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A 25 year old G3P2L2 at 15 weeks of pregnancy was referred to the emergency of our hospital from another Centre with diagnosis of pregnancy with acute abdomen. She had previous two uneventful vaginal deliveries at home with last child birth 3 years back. She presented to our emergency with pain abdomen for 2 days and vomiting for 1 day. There was no complaint of per vaginal bleeding. She gave no history of medical abortion or any instrumentation done. She had gone to a rural hospital for this complaint where she was conservatively managed and an obstetric ultrasound was done documenting a 15 weeks single live intrauterine pregnancy. But when her condition started deteriorating, she was referred to our centre for further management. Our hospital, B.P. Koirala Institute of Health Sciences is a tertiary care referral hospital in Eastern Nepal.\nOn examination in our Obstetric emergency, she was ill looking with severe pallor. Her pulse was 140 beats/min, blood pressure 90/50 mmhg, respiratory rate 28 cycles/min. On abdominal examination, there was generalized tenderness. Bowel sound was present. On per speculum examination of vagina, there was no cervical pathology and no active bleeding was noted. On per vaginal examination, the uterine size could not be assessed due to tenderness and voluntary guarding. There was fullness in anterior fornix and tenderness in bilateral fornices.\nAfter admission, investigations were sent, blood arranged and resuscitation started immediately. Investigations sent in our hospital were: B positive blood group, Hb: 2.8 gm/dl, RBS: 78mg/dL, HIV, HepBsAg and VDRL negative. Ultrasonography done in emergency showed intrauterine pregnancy of 15 weeks period of gestation with no cardiac activity and hemoperitoneum. The patient was immediately prepared for emergency laparotomy. Abdomen was opened by midline infraumbilical incision. There was hemoperitoneum of approximately 1500ml. On further exploration, there was a fetus lying within the intact amniotic sac () in the ruptured right rudimentary horn of the unicornuate uterus. There was complete rupture of right rudimentary horn and was connected to the unicornuate uterus by a fibromuscular band (). No intracavitary connection was noted between the unicornuate uterus and the rudimentary horn. The left ovary and fallopian were attached normally to the unicornuate uterus. Excision of the ruptured right rudimentary horn and right fallopian tube with conservation of the right ovary was done. Hemostasis was achieved and drain was kept. She was shifted to the maternal intensive care unit where she received total 8 units of blood transfusion. Her post-operative period was uneventful and she was discharged on the fifth postoperative day in a stable and satisfactory condition.
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. ).
A 12-year-old girl presented to the Outpatient Clinic of Children's Hospital, Ain Shams University, Egypt, complaining of severe headache and tenderness over the scalp and right temporal area extending to the occipital region, of gradual onset, progressive course, and a 3-month duration. The headache was bursting, having its summit after school days, and was not associated with vomiting or blurring of vision. Ever since birth, the child was normal. At the age of 9, she suffered recurrent attacks of generalized edema diagnosed at another hospital as nephrotic syndrome for which she received several courses of daily oral prednisone at doses reaching 1.5 mg/kg. The response was incomplete but the patient's mother admitted that her daughter's compliance to therapy was poor and was off therapy for the preceding 2 months.\nThe child experienced frequent attacks of vertigo, fatigability, and bilateral knee arthralgia. There were no claudications, no Raynaud's phenomenon, and no history of rashes or photosensitivity or other system involvement. There was no history of a preceding viral infection or drug intake, and the patient denied any previous contact with animals. The family history was irrelevant.\nOn examination, her general condition was fair; she was fully conscious with puffy eyelids. Her temperature and respiratory rate were normal, but her radial pulse was bounding regularly at a rate of 86/minute and was equal on both sides. Her BP, measured at the arms, was 140/100 mmHg. Her weight was 30 kg and her height was 130 cm. The patient had a cord like nodular right superficial temporal artery over which the pulsations were barely felt and significantly decreased compared to the left side (). Several small tender nodular swellings were felt over the occipital area which the mother reported to have noticed a few months before. An audible bruit was heard by auscultation over the artery. The overlying scalp was tender. Pulsations over large arteries were normal with no audible bruits. There were no rashes. Ophthalmologic examination was normal with no audible bruit over the eye balls. There was bilateral pitting lower limb edema up to the thigh and ascites demonstrated by positive shifting dullness. No lipodystrophy was seen. Chest and heart were free. The joints were normal, there was no muscle tenderness, and she was neurologically free.\nThe investigations revealed high ESR (1st hr, 60 mm), hemoglobin: 10.2 g/dL, total leukocytic count: 6.900/mm3, and platelet count: 421.000/mm3. Her total serum proteins and albumin were 4.5 gm/dL and 1.4 gm/dL, respectively with a 24-hour proteinuria 2.7 gm and urinary protein/creatinine 4.3. Plasma protein electrophoresis showed hypoalbuminemia with increased alpha2 globulin and decreased gamma globulin. Serum creatinine was 0.6 mg/dL and creatinine clearance was 116 mL/minute. There were elevations in serum cholesterol, triglycerides ALT, and AST. The CRP was elevated (24 mg/L), p-ANCA was positive, and complement 3 decreased (41 mg/dL, normal: 55–120 mg/dL). Antinuclear antibodies and anti-DNA were negative. Plain X-ray chest and abdominal ultrasonography were normal.\nAn ultrasound-guided renal biopsy showed the following light microscopic findings. Renal tissue examined included 15 glomeruli. Four showed segmental sclerosis and periglomerular fibrosis. Other glomeruli were hypercellular with moderate to severe capillary wall thickening up to wire-loop formation. Foci of tuft necrosis were seen in 3 glomeruli. Hyaline thrombi were frequently seen in capillary lumina and occasional leucocytes. Tubules showed hyaline, RBC, and granular casts. Interstitial tissues showed clusters of foam cells and focal collections of chronic inflammatory cells yet there were no granulomas. Arterioles showed mild to moderate wall thickening. Electron microscopic examination revealed extensive electron dense deposits in subendothelial regions. Few mesangial and intramembranous deposits were seen as well. These findings were consistent with membranoproliferative glomerulonephritis (GN), Figures and .\nThe hypertension was controlled with antihypertensive drugs, but the headache and superficial temporal artery prominence and nodularity persisted. Hence, a temporal artery biopsy was planned but the parents did not consent to it. Duplex ultrasonography, performed 9 days after institution of steroid therapy, showed the diameter of the right superficial temporal artery to be narrower compared to the left (1.8 mm versus 2 mm) but with the same resistive index to blood flow (), whereas the carotid, vertebral, and abdominal arteries were normal.\nTreatment was instituted with oral prednisolone at 60 mg/day and the headache and temporal artery abnormalities gradually improved (); however, the nephrosis remained unresponsive. Oral cyclophosphamide was started at a dose of 50 mg/day with tapering of steroids and a significant response was observed.
