ventricular septal defect, and pulmonary artery hypoplasia (Figures –).\nBased on the aforementioned findings, she was admitted to the Obstetrics and Gynecology Clinic in Târgu Mure at 39 gestational weeks, where she underwent a cesarean section. The male newborn weighed 3,100 g with an APGAR score of 7. Clinically, he demonstrated a superior abdominal wall defect, a partial extrathoracic displacement of the heart, and a partially herniated liver (these structures being covered by a very thin skin layer), and also a diastasis of the sagittal suture (Figure ). The newborn was intubated, and we applied a saline-soaked gauze pad on the thoracoabdominal and cranial defects to maintain humidity.\nPostnatal echocardiography confirmed the prenatal diagnosis and also showed a partial extrathoracic and extra-abdominal displacement of the heart and liver, a large ventricular septal defect, great arteries originating from the left ventricle with the aorta situated anteriorly, a posterior deviation of the outlet septum causing severe subpulmonary stenosis, hypoplasia of the pulmonary artery, and a large hourglass-shaped left ventricle secondary to narrowing of the heart at the level of its extrathoracic displacement.\nWe also performed thoracoabdominal CT-angiography, which showed complex cardiac malformations consisting of large ventricular and atrial septal defects, an increased left ventricular volume, with apical extrathoracic aneurysmal dilatation below the xiphoid process at the level of the abdominal midline, hypoplasia of the right ventricle, and a reduced caliber of the pulmonary trunk artery. Abdominal CT revealed partial transparietal herniation of the left hepatic lobe adjacent to a left ventricular diverticulum, and an increase in the size of the right adrenal gland with hyperdense contents suggesting an adrenal hematoma. Cranial CT revealed a diastasis of the sagittal suture causing subcutaneous herniation of the venous sagittal sinus.\nFollowing admission to the Neonatal Intensive Care Unit, the newborn was administered ampicillin and amikacin, fluconazole, prostaglandin E, and phenobarbital (because he presented with multiple seizures), and also received daily dressing changes. During the first week of life, he showed multiple episodes of bradycardia and low oxygen saturation despite undergoing orotracheal intubation; therefore, surgical intervention was postponed until he was hemodynamically stable. He underwent surgical intervention at 14 days of age, consisting in the replacement of the heart inside the thorax via a systemico-pulmonary shunt procedure, with vascular prosthesis, the ligature of both persistent arterial canal and pulmonary artery trunk, and repair of the diaphragm defect. The abdominal wall defect was also sutured, but the thorax remained open. The surgical procedure was performed in extracorporeal circulation, and lasted 4 h and 15 min.\nPostoperatively, the newborn developed multiple episodes of tachyarrhythmia and low cardiac output suggesting an inability of the heart to adjust to the intrathoracic pressure. Unfortunately, the newborn died 5 h postoperatively secondary to progressive hemodynamic deterioration, metabolic acidosis, and hypoxia. |
An ill-appearing 19-year-old male with the one-year history of asthma presented to the emergency room with non-specific symptoms including fatigue, dyspnea, numbness in the right leg, nausea, vomiting, and dizziness. Two months prior to presentation, he had a sinus surgery and within few days after this surgery, he developed cough and dyspnea, so he was admitted to outside hospital for possible pneumonia. He was treated with cefuroxime, Tamiflu, and oral prednisone. He improved momentarily with steroids. Two weeks later, he returned to the outside hospital complaining of right foot plantar numbness and dyspnea, he was discharged home on Levaquin as they thought he may have some residual sinus disease left. One week later he was seen by a pulmonologist at outside hospital and they noticed that one of the cultures grew staph, hence started on Bactrim. He took Bactrim for three days and his mother noticed that he developed some mental status changes, hence Bactrim was stopped. After this, no more symptom of mental status change was noticed. Over the next few weeks, the patient noticed tachypalpitations, continued to have fatigue, shortness of air, and fatigue so the family decided to come to our hospital's emergency department for further workup. While in the emergency room, he was found to be in atrial fibrillation with the rapid ventricular response and elevated troponins. The patient spontaneously converted into sinus rhythm within 10 minutes. His vital signs were stable except for tachycardia with a heart rate of around 100 beats per minute. Physical examination was unremarkable with a normal sensation on right leg and foot. He was admitted to cardiac intensive care unit for further workup due to elevated troponin.\nSalient laboratory values and electrocardiogram\nThe patient’s initial complete blood count was remarkable for white blood cell of 28,800/ul with eosinophil count of 12,960/ul (45%) in spite of the use of low-dose oral corticosteroids for a few days prior to admission. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were both elevated at 35 and 4.14, respectively. His admission troponin was 16.28. His initial electrocardiogram (ECG) showed atrial fibrillation with a heart rate of 161 beats per minute, non-diagnostic Q waves in the inferior leads, T-wave inversions in the inferior leads and no significant ST segment changes noted (Figure ). His repeat ECG 10 minutes later when he converted to sinus rhythm showed sinus tachycardia with a heart rate of 100 beats per minute, Q and T changes as noted earlier, as well and no significant ST segment changes noted (Figure ). Other labs, including TSH, UDS, BNP, lactate, and renal function, were unremarkable. Rheumatological workup including anti-nuclear antibody (ANA), perinuclear antineutrophil cytoplasmic antibody (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA), rheumatoid factor, myeloperoxidase (MPO) antibody, serine protease antibody 3, and anti-cyclic citrullinated peptide (anti-CCP) IgG was inconclusive. However, the patient’s IgE and IgG were both markedly elevated. Several infectious causes, such as histoplasma, coccidioides, strongyloides, cytomegalovirus (CMV), human immunodeficiency virus (HIV), tuberculosis (TB), Epstein-Barr virus (EBV), hepatitis B, and hepatitis C, were explored and all were negative.\nImaging\nTransthoracic echocardiogram revealed an ejection of 55% with some apical hypokinesis. The transesophageal echocardiogram showed no evidence of endocarditis, thrombus, shunt, or atherosclerosis. Computed tomography angiography (CTA) of the chest with and without contrast showed moderate mediastinal and bilateral hilar adenopathy in addition to bilateral axillary lymphadenopathy, bilateral peribronchial thickening, and patchy ground-glass opacities most predominantly in the posterior lower lobes. There was no evidence of pulmonary embolism (Figure ). Cardiac magnetic resonance (CMR) showed several areas of delayed enhancement within the left ventricular myocardium and decreased perfusion in the mid to apical septal and inferior segments throughout the apex. It also revealed a small pericardial effusion and minimal hypokinesis of the lateral apical wall (Figures -). Due to the abnormal myocardial enhancement, a CT of the heart with coronary CTA was ordered which showed normal coronary artery anatomy with no evidence of stenosis, calcified plaque, or soft plaque (Videos -). Due to his reported neurologic symptoms, CT of the head without contrast was ordered and showed two areas of low-attenuation within right frontal white matter. MRI of the head was subsequently performed which showed many small bilateral punctate infarcts throughout cerebrum and a few additional ones in the cerebellum.\nBiopsies\nA bone marrow biopsy showed normocellular bone marrow for age and no concern for dysplasia; however, both the bone marrow biopsy and peripheral blood smear showed marked eosinophilia with leukocytosis. Several transbronchial cryobiopsies were taken from the left lower, upper lobes and lingula of the lung which showed patchy areas of eosinophilic venulitis with dense eosinophilic infiltrates involving many of the small venules. This process was happening in the background of chronic bronchiolitis with abundant eosinophils within small airways, smooth muscle hypertrophy, and goblet cell metaplasia (which suggests asthma). All these findings taken into consideration together suggested EGPA (Figure ).\nThe patient was initially started on 1000 mg of intravenous methylprednisolone for three days and then 1 mg/kg/day of oral prednisone for several months with a gradual taper. He was also started on cyclophosphamide for three to six months. Additionally, due to the patient’s young age, arrangements for sperm preservation were made prior to starting cyclophosphamide. The patient responded well to the treatment and at his one-month rheumatology follow-up, the patient continued to improve. His troponin-I reduced to 0.08 at one month visit. |
A 14-year-old male with DMD has been on daily oral steroid since 9 years of age. He weighed 53 kg (47th percentile) and was 147 cm tall (less than 3rd percentile). He became nonambulatory at 12 years of age. Forced vital capacity (FVC) was 2.37 L or 80% predicted. A polysomnogram was completed and revealed moderate obstructive sleep apnea. His echocardiogram (ECHO) was normal with cardiac magnetic resonance imaging (MRI) demonstrating normal left ventricular ejection fraction (LVEF). He participated in many school activities including wheelchair soccer.\nHe presented to the emergency department with confusion, tachycardia, tachypnea, and fever up to 39.2 degrees Celsius a few hours following soccer practice. During practice, he had been transferred from his wheelchair and his right leg had brushed the ground causing him to have to be placed on the ground until more help was obtained to put him back in his wheelchair. Brain computed tomography (CT) was negative for any acute intracranial process contributing to his current state. Chest computed tomography (CT) scan was negative for pulmonary embolism but showed patchy diffuse nodular airspace opacities seen scattered throughout both lung fields (). He had progressive acute hypoxic respiratory failure requiring mechanical ventilation. Shortly after intubation, he suffered cardiac arrest requiring 3 minutes of cardiopulmonary resuscitation (CPR) before return of spontaneous circulation. ECHOs were consistent with increased pulmonary vascular resistance including moderate right ventricle dysfunction and elevated pulmonary arterial pressure. Duplex ultrasound evaluation of lower extremities was without evidence of deep venous thrombosis bilaterally. X-rays of lower extremities revealed cortical step off at the proximal left femoral neck and nondisplaced fracture at the distal right tibial metaphysis and fibular metaphysis (). Ophthalmologic exam revealed Purtscher-like retinopathy. The patient was treated with 48 hours of antibiotics that were discontinued with negative blood, urine, and mini bronchoalveolar lavage (BAL) culture. The patient was treated with mechanical ventilation and right ventricular afterload reduction with milrinone and inhaled nitric oxide for pulmonary hypertension. These therapies were able to be weaned off, and the patient was able to be extubated on day five of admission. He was later transferred to the pediatric floor and later discharged home without any residual effects from the entire event. |
A 62-year-old female with no significant past medical history presented to the emergency department in November of 2017 with complaints of arthralgias, most notably in her right knee, left shoulder, and bilateral thighs that made it difficult for her to ambulate. She was also admitted due to a headache that was triggered primarily by coughing. Vital signs on admission were as follows: a blood pressure of 202/90 mmHg, a heart rate of 137 bpm, a respiratory rate of 20, and a temperature of 36.6 Celsius. Physical exam revealed Janeway lesions. She was found to have a neutrophilic leukocytosis, with white blood cell count at 20.4 cells/mm3 and neutrophils at 17.4 bil/L. Troponin was elevated at 1.85; this was deemed to be noncardiac in nature as the patient's pain was relieved with ibuprofen and her EKG showed no acute findings. ESR and CRP were elevated at 95 mm/hr and 24.8 mg/dL, respectively. A computed tomography of the brain showed a high-density mass in the right occipital lobe, with surrounding vasogenic edema. The patient continued to deny any visual changes or symptoms other than what was discussed above. An ophthalmologist was consulted to perform a dilated fundus exam, which was positive for small intraretinal hemorrhages that were deemed to be secondary to the patient's hypertension and less likely positive for Roth's spots. There was no evidence of disc edema. A brain MRI with and without gadolinium showed multiple small punctate bilateral areas of acute or subacute infarctions indicative of embolic phenomenon. The hemorrhagic area in the right occipital lobe was again identified, with subtle surrounding enhancement; the differential diagnosis consisted of neoplasm, vascular malformation, or embolic infarction with hemorrhagic conversion. A transthoracic 2D echo was without vegetation, so a transesophageal echo was ordered, and vegetation was shown on the posterior leaflet of the mitral valve. Two blood cultures from admission then came back positive for Rothia dentocariosa. Infectious disease was confirmed, and the patient's current antibiotics, which consisted of vancomycin and ceftriaxone, were switched to penicillin G on a continuous pump. The patient remained largely asymptomatic during her admission and was deemed to be stable for discharge from the hospital after a nine-day stay with penicillin G via a continuous pump for a total of six weeks and was planned for a follow-up MRI in three weeks. The repeat MRI came back showing new subacute strokes. The patient was reported, again, to be asymptomatic but was directed to come straight to the emergency department. A repeat transesophageal echo was done and showed the known vegetation on the mitral valve with new vegetation seen on the PICC line and an abscess between the mitral and aortic valves extending into the ascending aorta. The patient then requested transfer to another institution for further evaluation. A repeat transesophageal echo was completed at this outside institution which showed small anterior and posterior mitral leaflet vegetation with no significant destruction and no abscess. A cardiac MRI was then performed which showed a focal delayed enhancement in the apical inferior and lateral wall, likely secondary to coronary arterial embolization. The patient went on to complete the full six weeks of penicillin therapy, remained asymptomatic, and refused a mitral valve replacement. Her follow-up was continued in the cardiology clinic. |
A previously well 87-year-old Caucasian woman living in a senior assisted care center presented to the neurology clinic with complaints of six months of slowly progressing left sided weakness. Initial difficulty in ambulating and using the stairs progressed to being wheelchair bound. Neurologic exam revealed diffuse 3/5 left sided weakness, left leg drift, and left facial droop. Brain magnetic resonance imaging (MRI) revealed a large confluent white matter T2-hyperintensity in the right frontal lobe with multifocal nodular enhancement of the left cerebral hemisphere (). Foci of enhancement were also identified in the cerebellum and leptomeninges. The radiologic differential diagnosis included vasculitis, lymphoma, and CNS sarcoidosis as the most probable causes of the multifocal disease process, with glial neoplasm, demyelination, and metastases considered less likely.\nAll sample analysis described below were performed on material obtained by brain biopsy as part of clinical care. All samples were obtained with appropriate consent.\nA biopsy of the mass was performed and revealed extensive parenchymal lakes and vascular and perivascular deposition of amorphous, amyloid like material (). Congo-red positive staining and apple-green birefringence (not shown) of the amorphous material upon polarization confirmed that the amorphous material was amyloid (). Also present in the resected tissue were a number of small intraparenchymal blood vessels with perivascular lymphoplasmacytic infiltrates (). The initial histologic differential diagnoses included cerebral amyloid angiopathy-inflammatory type (CAA-I) and lymphoma associated amyloidoma. To identify the underlying etiology of the amyloid accumulation, a number of additional analyses were performed.\nLiquid chromatography tandem mass spectroscopic analysis identified the amyloid as AL λ-type and not β amyloid or an amyloid associated with a hereditary amyloidosis. Further analysis of the perivascular lymphoid populations was undertaken. Histologically, the monotonous populations of perivascular lymphoid cells demonstrated a lymphoplasmacytic appearance (). Immunohistochemical analysis demonstrated that the lymphoid cells were CD20 positive (). Tumor cells were negative for CD3, CD5, BCL1, and CD23. The tumor Ki67 proliferation index was low (3%). The more plasmacytoid appearing cells were CD138 positive and were shown to be lambda light chain restricted by kappa and lambda chromogenic in situ analysis (Figures and ). An immunoglobulin heavy chain (IgH) gene rearrangement analysis of the brain tissue from this case was positive for a clonal process with a 253-base pair peak in the FR2 region. A MYD88 L265P mutation analysis by PCR-based pyrosequencing on the brain tissue from this case was negative. A diagnosis of a low grade, lymphoplasmacytic lymphoma (LPL) was rendered. The identification of this CNS low grade lymphoplasmacytic lymphoma confirmed the cause of the amyloidoma to be a lambda light chain producing lymphoplasmacytic lymphoma.\nTo determine if an extracranial/systemic lymphoplasmacytic lymphoma was the source of the CNS neoplasm, a bone marrow biopsy was performed. The bone marrow biopsy showed normal trilineage hematopoiesis and no evidence of lymphoma, myeloma, or amyloidosis. Cytogenetics and fluorescent in situ hybridization studies on the bone marrow were negative for genetic aberrations. Urine protein and serum immunoglobulin levels were within normal limits. A biopsy of subcutaneous abdominal adipose tissue was negative for amyloid, demonstrating lack of evidence of systemic amyloid deposition. Interestingly, an IgH gene rearrangement analysis on the bone marrow was positive for a clonal gene rearrangement with two peaks: a 282-base pair peak in FR2 region and a 120-base pair peak in FR3 region in a polyclonal background, which importantly were markedly different from the IgH gene rearrangement identified in the CNS lymphoplasmacytic lymphoma. Since the two-small bone marrow clonal peaks are present in a polyclonal background, their significance is uncertain and may be age related.\nOur patient received one cycle of chemotherapy with Rituximab for Primary CNS lymphoplasmacytic lymphoma. Two months after diagnosis, she developed a hemorrhagic infarct on the left frontal white matter and was transferred to hospice care. |
A 39-year-old left-handed woman presented to the neurosurgery clinic with a history of right-sided arm and shoulder numbness for almost 5 years. Her occupation involved desk work. She had been previously diagnosed with neurogenic TOS due to the presence of numbness in her right shoulder and arm radiating down the hand and fingers in association with right neck tightness. She underwent physical therapy that included therapeutic exercise, soft-tissue and joint mobilization, neuromuscular reeducation, and taping, but these did not alleviate her symptoms. Specific discomfort affected the right shoulder both anteriorly and posteriorly, radiating down the arm, occasionally affecting either the medial two digits or the lateral three digits, with sporadic numbness in all digits. There were no symptoms in the left arm, except very mild infrequent numbness. Symptoms did not change according to the weather, tended to worsen while sleeping or with overhead movements, and improved with physical activities in general, except running. She had a past medical history of migraine.\nOn physical examination, motor examination showed 5/5 strength in all muscle groups. Deep tendon reflexes revealed biceps reflex spreading bilaterally, otherwise normal reflexes were elicited in both upper and lower extremities. She had a right-sided Hoffmann's sign. All thoracic outlet maneuvers were positive, including Adson's test, Wright's test, and Roos' test, with sensation of cold and numbness with arm elevation on the right side. She had a Tinel's sign over the right infraclavicular brachial plexus and percussion tenderness over the right supraclavicular brachial plexus.\nNerve conduction studies revealed mild right spinal accessory neuropathy, which is considered to be incidental.\nCT scan of the cervical spine showed a cervical rib bilaterally at C7. Doppler vascular studies showed bilateral impingement on the subclavian artery with thoracic outlet maneuvers. Magnetic resonance imaging of the cervical spine and of the brachial plexus ruled out both foraminal stenosis and the presence of any masses.\nIn light of the failure of conservative therapy, the patient was offered a right thoracic outlet decompression with removal of the cervical rib.\nSurgery was performed through a standard right supraclavicular approach. The anterior scalene muscle was resected including the fascial band underneath. The C7 rib was identified and resected with Kerrison rongeurs. The dorsal scapular artery was noticed coming off of the subclavian artery, and ran directly over the inferior portion of the brachial plexus, clearly compressing it. It was thought that pulsations from this artery could add to the compressive pathology on the nerve, and shredded non-adherent cotton was slipped underneath the artery to separate it from the nerve (\n). Upon detailed examination, the artery felt widely decompressed and free, and vascular decompression was thought to have been achieved at this point. Closure was performed per routine.\nAt 3-month follow-up, the patient reported a 70% improvement in numbness. At 1 year, her numbness was almost completely gone, and electromyography performed 2 years after the surgical procedure was normal. At 30-month follow-up, the patient had complete resolution of pain. |
A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition. |
A 34-year-old women in the second trimester of pregnancy was admitted to the emergency unit complaining about intensive moderate pain on lower portion of the abdomen with predominance to the right side for 3 weeks that worsened in the last 2 days. The patient did not present other symptoms such as fever, inappetence, nausea, or vomiting. Based on the abdominal pain diagnosis with predominance in the right Iliac fossa, laboratorial exams and a total abdomen ultrasonography (USG) were requested in the radiology emergency unit to assess inflammatory and infectious disease activities.\nThe USG confirmed the pregnancy without evident alterations related to the pregnant uterus, to the placenta, and to the fetus ( ). The directed evaluation of the right iliac fossa did not characterize suggestive signs of acute appendicitis. In this context, these signs would be hyperechogenicity and densification of the pericecal fat, locoregional lymphonodomegaly of reactional aspect with cortical thickness and, perhaps, the increase of the flow mapping with colour Doppler, and the presence of laminar free liquid, or locoregional collection. Caecal appendix was thicker in the body portion, reaching 9.2mm of maximal transverse diameter (the normal value is considered up to 6.0mm) and segment increase of the caliber in this region, without other inflammation characteristics ( ). In the thickened region, the light of the appendix was collapsed, and the wall of the organ had an increased echogenicity, in a homogeneous format, and with similar echotexture to fat. The appendicular compressibility was slightly reduced. In the appendicular apex, the external diameter was within normal limits (4.5mm). In the Doppler colour mapping no increase of flow was observed. In addition, no signs of hypercogenicity of periappendicular fat were seen.\nThe laboratorial exams did not show alterations (leukogram and C-reactive protein values were within normal ranges). Findings from the USG was not suggestive to acute inflammatory process, however they showed changes with benign aspects probably due to the fatty infiltration. A MRI exam was requested to complement the diagnosis.\nThe MRI confirmed the findings described in the USG with focal appendicular parietal thickness without inflammatory signs ( and ). We observed diffuse hyper signal of appendix on in-phase sequence with loss of signal on out-phase sequence ( ), therefore, indicating the presence of expressive amount of fat in intracellular compartment; a characteristic of lipomatosis. No significant highlight in the appendix was observed from the MRI exam. The clinical and laboratory findings, the USG, and MRI enabled to confirm the diagnosis of lipomatosis. The patient was discharged with analgesia, and return of symptoms was not encountered during follow-up. |