A 23-year-old female patient reported to the Department of Periodontics and Oral Implantology with a chief complaint of missing tooth no. 46 [] for about 5 to 6 years. On clinical evaluation, it was found that the edentulous space in relation to tooth no. 46 was compromised due to drifting of the adjacent teeth and was measured to be about 7 mm at the cervical level and at the contact area the space was reduced []. The buccolingual width preoperative was 7 mm and the inter-occlusal distance was measured to be 6 mm. The patient was suggested orthodontic correction of drifted adjacent teeth to achieve space for placement of standard implant. The patient was unwilling for orthodontic intervention because of prolonged treatment duration. After discussing the pros and cons of the NDIs in the posterior region, the patient gave the consent for the same. Following thorough clinical [] and radiographic [] evaluation, it was decided to place a narrow diameter two-piece implant of size 3.0 × 13 mm.\nFollowing standard preparation protocol for implant surgery, local infiltration anesthesia lignocaine (1:80 000) was administered. Using a scalpel no. 15, a crestal incision was given and the full thickness flap was raised using a periosteal elevator. Osteotomy was started using the pilot drill of diameter 2.0 mm to a depth of 13 mm and parallelism was checked using a paralleling pin. The osteotomy was subsequently enlarged to 2.5 mm. A narrow diameter two-piece dental implant of size 3.0 × 13 mm was placed with good primary stability achieving a torque value of 45 Ncm []. Abutment was placed and trimmed to receive temporary restoration []. IRWL[] protocol was followed with no occlusal contact in centric occlusion and eccentric movements [Figures and ]. A 0.2% chlorhexidine mouth rinse was prescribed two/three times daily for two weeks following surgery; antibiotics and anti-inflammatory drugs were prescribed for 5 days.\nAfter uneventful healing of 3 months, the patient was evaluated clinically and radiologically. Soft tissue demonstrated satisfactory healing and intraoral periapical radiograph suggested no marginal bone loss after 3 months of healing process.\nThe temporary crown with abutment was removed and closed-tray transfer coping was placed and a radiograph was taken for analyzing the fit of the coping to the dental implant. A 1-mm shoulder narrow diameter abutment was transferred to the laboratory for milling and fabrication of metal coping prior to final prosthesis. The abutment and coping was tried in to check for marginal fit. Final porcelain-fused metal crown was placed [] with good emergence profile and radiograph revealed good marginal fit of the restoration [].\nThe patient was reviewed subsequently after 6 months clinically and radiographically [] with no bone loss. At one year, clinical evaluation revealed good soft tissue health and excellent emergence profile []; radiographic examination showed no marginal bone loss [].
A 65-year-old male presented to our department complaining of recurrent epigastric and upper right abdominal pain radiating to the back for the last 3 months. No other complaints were prominent. The patient had undergone an uneventful laparoscopic cholecystectomy 2 years earlier. His family history was consistent with a father who suffered lung malignancy and a mother who expired due to hepatoma.\nAn ultrasound performed prior to his admission revealed a hypoechoic 4 × 4 cm lesion close to the pancreatic head. On admission, abdominal examination revealed a soft, nondistended abdomen with local tenderness in the upper right quadrant. No other findings were noted. His blood tests were all within normal limits. CEA and CA 19-9 were normal. Abdominal tomography revealed a 5 × 5 cm, rounded, soft density lesion adjacent to the root of the superior mesenteric artery (SMA) and pancreatic head (fig. ). It was not possible to distinguish the origin of this lesion.\nThe patient underwent endoscopic ultrasound examination that demonstrated a hypoechoic-heterogeneous solid lesion behind and adjacent to the SMA and superior mesenteric vein. No other pathology was demonstrated in the pancreas itself (fig. ).\nPathology obtained by fine needle aspiration from the lesion revealed fragments of mesenchymal tissue that was positively stained for c-kit and actin. DOG immunohistochemistry was negative. Laparotomy revealed a large, 7-cm, hard tumor adherent to the medial border of the pancreatic uncinate process laterally, to SMA medially, posterior to the portal vein and anterior to the inferior vena cava and left renal vein (fig. ). The lesion was successfully dissected from these structures and was completely excised and removed.\nThe patient was discharged after an uneventful postoperative course. The pathological report revealed leiomyosarcoma stained to actin and desmin. The proliferative index Ki67 was 10%. He received radiotherapy, and at 4 years of follow-up, the patient is healthy and has no recurrence.
The patient is a 63-year-old Caucasian man. He is 1.61 meters tall and weighs 66 kilograms. After working as a car mechanic for 36 years, he had been receiving disability benefits for 11 years. He was working part-time as a caretaker averaging 10 hours per week until two and a half years ago. At the age of 35, the patient began having bouts of severe back pain approximately twice a year. When these episodes occurred, he took non-steroidal anti-inflammatory drugs for three to four weeks for pain relief. At the age of 40, lumbar spondylolisthesis was diagnosed by radiography. At the age of 50, he suddenly developed severe right hip pain. He suffered from substantial arthrosis on the right side, which was treated with a total hip replacement one year later.\nThree years ago, he began to develop neurological symptoms in both hands. Pain and loss of function of his fingers prevented him from working as a caretaker. The pain radiated from his neck bilaterally down to his fingers and was described as parasthesia-like in nature. He also complained of weakness in his hands. Left hand digit flexion was classified as J1 and right hand as J4. Digit extension was classified as J4 on the left and J2 on the right and abduction on the left was classified as J0 and on the right as J1. The patient also had difficulty walking (Nurick 4, EMS 10/18) as he had to support himself using his surroundings in order to stand upright and was unable to walk unassisted. He was referred to a neurologist by his general practitioner who in turn referred him to a neurosurgeon. Radiographs (Figure ) magnetic resonance imaging (Figure ) and computed tomography (CT) revealed a spondylolisthesis between C7 and T1. Using the Meyerding classification [], which was initially developed for grading the degree of lumbar spondylolisthesis, the patient would have been diagnosed with a cervico-thoracic spondylolisthesis of the second or third degree. The spondylolisthesis, measured using the method developed by Kawasaki et al. [], was 13 mm. In view of the special nature of the case, a collaborative treatment between neurosurgeons and orthopedic surgeons was favored and subsequently implemented.\nThe surgical management was performed as follows:\nThe patient was placed in the supine position. The intervertebral disk at C7-T1 was exposed using blunt dissection. The disk was then removed to the point of the ligamentum flavum. A subsequent resection of the lateral parts of the intervertebral discs significantly mobilized the spondylolisthesis.\nThe wound was closed and the patient was rotated to the prone position. First, the dislocation between vertebras C7 and T1 was reduced while positioning the head.\nSubsequently, the lateral masses were prepared from a dorsal approach between C5 and T3. Then, lateral mass-screws were inserted into C5 and C6. We had noticed considerable laxity between C6 and C7. In addition, C5 and C6 were naturally fused and we decided to include them in the instrumentation. There was also a rigid displacement between C7 and T1.\nBicortical screws were then inserted into the transverse processes of T2 and T3. These were inserted into at least two segments from both sides in divergent directions. The length of these screws was 10 to 12 mm, with a diameter of 3.2 mm.\nThis was followed by a laminectomy. Since the roots of C7 and C8 were exposed, the lateral masses of C7 and C8 had to be resected. Longitudinal rods were placed. Proper reduction in lordosis and the preservation of a 5 mm intervertebral space between C7 and T1 were confirmed by radiograph. Set screws were used to fix the instrumentation in the desired position. Chips of cortical and cancellous bone were placed lateral to the longitudinal rods.\nThe dorsal wound was then closed. The patient was again rotated to the supine position to reopen the ventral wound. Now, instead of the previously seen displacement, a large gap was visible between C7 and T1. The endplates between C7 and T1 were milled and the ligamentum flavum was resected using punches. Then, the spinal canal was exposed completely, using a Caspar opener.\nSubsequently, a Harms basket, filled with autograft bone chips, was inserted into the intervertebral space. Three drains were placed and the wound was closed. For prophylactic infection control, the patient was perioperatively given intravenous 1.5 g cefuroxime twice a day for the next four days. Intraoperative and postoperative radiographs (Figure and ) were taken to confirm correct placement of the instrumentation.\nFollowing the operation, the patient was transferred to the intensive care unit. His vital signs were stable enough for him to be transferred to the general postoperative recovery ward on the fourth postoperative day. Eleven days after surgery, the patient left the hospital and entered an orthopedic and neurologic rehabilitation program.\nThe pain and parasthesias in his fingers resolved after the operation. Two months postoperatively, his ability to walk had noticeably improved after completing his rehabilitation program (Nurick 3).\nToday, two years postoperatively, the patient is able to walk without assistance (Nurick 2). Flexion of the fingers on his left hand was graded as J3 and as J5 on the right hand. Extension of the fingers on his left hand was graded as J4 and as J2 on the right. Abduction was graded as J4 for his left hand and as J2 for his right hand (EMS 14/18).