A male patient, aged 22 years, sought treatment for upper and lower anterior protrusion. He had no significant medical or dental history. Pre-treatment records showed a convex profile and mentalis strain with protrusive upper and lower lip. He had a 2 mm over jet and 2 mm overbite. The maxillary and mandibular incisors were severely proclined. Molars and canines were in Class 1 relationship (Figures and ). The lower midline was shifted to the left by 2 mm.\nThe panoramic radiograph showed that all teeth were present. Ceplalomeric analysis showed a Class 1 skeletal pattern with low mandibular plane angle. The maxillary incisors were proclined by 6 mm and 14° and mandibular incisors were proclined by 12 mm and 19°. Based on these findings, the patient was diagnosed with Angle’s Class 1 malocclusion with bimaxillary protrusion.\nThe treatment objectives for this patient were to achieve a normal soft tissue profile and to maintain ideal over jet and overbite. By correcting the protrusion of maxillary and mandibular anterior teeth, the lip incompetence would be eliminated, and the acute nasolabial angle would be improved. This would lead to better facial aesthetics and maintain the normal incisal and canine guidance.\nThe treatment plan to achieve these treatment objectives was established as follows: The maxillary and mandibular anterior teeth would be retracted after extraction of maxillary and mandibular first premolars.\nA maximum or a Group A anchorage was essential to maintain a Class I molar and canine relationship throughout the entire period of treatment.\nThe maxillary and mandibular first premolars were extracted. All teeth were sequentially bonded and banded with 0.018 × 0.025 inch pre-adjusted edgewise brackets (Roth-type prescription). Initial alignment phase was completed in 4 months.\nFive months later, the maxillary and mandibular anterior teeth were retracted with a continuous tear drop loop (0.017 × 0.025 inch titanium molybdenum alloy) (). The anterior and posterior segments were stabilized separately. The loop was activated 2 mm initially alpha 15°and beta 25° and reactivated when a space of 1.5 mm was closed every month. This procedure was repeated until the extraction space was closed. After 9 months of retraction of the maxillary and mandibular anterior teeth, the extraction space was closed.\nAfter 16 months of treatment, the fixed appliance was removed with proper over jet and overbite and Class 1 molar relationship. Fixed retainers were given from second premolar to second premolar in maxillary and mandibular archs for retention.\nThe post treatment facial photographs showed marked improvement of the facial profile, and the patient’s smile improved. Maxillary and mandibular anterior teeth protrusion were corrected, and a Class I molar relationship and over jet and overbite was maintained (Figures and ). The upper incisors to SN plane had decreased from 36° to 22° and the lower incisors to SN plane decreased from 45° to 28°. The movement of the maxillary and mandibular incisors contributed to correction of the soft tissue profile, and mental is strain. |
A 70-year-old man was admitted to our institution in September 2003 because of aggravation of blood sugar control. Subsequent abdominal computed tomography (CT) showed dilation of the main pancreatic duct. He had no other symptoms. No other abnormality was apparent by CT, except dilation of the main pancreatic duct. Although the fasting blood sugar level and HbA1c were abnormal (230 mg/dL, normal range: 60 - 110 mg/dL, and 6.3%, normal range: 4.3 - 5.8%), other laboratory findings, including tumor markers (CEA and CA 19 - 9), were normal. Screening upper gastrointestinal endoscopy showed a flat elevated tumor with central ulceration in the 2nd portion of the duodenum (). Subsequently, duodenoscopy using a side-view endoscope showed that the tumor was located in the descending duodenum where the minor duodenal papilla should have been situated (the major duodenal papilla was normally visible); furthermore, absence of the minor duodenal papilla elsewhere was confirmed (). Biopsy results indicated moderately differentiated adenocarcinoma. Endoscopic retrograde pancreatography (ERP) via the major duodenal papilla revealed no apparent abnormality except a slightly dilated main pancreatic duct and obstruction of the accessory pancreatic duct (). To assess the tumor's origin and staging, endoscopic ultrasonography (EUS, 7.5 MHz, UM230; Olympus Medical Systems Corp., Tokyo, Japan) was performed using a deaerated water-filled method in the duodenum. Those results revealed an elevated hypoechoic mass in the minor duodenal papilla with retention of the muscularis propria of the duodenum (), suggesting that the tumor existed only to a limited extent in the minor papilla and did not infiltrate into the pancreas. According to TNM classification of the tumor in the duodenal major papilla, this case was inferred to be T1.\nFor treatment, pylorus-preserving pancreaticoduodenectomy was done. The tumor was found in the minor duodenal papilla and macroscopic examination of the resected specimen showed that the tumor was 11 mm × 8 mm. Microsopic examination showed a well-differentiated adenocarcinoma, and the tumor cells surrounded the orifice of the minor papilla with a slight invasion into the submucosa (). There was no finding of lymph node metastasis or infiltration into the pancreas parenchyma (). These findings were thought to support the EUS findings. Because of the above-mentioned findings, we diagnosed a primary adenocarcinoma of the minor duodenal papilla. No relapse of the tumor after surgery was identified for 32 months. |
A 27-year-old housewife, resident of Uttarakhand, presented to the dermatology department with complaint of progressive papulonodular lesions on the right leg for the last 8 years. Initially, she developed asymptomatic erythematous to brownish papules 5 x 5 mm in size on the lower part of the right leg, which later increased in number and size. No history of trauma or injury was recalled. She gave the history of prior treatment with terbinafine 250 mg BD for 6–7 months and itraconazle 100 mg BD for 1 year and 200 mg BD for 2 years. As the lesions remained static, she was referred to our tertiary care hospital in November 2014. On examination, multiple discrete, round, erythematous papules coalescing to form plaques were observed on the anterior aspect of lower third of the right leg. Each papule was 6 x 7 mm, firm, and nontender. The plaques were arranged in an annular pattern with central clearing. She was advised to continue itraconazole 200 mg BD for another 2 weeks. Failing to achieve clinical improvement in December 2014, the treatment modified to itraconazole 100 mg BD and ketoconazole 200 mg BD for 5.5 months. Initially, signs of clinical improvement were evident. There was reduction in erythema, lesions were flattened, and no new lesions developed. In May 2015, ketoconazole was stopped and itraconazole was increased to 200 mg BD. However, no evidence of improvement was further noticed. The rest of the history and physical examination were unremarkable. Routine haematological and biochemical investigations were within normal limits. The skin biopsy was taken from the lesion and sent for histopathological examination and fungal and mycobacterial culture. On histopathology, chronic inflammatory infiltrates comprising lymphocytes, plasma cells, and giant cells containing brown pigmented structures were seen. Microscopic examination of the biopsy specimen using 20% KOH showed small, round, thick-walled, brownish, septate sclerotic bodies measuring 6–12 μm. The specimen was cultured on Sabouraud dextrose agar (SDA) with antibiotics and incubated at 25°C and 37°C. Slowly growing colonies were observed at 25°C after 7–10 days. The colonies were jet black, velvety, and embedded in the medium with reverse black. Based on lactophenol cotton blue (LPCB) preparation and slide culture, the isolate was identified as F. pedrosoi. The identity of the isolate was confirmed by sequencing the internal transcribed spacer (ITS) region by using the ITS1 and ITS4 primers. Comparison of the nucleotide sequence of our isolate with the GenBank database using the BLAST algorithm yielded 99% homology with F. pedrosoi (accession number KX793110). The patient was started on liposomal amphotericin B (2 mg/kg x 18 days) and cryosurgery was done. The lesions regressed completely by the end of 11 months. |
A 21-year-old Asian man from north India presented with history of recurrent episodes of lower gastrointestinal bleeding over the past six years. He had intermittent episodes, one to two per year, of passing bloody maroonish stools with occasional hematochezia. Episodes were self limited lasting two to three days, but required repeated hospital admissions with multiple blood transfusions. He had no significant past medical or family history of similar complaints. Physical examination was unremarkable. He was admitted with a fresh episode of lower gastrointestinal bleeding, which subsided spontaneously. Coagulation profile, liver function tests and hepatitis serology were normal. Upper gastrointestinal endoscopy to the third part of the duodenum did not reveal findings suggestive of portal hypertension or any other bleeding source. Colonoscopy revealed several large dilated tortuous sub-mucosal varices extending from the upper rectum, sigmoid, the entire colon extending into the terminal ileum (Figure ). Our patient was examined for portal hypertension. Doppler ultrasound revealed normal liver size and echotexture, portal vein 10 mm, splenic vein 7 mm with normal hepatopetal flow and no evidence of collaterals. Magnetic resonance portovenogram revealed no evidence of cirrhosis or portal hypertension. Selective mesenteric angiography was carried out to search for any other vascular lesions in the gastrointestinal tract. All vascular territories were found to be normal and colonic lesions were undetected on an angiogram. Small bowel evaluation with enteroclysis was normal. However, capsule enteroscopy revealed evidence of tortuous dilated vessels in distal ileum (Figure ).\nAt surgery his liver was normal and there was no evidence of portal hypertension. Intra-operatively, portal pressure measured by cannulating mesenteric veins was normal. Small bowel was normal except for the terminal 15 cm, which showed evidence of dilated tortuous sub-serosal vessels with a clear demarcation from rest of small bowel marked by a meandering dilated mesenteric vein (Figure ), confirmed by intra-operative enteroscopy. Serosal aspect of colon was normal except few dilated veins at sigmoid (Figure ). There were no collaterals in the colonic mesentery or retroperitoneum. Sub-total colectomy including the terminal ileum was performed with an ileorectal anastomosis. The rectum was relatively spared of varices. The colectomy specimen revealed multiple dilated tortuous sub-mucosal vessels in the colon (Figure ). Histology revealed large dilated thin walled vascular channels in the submucosa. |
A 26-year-old male in good health was referred to the endodontic clinic of Kyungpook National University Dental Hospital for root canal treatment of the maxillary right lateral incisor. The root canal treatment had been initiated by a general dentist, who opted for a referral after observing its unusual root canal morphology. Clinical examination revealed a sinus tract labially to the lateral incisor and swelling at this level. There was tenderness to palpation and percussion. Radiographically, a diffuse radiolucent area was noticed at the apices of roots of the maxillary right lateral incisor (). The sinus tract was traced with a gutta-percha cone to tooth #12 that showed a second root surrounded by a radiolucent area. The pulpal diagnosis was previous endodontic treatment and the periapical diagnosis was chronic apical abscess with sinus. There appeared to be two roots that had distinct root canal space.\nA rubber dam was applied, and temporary sealing material was removed. The principle canal was found easily but the second canal was not negotiated. At the following visit, because the second canal was not negotiated again while the patient had labial swelling with fever and pain, incision and drainage was performed at the labial swollen area (). At the third visit, the second canal was negotiated with ultrasonic instruments under an operating microscope. The second canal was located at the corresponding part of palatogingival groove in the pulpal chamber of the tooth. After removing dentin carefully with an ultrasonic device, an orifice was detected by using a size 10 stainless steel K-file (Mani, Tochigi, Japan). Both canals were cleaned and shaped with stainless steel K-files using the step-back technique. Radiograph revealed a narrow second canal, which was prepared with 2 mm increments per each size of file (.025 taper).\nAfter preparation, the root canals were irrigated with 17% ethylenediaminetetraacetic acid (EDTA) followed by 5% sodium hypochlorite solution and dried with sterile paper points. The root canals were obturated with gutta-percha using cold lateral compaction technique with an epoxy resin sealer (AH Plus, Dentsply De Trey GmbH, Konstanz, Germany) (). After verifying that the root canal filling was complete with a radiograph, the crown was restored with a composite resin (Gradia PA2, GC, Alsip, IL, USA). At six month examination, the tooth was asymptomatic and there was no radiolucency around the root apex (). |
A 19-year-old male patient complained of shoulder pain with no trauma history. He reported that the pain started about 6 months before, with progressive worsening. Pain was present in elevation and abduction, especially above 90°. The pain caused him to interrupt his physical activities, although he did not notice worsening during bodybuilding.\nA winged scapula was identified in physical exam, with scapulothoracic grade III dyskinesia, according to Kibler et al. []. He presented infraspinatus atrophy, complete range of motion, preserved upper limb strength, and discrete paraesthesia at the region of the medial border of the right scapula, with no other signs. There were no clinical signs suggestive of rotator cuff injury or glenohumeral instability ().\nThe imaging exams did not show significant changes. Shoulder MRI showed no rotator cuff lesion, labral lesions, cysts, or other soft tissue involvement. The cervical spine MRI did not show cervical discopathy or syringomyelia. Electroneuromyography with evoked potential of the scapular girdle evidenced diffuse axonal involvement of the long thoracic nerve, without other alterations.\nThe patient was then referred to the physiotherapy service, where he initiated a program of shoulder girdle rehabilitation focused on analgesia and passive mobilization. During ten weeks, he remained under the care of physiotherapists twice a week, but he did not notice an improvement in the pain. When he returned to the orthopedic clinic, he was informed about the possibility of surgical treatment.\nThe patient insisted on conservative treatment. We recommended strengthening of the shoulder girdle and swimming. During three months, he practiced swimming three times a week under the guidance of a physical education professional with experience in athlete training. In order to strengthen the periscapular muscles, he tried to practice the four classic styles of swimming, using floats in the lower limbs and increasing the demand on the upper limbs. Progressively, he noticed an improvement in his pain.\nThe swimming program consists of a 60-minute pool training three times a week, with increasing distances. The front crawl, breaststroke, and backstroke were alternated during training. The main set was a target mile, split in 200 m lengths with 1-minute rest (in a 25 m pool) and alternating strokes.\nHe returned after 90 days, free of pain. He presented dynamic stabilization of the scapula during elevation, and dyskinesia was no longer perceived. The force remained unchanged, but atrophy was no longer identified. The patient was satisfied with the progress made and was encouraged to stay in the muscle strengthening program in aquatic activities (). |
A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points (). |
A 76-year-old woman was referred to our hospital because of macrohematuria and right flank pain. The medical history revealed a slight mitral regurgitation as well as a substituted hypothyroidism due to a total thyroidectomy performed because of a benign pathology 40 years earlier. The patient was a nonsmoker and had no history of exposure to chemicals. Blood tests revealed no signs of systemic inflammation or renal insufficiency, only a moderate anemia was noticeable. We performed a cystoscopy, which brought no evidence of bladder pathology, but we noticed active bleeding from the right ureteral orifice. Sonography and an abdominal CT scan showed a hypodense mass with slow contrast enhancement, 80 mm in diameter and located in the upper pole of the right kidney and involving the pyelon. There was no evidence of metastases (fig. ). Suspecting an urothelial carcinoma of the right pyelon, we performed a retroperitoneoscopic nephro-ureterectomy.\nMacroscopic pathological evaluation revealed a right kidney weighing 300 g and measuring 12 × 6 × 5 cm. The upper pole tumor showed a greyish, partly hemorrhagic cut surface and a firm consistency. It measured 8 × 5 × 5 cm and showed a macroscopic infiltration of the renal parenchyma as well as a pedunculated growth into the pyelon, almost completely filling out the latter. Histological analysis revealed a monotonous tumor composed of spindle-shaped cells. There was prominent mitotic activity and a microscopic vascular invasion (fig. ). Resection margins were tumor free. Immunohistochemical analysis demonstrated positivity for Bcl-2, CD99, transducin-like enhancer of split 1 (TLE1), vimentin and focal positivity for cytokeratin (CK) 7. The other applied markers such as S100, HMB45, SMA, actin, CD34, desmin, CK22 and CK19 remained negative. The immunohistochemical pattern was typical for SCS. As the tumor revealed no epithelial component, it was classified as monophasic SCS. A definitive diagnosis was achieved by the confirmation of a SYT gene rearrangement using a SYT dual color break apart probe-based (Z-2097-50; Zytovision, Bremerhaven, Germany) FISH test (fig. ).\nThe postoperative follow-up was uncomplicated. The interdisciplinary tumor board decided not to perform adjuvant chemotherapy mainly because of the patient's age. Twenty months after surgery, the patient feels well and shows no clinical or radiological signs of recurrence. |
A 48-year-old female patient presents aesthetic problems related to the condition of the hard and soft tissue in the frontal maxillary sector (), in particular coronal fracture of 1.3, abnormality of shape, volume and color of 1.1, asymmetry of the gingival margin with relative height, and volume alteration of mesial and distal papilla. Considering the clinical and radiographic preoperative exams, we analyzed clinical case using the index PES/WES parameters. The authors [] have described the PES/WES index that combines both white and rose aesthetics parameters. In contrast to the original proposal [], the PES comprises the following five variables (): mesial papilla, distal papilla, curvature of the facial mucosa, level of the facial mucosa, and root convexity/soft tissue color and texture at the facial aspect of the site. The WES specifically focuses on the visible part of the restoration itself and is based on the five following parameters: general tooth form; outline and volume of the clinical crown; color, which includes the assessment of the dimension's hue and value; surface texture; and translucency and characterization (). All ten parameters are assessed by direct comparison with the contralateral tooth and a score of 2, 1, or 0 is assigned to all ten parameters. Thus, a maximum total PES/WES of 20 can be reached which represents the optimum condition of the hard and soft tissues of the rehabilitated site compared to the characteristics of the contralateral natural tooth.\nTo determine PES and WES, crown on 1.1 was evaluated clinically and was photographed with the contralateral tooth. The initial score is 5 as described in , given by the addition of the PES (3/10) and the WES (2/10) as shown in : the result of the PES is given by the incomplete presence of the mesial papilla (1/10), the complete presence of the distal papilla (2/10), and the major discrepancy of other parameters (0/10); the result of the WES is given by the minor discrepancy of tooth form (1/10), outline/volume (1/10), and the major discrepancy of other parameters (0/10). According to the patient we created and analyzed a study model with relative diagnostic wax-up that highlights what will be the advantages and disadvantages of the future prosthesis. The program includes the direct restoration of 1.3 using a composite resin body A2, lithium disilicate prosthetic crown on natural tooth 1.1. The crown was cemented with dual cement Variolink Esthetic using adhesive technique recommended by the manufacturer. The photographs were made using Nikon D90 and a 105 mm lens (AF micro Nikkor 105 mm 1 : 2.8 D, Nikon) with a ring flash (EM-140 DG, SIGMA-Nikon).\nWe found significant differences between the initial and final scores of the PES/WES or rather from 5 to 20 as described in Figures , , and . It is given by the addition of the PES (10/10) and the WES (10/10) as shown in : the result of the PES is given by the complete presence of the mesial papilla (2/10), the complete presence of the distal papilla (2/10) and no discrepancy of curvature of facial mucosa (2/10), no discrepancy of level of facial mucosa (2/10), and no discrepancy of root convexity/soft tissue color and texture (2/10); the result of the WES is given by the no discrepancy of tooth form (2/10), no discrepancy of outline/volume (2/10), no discrepancy of color (hue/value) (2/10), no discrepancy of surface texture (2/10), and no discrepancy of translucency (2/10). |
A 32 year old female Cameroonian gravida 4 para 3 at 34 weeks of gestation presented to the labour and delivery unit of Mbalmayo district hospital with 8 h history of severe generalized headache, expressive aphasia and right sided paralysis in an afebrile context. This was associated with blurred vision but no convulsions. There was no epigastric pain and no difficulty breathing and no history of trauma or fall. For this current pregnancy, antenatal care (ANC) was started at 18 weeks with a booking blood pressure of 100/70 mmHg. She did four ANCs and all were uneventful. During her routine four ANCs here blood pressure was always less than 140/90 mmHg and her urine dipsticks done during the four ANCs were all negative for proteinuria. She refused neurological symptoms such as headache during pregnancy. She has a history of gestational hypertension in her third pregnancy. There was no family history of chronic hypertension, diabetes and chronic kidney diseases. On examination she was afebrile with a blood pressure of 182/126 mmHg and pulse of 112beats/minute. Neurological examination revealed Glasgow coma score of 13/15, right sided hemiparesis and expressive Broca’s aphasia, no signs of meningeal irritation. The abdomen was distended by a gravid uterus with a fundal height of 35 cm, foetus in a longitudinal lie and cephalic presentation. The cervix was long, posterior, soft and closed with a station of − 1. We had a working diagnosis of severe pre-eclampsia complicated by stroke. Shown on Table are laboratory investigations done and their results.\nAn emergency obstetric ultrasound showed a life foetus with an estimated foetal weight of 2300 g at 33 weeks of gestation. Emergency cerebral non contrast-CT scan showed a 3.2 cm hyperdense region in the left parietal lobe with surrounding hypodensity due to clot retraction as shown on Fig. . Emergency management by the obstetrician consisted of MgSO4 using the Pritchard protocol [], which consisted of 14 g loading dose then 5 g maintenance every 6 h until 24 h after caeserean section; bethamethasone 12 mg intramuscular and reduction of blood pressure with nicardipine 5 mg/h. Four hours later an emergency caesarean section was done by the obstetrician under spinal anaesthesia and it let to the extraction of a life female with APGAR 8 and 10 at the 1st and 5th minute respectively and weight 2200 g. The management after caesarean section consisted of hospitalization in the intensive care unit with nicardipine titrated in an electric syringe at 2.5 mg/hour, ceftriaxone 2 g intravenous, Paractamol 1 g 8 hourly, and ringers lactate 6 hourly for 24 h. Post-operative management was done by a multidisciplinary team including a neurologist, cardiologist, intensive care physician, obstetrician, neonatologist and physiotherapist. On postoperative day 2 she was transferred from the intensive care unit to the maternity where she spends five additional days on nicardipine slow release 50 mg 12 hourly and paracetamol 1 g 8hourly and was later release after the ten days on nicardipine 50 mg daily and daily physiotherapy. Six weeks during routine postpartum visit the blood pressure was normal and patient was no longer aphasic and shet has regained the muscle strength partially. The baby was hospitalised in the neonatal unit for 10 days and discharged alongside the mother. |
A healthy 24-year-old male patient was reported to the Department of Conservative Dentistry and Endodontics with a chief complaint of broken, discolored tooth and swelling in the previously traumatized maxillary anterior region. The patient gave a history of fall and broken tooth 2 years ago. Medical history was noncontributory and clinical examination revealed a soft, fluctuant, and nontender swelling in the anterior region with fractured and discolored maxillary right central incisor []. Radiographic examination revealed well-defined radiolucency involving the coronally fractured and discolored maxillary right central incisor and lateral incisor.\nBoth the teeth presented a negative response to pulp testing and endodontic treatment for both the incisors were scheduled. The endodontic treatment procedures were conducted under magnification (loops × 2.5, Carl Zeiss, Germany). After teeth isolation the access cavities were prepared followed by shaping and cleaning and placement of intracanal medicament of calcium hydroxide (RC Cal, Prime Dental Products, India), which was renewed every 15 days for 3 months. Radiographic examination at 3 months proved no traits of healing and hence a periapical surgery was scheduled.\nThe teeth were then obturated using cold lateral condensation []. Raising a double vertical trapezoidal flap, a periapical surgery was performed and periapical pathosis was enucleated []. Maxillary right central and lateral incisors were apically resected 3 mm from the apex and retrograde cavity was prepared using ultrasonic tip. Biodentine™ was placed as retrograde restorative material of 3 mm thickness. The placement of the retrograde filling material was confirmed using a radiograph [] and the sutures were placed. Biopsy of enucleated lesion was sent for histopathologytests.\nThe patient was recalled after a week for suture removal. The patient was kept under symptomatic and radiographic observation for the purpose of postoperative evaluation of healing of the periapical intervention in intervals of 1, 3, 6, 12, and 18 months. Biopsy report confirmed the periapical pathology to be a periapical cyst. Periodic radiographic evaluation of the area of intervention showed progressive healing and 18 months follow-up X-ray showed complete bone formation and tooth was asymptomatic [Figures -]. The patient refused for an orthodontic treatment for correcting his misaligned teeth so the tooth was restored by an esthetic composite class IV restoration []. |