We present the case of a 48-year-old male, who was evaluated by the medical genetics service because he had noticed weakening of his voice with a high pitch since age 35, associated with premature graying since his 30s and skin lesions since about the age of 40. At the age of 32, bilateral cataracts were diagnosed and at 44 he was diagnosed with diabetes mellitus, currently on oral hypoglycemic agents. Additionally, he has hypothyroidism and hypertriglyceridemia in management and calcification of the Achilles tendon. Patient endorses lack of an early adolescent growth spurt; however, final stature is similar to his other 3 siblings (164 cm). Patient reports he had no child by choice.\nPatient is product of the union of consanguineous parents (second cousins) and has a 49-year-old brother with similar clinical characteristics, including voice changes since the age of 28, bilateral cataracts at age 29 (subsequently presents complications from corneal ulceration and is currently legally blind), and premature graying since age 33, moreover, scleroderma-like skin changes since his 30s and diagnosis of type 2 diabetes mellitus at age 35. His brother also endorses no child by choice. No other complications such as atherosclerosis, dyslipidemia, hypertension, osteoporosis, or tumors were reported.\nUnfortunately, patient's brother and parents declined genetic testing. There are no other relatives with clinical suspicion of WS.\nPatient states maternal aunt has unspecified type leukemia and father with a history of acute myocardial infarction at age 65 and a diagnosis of melanoma at age 85. Maternal uncle diagnosed with lung cancer at age 72 and maternal grandfather with prostate cancer diagnosed at age 73.\nOn initial physical examination, he appeared much older than his age with “bird-like” facial appearance, beak-shaped nose, and bilateral cataracts, his voice was high-pitched and his hair and eyebrows were scarce and markedly gray. He had thin upper limbs with decreased subcutaneous fat and truncal obesity (). Moreover, we found short stature, hypogenitalism, lower limbs with markedly atrophied skin and subcutaneous fat, abnormal pigmentation of the skin and hyperkeratosis, and flat feet (Figures and ).\nWRN gene sequencing identified the homozygous variant NM_00553.4: c.2581C>T (NP_000544.2: pGln861Ter). WRN gene sequencing report can be found in Supplementary . This variant generates a stop codon at position 861 and has been classified as pathogenic and previously described in homozygous status in a Caucasian patient from the United States in 2006 [].\nLaboratory findings included normal renal function, high blood glucose (164 mg/dl), elevated glycosylated hemoglobin (9.4%), and elevated triglycerides (324.6 mg/dl) with normal cholesterol (162.4 mg/dl). EKG showed an elevation of the J point by early repolarization. Abdominopelvic CT-scan showed bilateral renal cysts, small umbilical hernia, and no fatty liver. Testicular ultrasound showed decreased bilateral testicular volume mainly left side.\nRegular screening for malignancies is recommended for patients with WS, due to the high risk of early-onset neoplasms. Also, it is very important to rule out cardiovascular and metabolic diseases during the follow-up of these patients. Our patient is still under periodic clinical observation and follow-up. Currently, he is on treatment with oral hypoglycemic agents for DM2 with adequate glucose control and in treatment of hypertriglyceridemia. Until now no signs of atherosclerosis or cardiovascular disease have been detected. However, he was recently diagnosed with refractory cytopenia with multilineage dysplasia, a form of myelodysplastic syndrome, which has required multiple transfusions.\nAccording to a clinical history, the patient's brother is being monitored for inadequate control of diabetes mellitus and severe skin lesions that have been difficult to treat, but no cancer has been documented.
Our case is of a 52-year-old gentleman who presented with restricted tongue movement, dysarthria, and dysphagia that developed progressively over several months. He mentioned this at the Ear Nose and Throat clinic during follow-up of surgical drainage of a mucocele from the pterygopalatine fossa six months before. He also reported 15 kg of weight loss in the preceding four months. He is a nonsmoker with no medical history except for osteomyelitis following a meniscal knee repair four years before.\nAn MRI showed a 37 × 34 × 42 mm mass in the base of tongue extending to the geniohyoid muscle as well as the genioglossus muscle that was most concerning for squamous cell carcinoma as shown in .\nHis PET/CT scan showed a large intensely FDG avid tongue mass arising in the posterior of the tongue within the body of the tongue and extrinsic muscles and extending posterosuperiorly into the region of the lingual tonsils and midline tongue superiorly. There was moderate FDG uptake in adjacent nodes consistent with metastases.\nMultiple bilateral rounded pulmonary nodules were present within the lungs bilaterally, the largest of which measured 5 mm. These showed no significant FDG uptake and were below the resolution of the PET/CT to accurately characterise.\nThe lung lesions were too small for biopsy to confirm metastasis; however, it was judged to be metastatic given the rapidly progressive disease and bilateral neck metastases. The tumour was staged as T4N2cM1.\nTwo tongue biopsies performed were inconclusive; however, the third biopsy a month later showed an infiltrating biphasic tumour with solid nests composed of epithelial and myoepithelial cells as shown in . The tumour cells also demonstrated perineural invasion as shown in . There was nuclear atypia and prominent mitoses present. Tumour cells were positive for SMA, p40, p63, CK5/6, EMA (patchy and luminal), CEA (luminal), and CD43. Ki-67 reveals a high proliferation index (60%). The diagnosis was epithelial-myoepithelial carcinoma with myoepithelial anaplasia.\nSubsequently, the patient was offered radical local treatment with radiotherapy and chemotherapy. The patient declined these so as to maximise his quality of life. He was referred to the palliative care service for continued care and over a year later required a percutaneous endoscopic gastrostomy (PEG) tube for feeding. Approximately 18 months after the diagnosis, the patient passed away from obstruction of the airway.
We present the case of a 30-year-old man who originally noticed a progressively increasing swelling in his left thigh 6 years back. Magnetic resonance imaging (MRI) at that time revealed a soft tissue tumor in the left thigh, with no osseous destruction, and a core needle biopsy confirmed high-grade MFH. () .\nThe patient was treated with neoadjuvant chemotherapy and underwent tumor resection followed by adjuvant radiation. He remained well until 6 years, when he noticed a 4x3x2 cm swelling in the upper right thigh associated with pain and progressive increase in size, with the present size of 10x6x4 cm. A digital skiagram revealed a pathological fracture in the proximal shaft of right femur. Multiple lytic lesions were noted within the proximal half of the shaft involving the medullary cavity and cortex with scalloping of the endosteal margin and focal destruction of the cortices. Surrounding soft tissue mass was seen. The MRI of the right femur revealed a lytic expansile mass within the femoral shaft involving the medullary cavity from subtrochanteric to middle-third of the shaft for a length of 22 cm with endosteal scalloping, cortical thinning and focal destruction of the cortex at many areas. A large lobulated heterogenous soft tissue mass postero-medially contiguous with the medullary lesion via large cortical defect was seen in posterior cortex of the upper and mid-3rd junction of femur. Radiological features were suggestive of a fibrosarcoma. ()\nHistopathology revealed a cellular tumor, with the cells arranged in a pallisading and storiform pattern, consisting mainly of spindle to oval and round cells. The cells were showing moderate degree of pleomorphism, with formation of tumor giant cells. The cells showed very high mitotic rate, many of which were atypical. No bone formation could be identified in the tumor. However, the surrounding area showed fibroosseous callus formation. ().\nDifferential diagnoses of MFH, osteosarcoma, leiomyosarcoma, MPNST and poorly differentiated carcinoma were taken into consideration. On immunohistochemistry examination, tumor cells were positive for CD68, Vimentin [] and focally for SMA. However, SMA was negative in majority of the tumor cells as well as in the fascicles formed by the tumor cells (ruling out leiomyosarcoma), and tumor cells were negative for S-100 (ruling out MPNST), osteopontin (ruling out osteosarcoma), CK and EMA (ruling out poorly differentiated carcinoma).\nThus, a final diagnosis of high-grade MFH was made. Staging CT scans and PET scan preoperatively revealed no evidence of pulmonary, abdominal or other metastasis. The patient underwent neoadjuvant chemotherapy followed by wide excision of the right thigh lesion, which on biopsy was consistent with the diagnosis of high-grade MFH. The surgical margins of the resected thigh lesions were negative. Currently the patient is undergoing adjuvant chemotherapy and radiotherapy.
In late 2016, a 61 year old non-smoking female presented with exertional shortness of breath, mild pedal edema, distended neck veins, and a recent weight gain of 15 lbs over the prior 2 weeks. It was initially suspected that the patient was experiencing pulmonary embolism based on her clinical presentation and elevated D-dimer, but further testing revealed that she was experiencing cardiac tamponade with mild pulmonary hypertension and heart failure. Pericardiocentesis was performed without definitive diagnosis. In February 2017, the patient exhibited worsening respiratory symptoms without fever, hemoptysis, sputum production, B symptoms, or extremity edema. Baseline chest PET-CT images were reviewed by radiologists at both UCLA Oncology and Texas Tech University Health Sciences Center, demonstrating a large middle mediastinal mass encasing the main pulmonary artery, with pericardial and left pleural effusion (Figure & Figure ). Scattered pulmonary nodules and hypodense lesions were identified in the right lobe of the liver, consistent with a diagnosis of metastatic disease (Figure ). Biopsy of the mediastinal mass revealed a high grade undifferentiated malignant neoplasm composed of highly proliferative (ki67 staining ~50%) pleomorphic anaplastic epithelioid malignant cells with large areas of necrosis and fibrosis. Immunohistochemistry revealed strong antigenicity for CD31 and CD34, and weak antigenicity for D2-40 and Factor VIII indicating a diagnosis of angiosarcoma that was corroborated at both UC San Diego Health and MD Anderson.\nConventional treatment options were recommended, however the patient declined these based on low reported survival rates, and instead, requested the non-selective beta blocker propranolol as a single agent therapy. In May 2017, 40 mg/kg propranolol was administered daily and PET-CT scans were performed at regular intervals to assess the response of the tumor to propranolol. Assessment of tumor response was based on 18F-fluorodeoxyglucose (FDG) tracer uptake and measurements/assessments of the primary tumor and distant metastases. After 12 months of propranolol as a single agent therapy, significant debulking and decreased size of the residual mediastinal mass was observed on PET-CT scans, with resolution of pericardial effusion (Figure & Figure ). Pulmonary nodules were stable to regressed, and the nodules in the right lobe of the liver had completely resolved (Figure ). There was no evidence of residual hyper-metabolic activity based on FDG measurements in the primary lesion or in metastatic sites in the chest, abdomen, or pelvis on PET-CT.
A 50-year-old woman presented with a lesion in the left lung for 20 months on routine examination. Computed tomography (CT) at a local hospital showed that there was an irregular mass in the upper lobe of her left lung, about 2.8 × 1.9 cm in size, with a clear boundary, peripheral spiculation, and stretched adjacent pleura. The patient had no symptoms such as cough, sputum, dyspnea, fever, and hemoptysis. One month ago, she underwent surgery for the left breast cancer in the local hospital. A second chest CT showed no significant changes in the mass. Positron emission tomography-CT (PET-CT) revealed an irregular high-density mass in the left lung with increased fluorodeoxyglucose (FDG) uptake, no significant abnormal FDG metabolism in the rest of the body, multiple small lymph nodes in the left axilla, and slightly increased FDG uptake in some lymph nodes.\nFor further treatment, the patient was admitted to our department. There was no obvious abnormality on physical examination. The chest CT showed an irregular soft tissue mass in the anterior segment of the upper lobe of the left lung, and the border was not clear. The cross-sectional area was about 3.2 × 2.2 cm, and the spiculation sign could be seen. The plain CT value was 30 HU. Calcification of the patch was obvious after enhanced scanning, and the CT value was 98 HU. The mediastinal structure was clear, and no obvious enlarged lymph nodes were seen (Fig. ). There were no significant abnormalities in blood sample tests, including tumor markers. The patient had suffered from tetralogy of Fallot and underwent surgery for a ventricular septal repair35 years ago. Breast-conserving surgery was performed for her left breast cancer 1 month ago, and the pathological report confirmed a lobular carcinoma in situ. She had a family history of lung cancer (grandfather) and no smoking history.\nAccording to the patient’s medical history and imaging findings, this mass was highly suspected to be malignant. The patient underwent video-assisted thoracic surgery (VATS) instantly without preoperative CT-guided percutaneous needle lung biopsy. The intraoperative palpation of the lesion suggested that the lesion was too close to the hilum to receive limited resection. So we performed the lobectomy of left upper lobe directly. The procedure was successful and the mass was completely removed.\nThe dissected mass was gray-yellow, and a blue medical suture was visible inside (Fig. ). The surrounding tissue showed inflammatory changes, and local necrosis was found. The suture is about 15 cm in total length. The pathology report suggested that the mass tended to be an inflammatory pseudotumor, and no malignant cell component was seen. The structure of the lung tissue in the lesion area was damaged. The proliferative vascular components were visible, the wall thickness was accompanied by hyaloid and mucus degeneration, and fibrous tissue hyperplasia with hyaloid. Lymphocytes, plasma cells, and neutrophils infiltrated in the lesion area, and the remaining alveolar epithelium showed reactive hyperplasia. The submitted lymph nodes also showed reactive hyperplasia. Immunohistochemical staining results: CD38 (+), IgG (+), IgG4 (focal +), IgG4 / IgG less than 40% +. Special staining results: silver hexamine (−), Congo red (−).\nThe patient recovered well and discharged on the 3rd day after operation. Follow-up after 3 months showed no special abnormalities.