A ten-year-old boy diagnosed as having right eye (RE) infectious keratitis, not responding to antimicrobial therapy, was referred to our eye hospital for further management. The diagnosis of infectious keratitis was established one month prior to his presentation in an outside institution following an episode of acute corneal whitening, pain, and drop in visual acuity in his RE. A regimen of topical fortified antibiotics was implemented followed by a regimen of topical antiviral therapy with no improvement. The patient had a history of eye rubbing and progressive visual loss over the past year. However, no previous ocular examination was performed. At the time of presentation, the visual acuity in the RE was counting fingers near face (not improving with refraction) and the best-corrected vision in the left eye (LE) was 20/25 with a refraction of −6.75 + 4.75 × 55. The retinoscopy exam showed scissoring in the LE. However, retinoscopy could not be performed in the RE due to a poor red reflex. The slit lamp examination showed significant corneal protrusion with edema surrounding a rupture in Descemet's membrane in the inferior midportion of the cornea in the RE (). The cornea in the LE was clear. Anterior chamber was calm with no signs of infection. In both eyes, examination of the superior palpebral conjunctivae demonstrated mild generalized hyperaemia and a moderate papillary response, which were indicative of allergic conjunctivitis. Dilated ocular fundus examination confirmed normal posterior segment in the LE and was not visible in the RE.\nBased on clinical findings, acute corneal hydrops from advanced KC in the RE was highly suspected. Asymmetrical KC was confirmed with corneal topography (Pentacam 70700, Oculus, Germany) which revealed the presence of advanced KC in the RE and stage II KC (Amsler-Krumeich classification) in the LE (). To be noted, central corneal thickness was significantly reduced in the LE to 418 um with an inferiorly positioned corneal apex, consistent with KC. |
An eight-year-old girl presented to us with complaints of diffuse swelling of the right thigh and skin rash. Her parents denied history of fever, swelling anywhere else, any systemic complaints, bleeding from any site, or trauma. On local examination, her right thigh was diffusely swollen and tender to touch. There were no signs of inflammation. No abnormal mobility was found in the underlying femur. General physical examination revealed scaly, erythematous, brown to red papular lesions over the scalp, abdomen, inguinal region, and palms (). There was no pallor, icterus, lymphadenopathy, clubbing, edema, or swelling anywhere else. Rest of the general as well as systemic examination was unremarkable. X-ray of the limb showed a well-defined lytic expansile lesion in the diaphysis of femur with surrounding periosteal reaction (). Rest of her investigations including complete hemogram, liver function tests, coagulation profile, skeletal survey, and chest radiograph were unyielding. Patient was treated conservatively by us with a functional brace while being investigated for the cause. The skin lesion biopsy revealed inflammatory lesion with numerous uni-, bi-, and multinucleated histiocytes in the background of numerous eosinophils and lymphocytes. The patient was subjected to bone biopsy which showed similar findings consistent with Langerhans' cell histiocytosis. The follow-up radiograph of femur at two weeks showed signs of healing of the lesion. However, two weeks after presentation, the child developed alteration of sensorium and started having recurrent seizures associated with symptoms of raised intracranial pressure. There was no associated fever, signs of meningeal irritation, or focal neurological deficit. She succumbed to her illness within 72 hours. MRI head could not be done due to very sick general condition of child and CSF study was not done because of raised intracranial pressure and suspected space occupying lesion. Postmortem lumbar puncture as well as autopsy was refused by parents which would have thrown light on underlying CNS pathology. |
A 77-year-old female with hypertension, untreated hyperlipidemia, hypothyroidism, but without prior history of CAD or angina symptoms was referred to a cardiologist's office for a treadmill exercise test secondary to new onset palpitations. She denied any chest pain or pressure, shortness of breath, exertional dyspnea, or leg swelling. She quit smoking 36 years ago and has no family history of early cardiovascular diseases. She has a very distant cardiac work-up years ago, including a stress test and an echocardiogram, which the patient reported were unremarkable. Vital signs prior to the test were a blood pressure of 140/78, heart rate of 80, and a respiratory rate of 14. Physical exam was unremarkable except for a systolic ejection murmur that was graded II/VI at the base. EKG was at baseline with a normal sinus rhythm, normal axis, and occasional premature ventricular complexes (PVCs).\nThe patient underwent an exercise stress test using the Bruce protocol and was able to complete stage 1 with exercise for three minutes at a speed of 1.7 mph and a 10% incline. The test was terminated due to dyspnea and fatigue without chest pain. She reached a heart rate of 141 beats per minute which was 98% of predicted for her age. She accomplished 4.5 metabolic equivalents of exertion. With exercise, she had occasional atrial premature complexes and PVCs with a ventricular couplet in recovery. She started to notice tightness in her chest. Her peak blood pressure at the time was 218/90.\nThe patient was transferred onto a stretcher, and an IV line was started. She was given sublingual nitroglycerin, 325 mg of aspirin to chew, and one 5 mg IV push of metoprolol tartrate. She then received nitroglycerin paste and metoprolol tartrate IV every 5 min for two more doses. At that time, her EKG on the stretcher showed ST elevations in leads I, aVL, V5, and V6 with ST depressions in leads III, aVF, and V1-V3 consistent with a lateral wall evolving myocardial infarction (). She was transferred urgently to our institution for cardiac catheterization.\nThe patient underwent an emergent cardiac catheterization with left ventriculography and intravascular ultrasound (IVUS) within 2 hours after onset of symptoms. Troponin-I levels prior to the catheterization increased to 11.17 (normal less than 0.05 ng/ml). The rest of the laboratories were within normal limits including a thyroid-stimulating hormone (TSH) level. Coronary angiography showed nonobstructive coronary artery disease (pLAD 40%) and highly tortuous coronary arteries. IVUS of the proximal LAD revealed a minimal lumen area of 5.2mm2, and no ruptured plaques. Left ventriculogram revealed a left ventricular ejection fraction (LVEF) of 20% and severe mid-cavitary hypokinesis with basal and apical hyperkinesis (Figures and ). To our knowledge, this is the first case of treadmill exercise testing-triggered mid-left ventricular ballooning variant of takotsubo cardiomyopathy, whereby obstructive epicardial CAD and ruptured plaques were excluded with angiography and IVUS, respectively.\nThe patient was started on medical management with standard therapy for heart failure. A follow-up echocardiogram was done two days after the event which redemonstrated mid-left ventricular ballooning, with an improved LVEF of 35%. The patient remained asymptomatic during the course of her hospitalization and troponin levels trended down from a postcardiac catheterization peak of 16.06 ng/ml. An echocardiogram was repeated during an outpatient follow-up two weeks later which showed resolution of wall motion abnormalities and an LVEF of 45-50%. |
Case 1. A 40-year-old woman came to our observation because of a longstanding history of CD. History taking was remarkable for sudden onset of symptoms at age 26 years, with her neck turned to the left. At the time, a neurologist made a diagnosis of iCD. Pharmacological treatment (a 2-month trial with anticholinergic drugs and a single trial of botulinum toxin injection) failed. Two years later, at age 28 years, the symptoms spontaneously improved and remitted. After another 2 years, at age 30, CD recurred, with the neck turned to the right. Symptoms spontaneously improved over the following year and the patient stayed symptom-free for the next 8 years. CD then recurred 1 year before current presentation; pharmacological treatment comprising a 40-day course of therapy with an anticholinergic drug and a trial of botulinum toxin injection (Botox 45 units, U) into the left sterocleidomastoid muscle (SCM), 25 U into the right SCM, and 30 U into the right splenius capitis failed to relieve symptoms.\nHistory taking disclosed that 7 years after symptom onset her mother, too, had developed CD symptoms. The patient did not complain of pain, while stress and anxiety were reported to worsen the torticollis. Neurological examination (Video ) at current presentation revealed her neck turned to the right, with fluctuation of severity on turning the head. Neck turning worsened with attention and movement but disappeared with distraction maneuvers. Response to the sensory trick was absent. There was mild hypertrophy of the left SCM, some elevation of the right shoulder, and slight dystonic posturing of the right hand. Mild abasia during tandem gait was noted. Imaging studies (brain and cervical magnetic resonance) and laboratory tests for metabolic disorders were negative.\nThe inconsistency at the clinical history (acute symptom onset, long and recurrent periods of remission, head turning to the left and then to the right) and at clinical examination (positive response to distractive maneuvers, abasia), and lack of response to standard treatments, aroused suspicion of a possible functional overlay. A treatment decision was made. Psychological and physical rehabilitation brought progressive improvement of symptoms and complete remission within 1 month of the initiation of therapy. Follow-up assessment at 2 and 4 months after the end of treatment (Video – second and third segments) confirmed the absence of symptoms. |
A 47-year-old man presented to our referral center in November 2017, with a history of EMC of the right shoulder improperly excised with unplanned microscopic positive surgical margins (R1). The patient underwent debulking of the primary mass in September 2017 in a non-referral center, without a preoperative core-needle biopsy. The histological examination diagnosed EMC. No post-operative treatments were provided. At our first evaluation, the patient did not show macroscopic residual disease or distant metastasis both at the clinical and the radiological assessment. The pathological review of the excised specimen confirmed the diagnosis of EMC. After MTB discussion a “watch and wait” approach was adopted. After a 4-months follow-up, the clinical examination showed an elastic soft painless mass in the right shoulder. A Magnetic Resonance Imaging (MRI) of the shoulder was performed, showing a 35 × 15 mm intramuscular solid mass (Fig. ) within the right deltoid muscle. Computed Tomography (CT) did not reveal distant metastasis. The case was discussed in our MTB where the decision was made for preoperative RT, with a total dose of 50 Gy (fractionated in 200 cGy/die) which was a administered with Rapidarc technique. Post radiotherapy MRI performed 3 weeks later showed a partial radiological response, with a mass measuring 16 × 6 mm (Fig. ).\nIn July 2018, a wide surgical resection including the right anterior deltoid muscle and the lateral margin of the right pectoralis major muscle was performed, with a planned macroscopic margin of minimum 3 cm and the complete excision of the scar and the field of the previous surgery. The defect was reconstructed by right latissimus dorsi myocutaneous flap and polypropylene prosthesis (Figs. and ).\nThe size of the excised specimen was 23.5 × 10 × 3.5. Pathological examination was negative for viable tumor cells, showing only a fibrous subcutaneous scar. The patient had a complete response to preoperative RT. After MTB discussion, no further adjuvant treatments were provided. The patient did not show postoperative complications and was discharged on postoperative day 5. Neither relapse or systemic progression occurred in 24-months post-operative follow up. After the surgery the patient wore a shoulder brace for 15 days to avoid damage to the flap. Then a rehabilitation program consisting of 24 sessions (three times a week, lasting 1 h a session) was performed to improve the range of motion of the shoulder and motor performance of the shoulder girdle. No functional deficits were observed 6 months after surgery and at the 24-month follow up. Currently, after 2 years from surgery, at the clinical evaluation, the patient does not show differences in the flexion and abduction of the shoulder between right and left side (Fig. and ) and he confirms that he is leading a normal life without experiencing functional deficit or limitations in his daily life and his professional activities. |
An appropriate written informed consent for publication was obtained from the patient and his family.\nA chest X-ray during a routine medical examination of a 70-year-old male on September 2018 revealed a large tumor in the right lung field. Truncal contrast-enhanced computed tomography (CT) for further evaluation showed multiple tumors in mediastinal lymph nodes, lung, left kidney, para-aortic lymph nodes, and vertebral bodies, as well as in the right thoracic wall, which had been identified in the chest X-ray (Fig. A (A1, A2)). The left renal biopsy confirmed the diagnosis of renal cell carcinoma with multiple metastases. It was evaluated as clinical stage 4, T4N1M1 in accordance with TNM classification of Malignant Tumor 8th edition (Union for International Cancer Control, Geneva, Switzerland) and pathological grade 4. As a surgical operation was not applicable, a combination of the ICIs, nivolumab and ipilimumab, was chosen as the initial treatment (Fig. ). On the following day after the administration of the both drugs, erythema appeared on his dorsal hands and gradually spread to all extremities and body trunk on a daily basis. As the application of steroid ointment was not effective, 30 mg oral prednisolone was administered three weeks after the appearance of erythema. On the same day, nivolumab and ipilimumab were administered for the second time, because erythema was considered to be the only side effect of the first administration. Two days after the second administration, the patient began to have difficulty in elevating his upper limbs and standing up from a squatting position. Although erythema started to improve and oral prednisolone was reduced to 20 mg, he was referred to our Neurology Department by the urologist due to developing muscle weakness 1 week after the second administration. On physical examination, the patient did not have respiratory distress and was alert. He had Gottron sign, shawl sign, and mild erythema on the back, abdomen, and face. Neurological examination revealed proximal limb muscle and truncal weakness without easy fatigability. He had no gait disturbance but had difficulty in sitting up from the supine position. Cranial nerve abnormalities including impairment of ocular movement, ptosis, dysarthria, or dysphasia were not observed. All other neurological findings were normal. The blood test revealed marked elevation of creatinine kinase (CK, 17,386 U/L) (Table ) and positive for anti-Mi-2 (>150; normal range, 0–52) and anti-transcriptional intermediary factor 1-γ (anti-TIF1-γ) antibodies (50; normal range, 0–31). The patient had a lower titer of anti-Mi-2 antibody (124) and was negative for anti-TIF1-γ antibody before the administration of the ICIs. Anti-aminoacyl tRNA synthetase antibodies and an acetylcholine receptor antibody were negative. Electromyography showed active myopathic changes in the left biceps, right deltoid, and left paraspinal muscles. Low-frequency repetitive stimulation demonstrated no decrement in the left ulnar, facial, and accessory nerves. Magnetic resonance imaging of the left proximal upper limb showed edema and inflammation (Fig. B). Therefore, the patient was diagnosed with dermatomyositis, which most likely was an ir-AE (Grade 3, according to the Common Terminology Criteria for Adverse Events [version 5.0]) induced by the two ICIs, and admitted to our Neurology Department.\nAs his muscle weakness was progressing even after oral administration of prednisolone at 20 to 30 mg, he was treated with intravenous pulse therapy of 500 mg methylprednisolone for 3 days from the admission day (day 1), followed by 30 mg oral prednisolone (Fig. ). Both ICIs were discontinued. The reduction in CK level was limited to mild, and muscle weakness remained at the same level. In the evening of day 5, the patient had an acute episode of high fever (39°C) and started shivering. Systolic blood pressure dropped to 100 mmHg from 150 mmHg. As the blood test revealed elevated white blood cell count (27,800/μL) and C reactive protein level (CRP, 8.89 mg/dL), sepsis was suspected, and intravenous administration of cefepime was initiated. Next morning (day 6), the patient had pale extremities, respiratory failure, anuria due to acute renal failure, and mildly impaired consciousness, in addition to high fever. The laboratory tests showed considerable elevation of CRP, CK, D-dimer, and ferritin levels, which indicated disseminated intravascular coagulation and cytokine storm (Table ). Arterial blood showed lactic acidosis (lactate level, 6.2 mmol/L) and hypoxemia (PaO2, 57.4 mmHg). The patient's symptoms were getting worse hourly, and he was moved to the intensive care unit (ICU). His cardiac function was normal and the chest X-ray showed no abnormalities except for the known tumor in the right lung field. He was intubated due to acute respiratory failure and continuous hemodiafiltration (CHDF) was initiated for acute renal failure (Fig. ). Catecholamine was not needed to keep the blood pressure. All acute events that occurred simultaneously on day 5 were considered to indicate CRS (Grade 4, according to the Common Terminology Criteria for Adverse Events [version 5.0]) induced by ICIs, which would be steroid-resistant, rather than sepsis. Therefore, from the ICU admission day immunosuppressive therapy was strengthened with 2000 mg of mycophenolate mofetil (MMF), which is recommended in various steroid refractory ir-AEs, together with second methylprednisolone pulse therapy. In addition, plasma exchange was performed in order to remove abnormal cytokines and replace coagulation factors with fresh frozen plasma. IVIg therapy followed 6 times of plasma exchange. Blood cultures collected before the administration of antibiotics were predictably negative and sepsis was ruled out at this point. The immunosuppressive therapy and plasma exchange were effective. The patient was extubated 3 days after the intubation. CHDF was switched to intermittent hemodialysis, and he got off hemodialysis on day 29 and was discharged from the ICU. Laboratory parameters including CRP, CK, D-dimer, and ferritin levels improved gradually, and the levels of most cytokines decreased (Table ). Thrombocytopenia gradually developed and platelet count reached the minimum of 19,000/μL on day 16. Bone marrow biopsy revealed mild hemophagocytosis, but megakaryocyte count was normal. Thrombocytopenia slowly improved, possibly as a result of immunotherapy. Anemia gradually progressed and did not improve conceivably due to renal dysfunction. Additional problems that occurred a few days after the acute deterioration were intractable oral enanthema and diarrhea without abdominal pain. Tests for serum β-D glucan, cytomegalovirus antigenemia, and Clostridium difficile toxin in the ICU were negative. The biopsy of oral mucosa and tongue ruled out pemphigus vulgaris and pemphigoid, suggesting mucosal damage by the ICIs. Oral enanthema improved around day 60. Colonoscopy revealed mildly white colon mucosa, and colon biopsy showed mild lymphocytic inflammation with edema, which was compatible with colitis induced by ICIs. As it was difficult to continue enteral nutrition because of diarrhea, a central venous port was implanted for intravenous hyperalimentation. It was, of course, difficult to administer nivolumab and ipilimumab again due to the fulminant ir-AEs. Multiple tumors slightly enlarged within one month before the administration of the two ICIs, but remained almost the same size for two months after the admission, likely due to the positive effect of the ICIs. The muscle weakness did not improve possibly due to the complication of critical illness. The patient changed hospital for recuperation on day 93. |
The second case was a 48-year-old woman with an 8-year education. She was right handed. She reported subacute painless vision loss in her left eye, slow movement of the eyes, and ocular discomfort for 18 months before the neuro-ophthalmological examination. The family history did not indicate any problems; she did not report any history of previous neurological disease, tumor or traumatic brain injury. She was not a smoker. During the psychiatric evaluation, she denied any concurrent psychological issues. Co-existence of internal-medicine diseases (diagnosis of rheumatoid arthritis in 2006 and gastric banding for obesity with significant weight loss in 2014) were independent of visual field loss because of the evidence of the organic integrity of the afferent visual system. Indeed, she had mild bilateral astigmatism, not properly corrected. Her BCVA (20/20) and color vision were normal in both eyes; no afferent pupillary defect was observed in the affected eye. The external examination of orbits, slit lamp evaluation of the anterior segment, and intraocular pressure were normal in both eyes. Pupillary reactions and ocular motility were normal. Dilated fundus examination did not reveal any pathological changes in either eye. HVF revealed loss of peripheral vision in the right eye and mild and absolute loss of peripheral vision in the left eye (Cloverleaf visual field: mean deviation score of –10.95 dB for right eye and of -21.53 dB for left eye); this result is in line with the most common visual field complaints related to a concentric loss of peripheral vision, like “tunnel vision” (). SD-OCT did not show any changes in optic nerve or macular parameters. P-VEP, PERG, and ffERG revealed normal retinal function and optic nerve conduction. Brain neuroimaging showed only a few non-specific areas of altered signal in the frontal subcortical white matter. MRI did not show pathological changes in the orbits or optic nerves.\nThe procedure was conducted by FS, co-author of the work.\nTwenty-five right-handed healthy volunteers (16 women, Age M = 42 years; SD = 14; range: 23–61, Education M = 15; SD = 2; range: 8–18) participated in this study. They all reported normal or corrected-to-normal visual acuity and no history of neurological or psychiatric illness.\nAfter the experimental task, the participants completed self-report questionnaires. The Beck Depression Inventory (BDI) (; ) was used to measure the presence of depressive symptoms. The State-Trait Anxiety Inventory (STAI) was used to measure state- and trait- anxiety (; ). The Symptom Checklist 90-R (SCL-90) () was used to assess the presence of psychopathological symptoms, while the Toronto Alexithymia Scale 20 (TAS-20) (; ) was adopted to measure the level of alexithymia. No participant reported difficulties or required assistance in reading.\nThe experiment consisted of two tasks, the first was a recognition go-no go task of neutral visual stimuli, while the second involved a recognition of emotional visual stimuli. For both tasks, the participants were seated at a distance of ∼60 cm from a computer screen of which the vertical midline lay on the sagittal midplane of their trunk and head. They had to press the spacebar of a keyboard with their dominant hand to answer the questions according to the instructions. All participants completed the experimental test without any complaints about difficulties in their ability to look at the screen.\nThe stimuli were presented in black against a white background. A target (an empty square/an empty triangle) was presented in the upper or lower visual field in the following conditions: (1) in the unilateral condition, the target was presented on the right OR left of a fixation cross; (2) in the bilateral condition, the target was presented simultaneously on the right AND left of the fixation cross; (3) in the incongruent condition, the target was presented on the right OR left of the fixation cross while a distractor (an empty circle) was presented concurrently on the opposite side of the visual display. Moreover, catch trials (representing the no-go condition) in which a distractor (an empty circle) was presented unilaterally, bilaterally, or together with another distractor, were implemented in the experiment. An answer in these conditions represents a false alarm, since participants should not have provided any answer. The square and the triangle (the target) were shown independently in different blocks. Participants were required to respond as soon as possible after they noticed the target. The stimuli stayed until the participants answered or for duration of 1500 ms. The inter-stimulus interval varied randomly between 650 and 950 ms (Figure ). For each condition (unilateral, bilateral, incongruent), 32 valid trials and 16 catch trials were presented in 4 blocks (ABBA: square, triangle, triangle, square). Overall, 576 trials were administered. There was a 2- to 3-min break between blocks. Accuracy (% hits - % false alarms) and Reaction Time (RT) from stimuli onset were recorded for valid trials.\nPhotographs of male and female faces () with either an angry, a fearful, or a neutral expression, were presented in four different conditions: (1) in the unilateral condition, the target (anger/fear) was presented on the right OR left of a fixation cross; (2) in the bilateral condition, the target was presented simultaneously on the right AND left of the fixation cross; (3) in the neutral incongruent condition, the emotion target was presented on the right OR left of the fixation cross along with a neutral expression; (4) in the emotional incongruent condition, the target was presented on the right OR left of the fixation cross along with a different emotion. Moreover, in the catch trials, a distractor (represented in half the trials by neutral stimuli and in the other half by a contrasting emotion) was presented unilaterally, bilaterally, or in opposition to a neutral and another emotion stimuli. The emotions of fear and anger were studied independently in different blocks. Participants were required to respond as soon as they noticed the target. The stimuli stayed until the participants responded or for a duration of 1500 ms. The inter-stimulus interval varied randomly between 650 and 950 ms (Figure ). For each condition (unilateral, bilateral, neutral incongruent; emotional incongruent), 32 valid trials and 16 catch trials were presented in 4 blocks (ABBA: anger, fear, fear, anger). Overall, 768 trials were administered. There was a 2- to 3-min break between blocks. Accuracy (% hits – % false alarms) was measured. The negative scores on accuracy indicated a higher number of false alarms, meaning that the subject mislabeled an emotion stimulus as the target. Moreover, Reaction Time (RT) from stimuli onset was recorded relative to valid trials.\nThe analysis was conducted by FS (author of the present manuscript). The scores of each psychological questionnaire were computed according to the seminal articles (; ; ; ). For the non-emotional task, the data were collapsed together for the upper and the lower visual fields as well as for those relative to the square and the triangle. Regarding the control group’s RT, 1.2% of valid trials were eliminated due to omissions; in other words, when individuals did not erroneously provided any answer and then no information about accuracy or RT was available for the successive analyses. In terms of the emotional task, the emotions of anger and fear were studied independently. Concerning the control group’s RT, 12.62% of valid trials for anger and 7.7% of valid trials for fear were eliminated due to omissions. The two patients’ scores for each psychological subscale as well as for the experimental data were compared to the means and the SDs of the control group using Crawford’s t-test for single cases (; ). |