A 27-year-old Emirati male presented to Cleveland Clinic Abu Dhabi (CCAD) emergency department (ED) for the first time in Sept 2015 complaining of severe abdominal pain. The pain has been episodic for the last 4 years and had significantly affected his work and family life. He was seen and admitted to multiple hospitals across Abu Dhabi, including our own, attended different specialists, and underwent a wide range of investigations including blood tests (CBC and differential count, liver and renal profiles and CRP), gastroscopies, colonoscopies, and CT scans and a laparoscopy. The results of all his clinical assessments and investigations did not show any sign.\nOn one of his acute admissions to CCAD, the immunologist was asked to review the patient. Detailed examination of the patient's medical history starting from the onset of symptoms reveled that he used to get 2 swelling episodes every week affecting his face, hands, feet or scrotum and severe abdominal pain twice a week. These swelling episodes and abdominal pain appeared suddenly without any obvious triggering factor, developed over 36 hours and resolved spontaneously in 5-7 days without any medication including analgesics. He reported no laryngeal swellings or respiratory compromise. He denied any fevers, night sweats, weight loss, change in bowel habits or blood in his stools. His swelling episodes were occasionally associated with non-pruritic red skin rash that was mistaken for chronic urticaria for which he was treated with Omalizumab (300mg every 4 weeks) for 9 months without any benefit. Moreover, his response to different types of analgesia, high dose antihistamines, antibiotics and corticosteroids was unsatisfactory. He is a thalassemia carrier; otherwise he is fit and healthy and has no past medical history of note. He has no family history of immunodeficiency, inflammatory bowel disease, autoimmunity or FMF. On examination, he was in pain and his abdomen was soft, tender with guarding and decreased bowel sounds. There was no rebound, rigidity, distension or ascites. He had no peripheral swellings.\nDuring his acute admission to CCAD, a review of his blood tests was performed that showed reduced C4 and absent C1 inhibitor function (Table). These tests were performed a month prior to his admission but were not followed up. His abdominal CT scan showed diffuse swelling and long segment of enhancing mucosal thickening involving the proximal jejunum and gastric mucosa with minimal free abdominal fluid (). In addition, he had a colonoscopy which showed severe mucosal edema in the transverse colon with occlusion of the lumen (). A provisional diagnosis of HAE was made based on his limited complement studies. As he had severe abdominal pain for 24 hours prior to his hospital admission, C1 inhibitor concentrate (2000 units IV over 10 minutes) was administered and within 2 hours his pain had almost resolved.\nAfter his recovery, he underwent detailed immunological investigations that revealed markedly reduced C4 level and absent C1 inhibitor function (performed manually, read on Shimadzu UV-1700 equipment) with normal C3 and C1q levels and raised C1 inhibitor serum levels (). His ENA, total immunoglobulin, CBC and differential count, serum protein electrophoresis, liver function tests, hepatitis serology, lipase, amylase, tissue transglutaminase, stools tests and urinalysis did not show any significant abnormality.\nHe was diagnosed with type 2 HAE based on his abnormal complement studies () and was commenced on tranexamic acid for 3 months. He did not want to start with attenuated androgens because he was concerned about their adverse effects. However, he continued to get abdominal pain even when the dose of tranexamic acid was increased to 3 grams daily. He was then switched to danazol 100 mg twice daily with complete resolution of his abdominal symptoms.
A 76-year-old Caucasian male was admitted to our hospital with acute onset of left-sided weakness. His past medical history included osteoarthritis only, and his surgical history included bilateral knee replacements. There was no history of cholecystitis, gallstones, pancreatitis, or abdominal surgeries in the past. He denied any fever, chills, shortness of breath, chest pain, palpitations, abdominal pain, nausea, vomiting, and constipation at the time of admission. He was an active smoker (1 pack of cigarettes daily) but denied use of alcohol or illicit drugs. On neurological exam, there was contralateral hemiplegia and hemiparesis of left face, arm, and leg. Power was 0/5 in left upper and lower extremity, and reflexes were absent bilaterally. His speech was slurred, and he failed swallowing evaluation in the emergency department. He was not a tissue plasminogen activator (TPA) candidate as the time of onset of stroke was unknown. Magnetic resonance imaging of brain without contrast showed subacute to acute infarct in the right middle cerebral artery territory. Magnetic resonance angiography of the brain showed acute occlusion of middle cerebral artery in the proximal M1 segment. Carotid Doppler showed mild stenosis of internal carotid arteries bilaterally. Transthoracic echocardiogram was done to rule out embolic source of stroke, which revealed ejection fraction of 45% with no valvular abnormalities and shunting. His chest X-ray was unremarkable. Two days later, he suffered septic shock with a fever of 39°C and hypotension. Blood, urine, and respiratory cultures were obtained, and broad-spectrum antibiotics (vancomycin and zosyn) were started empirically. Two blood cultures were positive for Klebsiella oxytoca, which was sensitive to ceftriaxone, levofloxacin, and gentamycin and resistant to ampicillin and cefazolin. Urine and respiratory cultures did not grow any bacteria. Antibiotics were tailored to ceftriaxone based on the sensitivity results. Computed tomography scan of the abdomen, as part of workup for septic shock, showed dilated common bile duct. Right upper quadrant ultrasound showed cholecystitis with clinical suspicion of acute cholangitis. He did have right upper quadrant pain, fever, and elevated alkaline phosphatase suggestive of acute cholangitis. He underwent percutaneous cholecystostomy and an endoscopic retrograde cholangiopancreatography. Drainage culture showed carbapenem-resistant Escherichia coli. He continued to require vasopressors and fluid resuscitation despite repeat negative blood cultures. On hospital day 12, he developed a new-onset intermittent complete heart block with significant pauses (), requiring temporary transvenous pacing. Transthoracic echocardiography (TTE) detected severe aortic incompetence, with a large aortic vegetation measuring 1.7 × 0.6 cm (). This was a new finding compared to a TTE done on the second day of admission. He did not have any chest pain, shortness of breath, cough, or palpitations. No signs of IE such as petechiae, splinter hemorrhages, Osler nodes, Roth spots, or Janeway lesions were appreciated on physical examination. The patient died before obtaining a transesophageal echocardiography in the process of evaluation for cardiac surgery. Autopsy was declined by the family.\nThe diagnosis of Klebsiella endocarditis was inferred from positive blood cultures, aortic valve vegetation on TTE, and new-onset heart block. The likely source of Klebsiella bacteremia was acute cholangitis and cholecystitis. The presenting symptom of stroke was possibly secondary to emboli from the endocarditis. The patient progressed despite timely, appropriate intravenous antibiotics to manifest heart block, which progressed to asystole.
Miss AL, a six year old girl was seen in A&E with pyrexia, diarrhoea and vomiting, hip and shoulder pain pyrexia of two days duration. She had also complained of a sore throat and earache of four days duration. Her symptoms were felt to be consistent with a viral upper respiratory tract infection and gastroenteritis. No investigations were performed at this time and she was discharged with Diarolyte.\nThe patient returned four days later with persistent multi-joint pains; having now developed diffuse abdominal pain and distension and at this point a surgical opinion was sought.\nThe child was pyrexial and hypoxic, and clearly unwell. Ear, nose and throat examination was normal at this time as it had been one week previously. She had peritonitis localised to the right iliac fossa and an overlying cellulitis. Note was also made of an intermittent roving erythematous but non pruritic rash over her wrists and distal limbs.\nThe full blood count (white cell count 25.6, neutrophils 23.1 platelets 47) and C-reactive protein (248 mg/L) were consistent with fulminant sepsis.\nFollowing an equivocal abdominal ultrasound scan the patient was taken to theatre.\nLaparotomy revealed copious fibrin deposits and a congested caecum and appendix which were not felt to be due to acute appendicitis. appendicectomy was performed.\nDespite aggressive fluid resuscitation and broad-spectrum antibiotics (cefuroxime, metronidazole and gentamicin) the patient deteriorated rapidly, developing disseminated intravascular coagulation, acute respiratory distress syndrome and acute renal failure. She was kept intubated and required admission to the paediatric intensive care unit for inotropic and ventilatory support for 48 hours post operatively. Blood cultures from admission and peritoneal swabs from laparotomy grew a fully sensitive group A streptococcus at 48 hours, after which her antibiotics were rationalised and she was commenced on clindamycin. Her condition improved and she was discharged from hospital after two weeks with no post infection sequelae to date.
A 19-year-old Caucasian man was admitted to our emergency department with a critical ischemia of his right ring finger due to a crushing injury. His finger had been caught between a car and a car-jack while he was working as a mechanic. The first physical examination of the ring finger revealed an open wound of 3 cm on the dorsal side of the middle phalanx. Edges of the wound reached the ulnar and radial digital neurovascular bundle. The patient reported decreased sensibility of the end phalanx including painful range of motion (ROM) but without loss of function of all tendons. We found a restricted finger blood flow detected by nail bed compression in comparison with the uninjured fingers. An X-ray showed an undislocated transverse fracture of the middle phalanx (Figure ). The patient was immediately transferred to the operating room (OR) where the wound was examined. Severe damage of the surrounding soft tissue was seen. Microscopic examination revealed no defect of the crushed vessels, the radial digital nerve was intact, but an epineural lesion was apparent for the ulnar digital nerve. A minimal osteosynthesis with two Kirschner-wires (K-wires) was performed. X-ray imaging showed an anatomical retention and fixation of the fracture (Figure ). The dorsal wound of the ring finger was cut out and sutured. The patient was discharged from the hospital on the fourth day without any sign of wound healing disturbance. The ring finger was immobilized with a finger splint including the proximal interphalangeal joint (PIP) and distal interphalangeal joint (DIP).\nThe patient was seen five months after initial treatment in our Out-Patient Clinic and reported painful moving of the right ring finger. The examination showed an instability of the middle phalanx with a passive lateral movement. Twenty degrees ulnar deviation of the ring finger with decreased ROM of the PIP joint (E/F 0-0-60˚) was apparent. In addition, the ring finger tended to cross over and overlap the adjacent finger when making a fist. The patient reported that ulna deviation of the ring finger started when K-wires had been removed by an office-based orthopedic surgeon two months after the initial surgery.\nSince that time the finger was fixed again with a splint. He also complained about tingling, coolness and numbness of the injured finger. Since the X-ray showed a nonunion (Figure ) we started treatment with a LIPUS device (Fa. Melmak GmbH, Munich, Germany) three times daily for 20 minutes including the following parameter configuration:\nAverage intensity I = 30 mW/cm² (SATA), ultrasound frequency F = 1.5 MHz, signal impulse duration 200 microseconds, repetition rate 1 kHz, effective radiating area 3.88 cm2, temporal average power 117 mW.\nThe ring finger was immobilized with a fitted personalized thermoplastic splint (Figure ) for six weeks. Six weeks after LIPUS treatment the patient reported to be without pain when moving his ring finger and clinical examination revealed a slight persistent ulna deviation of five degrees. The clinical examination showed a stable middle phalanx compared to the result six weeks previously. After four weeks of physiotherapy the ROM of the PIP joint was E/F 0-0-85° and the fist closure was full (Figure ), except for the pre-existent limited ROM of the DIP-Joint (E/F 0-0-15˚). An X-ray confirmed fracture healing and calcification of the soft callus was clearly evident (Figure ).