A 70-year-old man presented to the emergency department (ED) after falling twice at home. While standing after eating a light meal, he had two separate episodes of brief loss of consciousness. On the second fall, the patient had hit his right shoulder, cheek, and foot, prompting the visit. The patient had received a colonoscopy earlier in the day for routine cancer screening. He had followed proper protocol regarding his bowel prep and had not had any immediate complications related to the procedure or anesthesia. Since the colonoscopy, he had continuous bouts of cramping abdominal pain. He had also felt dizzy throughout this period but attributed it to dehydration related to his bowel prep. The patient denied striking his head, chest pain, shortness of breath, nausea, vomiting, or blood in his stools. His past medical history was significant for coronary artery disease, hyperlipidemia, and hypertension. The patient took his benazepril, aspirin, and atorvastatin on the day prior to colonoscopy.\nOn physical exam, the patient was afebrile with a heart rate of 87 and blood pressure of 130/78. The patient's head, neck, chest, and neurological exams were entirely normal. His abdominal exam was remarkable for tenderness in the right and left lower quadrant with some voluntary guarding, but no masses or rebound was appreciated. His orthopedic exam demonstrated tenderness to his right acromioclavicular joint and ecchymosis of his right 5th metatarsal with no deformity and normal range of motion at all joints.\nOn laboratory evaluation, the patient's hemoglobin was 12.4 g/dL, with normal platelets and chemistry studies. Head computed tomography (CT) and shoulder and chest radiography were normal. Foot radiography demonstrated a nondisplaced 5th metatarsal fracture. CT of the abdomen and pelvis demonstrated moderate hemoperitoneum with splenic laceration (Figures , , and ).\nThe patient was admitted to the intensive care unit and underwent serial abdominal exams and every four-hour hemoglobin measurements. His hemoglobin fell to 8 g/dL at 48 hours after admission but subsequently stabilized. The patient was observed in the hospital and ultimately discharged home on hospital day 3 in good condition. |
Carl was a 58-year-old African-American/Black man staying in a homeless shelter at the time he enrolled in the study, where, instead of a bed, he slept upright in a chair because of overcrowding. Carl reported he had not been engaged in any type of medical care for as long as he could remember, only visiting an emergency department if it was unavoidable. Further, he indicated he suffered from some cognitive impairment due to an earlier traumatic brain injury, making it difficult for him, for example, to plan or enact sequences of new activities. Moreover, at the time he entered the study, Carl felt run-down and sick. He had trouble walking and had been drinking heavily over the last several months. Carl reported he had never been tested for HIV, but that he suspected that he might be HIV infected. He noted that after the death of his wife 15 years prior, he had started to drink daily and engage in what he considered risky sexual behaviors with a number of partners. When Carl was offered an HIV test in the study’s first phase, he began to prepare himself emotionally for the possibility of receiving an HIV diagnosis.\nAfter receiving his preliminary positive HIV test results from the rapid test, Carl was upset, but remained calm and maintained perspective. He later recalled,\nIt really hurt when I found out I had it. It was a big blow. But I’m gonna take one step at a time. It’s a long, long road.\nImmediately after learning his status, Carl stated he was willing to obtain HIV primary care, but explained that he was unable to do so because he had no phone and no means of transportation—common barriers to HIV care among this population. After providing him with the confirmatory test results and post-test counseling in session 3, he was also given a phone and funds for round-trip local public transportation—simple and practical, yet critical, forms of assistance.\nMoreover, cognitive challenges related to the traumatic brain injury compounded the difficulties Carl faced linking to HIV care and other social services. Yet, with concrete guidance from his study interventionist, including assistance with paperwork to apply for public insurance (Medicaid), Carl was referred by the study to a comprehensive care social service agency, which provided case management, nutrition support, support groups, housing assistance, and HIV primary care—a setting that seemed optimal given Carl’s needs.\nSoon after, Carl attended his initial HIV primary care appointment at this multi-service organization. Much to his surprise, in addition to HIV infection, he was also diagnosed with diabetes. He then initiated ART and treatment for diabetes and reported being pleased with a rapid and dramatic improvement in his health. He noted, “I always take my medication. I feel healthy [now], like I was 17.”\nHowever, at times it was challenging for Carl to adhere to his HIV medications. For example, at one point he took a trip to visit family who did not know about his HIV status. Because Carl was not yet prepared to disclose his HIV status to these family members, he left his HIV medication at home rather than risk his family members’ accidently learning of his HIV status. Consequently, Carl’s VL, which had dropped dramatically in response to the ART regimen, rebounded, as shown in Figure .\nPeriodic contacts during the navigation period were used to help him identify barriers to maintaining good adherence to ART and making good decisions about his health behaviors. Upon learning of the effect his non-adherence to the ART regimen had on his VL levels, Carl was upset and re-committed to adhering well to the medication. Further, Carl enrolled in a conditional economic incentive program at the organization; that is, an intervention that reinforces adherence to HIV medication regimens with economic incentives provided when participants achieve undetectable VL. Carl found the program beneficial, as he stated:\n(I) just take my medicine, take one pill. When I miss a lot of days … [my viral load] goes back up. My [viral load] when I first went in, was like, over 100,000. Now I got it down low. I think it’s down to 22,000. But I got to get it back to 40 (undetectable). Because, see once I get it to 40 again, I get $150 bonus card.\nImportantly, over the course of his participation in the study, Carl developed positive relationships with his doctor and case manager, and reported having “complete faith” in the information they provided to him regarding management of his HIV infection. Further, Carl moved from the shelter into an apartment with a life-long friend. In navigation contacts, he weighed the pros and cons of sharing his HIV status with this person and decided to do so. In fact, this friend was an ongoing source of support and was the only person to whom Carl disclosed his status during the study. Further, through the social service organization where he received services and HIV care, Carl applied for supplemental security income and housing for HIV-infected persons. Unfortunately, though, before his city-sponsored HIV housing came through, Carl had to leave his friend’s apartment. He acknowledged this was stressful for him, saying, “I lost weight. Probably stress. Because I got to go back to the shelter.” Carl asserted, however, that he was able to maintain his health and good ART adherence while waiting for housing, even if he had to live in a shelter, noting, “no, I’ll take my medication with me. I still see my doctor.”\nCarl stated he was motivated to reduce his chances of transmitting HIV to others by taking ART, and by refraining from sexual behavior at this point. He was still considering how he might negotiate condom use and disclosure to sexual partners in the future, as he stated, “Because I wouldn’t give them the HIV. You don’t want nobody to give you that, right?”\nOver the course of the study, Carl’s physical health showed improvement. His CD4 increased. Yet consistent with his reports of inconsistent adherence, his VL data were variable (Figure ).\nCarl’s case highlights a number of themes common among participants receiving a new diagnosis. First, while Carl may have been concerned he was infected with HIV, he had never been tested for HIV. He was brought to the study through active outreach from a peer as part of RDS and agreed to be tested. With respect to the peer recruitment component of the study he noted,\nNo, I didn’t know [I was infected with HIV]. I really didn’t. So, in a way that guy like saved my life when he give me the card to come here.\nFurther, like many participants in the study, Carl was faced with a number of potent structural barriers to his timely engagement in HIV medical care, including lack of transportation and difficulties applying for public health insurance. There is growing awareness in HIV testing and clinical settings regarding the need to resolve or circumvent such barriers to health care, similar to the approach taken in the present study. Once linked to a multi-service organization, Carl developed an open and trusting relationship with his health-care and social service providers. This setting provided him with high-quality HIV care and assisted him with managing his other significant life stressors, in particular his diabetes and housing instability. Moreover, similar to Jerry’s experiences, and results in the sample more broadly, fear of stigma was a primary theme. Finally, Carl lacked confidence in his ability to negotiate safer sex and struggled to find a way to have a satisfactory sexual and romantic life while living with HIV. |
In a 19-year-old, mentally disabled male, chest radiography was done because of a sudden episode of cough. Metallic, hook-shaped foreign bodies were identified in both the main bronchi.\nThe right-sided FB was removed by fiber-optic bronchoscopy in the regional hospital, whilst the left-sided extraction failed with the left-sided FB persisting in the left main bronchus ().\nUpon urgent admission in a tertiary institution, extraction was attempted by rigid bronchoscope under general anesthesia. Bronchoscopic extraction failed, associated with some bleeding and subcutaneous emphysema immediately after the intervention. The increasing mediastinal and subcutaneous emphysema raised suspicion about an iatrogenic airway lesion, so surgery was indicated. Esophageal injury was previously ruled out by esophagoscopy, revealing many metallic FBs in the stomach. At thoracotomy, a significant mediastinal emphysema (), together with diffuse adhesions, was noticed. After the lung liberation, a proximal 0.5 cm of the noncurved part of the metallic hook was found to protrude through the perforated membranous wall of the left main bronchus, 1 cm away from the descending aorta (, arrow). The part of the FB protruding outside the bronchus was grasped by the clamp and, by following the curved shape of the FB, gentle maneuvers were applied by pulling the sharp end (hook) of the FB in the direction outside the bronchus. The FB was removed from the bronchus (insert on ) without the need for additional bronchotomy. The remaining 10 × 1 mm defect in the bronchial wall, caused both by manipulations during a bronchoscopic extraction attempt and subsequent surgical extraction, was sutured by interrupted PDS 3-0 stitches, and the lung fully inflated. No air leaks appeared during the water test. Having in mind the dimensions of the defect and tensionless suture, no suture-line protection was performed.\nAfter the thoracotomy closure, laparotomy was done and several sharp metallic pieces of different shapes were removed from the stomach (Figures and ). This was followed by an uneventful postoperative course and the discharge after 14 days.\nAfter three years, the patient was urgently readmitted for the new episode of the metallic foreign body aspiration (Figures and ). The abdominal radiography revealed metallic pieces in the digestive tract as well (). With the surgical team on site, rigid bronchoscopy under general anesthesia was done. As the tip of the FB was not impacted in the mucosa, it was possible to grasp it with the rigid biopsy forceps and to withdraw it up to the tip of the bronchoscope. Because of the curved shape of the FB and the impossibility to remove it through the instrument, the FB and bronchoscope were pulled out from the patient together, with the FB firmly grasped, followed by reintubation with the same bronchoscope (Figures and ). After a careful check-up for bleeding and mucosal damage, the patient was extubated.\nMetallic pieces left the digestive tract spontaneously after a couple of days. |
A 39-year-old man with no previous illness visited a local medical center for healthcare checkup. He exhibited no other symptoms and was observed to harbor a mass-like lesion on which a punch biopsy was performed. The initial biopsy revealed an inflammatory lesion; to further evaluate for the possibility of cancer, he was referred to a tertiary referral center. Informed consent was obtained from the patient, and the case was approved by the Institutional Review Board (No. 2019-02-046).\nThe patient underwent repeat colonoscopy, biopsy, and abdominal computed tomography (CT) in the gastroenterology department to rule out malignancy. The colonoscopy, which was performed for confirmation, revealed a fungating mass-like lesion with hyperemic mucosa around the appendiceal orifice that was not compatible with the usual findings of colonic adenocarcinoma (). The abdominal CT findings were described as severe edematous changes in the appendix, with appendicolith, suspected chronic inflammation, and wall thickening of the cecal base (). A radiologist recommended surgical evaluation to exclude cecal malignancy. Pathology of the colonoscopic biopsy revealed simple inflammatory change with chronic colitis. Regarding the differential diagnosis for Crohn’s disease, there were no specific colonoscopic findings such as skip lesions, cobblestoning, ulcerations, or strictures and no pathologic findings including transmural inflammation, granulomas, or crypt abscesses to justify that diagnosis. Even though the colonoscopic and radiologic findings did not direct the diagnosis to colon adenocarcinoma, a gastroenterologist recommended surgical evaluation of the mass to rule out malignancy of the colon. After the preoperative workup and colorectal multidisciplinary team meeting, we planned to perform single-port laparoscopic cecectomy followed by right hemicolectomy if the final surgical biopsy report revealed signs of cancer.\nRegarding surgical technique, a single vertical incision was made through the umbilical skin, and an S Port (Eraesi Medical, Hanam, Korea) was placed in the abdominal cavity through the umbilical incision. The mesoappendix was divided through sequential use of a Covidien Sonicision (Covidien, Mansfield, MA, USA) device. The cecum was divided with application of an Endo-GIA Stapler (Covidien). The appendix was removed through the umbilical incision and placed into the sterile bag component of the single-port ().\nThe resected appendix was transported to the pathology department. Microscopic findings revealed a thickened wall and purple sulfur granules in the submucosa and muscular wall as well as in the lumen (). As a result, this patient was diagnosed with appendiceal actinomycosis. After diagnosis, the patient was referred to the medical department for further antibiotic treatment for actinomycosis. After undergoing 1 month of treatment with Augmentin (Amoxicillin-Clavulanate), the patient’s follow-up CT exhibited no signs of recurring infection. |
A 31-year-old male patient attended our outpatient department with asymptomatic brownish macules and patches [] and purpuric lesions distributed on both legs near the lateral malleoli of five months duration. A few similar lesions were present on the anterior aspects of both legs []. According to the patient, lesions started as red spots and later changed in color as new lesions continued to appear. He neither gave any history suggestive of bleeding disorder or venous insufficiency nor was he on any medications. Doppler study of arterial system was within normal limits, whereas venous Doppler revealed incompetence of above-ankle perforators bilaterally. Complete hemogram, peripheral smear study, urine routine examination, bleeding and clotting time, prothrombin time and international normalized ratio were within normal limits. With the clinical diagnosis of pigmented purpuric lichenoid dermatosis, the brownish macule was biopsied, which revealed a heavy lymphoid infiltrate in the superficial and mid-dermis with epidermotropism and occasional Pautrier microabscesses [Figure and ] indicating mycosis fungoides. Perls Prussian blue stain revealed hemosiderin deposits in dermis indicating extravasated red blood cells []. Immunohistochemistry showed the lymphocytes to be CD3 [], CD4 [], and CD5 positive with few CD8 positive cells []. The patient was evaluated in detail in consultation with the hematologist. Serum calcium, liver and renal function tests, chest X-ray, bone marrow analysis, ultrasound examination of abdomen and pelvis, and contrast-enhanced tomogram of the thorax were all within normal limits with marginal elevation of serum lactate dehydrogenase. Gene rearrangement studies identified polyclonal T-cell population.\nSince new lesions continued to appear despite treatment with topical steroids, he was offered systemic psoralen 20 mg followed by ultraviolet A therapy twice a week, which after 4 weeks achieved some clearance of lesions and cessation of appearance of new lesions. The patient is presently under regular follow up with thorough clinical evaluation and complete hemogram and peripheral smear analysis every three months. |
A 36-year-old male patient was referred to our internal medicine unit because of severe thrombocytopenia. He is known to have had Addison’s disease for 11 y and was kept on oral dexamethasone 0.5 mg daily; as a replacement therapy. Six years before he had right lower limb deep venous thrombosis, for which he was prescribed warfarin for six months. Three years before he had another deep venous thrombosis in the left lower limb and was given warfarin for three months. Three months before presenting to our unit, he had another right lower limb deep venous thrombosis and was kept on warfarin till he was admitted. A few weeks before admission, he started to notice a rash over both forearms, but gave no history of spontaneous bleeding. No similar conditions were reported in his family.\nOn admission, his blood pressure was 120/75 with no postural drop. His weight was 72 kg and body mass index 25.8 kg/m2. Purpuric rash was visible over both upper and lower limbs. Initial laboratory investigation showed platelet count 14,000/cmm, prothrombin time 32 s and international normalized ratio 3.4 (on warfarin 5 mg/day). His haemoglobin level, white cell count and blood chemistry and thyroid function test results were all within normal limits. Lupus anticoagulants were positive but anti-nuclear, anti-ds DNA and anti-cardiolipin antibodies were all negative. Peak cortisol level was 1.9 µg/dL; 60 min after adrenocorticotropic hormone stimulation. Adrenal haemorrhage was ruled out by magnetic resonance imaging, which revealed marked thinning of both suprarenal glands. Duplex ultrasonography showed remnants of old femoral and popliteal thrombi in both lower limbs.\nThe patient was diagnosed to have primary anti-phospholipid antibody syndrome. Dexamethasone was stopped and oral prednisolone 60 mg/day was started. Warfarin was also stopped, and low-molecular weight heparin was given instead. Significant improvement of the platelet count was noticed within two weeks. When platelet count reached >100,000/cmm, gradual tapering of prednisolone dose was started. |
A 52-year-old male presented with a 3-week history of flashes and floaters followed by painful vision loss in the left eye 1 day prior to presentation. He had no history of myopia, previous ocular trauma, or surgery but reported a family history of retinal detachment in 2 immediate family members. On initial examination, his visual acuity was 20/200 OD and hand motion OS with intraocular pressures of 24 mm Hg OD and undetectable by hand-held tonometry OS. Examination of the unaffected right eye was within normal limits, with a 3+ brunescent nuclear sclerotic cataract, no evidence of intraocular inflammation, and an attached retina. Examination of the left eye revealed rare anterior chamber cells and 4+ brunescent nuclear sclerotic cataract which prevented adequate viewing of the posterior pole. B-scan ultrasonography revealed a funnel retinal detachment, with homogenous choroidal echogenicities suggestive of hemorrhagic choroidal detachment as shown in Figure . His medical history was significant for type 2 diabetes and hypertension but negative for autoimmune diseases and systemic malignancy. Complete blood count, basic metabolic panel, HIV, T-spot, syphilis screen, and antinuclear cytoplasmic antibody panel were all normal. Computed tomography of the brain and maxillofacial structures did not reveal any intraocular mass but demonstrated prominent choroidal detachment as shown in Figure . Chest X-ray was within normal limits. The patient was started on 60 mg oral prednisone and re-evaluated every 2 days. Serial ultrasonography revealed persistence of the choroidal detachment. One week after initial presentation, the patient underwent pars plana vitrectomy combined with phacoemulsification, drainage of serous and hemorrhagic choroidals, placement of an encircling band, and injection of a C3F8 gas bubble. Intraoperatively, inspection of the retina revealed a total retinal detachment with retinal breaks in the inferior and superior periphery. At the 1-month visit, the retina remained attached (Fig. ) and the patient's vision had improved to counting fingers near face. |