A 17-year-old female presented with dull pain in the left upper chest alternating with brief periods of chest discomfort lasting a few months. She and her parents were nonsmokers with no history of radiation or chemical exposure. She had no serious medical or surgical history and grew up in normal residential and social environments. She had no shortness of breath or cough. There was no history of weight loss, fever, or night sweats. Her vital signs and physical examination were unremarkable. All laboratory data including blood tests, standard biochemical tests, and urinalysis were within normal limits.\nChest radiographs ( and ) revealed a well-defined round mass in the left upper hemithorax. The mass was at an obtuse angle to the chest wall and was approximately 8 cm long. There was no other abnormality in the lung parenchyma or bony thorax.\nChest CT ( - ) confirmed a well-defined oval-shaped mass with heterogeneous enhancement in the left upper hemithorax abutting the pleura. In the pre-contrast image, the lesion showed homogeneous soft tissue attenuation and a lack of calcification, but a small amount of high-attenuation fluid was present in the left pleural space, suggestive of hemothorax. After contrast enhancement, the mass showed heterogeneous enhancement, with attenuation similar to that of the back muscles, and contained subtle low densities suggestive of necrosis or hemorrhage. There was no evidence of adjacent bone destruction or chest wall invasion. The mediastinal and hilar lymph nodes were not enlarged. In the scanned portion of the abdomen, there were no masses, lymphadenopathy, or ascites.\nF-18 fluorodeoxyglucose positron emission tomography-CT (18F FDG PET/CT) revealed increased FDG uptake in the mass, with a maximum standardized uptake value of 31.7 ().\nFrom these imaging findings, the diagnoses of a localized fibrous tumor of the pleura, malignant mesothelioma, metastatic pleural malignancy, and rare primary pulmonary sarcoma (e.g., pleuropulmonary synovial sarcoma, fibrosarcoma, leimyosarcoma, sarcomatoid carcinoma, malignant nerve sheath tumor, hemangiopericytoma, and malignant fibrous histiocytoma) were considered. These diagnoses were based on the tumor’s appearance as a sharply marginated, heterogeneously enhanced mass with low attenuation foci and no involvement of the bone or calcifications. CT-guided core needle biopsy of the mass yielded two samples of yellowish gelatinous material. Microscopic examination showed relatively uniform spindle-shaped cells with occasional mitoses arranged in tight fascicles. The tumor cells were immunoreactive to vimentin and CD99 and were focally positive for epithelial membrane antigen (EMA), suggesting a monophasic synovial sarcoma. Medical and surgical oncologists agreed to treat the tumor with surgical resection and to initiate a combination chemotherapy regimen consisting of five cycles of vincristine, doxorubicin, and cyclophosphamide, alternating with four cycles of ifosfamide and etoposide. A thoracotomy was performed, and the mass was found to arise from the visceral pleura and to adhere to the left first to third intercostal spaces, with invasion into the left first intercostal muscle with adjacent hemothorax. The surgical specimen consisted of an 8.0 × 6.5 × 5.5 cm well-circumscribed but unencapsulated tumor. The tumor was whitish-yellow, soft, and fleshy with cystic degenerative changes and hemorrhage. There was no calcification in the tumor. Histologically, the tumor was composed of densely packed, cellular sheets of spindle cells, and some of the tumor cells were arranged in intersecting fascicles in a herringbone pattern, suggesting fibrosarcoma-like changes ( - ). Loose and hypocellular focal areas were found at the periphery of the tumor. The tumor cells were relatively uniform with ovoid nuclei, scant cytoplasm, and occasional mitotic figures. Immunohistochemical staining revealed immunoreactivity of the tumor cells to cytokeratin, EMA, and CD99 but not to CD34 or desmin ( - ). A final diagnosis of a monophasic synovial sarcoma from the visceral pleura was made based on the histological and immunohistochemical findings. She was discharged after surgical resection without complications, after which she underwent adjuvant chemotherapy and radiotherapy.\nThe patient remained stable for 28 months until follow-up contrast-enhanced chest CT revealed a 1.6 cm low attenuation nodule with poor enhancement in the left pericardial area and a small amount of left pericardial and pleural fluid (). Subsequent 18F FDG PET/CT showed slightly increased FDG uptake in the developing nodule with suspected metastasis. Five months after discovery, follow up contrast-enhanced chest CT demonstrated a well-circumscribed polycyclic marginated mass with heterogeneous enhancement in the left pericardial region that had enlarged from 1.6 cm to 5.1 cm (). Although MRI is the most accurate technique for demonstrating cardiac mass, oncologists found the following MRI might be ineffective for choosing further treatment for the pericardial mass due to high cost. They had no choice but to do surgery for the mass whether to perform MRI or not. Additionally, CT also provided proper information for ongoing surgery. Therefore, they agreed to operate surgical resection to treat pericardial mass without obtaining cardiac MRI.\nA second mass resection was performed. The tumor specimen was a 6.0 × 5.0 × 2.0 cm well-circumscribed, whitish-yellowish, fleshy to rubbery mass with zones of necrosis and hemorrhage. The tumor was lateral to the left ventricular wall with invasion into the pericardium, myoendocardium, and endocardium of the left ventricle. Microscopically, the tumor contained tight clusters and fascicles of spindle cells with scant cytoplasm, identical to the PPSS, confirming a metastatic synovial sarcoma with left pericardial, myoendocardial, and endocardial involvement.\nAfter metastatectomy, the patient received adjuvant chemotherapy and radiotherapy. The patient’s postoperative period was complicated by recurrent pneumonia. Next, hematogenous metastases were detected in the liver, breast, and pleura. The patient died from sepsis 37 months after the initial diagnosis.
An 18 year old woman was referred to our outpatient clinic of Endocrinology, University-Hospital of Naples Federico II because of hypothyroidism due to Hashimoto's thyroiditis. Blood samples showed high levels of thyroperoxidase and thyroglobulin antibodies and normal calcitonin serum levels. The patient was euthyroid with normal serum levels of thyroid-stimulating hormone (TSH), free triiodothyronine (FT3), and free thyroxine (FT4) during replacement therapy with L-T4. At physical examination, a palpable nodule of ~2 cm in size was detected in the isthmus of the thyroid. There were no palpable cervical lymph-nodes. An US evaluation confirmed an isolated lesion located in the isthmus, showing an isoechoic solid nodule with smooth margins; its size was 18 × 13 × 6 mm with intra and perilesional vascularity (Figure ). Therefore, a FNA was performed and cytological results revealed a TIR3A lesion. The cytological specimen showed an increased cellularity with some microfollicular structures in the background of scant colloid (Figure ). Thus, we assessed the risk factors associated with the isolated TIR 3A nodule of our patient. According to the ATA guidelines we repeated the FNA which confirmed the same result (TIR3A). The second US (after 6 months) showed that there were no clear signs suggesting malignancy such as microcalcifications or taller than wide-shaped nodules. However, we found a small hypoechoic cranial component in the nodule with blurred margins and elastography revealed an increased stiffness in this cranial component. No nodules were detected in the contralateral lobes by US; cervical lymph nodes were normal. Among the possible risk factors, our patient referred a familial history of thyroid cancer. Her mother was submitted to total thyroidectomy for a follicular variant of PTC twenty years ago; our subsequent evaluation showed that she was disease free at the moment.\nOn this basis, we decided that a surgical treatment was indicated for our patient and assessed the risk/benefit of total thyroidectomy vs. isthmusectomy.