A 36-year-old woman (height, 147 cm; weight, 50 kg) with CIPA was scheduled for revision of left total hip arthroplasty. She was diagnosed as having CIPA because of recurrent episodes of unexplained fever, anhidrosis, burns, and bone fractures after birth. She had previously undergone 7 operations for spinal deformity and 1 operation of total hip arthroplasty in both the left and right sides. Although lack of general diaphoresis and thermal nociception were observed, the patient performed body surface cooling at her own discretion when she felt she was at a risk of hyperthermia, and her body temperature was kept approximately 36°C. No signs of mental retardation or orthostatic hypotension were observed. No abnormality was detected on chest radiographs and electrocardiograms. Blood biochemistry revealed no abnormality except mild anemia indicated by a hemoglobin level of 10.6 g/dl.\nNo premedication was administered. After the patient was brought into the operating room, routine monitoring and measurement of the bispectral index (BIS) were started. Body temperature was measured at 3 different sites (urinary bladder, esophagus, and precordial skin) and controlled by a hot-air-type heater. Propofol was administered at an effect-site concentration of 4 μg/ml by target-controlled infusion. After muscle relaxation had been achieved by administration of 50 mg of rocuronium, the trachea was intubated. Immediately after endotracheal intubation, systolic blood pressure increased from 130 to 145 mmHg, and heart rate increased from 60 to 95 beats per minute (bpm). Two minutes later, systolic blood pressure had decreased to 125 mmHg. Propofol was continuously infused intravenously at a target concentration of 2 to 4 μg/ml () and BIS levels were maintained between 40 and 60. After an arterial catheter had been placed, her position was changed from the supine to right lateral position. Surgery was then started.\nSince no circulatory change associated with pain occurred during surgery, opioids were not administered. Regarding hemodynamics, when 600 ml of blood was rapidly lost within 20 minutes, blood pressure decreased from 113/66 to 93/55 mmHg and heart rate increased from 55 to 70 bpm ( a). Similarly, when 850 ml of blood was lost within 30 minutes, systolic blood pressure decreased from 108/65 to 95/60 mmHg and heart rate increased from 66 to 74 bpm ( b). Administration of 0.1 mg of phenylephrine increased blood pressure from 87/55 to 117/76 mmHg and decreased heart rate from 70 to 65 bpm ( c).\nThe operative time was 6 hours and 49 minutes, and the duration of anesthesia was 8 hours and 41 minutes. The volume of blood loss was 3350 ml. Blood transfusion was performed with 1600 ml of preoperatively donated autologous blood, 900 ml of salvaged blood, and 720 ml of fresh frozen plasma. Intraoperative body temperature was controlled and kept between 36.0°C and 36.9°C at all 3 measurement points. After surgery had been completed, the patient was returned to the supine position and she was extubated. Since she did not complain of any pain after the surgery, no analgesic was administered. She was discharged at 6 weeks after the operation.\nBlood samples were collected 3 times: before anesthesia induction, after the start of surgery, and at the end of surgery. The levels of catecholamine fractions and cortisol were measured. Norepinephrine levels were below the normal range at all time points, and the levels of epinephrine and cortisol were within the normal ranges at all time points (). |
A 34-year-old female with no significant past medical history presented to our clinic after experiencing a left second metatarsal stress fracture (Figure ). One year prior, while running errands around town, she suddenly felt a sharp pain in her left midfoot and promptly consulted an orthopedic surgeon who placed her in a boot. Six months later, after experiencing minimal improvement in her pain, a different orthopedic surgeon performed an open reduction and internal fixation by injecting 1 mL of bone cement into the diaphysis of the second metatarsal.\nOver the next six months, she noticed no meaningful improvement in her pain. At this point, she presented to our clinic for a third opinion. During our initial visit with her, she stated that her left foot felt different than her right at baseline.\nOn physical exam, there was no gross deformity of her left lower extremity. The skin was intact with a healed incision over the dorsal midfoot, and there was point tenderness to palpation over the second metatarsal. Active and passive range of motion of the ankle and transverse tarsal joint was full and painless. Strength was 5/5 in dorsiflexion, plantarflexion, inversion, and eversion. Sensation to light touch was intact, Achilles reflex was present, and dorsalis pedis and posterior tibialis pulses were palpable.\nLaboratory work revealed an elevated erythrocyte sedimentation rate of 36 (reference range: 0–20) and C-reactive protein of 34.74 (reference range: 0–10.9). Plain radiographs and a computed tomography (CT) scan of the left foot showed diffuse sclerotic changes and cement within the left second metatarsal (Figures , ). Magnetic resonance imaging (MRI) showed diffuse edema of the left second metatarsal with a non-displaced fracture line (Figure ).\nAll treatment options were discussed with the patient and she agreed with undergoing operative fixation. In the operating room, cultures and a bone biopsy of the left second metatarsal were taken. After performing an osteotomy, curettage was performed to remove the injected cement. Open reduction and internal fixation was performed utilizing a plate and calcaneal bone graft (Figure ). The patient was discharged home on the same day with adequate pain control and a bone stimulator. X-rays taken at two weeks post-revision surgery are shown in Figure .\nAt one-month follow-up, her incision was healing well without signs of infection and she had no complaints of pain. At her most recent appointment—three months post-revision surgery—she again reported no pain and good functional recovery with physical therapy. CT scan at three months post-revision surgery showed appropriate alignment of the healing second metatarsal with intact hardware (Figure ). |
The patient is a 19-year-old man, a seasonal agricultural worker daily in contact with sheep, living in Burgundy and with no history of travel neither abroad nor in the south of France during the previous months. At the end of September 2016, the patient performed farm work in contact with sheep when he had an ocular traumatism caused by a fly. Three hours after the ocular traumatism, the patient complained of a painful right eye discomfort, with sensation of moving foreign. Upon arrival at the department of ophthalmic emergency of the University Hospital Center of Dijon within hours of the onset of the first symptoms, the clinical examination showed a red and irritated conjunctiva in the right eye with the observation of mobile and translucent larvae in the conjunctival fornix. The rest of the ophthalmologic examination was normal. Eight larvae were extracted using Bonn hook forceps under local anesthesia. All larvae were sent to the Parasitology-Mycology Laboratory of the University Hospital Center of Dijon for identification.\nThe parasitological diagnosis allowed the identification of stage 1 Oestrus ovis larvae (L1). Indeed, the macroscopic examination revealed larvae of white color and about 1 mm length. Microscopically, these larvae were composed of eleven metameres, each of these displaying 4 rows of spines (Fig. a). The cephalic segment had two large black buccal hooks (Fig. b), while the posterior segment consisted of two tubercles, each containing about ten curved spines (Fig. c) which is concordant with the morphological description of L1 Oestrus ovis larvae in the literature [].\nAt the first visit, the patient received a local treatment based on the administration of oxybuprocaine and antiseptics (Biocidan®) as eye drops. The curative treatment consisted of the mechanical removal of all of the eight larvae present at the level of the conjunctiva using a forceps, as mentioned before. Subsequently, the treatment was supplemented by the administration of antiseptic eye drops (i.e. desomedine) and antibiotics (i.e. ofloxacin). Removal of the larvae resulted in rapid relief and no complication was further reported. |
A 42-year-old Caucasian female underwent laparoscopic Roux-en-Y gastric bypass in February 2007. She subsequently developed gallstones, a known complication of bariatric surgery due to rapid weight loss. This was managed by an open cholecystectomy in November 2007. An abdominoplasty was performed in 2010 to remove excess skin.\nShe presented to our unit in February 2012 with a week long history of right upper quadrant pain associated with vomiting. Blood tests revealed deranged liver function tests: alkaline phosphatase of 169 U/l and alanine aminotransaminase of 188 U/l. Interestingly, her Bilirubin was <5 U/l. Ultrasound revealed a mildly dilated common bile duct of 9.5 mm. A computed tomographic scan was organized confirming the appearances of a dilated common bile duct and the potential of a calcified stone in the common bile duct.\nShe was managed conservatively with analgesia and ursodeoxycholic acid to attempt to dissolve the stones. Her liver function tests and pain improved, and she was discharged with an outpatient magnetic resonance cholangiopancreatography (MRCP).\nIn March 2012, her MRCP showed a dilated common bile duct of 10 mm and two small filling defects suggestive of gallstones. She represented to hospital in April 2012 with similar symptoms. On this occasion, a percutaneous transhepatic cholangiogram was arranged. Again, this confirmed the dilated common bile duct, but also showed free drainage of bile into the duodenum and no gallstones; hence, therapeutic interventions were not undertaken.\nShe was referred to the gastroenterologists for further advice, but as of June 2012 she was still symptomatic.\nUltimately, an endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy was deemed to be necessary to alleviate her symptoms.\nOwing to the altered anatomy post Roux-en-Y gastric bypass, this was to be a technical challenge. Post procedure, the patient is left with a small gastric pouch. The bypass of the distal stomach and proximal small bowel is achieved by anastomosis of the gastric pouch and the jejunum known as the Roux alimentary limb. The biliary pancreatic limb is then anastomosed to a small bowel []. As a result it is technically challenging to perform an ERCP in the conventional manner, success rates have been quoted as well below 70% [].\nWhat was performed was a laparoscopic-assisted transgastric ERCP and sphincterotomy. We shall describe our technique as follows.\nThe patient was positioned in the classic Lloyd Davies position. She was prepped and draped in the conventional manner. Carbon dioxide pneumoperitoneum was achieved through the standard 10-mm umbilical port. This was technically challenging in our patient due to the altered anatomy post abdominoplasty. Two subsequent port sites were used: a 5-mm port site in the right upper quadrant and a 10-mm port in the left hypochondrium (Fig. ).\nThe port site in the left hypochondrium allows the gastric remnant to be visualized. A site on the greater curvature of the stomach was chosen. A gastrotomy was performed using diathermy. Stay sutures were placed in a diamond-shaped configuration around the gastrotomy site—these can allow both traction and counter-traction.\nA sterile ERCP scope inserted manually through a left 10-mm port site all under laparoscopic visualization. ERCP and sphincterotomy were performed in the conventional manner by the consultant gastroenterologist. There were no obvious gallstones in our patient (Fig. ).\nThe gastrotomy site was closed with a two layer closure: the first layer as a running stitch and then as a horizontal mattress with 2.0 vicryl. The procedure was uneventful. She was admitted for an overnight stay and discharged the following day. Upon review of the patient in a routine 6-week follow-up clinic, she was asymptomatic and was discharged back to the care of her general practitioner. |
Diana (a pseudonym) is a young woman of 25 years old that reached the Eating Disorders Centre, Division of Endocrine and Metabolic Diseases, San Luca Hospital in Milan, following a dramatic weight loss. Diana reached the Centre with a BMI of 16.06 kg/m2 reporting several disruptions in her eating patterns and several distressful alterations in her body image perception. As reported in the clinical history, Diana’s first eating related crisis was dated back 2 years before her current admission, with a subtle episode when she started a diet to lose some weight after health issues related to her thyroid. During that period, Diana was located abroad for work and – under moderate stress – she began a restrictive diet with a low caloric intake that brought her to lose 10 kg in 6 months. Diana’s weight remained constant in the following months but she developed an obsessive attention to the caloric intake along with intrusive thoughts regarding her weight and regarding specific types of foods. Diana also reported body-related image distortions such as overvaluation of her weight, mirror and body checking, and avoidance of body exposure. Moreover, during the crises, she reported frequent crying spells observing her body in front of a mirror.\nIn the last year, Diana reported a stressful situation at the University that heightened her psychological symptoms. Following these new difficulties, Diana re-enacted the restrictive conducts, reducing the caloric intake with a consequent weight loss of 4 kg in a month. In the period before the admission, the restrictive conduits were accompanied by self-induced vomit and daily binge episodes.\nDiana matched all the DSM-5 criteria for a diagnosis of AN, binge-purge subtype. Compatible with the diagnosis, Diana presented distortions in her body perception and obsessive thoughts regarding her weight and her body image; for these reasons, she was considered as an optimal candidate for the interoceptive assessment.\nAt the begin of the rehabilitative protocol, Diana’s blood panels showed no signs of metabolic distress, with values in normal ranges. At her admission, Diana’s thyroid levels were within normal range and they remained within the normal range during the curse of the treatment. Endocrinologist suggested a chronic autoimmune normal-functioning condition. The psychiatric assessment indicated mood alterations toward a depressive condition accompanied by severe sleep difficulties and insomnia.\nThe rehabilitative program was composed of a multidisciplinary approach that included several experts in different fields: endocrinology, psychiatry, psychology, and nutrition. The specialists collaborated in an outpatients service tailored to the specific users’ needs. The rehabilitative program could extend from two to four cycles of treatment. Diana followed a two-cycle rehabilitative protocol with a frequency of 3 sessions a week for a total of 37 sessions. The protocol was composed of psychological intervention with group psychotherapy and individual sessions focused upon a psyco-corporal therapy approach (body-oriented psychotherapy). Psychological intervention was accompanied by psychiatric and pharmacological support (citalopram and mirtazapine), to moderate Diana’s mood alterations. The rehabilitative protocol was integrated with alimentary education sessions provided by the nutritionist. Additionally, the protocol was also accompanied by a nutritional program with fixed meals (both in quantity and composition) that Diana consumed under supervision. Scheduled assessment sessions ensured an adequate monitoring of the progress.\nFrom the beginning, Diana showed a deep insight regarding her condition. Nonetheless, from her first session, Diana showed severe difficulties in following the assigned rehabilitative diet due to obsessive thoughts connected to her body weight and to certain types of food. In the following weeks, Diana improved her adherence to the recovery protocol with a better ability to follow the changes in the diet both on quantitative both on the qualitative level (e.g., types of foods consumed). Diana reached a BMI of 19.00 kg/m2 at the end of her second cycle of rehabilitative treatment. Considering the noticeable improvements regarding her eating behaviors and her general clinical condition, Diana was dismissed from the Centre and continued her program following only monthly assessments accompanied by individual psychotherapy. |
A 17-year-old female was admitted to the hospital due to severe suicidality. At the time of admission she complained about an irritating feeling in her nose, which made her constantly grimace in the area around the nose. She was excessively worried about having a serious illness of her nose (secondary hypochondriacal delusions) and was suicidal as a consequence. Her belief persisted even after any underlying medical condition of the nose has been ruled out by extensive medical examinations. She also presented with disorganized behavior, stereotypical movements, emotional instability and lability, and a below average level of intelligence during hospitalization. On the PANSS, her symptoms scored 29/23/70 (for the Psychotic, Negative and General Psychopathology Scale, respectively). Brief neurological examination revealed no abnormal neurological signs. As ascertained by the history taken from the patient and her mother, she had a history of school phobia that began at the age of 12 years, emotional disorders, normal cognitive and physical development, and a three-year history of chronic headache. She managed to complete primary and secondary education with the help of school counseling services given to her on account of school phobia. She had not received any psychiatric care before the described admission. A diagnostic evaluation for chronic headache at the University Children’s Hospital was undertaken a year before admission.\nCalcium, phosphate and parathyroid hormone blood levels were normal. Vitamin D levels were decreased with decreased calcium levels in the urine. No signs of calcium depositions in organs other than the described brain regions were determined by ultrasound. Ophthalmological, ear-nose-and-throat examination and electroencephalography were also normal.\nDetailed neurological examination revealed dysfunction of pursuit eye movement, dystonic positioning of both arms when stretched ahead, discrete ataxia of the arms and legs, and a pathological extensor response of the left big toe.\nBilateral symmetrical calcification in head, body and tail of the caudate nucleus and ventral part of the thalamus were determined by computerized tomography (Figs. and ). MRI was preformed twice over a two-year period using the same protocol. Corresponding MR T1 sequences showed hyperintense calcifications in the same regions as those found on CT examination (Figs. and ). Both MR examinations showed a high signal of calcified areas on T1 weighted sequences due to the surface area of calcium crystals. The same areas had an isointense signal on T2 weighted sequences. No other abnormalities of the brain were detected on the MRI.\nDuring psychometric evaluation, the patient’s cognitive abilities were assessed with RPM, TOL-II, d2, CTMT and Stroop tests. The patient performed significantly worse than her normative age group in terms of general cognitive abilities, coming in below the 10th percentile. She was unable to perform problem-solving operations that require abstract thinking. Assessment of her attention performance showed below-average results in scanning and alternating attention. She also showed below-average performance in her sustained and divided attention, with her concentration performance in the 8th percentile. Her planning abilities were significantly worse in comparison with her normative group, where she was unable to construct a problem-solving strategy. Her approach was a trial-and-error strategy and she failed to solve the problem within the time limit. The patient had no significant difficulties with inhibition of dominant response, reaching a borderline average result. We performed a retest after a year of treatment and the results showed no significant changes, although she was slightly better, but unfortunately without significant improvements, in her planning abilities and in her sustained attention.\nA whole blood EDTA sample was used for extraction of genomic DNA according to established laboratory protocols using the FlexiGene DNA isolation kit (Qiagen, Germany). Whole exome sequencing a trio (index patient and her parents) was performed in collaboration with NovoGene Corp. Inc. (Davis, CA, USA) using an Agilent Sure Select Human All Exon V6, 5191–4004 kit for whole exome enrichment preparation together with an Illumina Platform PE150 (Illumina, San Diego, USA) to perform the whole exome sequencing. Genetic variants with coverage >15x were analyzed using Variant Studio 3.0 software (Illumina). Evaluation of variants was firstly restricted to those located in eight genes related to Fahr’s disease (SLC20A2, PDGFRB, PDGFB, XPR1, KRIT1, SLC19A3, TREX1, MYORG). We reached 99.9% with at least 10X coverage for the patient. A search tool for the retrieval of interacting genes/proteins (STRING, ) was used to construct the protein-protein interactions that are involved downstream and upstream in Fahr’s syndrome (GNAS, ERCC8, PDGFB, CYP2U1, GNA11, SLC20A2, IFIH1, PSMB8, PDGFRB, CA2, ERCC6, SAMHD1, TREX1, CASR, TREM2, TYROBP, GJA1, ERCC3, FAM111A, RNASEH2B, SLC46A1, SLC7A7, ATP13A2, PARK7, HMBS, KRIT1). No causative mutations were found in the selected genes in the patient.\nComputerized tomography scans of the heads of the patient’s parents were normal.\nThe patient was treated symptomatically with quetiapine sustained release (initially 200 mg and gradually increasing to 900 mg daily) and sertraline (150 mg daily, gradually increasing to 200 mg daily). We did not observe any side effects with the use of quetiapine, although special attention was given to the possible exacerbation of extrapyramidal symptoms. Psychotic (PANSS scores at discharge were 8/13/27, PANSS scores after two years were 9/16/34 for the Psychotic, Negative and General Psychopathology Scale, respectively), affective and behavioral symptoms were improved; she was no longer suicidal and remained stable on gradually increasing doses of antipsychotic medication within two years of treatment, however, her intellectual abilities were not improved. Even though the patient completed secondary professional education and intense professional rehabilitation efforts were made, she has not been able to start working, mainly due to emotional instability. The patient was transferred to adult psychiatric services at the age of 21. |
A 49-year-old male was referred to the head and neck service with a 6-week history of hoarseness. His past medical history included anxiety and hypertension. He lived alone, smoked 15 roll-up cigarettes daily and consumed a moderate volume of alcohol per week. He had a World Health Organization performance status score of 1 and a Malnutrition Universal Screening Tool score of 0. Direct examination and subsequent computed tomography of the head and neck confirmed a T3 transglottic lesion with no evidence of distant disease; biopsy of this lesion confirmed a moderately differentiated SCC. His case was discussed at the regional head and neck multidisciplinary team meeting, and a total laryngectomy and bilateral neck dissection was proposed, to which he subsequently agreed.\nHis operation was planned for the end of March 2020 for social reasons; however, two weeks prior to his operation date the Covid-19 pandemic was declared. The patient underwent standard pre-operative investigations including blood work, electrocardiography and an echocardiogram, the findings for all of which were normal. British Association of Head and Neck Oncology guidance for laryngectomy during Covid-19 was followed; the patient was advised to self-isolate for 14 days and asked to attend his local ENT department for two Covid-19 swab tests within the week prior to surgery. His first swab was negative; however, the second swab, performed 4 days later, was positive for SARS-CoV-2. His surgery was postponed and he was asked to self-isolate for a further 14 days. He was asymptomatic at the time of testing and remained so throughout his isolation period.\nDuring the 14-day self-isolation period, he underwent Covid-19 swab testing on days 7 and 14. Both swabs were negative and therefore his operation was rescheduled to take place 2 days following his most recent negative swab test. The day before surgery, he was admitted to a ‘clean’ tertiary centre with no accident and emergency or dedicated Covid-19 ward. He was again swab tested on admission and found to be negative.\nThe patient successfully underwent total laryngectomy and bilateral neck dissection following clearance of Covid-19, only two weeks later than initially planned. Despite his negative swab results, enhanced personal protective equipment (PPE) was worn throughout the surgical procedure by operating theatre staff, including a filtering facepiece code 3 (FFP3) mask, face shield, disposable cap, fluid-resistant gown and gloves. There were no surgical or anaesthetic-related peri-operative complications.\nThe patient progressed well on the ward following surgery. Healthcare professionals wore enhanced PPE, including FFP3 masks, face shield, fluid-resistant gowns and gloves when carrying out AGPs (e.g. suctioning and stoma care). On morning ward rounds, he was reviewed by a senior member of the medical team who wore a fluid-resistant surgical mask, face shield, apron and gloves. There were no post-operative complications and the patient was safely discharged two weeks after his surgery.\nThe patient was reviewed in clinic one month post-operatively; his wounds were healing well and he was eating and drinking without issue. His final pathology report revealed a completely excised, moderately to poorly differentiated laryngeal SCC, pathologically confirmed as tumour–node stage T3N0 (based on the 8th edition of the Union for International Cancer Control tumour–node–metastasis (TNM) classification). Following discussion with the oncology department, the patient decided against adjuvant radiotherapy. The British Association of Head and Neck Oncology advises against same-day tracheoesophageal puncture and speech valve insertion during the pandemic, to reduce the length of hospital stay and associated complications; he therefore remains on the waiting list for this. |
A 73-year-old Asian woman with an underlying anxiety disorder, functional headache, and hypertension was prescribed escitalopram and lorazepam when she presented with progressively worsening headaches to her primary care doctor. Her symptoms did not improve with the medications, and she was unable to eat well and required bed rest. She was transported to our hospital 4 days later after developing chest and back pain with altered consciousness. She was a housekeeper, had no allergies, and had no alcohol or tobacco smoking history. On arrival, her Glasgow Coma Scale score was 3/15 (E1V1M1); both pupils were approximately 4 mm in diameter and reactive. Her blood pressure was too low to be measured, her carotid artery pulse was palpable, her heart rate was 112 beats/minute, and her respiratory rate was 30 breaths/minute. Her conjunctiva was pale. An auscultation of breath sounds did not reveal upper and lower airway obstructions and was within normal limits. Her abdomen was soft and flat without tenderness. She had no skin abnormalities (such as rash). Both legs had no edema. Echocardiography on arrival was performed as point of care ultrasound and revealed a hypercontractile left ventricle with an eliminated left ventricular cavity and a collapsed inferior vena cava without right ventricular dilation. There was no pericardial effusion or obvious large regurgitant jet observed on color Doppler. In response, we immediately inserted a peripheral venous catheter and began introducing fluid resuscitation; however, she developed PEA. Conventional CPR according to the adult advanced cardiovascular life support guidelines (including adrenaline) was initiated and a return of spontaneous circulation (ROSC) occurred. However, her blood pressure was unstable and PEA returned, prompting repeated CPR with immediate administration of fluids and three adrenaline injections. Venoarterial extracorporeal membrane oxygenation (VA-ECMO) was initiated for refractory PEA. Whole-body contrast-enhanced computed tomography was unremarkable, and the admission laboratory results were also unremarkable, except for anemia (Table ). Her hemoglobin level decreased from 7.1 g/dL to 3.5 g/dL 1 hour later without obvious signs of gastrointestinal hemorrhage. Therefore, 8 units of packed red blood cells were transfused for 1 day, after which her hemodynamic status stabilized. She was in a coma without sedatives; thus, targeted temperature management at 34 °C was initiated on admission to an intensive care unit. Echocardiography in the intensive care unit showed a thickened interventricular septum (which was 12.8 mm), prolonged anterior mitral valve, and contact between the bodies of the anterior and posterior mitral valves, suggesting that the left ventricular obstruction could have potentially occurred through this redundant anterior mitral valve. VA-ECMO was terminated on day 3, and after stabilizing her hemodynamics, transthoracic echocardiography showed a sigmoid septum with normal left ventricular function (ejection fraction, 75%) (Fig. ). On day 26, dobutamine-infused (30 μg/kg per minute) Doppler echocardiography revealed a significant outflow gradient (236 mmHg) accompanied with chest pain (Fig. ) and intermittent systolic anterior motion (SAM) of the mitral valve; continuous monitoring during Doppler echocardiography showed a Brockenbrough–Braunwald sign (Fig. ), which is a fall of arterial blood pressure after premature ventricular contraction; these findings confirmed a diagnosis of latent LVOTO due to a sigmoid septum. The significant LVOTO was not dependent on SAM but might have occurred due to the greatly thickened interventricular septum. As a result, carvedilol was initiated with gradual increment up to 10 mg/day on day 35. In addition, verapamil (120 mg/day) was administered on day 29. A follow-up dobutamine-infused Doppler echocardiography on day 40 showed a reduction of the outflow gradient to 14 mmHg, indicating a successful medical therapy.\nThe worsening anemia that was identified at admission was suspected to be hemolytic anemia (HA) based on results of the blood test. The results of the laboratory examination, including total bilirubin, direct bilirubin, lactate dehydrogenase, reticulocyte count, and haptoglobin, and a direct Coombs test on day 1 are presented in Table . Her drug lymphocyte stimulation test was positive, and agglutination occurred when her serum reacted with lorazepam. Therefore, lorazepam-induced immunological HA was diagnosed. Her anemia improved following discontinuation of lorazepam. Her hemoglobin level was 11.9 g/dL on day 64, and she was eventually discharged on day 68 without any sequelae. |
A 67-year-old female with history of chronic tobacco use, chronic obstructive pulmonary disease, hypertension, and hyperlipidemia, presented to the ED with symptoms of TIA. The patient described the acute onset of left-sided facial weakness that waxed and waned, recurring several times throughout the day, and lasting 2–3 minutes at a time. The left facial weakness was also associated with mild, left-arm weakness and “clumsiness” involving fine motor function of her left hand. She noted lightheadedness but denied leg weakness, headache, visual changes, chest pain or shortness of breath. She also noted that symptoms were brought on by use of her upper extremities and when she changed her body position from lying to sitting. She denied any similar symptoms previously or stroke history. Of note, she noticed a rapid improvement in her symptoms to resolution just prior to ED presentation.\nOn examination, her blood pressure (BP) was 183/86 millimeters of mercury (mmHg). She was awake, alert, oriented, and able to describe a detailed history. Her cranial nerves were intact, motor strength was 5/5 bilaterally, and fine motor movements in both her hands were normal. There was no ataxia, extraocular muscle dysfunction, or indication of posterior circulation involvement.\nJust after her initial asymptomatic presentation to the ED, her symptoms recurred when her systolic BP dropped by 20 mmHg upon standing from a supine position. Emergent computed tomography angiogram (CTA) of the head and neck demonstrated a severe flow-limiting lesion of the innominate artery (). Further investigation with magnetic resonance imaging demonstrated decreased signal intensity within the right internal carotid artery at the cavernous sinus and petrous segments, a finding that potentially represented slow flow ().\nThe patient subsequently underwent emergent cerebral angiogram, which demonstrated occlusion of the proximal innominate artery () at the aortic arch with resultant left to right vertebral artery steal phenomenon supplying the right subclavian artery (). The distal brachiocephalic artery flow was reconstituted via the subclavian artery and secondary steal phenomenon occurred into the right common carotid artery, causing delayed flow to the right cerebral hemisphere ().\nThe patient was maintained on a norepinephrine bitartrate infusion to increase BP, and her symptoms subsequently resolved. The symptoms recurred when she was positioned supine, but upon being placed in the Trendelenburg position her symptoms again resolved. The patient was therefore maintained with systolic BP goals between 160 and 210 mmHg. She remained asymptomatic during this period of elevated BP management. For definitive care, she underwent elective left carotid to right carotid “necklace” bypass surgery with complete and permanent resolution of her symptoms. |
Our patient is a 78-year-old white male with a past medical history of hypertension and benign prostatic hypertrophy (BPH) who had undergone cystoscopy and transurethral resection of bladder tumor by a urologist. The pathology showed non-muscularis propria involving high-grade papillary urothelial carcinoma for which he was undergoing treatment with intravesical BCG administration to prevent a recurrence. He tolerated the initial treatment sessions without any complication but during one of the sessions, he needed multiple attempts to insert the Foley catheter which was painful for him. Subsequently, a “coude” urinary catheter was placed for BCG administration. The patient was hospitalized the same day for intense dysuria and low-grade fevers with chills. At that point, his urinalysis showed elevated white blood cells and red blood cells but no bacteria. He was admitted with a diagnosis of a complicated urinary tract infection (UTI) and initially was started on intravenous Cefepime. He was seen with infectious diseases and was switched to Levofloxacin the next day to cover BCG cystitis and other bacterial organisms for UTI. Cultures remained negative at that time and he was discharged to a rehabilitation facility where he completed 14 days course of Levofloxacin treatment.\nThe patient complained of intermittent chills and dysuria throughout his stay at the rehabilitation facility where he stayed for almost three weeks. Within three days after discharge from the rehabilitation center, he came back to the emergency department (ED) with severe disabling chills and rigors, fever in the range of 101-103 °F, and generalized weakness. On initial evaluation, he was found to be febrile with a temperature of 101 °F, heart rate of 110 beats per minute, blood pressure of 130/80 mmHg, and pulse oximeter saturation reading of 95% on room air. The patient was very uncomfortable but denied any localizing symptoms like chest pain, shortness of breath, coughing, nausea, vomiting, diarrhea, headache, skin rash, etc. He reported mild dysuria which was an ongoing issue since his last presentation. His blood work showed leucopenia with a white blood cell (WBC) count of 3000/µL (NL 4500-11,000/µL), absolute neutrophil count of 800/µL (NL 2500-7500/µL), thrombocytopenia with a platelet count of 63,000/µL (NL 150,000-450,000/µL), transaminitis with elevated aspartate transaminase (AST) and alanine transaminase (ALT) in the range of 200 unit/liter (NL 5-40 U/L) and elevation in alkaline phosphatase (ALP) 250 U/L (40-150 U/L). A computed tomography (CT) scan of chest, abdomen, and pelvis with contrast was done which showed new moderate splenomegaly. He was started on broad-spectrum antibiotics from ED with intravenous vancomycin, piperacillin/ tazobactam, and levofloxacin. The next day he was examined by various specialists including Infectious diseases, hematology, gastroenterology, and urology. Extensive workup for infectious etiology was ordered including blood and urine cultures, cultures for acid-fast bacilli, Lyme serology, infectious mononucleosis panel, respiratory viral panel, and blood parasite smear, the results for which remained unremarkable. Additional workup by that time involved tagged WBC scan and flow cytometry which were negative as well. Despite being on broad-spectrum antibiotics patient continued to have recurrent fevers in the range of 101-103 °F associated with disabling chills.\nThere was a strong suspicion of disseminated BCG infection, but the team was hesitant about starting on usual anti-mycobacterial medications because of transaminitis. On day 4, antibiotics were switched to levofloxacin, amikacin, and ethambutol which are all active agents against BCG. The patient’s WBC and platelet counts showed slight improvement and liver function test (LFT) showed some improvement over the course of the next few days and the patient was then transitioned to Ethambutol (dose 15 mg/kg oral per day), Rifampin (dose 600 mg oral per day), Isoniazid (INH; dose 5 mg/kg oral daily)/vitamin B6 after multidisciplinary discussions. The patient showed definitive improvement in symptoms subsequently with improvements in fevers, chills, and rigors and overall started to feel much better. After approximately six days of getting Rifampin, AST/ALT showed an increase to 400 U/L and thus rifampin was stopped and switched to levofloxacin (dose 500 mg oral per day). His dose of INH and Ethambutol was also reduced to one-half of the initial dosage. A decision was made to continue levofloxacin for two months and INH and Ethambutol for six months.\nAs the patient continued to show symptomatic improvement, he was discharged to a rehabilitation facility after a few days and was followed up as an outpatient with infectious disease and hematology. His cell counts and LFTs eventually normalized and repeat imaging showed complete resolution of splenomegaly. |
A 51-year-old female presented with right hip pain increasing over several years. She reported difficulty putting her socks and shoes on, and could no longer exercise due to her hip pain. The patient had no other significant past medical or past surgical history. She was also a Caucasian and her BMI was 33. Her physical exam demonstrated painful, limited range of motion. Right hip flexion was 85 degrees, internal rotation less than five degrees, and external rotation less than 10 degrees. Severe degenerative changes were observed on radiographs.\nThe patient elected to undergo hip SRA in late 2006. A 46 mm femoral head was used along with a 54 mm acetabular cup (Smith & Nephew Orthopaedics, Ltd, Bromsgrove, United Kingdom). Simplex P cement with Tobramycin was used for fixation of the femoral component. After an uneventful hospital stay she was discharged with posterior hip precautions as described in Case #1. Radiographs from the 6-week post-op visit showed satisfactory alignment and no evidence of fracture, loosening, or subsidence (Figure ). At this time her posterior hip precautions were discontinued, and she began outpatient rehabilitation with a therapist in her community.\nShe was seen again three months post-op and reported doing very well, able to perform ADLs, and with sufficient functional status to return to full time work. She had no pain, but reported having less than expected range of motion in physical therapy. In particular, she was having difficulty with advancing full external rotation, similar to that noted pre-operatively. This limitation was noted despite consistent flexion, abduction, and external rotation exercises implemented immediately post-operatively.\nApproximately twenty weeks after the hip resurfacing, the patient sustained a painful groin injury during physical therapy. She was undergoing forceful passive external rotation manipulation with her therapist when the injury occurred. The patient started in a "figure of four" position (knee and hip flexed about 90 degrees with the ipsilateral ankle resting on the contralateral thigh). From this position the therapist used steady force to passively flex and externally rotate the hip further. The force that was applied was described as in excess the patient's own active or passive ROM attempts. She reported initial pain after this, but was ambulatory upon leaving the physical therapy clinic. However, the following day she was unable to bear weight and was seen urgently back in clinic, where radiographs confirmed femoral neck fracture (Figure ).\nTreatment options for the peri-prosthetic fracture, including conversion to THA and non-operative treatment with limited weight bearing were presented to the patient. She elected to pursue non-operative treatment, and maintained non-weight bearing for a period of six weeks. After the six weeks she had very little pain. Radiographs at that time showed no significant change in hip SRA placement, but mild lower extremity shortening due to initial positional change of the femoral component. Her weight bearing status was then advanced to fifty percent from 6 to 12 weeks following fracture, then full weight bearing 12 weeks post-fracture.\nAfter advancing to full weight bearing, she did have a pinching discomfort when ambulating and she used a cane for walking longer distances. Range of motion on examination was 100 degrees of flexion, 30 degrees of external rotation, and 20 degrees of internal rotation, and radiographs at that time showed no further change in implant positioning (Figure ). No restrictions in weight bearing or ROM were maintained at this point. She was happy with her pain-free status and ROM progress, improved function, and gait. Non-operative treatment continued.\nAt nearly one year post-fracture, the patient's pain slowly worsened with more compelling evidence of implant bone collapse on radiographs (Figure ). The patient ultimately chose to undergo revision to THA at an outside facility in an identical fashion to Case #1 and is now pain free. |
A 21-year-old woman presented to the surgery outpatient department (OPD) with clinical history of right upper abdominal lump for 6-7 years, which was gradually increasing in size. It was associated with dull aching type of pain, which was on and off in nature. There was no history of fever, weight loss, altered bowel habits, or urinary symptoms. Her menstrual history was within the normal limits. There was no history of twin birth or teratoma in the family. Her general physical examinations were within the normal limits. On abdominal examination, as done in surgery OPD, there was a lump involving the right upper quadrant of the abdomen. It had ill-defined margins with soft to firm consistency and irregular surface. It was not moving with respiration. On evaluation, beta-human chorionic gonadotropin (β-HCG), alpha fetoprotein (AFP), and routine blood investigations were found within the normal limits. For further diagnostic evaluation, ultrasonography (USG) was done. Prior imaging examinations were not done in this patient. USG showed a large, ill-defined heterogeneous mass in the right side of the abdominal cavity adjacent to the liver, having multiple hypoechoic areas and echogenic structures with no internal vascularity. With inconclusive diagnosis, she was admitted in surgery ward for further evaluation.\nLater, contrast enhanced CT scan of the abdomen and pelvis revealed a large heterogeneous retroperitoneal mass in the right side of the abdomen, measuring approximately 22.5 × 14.1 × 12.5 cm having cystic areas, fat attenuating areas, foci of calcifications, malformed skeletal parts resembling long bones, ribs and vertebrae, and heterogeneous soft tissue fetal components. Soft tissue components showed minimal peripheral enhancement. The mass was lying predominantly in the posteroinferior aspect of the right lobe of the liver causing its displacement anteriorly and to the left side and extending superiorly up to D8 vertebral body causing superior displacement of the right hemidiaphragm. It has had extension inferiorly up to L3 vertebral body with inferiorly displaced and malrotated right kidney. Inferior vena cava was displaced anteriorly and medially. Bowel loops were also displaced inferiorly and to the left side. It has had maintained fat plane with the surrounding host organs (). The uterus and bilateral adnexa were normal. With the features of well-organized axial skeleton and long bones (), CT findings were suggestive of fetus in fetu with various mass effects. Organized teratoma was kept as differential diagnosis.\nMass being huge with its retroperitoneal location, difficult surgical planes, and surrounding structural relationship, laparotomy was planned over laparoscopy for further diagnostic and therapeutic purposes. It was performed and mass was excised completely. Grossly, it was enclosed in a sac containing fat, malformed bony and cartilaginous tissues, soft tissue components along with large amount of serous fluid (). The uterus and bilateral adnexa were grossly unremarkable. Total operative time was around 4 hours, and total amount of blood loss was approximately 300 ml. Her total postoperative hospital stay was 5 days and uneventful.\nThe mass was sent for histopathological examination. Histopathology showed mixture of variable tissues in variable proportion. There were areas of tissues lined by respiratory epithelium, intestinal epithelium, and stratified squamous epithelium. Muscle bundles, cartilages, bones, glial tissues with psammoma bodies, adipocytes, thyroid follicles, and lymphoid aggregates were also seen. Multiple cystic spaces lined with ciliated columnar epithelium were also noted. However, there were no immature components and no features of malignancy seen (). Gross and histopathological findings of mass confirmed it to be fetus in fetu.\nShe has been under regular follow-up with normal level of tumor markers β-HCG and AFP, and she is doing well. Her last follow-up was around 3 months after the surgery. |
A 51-year-old man was referred with a 4-month history of dysuria, haematuria and increasing pelvic pain. He had a background history of ESRD secondary to polycystic kidney disease requiring dialysis. At the time of initial review he had performance status 2 due to pain but had previously been fit and well.\nRectal examination revealed a nodular prostate but nothing else of note. Blood results were within normal range, including a PSA at 1.2 µg/mL, and a cystoscopy was unremarkable. Transrectal ultrasound-guided biopsies however identified small-cell carcinoma. A pelvic MRI scan showed disease extending outside of the prostate into the right seminal vesicle, but without evidence of nodal disease. Further CT imaging showed no evidence of metastasis. The case, reviewed at a multi-disciplinary consultation with urology, medical oncology, radiation oncology and pathology, was felt to represent limited stage small-cell carcinoma of the prostate.\nTo maximize the patient's chance of cure, it was intended to treat his disease with sequential chemoradiotherapy to the prostate. In our centre, this would usually involve a regime including cisplatin chemotherapy. This however has significant nephrotoxic potential. Carboplatin, an alternative platinum-based chemotherapy agent which is less nephrotoxic and reported (albeit in series of no more than three patients) to be well tolerated by haemodialysis patients [–], was considered. Nephrology advice was sought early to adjust the haemodialysis prescription to the kinetics of carboplatin in ESRD.\nCarboplatin 300 mg/m2 on Day 1 and etoposide 50 mg/m2 on Day 1 and Day 3 of a 21-day cycle were administered. Dialysis was carried out on Day 1 and Day 3, 2 h after chemotherapy (early in the drug elimination phase, when protein binding is low). Sequential radical radiotherapy to the prostate and seminal vesicles was given using standard hypo-fractionated intensity modulated radiotherapy of 57 Gray in 19 fractions over 4 weeks.\nThe patient, who was on extended haemodialysis schedules, had a body weight of 124 kg with significant residual renal function (RRF 1 L/day) with minimal interdialytic weight gains, the volume management complicated by steroid therapy. He would perform six 3-h sessions per week independently using constant site cannulation, high-flux FX100 polysulphone dialysers (Fresenius™), Tinzaparin anticoagulation and A232 dialysis fluid. Post-chemotherapy he underwent high-flux haemodialysis using an identical prescription but for over 4 h, using blood flow of 320 mL/min and dialysate flow of 800 mL/min. The high flux, larger dialyser surface area and increased time were adjustments made given the higher total body water to maximize diffusive clearance of the drug.\nAfter the first of four intended cycles of chemotherapy, the patient enjoyed a complete resolution of his severe pelvic pain. He developed Grade 3 thrombocytopenia (carboplatin has a well-known myelosuppressive effect), and was moved to a 28-day cycle from cycle 2. Interval scans after two cycles showed a complete disease response. Treatment was stopped after three cycles due to persistent grade 2 thrombocytopenia, but he proceeded to radiotherapy to the prostate and seminal vesicles with concurrent prophylactic cranial irradiation (25 Gray in 10 fractions over 2 weeks). All radiotherapy was well tolerated without significant toxicity. At an interval of over 12 months post-treatment there is no clinical evidence of recurrent disease and the patient continues to be on frequent haemodialysis with well-maintained RRF. |
A 49-year-old woman presented in June 2014 with gradual progression of exophthalmos of the left eye, a painless palpable mass under the left orbital rim, double vision, and epiphora for the last 6 months. The patient’s visual acuity was 20/20 in the right eye and 20/25 in the left eye. Hertel exophthalmometry was 12 mm for the right eye and 15 mm for the left eye. The mass was palpated at the superior, temporal, and inferior orbital rim of the left eye featuring well-defined and regular margins, a smooth surface, and it was slightly hard. Movement of the left eye was restricted in the left field of gaze. Examination of the globe did not show obvious abnormality. The intraocular pressure was 13 mmHg in the right eye and 15 mmHg in the left eye.\nCT scans showed an irregular orbital mass with ill-defined margins, and featured low and heterogeneous density in the extraconal space of the left orbit (). , and are different CT sections to show the shape of mass. The lesion contained an ill-circumscribed calcification with a size of 11×7 mm near the lateral rectus muscle (). The upper eyelid was involved and the surrounding soft tissue was thickened. The medial wall of the left orbit showed local destruction (). On MRI, the mass appeared hypointense on T1WI and T2WI, which was enhanced following contrast administration (). Serum tumor marker examination showed increased CEA (8.4 ng/mL) expression.\nThe patient was diagnosed with an orbital carcinoma (nature undefined) in the left orbit. She then underwent excisional biopsy. A histopathological examination revealed a poorly differentiated adenocarcinoma.\nWhole-body PET, which was performed to determine the degree of metastasis, showed irregular and slightly elevated fluorodeoxyglucose uptake by the left orbital mass with a maximum standardized uptake value of approximately 3.1. The lesion grew in the extraconal space, involving the superior rectus, lateral rectus, inferior oblique, and internal rectus muscles. No systemic metastasis and bone destruction were present in this case (). Mammography examination showed hyperplasia of the mammary glands (figure not shown).\nExenteration with radical orbitectomy and postoperative radiotherapy was performed 1 month after biopsy. The specimens underwent histopathological and immunohistochemical analyses. The patient had no evidence of recurrence or metastasis during the 9-month follow-up. |
A 23-year-old man (weight 65 kg, height 175 cm, and BSA 1.8 m2) with a diagnosis of primitive right atrial enlargement from foetal age was referred to our Centre for cardiological evaluation. Cardiac examination showed increased heart size on percussion and a grade II/VI Levine systolic murmur. No significant pathological findings were found on pulmonary examination. Electrocardiography showed a regular sinus rhythm with a rate of approximately 60 beats/min associated with an abnormal morphology and duration of P wave (enlargement of P wave with duration of 130 msec), together with a low amplitude of QRS complexes in the limb leads. All routine laboratory studies were within normal limits. Chest radiography showed an abnormal cardiac silhouette with increased convexity in the lower half of the right cardiac border and cardiomegaly ().\nTransthoracic two-dimensional echocardiography demonstrated a huge right atrium of about 6.2 cm and a volume of 230 ml/m2, with a thick smoke pattern and mild tricuspid regurgitation. The pulmonary arterial pressure was normal (). The tricuspid valve was normal without significant annular dilation. No stenosis or abnormal displacement of the tricuspid valve leaflets was detected. No significant regurgitation of the tricuspid valve was found despite a partial distortion of the anterior leaflet and compression of the right ventricle inflow. The right ventricle appeared small and compressed anteriorly by the right atrium (area of RV: 11 cm2).\nCardiac magnetic resonance imaging showed a marked right atriomegaly (right atrium area: 66.50 cm2, volume: 220 ml/m2) and normal size of the left atrium (left atrium area: 7.02 cm2). The right ventricle was regular in size and global contractility but was partially compressed and dislocated posteriorly, due to the massive enlargement of the right atrium. The left ventricle was regular in dimension, thickness of the wall, and global/segmental contractility (FE VS = 61%). No evident transvalvular jets or areas of late gadolinium enhancement were found. The pericardium was visualized without focal abnormalities or pericardial effusion ().\nDue to the high risk of arrhythmias and thrombus formation in the right atrium, which is a potential risk for pulmonary embolism, the patient underwent cardiac surgery. Through a median sternotomy, cardiopulmonary bypass was established with standard aorta and bicaval cannulation. After the pericardium was opened, the entire anterior surface of the heart was found to be covered with a thin wall in continuity with the right atrium. No atrial appendage as such was apparent. The right atrium was fully opened. The inferior border of the atriotomy was sewn around the anterior part of the tricuspid annulus, and the superior border was brought over the lateral wall of the right atrium as a flap and sewn near the interatrial groove. This provided adequate reduction of the atrial size and reinforcement of the atrial wall ().\nThe histology of the resected atrial wall showed focal hyperplasic areas of smooth muscle cells with polymorphic nuclei surrounded by a few scattered areas of hypertrophic fibrous tissue.\nPostoperative transesophageal echocardiogram showed a significant reduction of the right atrium area (23 cm2, volume: 93 ml).\nThe patient was extubated 11 hours after surgery. Complications arose postoperatively with the early appearance of pericardial effusion with leukocytosis and elevated inflammatory markers. This was resistant to conventional medical therapy, which in the end required surgical drainage. Medical therapy of the postpericardiotomy syndrome (ibuprofen 600 mg/TID and colchicine 1 mg/OD) was continued over the subsequent 6 follow-up months without further recurrence of pericardial effusion. |
73-year-old female with history of hypertension, and a recent diagnosis of right lower lobe (RLL) lung mass, presented to the emergency department (ED) complaining of progressively worsening dysphagia for few weeks. The patient had difficulty in swallowing both liquids and solids. She reported decreased appetite, weight loss, and multiple episodes of nonbloody vomit. She was scheduled to have an endoscopy as an outpatient on the same day of admission; however, she decided to come to the ED instead, as her symptoms became more severe.\nThe patient had a remote history of 20 pack-year of cigarettes smoking. Her medications included Aspirin, Iron Pills, Atorvastatin, and Lisinopril. On physical exam, she was hypothermic with a temperature of 35.6 degrees Celsius and tachycardic with a heart rate of 103. The patient looked cachectic and chronically ill. She also had dry oral mucous membranes. The initial laboratory results showed significant leukocytosis with a white blood cell count of 29.3 thousand/ul and hyponatremia with a sodium of 127 mmol/L. The urine was cloudy in appearance, and it contained leukocytes esterase and more than 50 white blood cells. The chest X-ray revealed RLL mass, with no evidence of pulmonary consolidation. The patient was admitted to a telemetry bed and was started on aggressive IV hydration and IV ceftriaxone as a treatment for severe sepsis syndrome. The source of the sepsis was thought to be a urinary tract infection as the urine culture grew Kluyvera ascorbata and Streptococcus agalactiae (group B), and both organisms were sensitive to ceftriaxone. The hospital course was prolonged and complicated. During the first 24 hours, the patient went into atrial fibrillation with rapid ventricular rate and was started on IV amiodarone and heparin drip. She also had an abrupt onset of cold and discolored left leg that required an emergent vascular surgery evaluation. The patient was found to have left femoral artery occlusion and underwent left iliac artery stenting and left femoral endarterectomy.\nFurthermore, she had a CT-guided biopsy of the RLL mass and the pathology was consistent with primary adenocarcinoma. The patient was intubated for the surgical procedure and the biopsy. Few days later, she was briefly extubated; however, she continued to have significant hypoxia and hypotension. There was also a concern for aspiration pneumonia. Palliative care team was involved and after an extensive discussion with family, the decision was taken to change the code status to DNR. Patient expired after 2 weeks of hospitalization. The endoscopy was never done as the patient was never in a stable condition throughout her hospital stay. |
A 74-year-old male presented in February 2016 with increasing dysphagia. There was no history of prior abdominal infection or surgery. On esophagogastroscopy, a necrotic and circumferential friable tumor was seen at 33 to 40 cm from the incisors, with an endoscopic appearance of involvement of gastroesophageal (GE) junction and the proximal 2 cm of the stomach. Biopsies of the distal esophageal tumor confirmed poorly differentiated adenocarcinoma. The patient was anemic with a hemoglobin of 89 g/L. Staging endoscopic ultrasound suggested a breach of muscularis propria and four enlarged paraesophageal nodes. Neoadjuvant chemoradiotherapy followed by esophagectomy was initially considered; however, a staging positron emission tomography (PET) scan demonstrated 18-fluorodeoxyglocose (FDG) uptake not only in the primary tumor, but also in the paraesophageal region near the GE junction and upper abdominal lymph nodes extending as far inferiorly as the right renal vessels, in a retrocaval location (Figure ).\nA radiation oncology consultation was sought regarding treatment options of such extensive lymphadenopathy. Palliative radiation therapy (RT) was recommended. The patient was also evaluated by a medical oncologist who advised that chemotherapy may be considered after assessing the response to palliative radiotherapy.\nFrom March 21, 2016 to April 5, 2016, the patient received palliative RT to the symptomatic primary tumor and closest adjacent nodes using a pair of anterior and posterior fields. A total dose of 30 Gray (Gy) was prescribed over 10 daily fractions. As the lymphadenopathy in the lower abdomen was not symptomatic, and would have contributed to increased toxicity, this region was deliberately excluded from the high dose RT volume (Figure ). Other than very mild odynophagia, the patient had no other RT-related side effects. On the first follow-up visit, one month following treatment completion, he had improved swallowing function and a weight gain of six pounds.\nFollow-up computed tomography (CT) scan was obtained on May 24, 2016 to evaluate for the suitability of chemotherapy and to serve as a baseline during systemic therapy. This demonstrated persistent thickening of the lower esophagus, with lymphadenopathy reported to have decreased in size and no significant retroperitoneal adenopathy. When given the option of receiving palliative chemotherapy, the patient declined and chose to continue on observation only. Further CT scans in August and October 2016 showed a complete response in the irradiated primary tumor and nodes, with a stable 10 mm lymph node at the right renal vein.\nIn January 2017, due to symptoms of increasing dysphagia, the patient was assessed by a thoracic surgeon for consideration of esophageal stent placement. Endoscopy on January 12, 2017 noted that there was a possible small amount of residual tumor at the GE junction, but there was no significant narrowing or stricture, and no biopsies were taken. A further CT scan on April 10, 2017 showed minor circumferential thickening of the distal esophagus, but unchanged from previous. Paraesophageal lymphadenopathy was reported to be unchanged. The PET-positive lymph node at the renal vein decreased from 10 mm to 5 mm.\nThe patient’s symptom of dysphagia resolved spontaneously, and an evaluation was made with a further PET scan on May 19, 2017 (Figure ). This demonstrated mild residual FDG activity within the distal esophagus, more likely inflammatory change rather than malignancy. The FDG activity within all the lymph nodes, both treated and untreated, had unexpectedly resolved. |
A previously healthy 6-year-old boy visited Samsung Medical Center with complaints of dyspnea and a barking cough lasting for 3 days. Chest radiography revealed a mass-like lesion in the right upper mediastinum. Chest computed tomography demonstrated a well-defined posterior mediastinal mass measuring 4 cm, which was suspected to be a neural foraminal extension to the thoracic spine (T3–T4). Magnetic resonance imaging was conducted for further evaluation; it showed a right upper paravertebral enhancing mass with an adjacent neural foraminal extension to the T2–T5 spine (). A preoperative neurologic examination revealed that the patient had no abnormalities. He underwent mediastinal mass excision by video-assisted thoracic surgery. The mass was in the right posterior mediastinum, was located at the T2–T5 level of the spine, and did not extend to the neural foramen. It was resected by electrocautery. The sympathetic chain at the T2 level was inevitably resected as part of the complete resection of the mediastinal tumor during the operation, because the tumor originated from this sympathetic chain. The intraoperative course was uneventful, with a stable hemodynamic status throughout the surgery. After the patient was sent to the post-anesthesia recovery unit, a sharp midline facial demarcation was observed, and the left face, neck, and chest (contralateral to the operation site) became flushed and warm (). These findings were noted to increase in intensity when the patient cried. In contrast, the right face, neck, and chest (ipsilateral to the operation site) were pale and cool and did not change in color when the patient cried. A neurologic examination including that of the cranial nerves was normal, and miosis and ptosis were absent. The patient’s vital signs and laboratory tests were normal. The symptoms, including the color and the warmness of the face, started to show minimal improvement within an hour and completely resolved without any treatment 3 hours after surgery. The patient was discharged on postoperative day 2. The permanent pathologic findings revealed that the resected tumor was a ganglioneuroma. During follow-up on postoperative day 30, there was no sign of color change on his face, neck, or chest. |
A 56-year-old man with diabetes, hypertension, and coronary disease presented with severe mitral regurgitation and underwent mitral valve replacement with coronary artery bypass grafting (CABG) in April 2019. The surgeon initially performed a vertical incision in the left atrium (LA) between the right pulmonary veins and the interatrial septum in the interatrial groove (Waterston’s groove). However, because of poor visualization, another horizontal incision was made in the posterior RA and included the atrial septum (. Thereafter, mitral valve replacement was performed through this larger incision involving the RA, septum, and LA. Postoperatively, the patient developed atrial fibrillation and was placed on amiodarone for several weeks and then taken off the medication. Several months later, he developed an atrial flutter as shown in . The ECG showed a slow atrial flutter with morphology consistent with that of a typical atrial flutter.\nThe patient was placed on anticoagulation and, after discussion, wished to proceed with a cardiac EP study. The patient was brought to the EP laboratory and catheters were placed in the coronary sinus (CS) and anterolateral RA (proximal catheter in the high lateral RA, with distal electrodes inferior toward the floor of the RA along the low lateral wall), along with a His-bundle catheter (HBE).\nEntrainment mapping was performed from the proximal CS (PCS) (, low lateral RA (Deca 1–2) (, and distal CS (DCS) (. The postpacing interval (PPI) for each of the sites was within 20 ms of the tachycardia cycle length (TCL), suggesting a biatrial tachycardia as depicted in . The ablation catheter (Abl) was then placed on the CTI (. Given that both adjacent sides (RA low lateral wall and PCS) of the CTI appeared to be within the circuit, ablation was conducted along the CTI, prolonging the CL from 340 ms to between 370 and 380 ms, but did not terminate the tachycardia.\nAs a result, repeat entrainment mapping was performed from the low lateral RA (Deca 1–2), PCS, and DCS with the longer CL atrial flutter and the PPIs were found to be within 30 ms of the TCL, again suggesting the involvement of both atria in the tachycardia.\nA multielectrode catheter (Pentaray; Biosense Webster, Diamond Bar, CA, USA) was used to perform high-density activation mapping in the RA and in the CS. show activation occurring from the anterior RA down the lateral wall of the RA, then moving to the posterior RA above the inferior vena cava (IVC). Subsequent activation of the septum then occurred, followed by proximal to distal CS activation. High-density voltage mapping ( was also used to delineate scar from prior surgery. When combined with high-density activation mapping, the electroanatomic map showed activation occurring through a channel on the posterior wall that was bordered by scar. It also revealed that a portion of the circuit was still missing (light blue color) between the DCS and anterior RA, consistent with activation over Bachmann’s bundle (BB). Mapping of this remaining portion of the circuit would have required access to the LA and was not deemed necessary and was not performed to avoid unnecessary procedural risk to the patient.\nEntrainment was performed adjacent to the posterior RA channel (, with a PPI of 370 ms that was identical to the TCL. Pacing from the ablation catheter at a high output of 15 mA was also performed to exclude the possibility of ablation of the phrenic nerve. Ablation in this area slowed and terminated the tachycardia, which could not be induced at the end of the procedure.\nIn addition, importantly, while attempting to check the line of block at the CTI by pacing the lateral portion of the CTI, activation of the LA was noted to change postablation (, occurring over BB with DCS activation occurring prior to PCS as compared to while in tachycardia. |
Case 1 was a 60-year-old woman. She was raised in a coastal area in a small town, in what is described as a well-functioning family, and has been trained as a healthcare assistant. Prior to moving into the nursing home, she had been married and had two adult children, with whom she had no contact. She had gradually increased her consumption of alcohol over the years, and in spite of several contacts with outpatient services for alcohol dependence, her drinking had steadily increased. After losing her job, she became increasingly socially isolated. Prior to moving into the nursing home, her home nurses visited several times per day, and they often found her in severe withdrawal, occasionally convulsing. She was underweight and incontinent. Her apartment was untidy and rarely cleaned, smelled of urine and feces and evinced her lack of personal hygiene. She was depressed and talked about suicide.\nAfter moving into the home, she gradually became stable, and was able to manage her personal hygiene with minimal assistance. She ate at meals and began to look better. She was still drinking, but at a level that did not cause problems with other residents. Occasionally, she drank heavily for 1–2 weeks. Her contact with other residents and staff stabilized, and she participated in simple practical activities. She seemed less anxious, and did not go through serious withdrawal.\nDuring the 18-month period before moving into the nursing home, she had been hospitalized nine times for periods ranging from 1 to 19 days; in total, she spent 43 days in hospital, had one outpatient visit and several ER visits. The total cost of her hospital-based care was estimated to be 154,649 DKK (20,798.74 Euros).\nAfter moving into the nursing home, she was admitted to inpatient treatment on two occasions for a total of 4 days. The total cost was 25,226 DKK (3392.64 Euros). She also had two visits at an emergency room (ER) and four outpatient visits. |
A 40-year-old Hispanic man with a past medical history of human immunodeficiency virus (HIV) was brought to the emergency department complaining of right upper extremity (RUE) weakness and numbness for four days with associated bitemporal headache and generalized fatigue. The patient reported first time use of intranasal cocaine and heroin, after which he lost consciousness and woke up approximately four hours later with new onset RUE and headache. His cluster of differentiation 4 (CD-4) count was reported above 500 cells/mm3 and viral load (VL) was undetectable. The patient did not have any known CNS complications in the past.\nOn physical examination, his blood pressure was 151/97 mm Hg and pulse was 82 and regular. He was alert and cooperative. His cranial nerves were intact. His motor exam, however, was abnormal in the RUE with 3/5 arm strength and wrist drop; the strength and tone of the other extremities were normal throughout. Deep tendon reflexes were normal bilaterally, but his gait could not be evaluated. His sensory function decreased to pin sensation at the RUE and normal sensation was noted in the rest of the extremities and face. Laboratory testing was normal except for an elevated creatinine of 6.9 mg/dl, creatine phosphokinase (CPK) of 7855 IU/l, alanine transaminase (ALT) of 139 IU/l, and aspartate transaminase (AST) of 109 IU/l. Urine toxicology was positive for metabolites of cocaine and heroin. Magnetic resonance imaging (MRI) of the brain was done and it revealed two areas of increased T2/FLAIR signal within the medial aspect of both basal ganglia, measuring 16 mm in the right and 12 mm on the left involving each globus pallidus and the genu of the internal capsule, as can be seen in Figures -. His chest radiography was normal, computerized tomography (CT) of the brain, as can be seen in Figure , and cervical spine were normal. His electrocardiogram was normal.\nIn the subsequent days, his kidney function and rhabdomyolysis improved. The patient remained fully awake, alert and oriented, but the weakness of his RUE persisted. The patient decided to leave against medical advice despite full explanation of the risk of leaving.\nThe patient was contacted over the phone and he informed us that he followed up with his primary care physician and reported improvement of the weakness. He received physical therapy and was independent in all activities of daily living and functional mobility. His only limitation was a moderate decrease in fine motor coordination of the RUE. |
A 23-year-old female patient came to our orthopedic clinic with chief complaints of pain and swelling in her left knee for four months. The pain on the left knee was gradual in onset, progressive in nature, and dull aching in character. Pain usually increased at night, was aggravated by walking, and was relieved by rest. On examination, there was antalgic gait with diffuse swelling over left knee, and tenderness was present over medial condyle of left femur. Knee ROM was five to 45 degrees. Anteroposterior and lateral view radiographs of the knee were obtained (Figure ).\nRadiographs revealed a small radiolucent lesion on the lateral aspect of the medial femoral condyle of the distal femur. MRI also showed a well-defined lesion of increased signal intensity on the lateral aspect of the medial femoral condyle (Figures -).\nSince signs of synovitis were present, arthroscopically assisted drainage was planned. The lesion was located on the lateral aspect of the medial condyle and was easily approachable using the arthroscope. Standard anterolateral and anteromedial portals were made. A guide wire was passed into the abscess cavity (Figure ) via the anteromedial portal, and the position was checked using fluoroscopy. Then a cannulated drill was passed over the guide wire to decompress the cavity (Figure ). Pus was seen extruding from the cavity. An arthroscopic burr was used to clean the cavity wall thoroughly.\nPostoperatively, intravenous antibiotics were given for eight days followed by oral antibiotics for another two weeks. Physiotherapy was started from the third postoperative day. The drain was removed after 72 hours, and the patient was discharged on the eighth postoperative day. At the time of discharge, knee ROM was zero to 45 degrees, and at the one-month follow-up, knee ROM was zero to 90 degrees. At the two-year follow-up, there was no pain, with ROM from zero to 120 degrees. |
A 63-year-old man presented for a second opinion on the management of a comminuted C1 burst fracture he had sustained after a fall down a flight of stairs before 6 months. Of note, the patient had a history of an uncomplicated C3 to C6 anterior cervical disectomy and fusion for radiculopathy done by an outside surgeon a few years before sustaining this injury. In addition to the Jefferson fracture, the patient also sustained an unstable C6 facet fracture. This was treated with a C3-T4 posterior decompression and instrumented fusion two days after his initial injury by a separate surgeon. At that time, the Jefferson fracture was treated nonsurgically in a cervical collar. The decision to treat only the C6 fracture was because of the patient's request despite the recommendation of surgical intervention by the initial treating surgeon. Over the next 6 months, follow-up imaging noted continued nonunion and increased right-sided displacement (Figure ). The patient was seen for a second opinion because of his continued pain and torticollis and was subsequently offered surgical intervention. The patient was admitted the day before surgery and placed in 15 pounds of cervical traction with the goal of attaining improved alignment before surgery. On the day of surgery, anesthesia was induced and video-assisted laryngoscopy was used to attain endotracheal intubation. No difficulties were noted during the intubation. The patient was then transferred to the operating table and placed in the prone position. Using a posterior approach, occiput to C3 posterior spinal fusion was done (Figure ). Connectors were used at C3, C4, and C5 to attach rods from the occipital plate to the previously placed construct. Owing to the chronicity of the patient's malalignment, the cervical traction was the sole method of reduction. The Gardner-Wells tongs were then removed, and the patient was extubated and transferred back to the hospital bed. The patient was noted to have been intubated for a total of 183 minutes. No complications were encountered intraoperatively, and the patient remained hemodynamically stable throughout the perioperative period. Approximately 2 to 3 hours postoperatively, the patient was observed to have developed significant dysarthria and difficulty swallowing. On further evaluation, the patient was also noted to be unable to protrude his tongue. A neurologist was consulted, and the patient was diagnosed with an isolated bilateral hypoglossal nerve palsy. Perioperative MRI imaging of the patient's brain and neck along with both a head and neck computed tomography angiography (CTA) were reviewed and were negative for any identifiable cause including vascular abnormalities, compressive pathology, or entrapment of the nerve in the fracture.\nOwing to the patient's dysphagia, speech therapy and a nutritionist were consulted to provide and assess the safest method for the patient to attain adequate nutrition. After a thorough evaluation, the patient was deemed able to attain sufficient nutrition with nectar consistent foods administered with a syringe.\nAlthough discussing the patient's diagnosis, the patient recalled he had sustained a similar injury after his C3-T4 posterior spinal fusion that had lasted only a few days. Per the patient, he developed mild dysarthria and difficulty swallowing, which he thought only affected the right side of his tongue. These symptoms completely resolved over the next few days after the initial onset. In retrospect, this may have been an unilateral palsy secondary to airway management or because of continued fracture displacement.\nThe patient was discharged on postoperative day 5. He made slow improvements over the next several weeks. At 6 months postoperatively, the patient's speech had significantly improved; however, he still had some residual difficulty with swallowing and required continued speech therapy on a weekly basis. |
A 9-month-old girl was referred for evaluation of bilateral, intraocular white tumors that were noted during evaluation for strabismus by a pediatric ophthalmologist. Past family, medical, and ocular histories were unremarkable. Anterior segment examination was normal in both eyes. Fundus examination of the right eye disclosed a white retinal tumor centered on the macula extending to the optic disc, surrounded by shallow subretinal fluid containing a few small subretinal seeds []. Fundus examination of the left eye showed three discrete white retinal tumors, the largest of which involved the entire macula and prevented visualization of the optic disc []. The tumor complex was surrounded by subretinal fluid that contained numerous subretinal seeds. Magnetic resonance imaging of the orbits and brain confirmed bilateral enhancing intraocular masses consistent with retinoblastoma, without evidence of retrobulbar or intracranial extension. Based on the International Intraocular Retinoblastoma Classification (IIRC), the patient was diagnosed with group C disease in the right eye and group D disease in the left eye.\nAfter discussion of potential benefits and risks of currently available management options with the patient's family, bilateral SOAIC was chosen as initial treatment. Monthly tandem SOAIC was initiated with single-agent melphalan at a dose of 6 mg. After the first two cycles, both eyes showed partial regression of all retinal tumors and subretinal seeds with complete resolution of secondary serous retinal detachment. However, examination after the third round of SOAIC showed no further shrinkage of still viable-appearing retinal tumors. The melphalan dose was increased to 8 mg and carboplatin (60 mg) and topotecan (0.6 mg) were added to the regimen. Four more cycles of SOAIC, eight total, and two sessions of local consolidation in the form of transpupillary thermotherapy (TTT) were administered in both eyes. No evidence of systemic toxicity was appreciated clinically, and complete blood count revealed mild myelosuppression.\nDespite treatment, calcific lesions with fleshy, residual areas of viable tumor were present in both eyes [], and intravenous chemotherapy with carboplatin (18.6 mg/kg), etoposide (5 mg/kg), and vincristine (0.05 mg/kg) was initiated as salvage therapy. Complete clinical regression was achieved in each eye following the third cycle of systemic chemotherapy [], and one additional cycle of systemic chemotherapy was administered for consolidation. The patient has remained in clinical remission 24 months after completion of intravenous chemotherapy, with rare episodes of emesis in the immediate post-treatment period and limited myelosuppression not significant enough to warrant a transfusion. |
An 81-year-old female patient presented to our emergency department with acute onset of hematemesis and melena. On admission, the patient appeared to have a poor general health condition and was hemodynamically compromised. Her initial laboratory exams revealed a hemoglobin concentration of 7.7 mg/dl without leukocytosis or C-reactive protein (CRP) elevation. The patient had a history of a 6.5 cm gastrointestinal stromal tumor (GIST) of the cardia, for which she initially received downsizing treatment with imatinib 400 mg/d, followed by surgical resection of the gastroesophageal junction and reconstruction with a jejunal interposition (Merendino procedure) 4 years earlier (). Histopathological studies of the tumor revealed a T3-tumor with a positive c-Kit mutation in exon-11 and a Ki-67 proliferation rate of 15%. The risk for disease progression based on Miettinen's criteria was determined as high (size > 5 cm; mitosis rate > 5/HPF), and the patient was subsequently placed on 1st line adjuvant therapy with imatinib 400 mg/d []. After two years on 1st line treatment, the patient developed hepatic and peritoneal metastasis and was placed on sunitinib as 2nd line therapy for metastatic GIST. Treatment with a proton pump inhibitor (PPI) was suspended for an unknown reason two years prior to presentation.\nThe patient was immediately transferred to our intensive care unit, where she was intubated and received two units of packed red blood cells (pRBCs). An emergency gastroscopy was carried out, which revealed active bleeding from a vessel stump in the jejunal interposition, corresponding to a Forrest stage I b upper gastrointestinal bleed. The bleeding was successfully stopped endoscopically by local injection of adrenaline and the application of polymer powder. A CT scan of the thorax and abdomen showed no signs of active bleeding or free abdominal fluid (). The known hepatic and peritoneal metastasis were described as constant in size, but increasingly necrotic compared to a previous CT scan. Due to a renewed drop in the hemoglobin concentration during the course of the day, a repeat gastroscopy was performed. This time, it showed diffuse bleeding without a circumscribed source. As a result, we acted to stabilize the coagulopathy by transfusing the patient with 9 units of pRBCs, 6 units of fresh frozen plasma (FFPs), and 6 units of platelet concentrates. In addition, the patient received 3 g of fibrinogen and 4000 IU of PPSB®, a prothrombin complex concentrate containing the coagulation factors II, VII, X, and IX.\nOn the second day after admission, a temporary improvement in the clinical condition of the patient was observed. It was possible to extubate the patient, who was hemodynamically stable with no signs of active bleeding. A phase of atrial fibrillation was cardioverted following treatment with a beta blocker, digoxin, and amiodarone. On the third day following her admission, the patient's condition deteriorated rapidly with the occurrence of fever, gross hematuria, and decreased oxygen saturation. A delayed hemolytic transfusion reaction was suspected, and positive Rh antibodies (anti-c antibody) were detected. Clinically as well as biochemically, the patient was suffering from a hemolysis with a decline of the hemoglobin concentration to 4.8 mg/dl and an increase of lactate dehydrogenase (LDH) to 3842.0 U/l. Given the lack of a septic focus, only a marginal increase in the inflammatory parameters, and pending blood culture results, no antibiotic treatment or surgical therapy was initiated. Due to imminent respiratory failure, the patient was reintubated. Vasopressors, atropine, and crystalloid solutions were administered to treat bradycardia and shock. However, the patient died on the same evening, following an unsuccessful cardiopulmonary resuscitation.\nThe results of blood cultures taken on the day of the patient's death revealed gram-labile rods without bacterial growth after two days. The subsequent external analysis confirmed bacteremia with C. perfringens and the detection of the alpha toxin gene by polymerase chain reaction, but without any traces of the beta toxin, enterotoxin, epsilon toxin, or iota toxin. The autopsy of the patient revealed a 6 cm sized local recurrence of the GIST and multiple necrotic liver metastases. In addition, a diffuse spread of C. perfringens in multiple organs with advanced tissue lysis was histologically confirmed (Figures –). The mucosal ulcer of the jejunal interposition was located 1.5 cm distal to the esophagojejunal anastomosis, which itself was intact. Death due to a septic-toxic shock caused by C. perfringens sepsis was determined as the cause of death. A contamination of the administered blood products with C. perfringens as the source of the infection was excluded by a subsequent analysis, which was confirmed by an external laboratory. |
A 20-year-old female with a 2-year history of systemic lupus erythematosus (SLE) presented with complaints of painless progressive decrease in vision in her right eye of 5 months duration. She had been diagnosed with SLE 5 years back, and had additionally developed lupus nephritis and pulmonary artery thrombosis, for which she was on treatment in the form of systemic corticosteroids, immunosuppressive medication mycophenolate mofetil at a dosage of 2 g a day (divided), tablet hydroxychloroquine 400 mg a day and an anticoagulant. Her systemic condition was well controlled, according to her rheumatologist. A year earlier, she had undergone treatment for SLE retinopathy with intravitreal ranibizumab and pan-retinal photocoagulation in both eyes, elsewhere. She had undergone a carotid doppler test at that stage, which had been reported to be within normal limits.\nHer best corrected visual acuity was 20/20 with a normal intro-ocular pressures and an anterior segment exam. Fundus evaluation revealed a rhegmatogenous retinal detachment in the right eye. Left eye revealed a healthy disc with no disc pulsations, a healthy macula on direct examination with a 78 D lens, and clinically normal retinal vessels with no active neovascularization noted at the disc or elsewhere. Trace old vitreous hemorrhage inferiorly with peripheral laser scars and regressed neovascularization was noted in the temporal periphery. An FFA [ and ], multicolor imaging, optical coherence tomography (SD-OCT), and SD-OCT angiography (OCTA) (Spectralis, Heidelberg, Germany) was performed. Two interesting features were noted.\nFirstly, the FFA was unremarkable till the peak phase. At 27 s an increase in arteriolar fluorescence in all the four major arterioles, with a simultaneous decrease in venular fluorescence without a change in vessel caliber was seen. Fluorescence increased in the venules at 29 s, with a reduction in arterioles. This cycle repeated at 43 s. Peripheral areas of nonperfusion with areas of leakage were seen suggestive of neovascularization.\nSecondly, the multicolor image of the left eye [] showed an undulating retinal surface infero-temporal to the disc, apparent in the blue and green channel, absent in the infrared. Corresponding changes were seen on the SD-OCT [] as an area of inner retinal thinning, on the enface OCT image as dark patches, on the OCTA [] as a flow void in the superficial and deep capillary plexus and on the FFA as a subtle ill-defined hypofluorescence in the early phase, persisting into the mid and late phase of the angiogram.\nNo conjunctival blanching was noted when the patient was examined earlier or after the fluorescein angiography. The patient was re-evaluated by the rheumatologist who advised no additional treatment. The right eye retinal detachment was managed surgically and the left eye was treated with additional laser photocoagulation. |
A 31-year-old woman with a singleton pregnancy missed the regular prenatal checkups (including her second trimester ultrasound) due to the COVID-19 pandemic and came to our hospital for a fetal ultrasound examination at 34+6 weeks of gestation. A 1.3 × 1.4 cm cyst was found on the right side of the fetal thorax (). Further, a fetal MRI was performed at 35+3 weeks of gestation. The MRI showed a 1.5 × 1.2 cm × 1.5 cm cystic lesion in the right thoracic cavity of the fetus, which was considered as a congenital BC (). The woman was not able to undergo prenatal genetic diagnosis because of the late gestational age. A multidisciplinary consultation was implemented by the pediatric surgery, radiology, and obstetrics departments, and a regular follow-up was recommended. There was no significant change in cyst size during the follow-up. A planned cesarean section (C-section) was performed at 38+5 weeks of gestation due to a previous C-section and rejection of vaginal delivery. The reaction of the baby girl was good, with Apgar score 9 at 1 min and 10 at 5 min after birth.\nA postnatal chest CT scan confirmed a cystic lesion of about 1.6 cm on the right upper mediastinum (). The child was breastfed and had a normal growth curve during the follow-up. A reexamination of chest enhanced CT scan was performed at 4 months of age. A 2.4 × 2.3 × 2.1 cm low-density shadow was shown on the right upper mediastinum, without enhancement after injection of a contrast agent. Considering the gradual enlargement of the cyst, the multidisciplinary team proposed a surgical treatment plan. With COVID-19 testing negative, doctors for infectious disease evaluated the risk of infection and took preventative measures during the COVID-19 pandemic. Pediatric surgeons carried out a video-assisted thoracoscopic surgery with closed thoracic drainage for the 5-month old girl. Subsequent pathological findings () confirmed the diagnosis of BC. The patient's recovery was smooth, and she was discharged after 6 days. The girl recovered well with normal growth and development at present. |
A 60-year-old male was referred to the ophthalmology clinic with recurrent uveitis of the right eye and severe corneal edema. He had undergone cataract surgery in both eyes 15 years previous. He denied any history of ocular trauma or ocular surgery other than cataract surgery. He had been diagnosed with anterior uveitis one month previous at a private eye clinic, for which administration of topical 1% prednisolone acetate led to remission. Anterior uveitis recurred with severe corneal edema one month after the first episode. Despite topical and systemic steroid treatment, the anterior chamber (AC) inflammation and corneal edema worsened, leading to the referral of this patient to our clinic.\nAt initial presentation, visual acuity was 0.2 OD, and 0.9 OS using a Snellen visual acuity chart. Intraocular pressure (IOP) was 15 mmHg OD and 17 mmHg OS as measured with a non-contact tonometer (NCT). An OD slit lamp examination revealed grades 1 and 2 AC reactions with flare and severe corneal edema. A small whitish fragment, previously diagnosed as hypopion at the private eye clinic, was observed in the inferior portion of the AC (). After dilation with mydriatics, a retained lens fragment was identified in the superior capsule. The OD vitreous and fundus were not visualized due to severe corneal edema, but B-ultrasonography revealed no remarkable signs. Specular pachymetry of the right eye was not possible due to the severe corneal edema and endothelial decompensation. It was concluded that the whitish material in the inferior angle of the AC was a retained lens fragment, because a retained lens fragment was also discovered in the superior capsule.\nSurgery was performed on the right eye under local anesthesia. A clear corneal incision was made at the superior cornea, and hyaluronate sodium (13.8 mg/0.6 mL/SYR, Healon 5 inj; Meditip Co., Seoul, Korea) was injected into the anterior chamber. The lens fragment was removed with lens forceps, and the remaining hyaluronate sodium was removed with irrigation and aspiration (Stellaris Vision Enhancement System; Bausch & Lomb, Rochester, NY, USA).\nOn postoperative day 1, OD corneal edema was slightly aggravated, and visual acuity was limited to a finger count at 10 cm. Measurement with NCT revealed an IOP of 15 mmHg. After one month, OD corneal edema was much improved, and visual acuity had increased to 0.4 by Snelln visual acuity chart. A two-month post-operative OD follow-up examination revealed reduced corneal edema and subsequent improvement of visual acuity to 0.7 by Snellen visual acuity chart (). Although the OD cornea was clear, an endothelial cell count showed 458 cells/mm2, and specular pachymetry revealed significant loss of hexagonality (). |
A 57-year-old man presented with a flu-like syndrome and right neck swelling. Fine-needle aspiration of the right neck mass revealed squamous cell carcinoma, and a CT scan of the head and neck revealed an enlarged right jugulodigastric lymph node (2.9 × 2.5 cm). A CT scan of the chest showed mediastinal and bilateral hilar lymphadenopathy with bilateral interstitial nodular opacities in the upper lobes. A diagnostic PET scan showed the right jugulodigastric node to have a standardized uptake value (SUV) of 5.98 with bilateral oropharyngeal radiotracer activity. Surprisingly, radiotracer uptake in the oropharynx was higher in the left tonsil compared to the right. The patient underwent two rounds of panendoscopy with biopsies which revealed, respectively, mild dysplasia of the right tonsil and a friable and nodular inferior border of the right tonsil containing carcinoma in situ. Diagnostic right tonsillectomy showed extensive squamous cell carcinoma in situ with a high suspicion of invasion. The left tonsil was specifically noted to be clinically unremarkable.\nA repeat PET/CT was performed for the purposes of radiotherapy planning and this study confirmed the presence of a 3 cm right neck level II lymph node with an SUV of 7.3, with central necrosis (). A diagnostic head and neck contrast-enhanced CT was included as part of the PET/CT study, as is our institutional practice and review of the CT elicited concern for extracapsular spread. FDG-avidity was again noted bilaterally in the oropharynx, with the intact left tonsil showing a maximum SUV of 8.7 as compared to maximum SUV of 6.6 in the tonsillar bed on the right (). However, the left tonsil was noted to be unusually bulky and irregular on the CT portion of the imaging study (). The PET/CT also demonstrated mild, symmetric FDG avidity in the mediastinal and hilar regions, which was considered more consistent with an inflammatory rather than neoplastic process, given the low FDG uptake (). High-resolution chest CT also suggested sarcoidosis. Due to the possibility that the asymmetric uptake and enlargement of the intact left tonsil might have been caused by the recent right tonsillectomy, the patient underwent a diagnostic left tonsillectomy. While the appearance of the left tonsil was still normal on examination, pathologic analysis revealed extensive in situ and invasive squamous cell carcinoma (). Immunohistochemical staining for p16 was diffusely and strongly positive (). Concurrent cisplatin was recommended due to the radiographically identified extracapsular extension, but the patient chose cetuximab in conjunction with intensity-modulated radiotherapy. Radiation treatment targeted the bilateral tonsillar beds and the right neck lymph node. The prescription dose was 6996 cGy, delivered over 33 fractions of treatment, with weekly localization using conebeam CT imaging to position the patient on the radiation therapy table. The concurrent cetuximab was delivered over 8 weekly infusion cycles and was well tolerated, with development of a moderate skin rash as is typical for this class of targeted therapy. After 2 years of followup, the patient is well with no evidence of recurrent cancer and his sarcoidosis remains stable. |
A six-year-old boy presented with symptoms that included shock-like jerky movements of the limbs, inability to hold his neck, functional urinary incontinence, and dependence on the mother for most daily activities. There was no visual deficit, with normal ophthalmoscopic examination. A physical examination revealed hypertonicity and hyperreflexes in both upper and lower limbs with a positive Babinski sign. He was unable to speak but otherwise appeared to have normal intellectual ability. The family history reveals a third degree consanguineous parents with a history of Krabbe disease. The other child of the same parent was observed to be normal. Initially, the patient's mother found a defective suckling of milk at birth, which was compensated by bottle feed and weaning. The milestones were normal until the age of two when the patient developed fever associated with seizures. This episode of seizure was misdiagnosed as a common febrile seizure. After this episode there was progressive spastic tetraparesis that made him unable to walk, talk, and caused difficulty in swallowing. The patient was reported to suffer from approximately twenty episodes of seizures a day. He had great difficulty in swallowing and had to be fed only a semi-solid and liquid diet.\nA brain magnetic resonance imaging (MRI) showed demyelination in the brain stem and cerebellum. A brain magnetic resonance (MR) spectroscopy revealed elevated myo-inositol and choline-containing compounds with decreased N-aspartyl aspartate in the affected white matter area, and the grey matter showed neuronal degeneration. Mutational analysis by deoxyribonucleic acid (DNA) sequencing revealed a missense mutation on chromosome band 14q31.3, coding for GALC gene confirming Krabbe disease. The patient was advised to undergo physiotherapy to help maintain muscle tone and circulation. Cerebrospinal fluid evaluation, electroencephalography (EEG), and electromyography (EMG) tests could not be performed due to patient's concerns.\nThe patient was prescibed anticonvulsant drugs to minimize the seizure episodes and drugs to ease muscle spasticity. |
A 17-year-old male suffered a severe motorcycle accident with a deep upper abdominal blunt trauma. Soon after the accident he was resuscitated and taken for emergency laparotomy at the nearest Trauma Centre. A massive right-sided retroperitoneal hematoma was detected, with active haemorrhage from a severely disrupted pancreatic head, duodenum and lower biliary tract. An extended Kocher manoeuvre was performed while compressing the hepatoduodenal ligament for haemostasis. At that time a main pancreatic duct disruption was demonstrated, without any evidence of the portal or the superior mesenteric vein involvement. The patient was hypothermic and moderately acidotic, fostering a DCS. After multiple sutures around the avulsion areas, the active bleeding was stopped and a cholecystectomy, complemented with a silicone T-tube drainage of the main bile duct and a gastroenterostomy were performed, as well as a duodenostomy with a Foley catheter. Two large silicone drains were placed and the patient was transferred to Lisbon by plane, with a laparostomy.\nThe patient was admitted in the Intensive Care Unit (ICU) of Curry Cabral Hospital where supportive care was initiated. Improvement of physiological parameters and hemodynamic stability was achieved in 48 h, despite a progressive biliary drainage from the silicone rubber drains.\nA second surgery was then decided, revealing bile staining throughout the entire peritoneal cavity, revealing slight oedema of the bowel loops without contamination. A Whipple’s resection was performed, exposing a main bile duct of around 5 mm diameter and a centred and easily located 3 mm main pancreatic duct: they were both cannulated with seized calibre silicone drains exiting the abdomen through the skin. Before closing the abdomen both catheters were secured to the surrounded parenchyma with a nylon transfixed suture, and the right upper quadrant was drained with two closed suction systems (Fig. ).\nThe post-operative course was uneventful. Fourteen days later the reconstructive operation was performed without any unusual factors, and a classical Whipple operation was completed after removal of the well-placed silicone endoluminal catheters. Again, the post-operative course was uneventful and the patient was discharged from the hospital at post-operative day 8.\nThis patient was a 47-year-old male paddle-surfer who suffered a severe deep and blunt trauma in the upper abdomen with his paddle-surf board. He was immediately transported to the nearest emergency room (Trauma Centre of Portimão Hospital), where he was submitted to a first medical evaluation. At that time, he was hemodynamically stable but with severe abdominal tenderness and acute abdomen signs. An abdominal CT scan revealed a haemopneumoperitoneum with distinctive evidence of pancreatic contusion in the uncinated process and duodenal rupture. No main pancreatic duct disruption was shown. He was then sent to emergency surgery and a median laparotomy was performed. There was a 300 cc haemoperitoneum with its origin in the duodenum and the head of the pancreas, but as in the first clinical setting, there was no lesion detected in the portal or the superior mesenteric vein. A more than 75% rupture of the second duodenum was confirmed, with alleged bile duct integrity and a complete main pancreatic duct disruption in the neck (Fig. ). A DCS was performed, with haemostasis, a duodenostomy with a Foley catheter, a silicone T-tube drainage of the main bile duct (after a normal cholangiography), cholecystectomy and a gastroenterostomy (Fig. ). Again, two large silicone rubber drains were placed and the patient was transferred by plane to the HPB Unit of Curry Cabral Hospital, with a laparostomy.\nThe patient was admitted in the ICU of Curry Cabral Hospital, obtaining an improvement of physiological parameters and achieving hemodynamic stability in 48 h, once again with a progressive drainage rich in amylase from the silicone rubber drains.\nAs the drainage turned biliary, a second surgery was then decided, confirming bile content throughout all the peritoneal cavity, with considerable oedema of the bowel loops. A Whipple’s resection was performed, exposing a main bile duct of around 4 mm diameter and a centred and easily located 3 mm main pancreatic duct with a normal consistency parenchyma. The same surgical protocol was used, cannulating both ducts with seized calibre silicone drains exiting the abdomen through the skin (Fig. ).\nAgain, both catheters were secured to the surrounded parenchyma with a nylon transfixed suture, and the right upper quadrant was drained with two closed suction drains. The abdomen was then closed.\nThe post-operative course was uneventful. Sixteen days later, the reconstructive operation was performed just as in the previous case, and a classical Whipple was completed after removal of the well-placed silicone endoluminal catheters.\nThe post-operative course was uneventful until the 5th day. At this point, there was progressive abdominal pain and a high debt biliary fistula, both from the drain and the abdominal wall. A relaparotomy was then performed, with extensive peritoneal lavage, confirming an impenetrable and sealed upper abdominal compartment: the suction drains were changed and the laparotomy was closed with separated full-thickness stiches.\nAfter conservative measures and serial image control, the fistula underwent a progressive closure in 15 days, and the patient was discharged from the hospital at post-operative day 50. He reported returning to his normal lifestyle without any limitations. |
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