A 25-year-old man presented to our department with long-standing left-sided hearing loss. He underwent bilateral grommet insertion in childhood for recurrent otitis media complicated by a discharging left ear postoperatively. On grommet extrusion, he had a persistent left small central tympanic perforation with granulations, with subsequent unsuccessful fat graft myringoplasty at the age of 16. Conservative management witnessed spontaneous closure of the perforation, but with the development of a distorted appearance of the posterior tympanic membrane over the ensuing 7 years. He continued to have intermittent left otorrhoea throughout this period, but eventually achieved a dry ear with improvement of his pure tone audiogram threshold from a 40-dB conductive loss to 20 dB. He was discharged from otology at this point, but presented to our services 8 years later following deterioration in his left-sided hearing over the preceding year.\nOn assessment, he denied otorrhoea or other otological symptoms. His general medical history was unremarkable. His left tympanic membrane had a normal anterior appearance, but the posterior half of the external auditory canal was obliterated. His right ear was unremarkable, except for a patch of myringosclerosis. Audiometry revealed a right mild high tone sensorineural hearing loss (presumed secondary to previous otitis media) and a left severe mixed hearing loss (pure tone average 79 dB) associated with a type B tympanogram. CT imaging confirmed a normal middle ear cleft, with a plug of soft tissue containing dense calcification 8 mm lateral to the tympanic membrane (Fig. ), consistent with a diagnosis of left chronic fibrosing otitis externa.\nTo improve his hearing, he underwent a left external auditory canaloplasty. At operation, the skin overlying and immediately lateral to the posterior tympanic membrane was thickened and fibrosed, confirming the radiological findings. A 4-mm smooth bar of bone was found to be traversing the deep external auditory canal immediately lateral to the inferior tympanic membrane (Fig. ). The calibre of the external auditory canal was otherwise normal and symmetrical with that of the right ear. The bony bar was drilled away taking care not to damage the tympanic membrane. Upon peeling off the remaining mass of fibrosis, the underlying tympanic membrane was intact. A partial-thickness graft harvested from postauricular skin was used to cover the de-epithelialized tympanic membrane and exposed bone of the deep external auditory canal. Postoperative audiometry showed an improvement in conductive hearing with a pure tone average of 55 dB.
A 66-year-old Japanese man who had no past medical or medication history complained of gross hematuria and visited a nearby hospital in October 2013. He had no habit of drinking alcohol or smoking tobacco. He was diagnosed as having a right renal tumor and underwent right nephrectomy laparoscopically. The pathological diagnosis was right renal cell carcinoma (RCC), clear cell carcinoma, pT1bN0M0, v1 (Fig. ). One and half years later, lymph node swelling was detected at hepatic portal region and he underwent lymphadenectomy. The pathological diagnosis was a metastasis from RCC. Two years after diagnosis, he was suspected of lung metastases and started treatment with interferon α. Three years later, the multiple lung metastases grew larger and were determined as progression despite interferon α therapy. At this point, he was referred to our hospital in October 2016. There were no abnormalities on physical examination and his vital signs were normal. He started treatment with sunitinib 50 mg/day on a schedule of 4 weeks on treatment and 2 weeks off; however, adverse events including grade 3 thrombocytopenia (platelet count, 49,000/μL), gum swelling, and hoarseness became intolerable 2 weeks after starting sunitinib. Four weeks after cessation of sunitinib 50 mg/day, he was started on a dose of sunitinib 25 mg/day on a schedule of 2 weeks on and 1 week off. Computed tomography (CT) findings in January 2017 revealed that his lung metastases had shrunk; however, he continued to experience the same adverse events. Therefore, the dose of sunitinib was further reduced to 12.5 mg/day on a schedule of 2 weeks on and 1 week off. CT findings in May 2017 revealed new metastases in the pleura, diaphragm, and the right paracolic gutter (Fig. a, b). As a result, the treatment was changed from sunitinib to axitinib and he started treatment with axitinib at 10 mg/day; however, adverse events including gum swelling, dysphonia, hypertension, diarrhea, and thrombocytopenia became intolerable (Fig. ). Two weeks after cessation of the drug, the dose of axitinib was gradually reduced from 6 mg/day to 4 mg/day. CT findings in September 2017 revealed the metastases had diminished in size and lung metastases were maintained at a diminished size (Fig. c, d); however, the adverse events could not be controlled and he discontinued axitinib treatment. His adverse events improved after discontinuation of axitinib; however, CT findings in December 2017 revealed the size of metastases had increased again (Fig. e, f). Consequently, he was started on fourth-line therapy with nivolumab (3 mg/kg every 2 weeks) and did not experience any adverse events. However, after he had received eight cycles of nivolumab, his metastatic lesions had grown, peritoneal dissemination appeared in his pelvic region, and pleural effusion appeared (Fig. g, h), so nivolumab was discontinued. After giving a detailed explanation of treatment options to our patient, he decided to rechallenge with axitinib 4 mg/day. However, adverse events including gum swelling and dysphonia became intolerable. After that, the dose of axitinib was reduced to 2 mg/day, and he experienced relief of adverse symptoms except for hoarseness. CT findings in August 2018 revealed metastases in lungs, pleura, diaphragm, and the right paracolic gutter had diminished in size (Fig. i, j). He has been continuously receiving a low dose of axitinib at 2 mg/day for 10 months with metastases maintained at reduced size.
A 38-year-old man was admitted to the Division of Surgery in February 2006 because of persistent cough and haemoptysis. Bronchoscopic examination, bronchoalveolar lavage, sputum and bronchial aspirate were negative for malignancies. Contrast-enhanced computed tomography scan disclosed a single expansive cystic mass, 7 cm in diameter, with air fluid level, occupying the lateral segment of the right middle lobe of the lung with compression of the medial segment and of the adjacent segments of the lower lobe. The cystic wall showed intraluminal projections and a sessile nodule 1 cm in diameter (Fig ).\nThe patient underwent a middle right lobectomy without complications. The macroscopic examination of the surgical specimen revealed a 7 × 6.5 × 6 cm single cyst, surrounded by haemorragic pulmonary tissue. The cavity contained 10 cc of clear fluid. The outer surface of the cyst was smooth and the inner surface was characterized by small papillary protrusions. Histologically, the cystic wall consisted of vascularized fibrous tissue lined by cuboidal or columnar respiratory epithelium with focal gland-like appearance (Fig ). Rare smooth muscle bundles and elastic fibers were present. Islets of cartilage were not found. Intraluminal projections of connective tissue were observed (Fig. ). The adjacent pulmonary parenchyma revealed areas of atelectasia and intra-alveolar essudate.\nThe diagnosis was CCAM, type I, according to the modified Stocker's classification [].\nThe patient was discharged from the hospital one week later with a completely uneventful post operative course. Nine months after surgery the patient was free from symptoms.\nCCAM is a relatively rare malformation of the terminal respiratory structures, first reported by Ch'in and Tang in 1949 []. The lesion consists of cysts and solid airless tissue, with no cartilage in the wall. It may affect partially or entirely the pulmonary lobes []. Many lesions described in the past as congenital cystic disease, bronchiolectasis, and a variety of related entities probably were adenomatoid malformations [,].\nCCAMs have been classified, depending on the size and number of the cysts, into three types:\n1. Type I (macrocystic type) accounts for 50% to 70% of cases, is characterized by multiple large cysts, (up to 10 cm) or a single dominate often multiloculated cyst, with a pseudostratified ciliated columnar epithelium, resembling the distal bronchial tree and proximal acinus, with normal alveoli between the cysts. Radiographic analysis may preoperatively suggest the diagnosis, especially when a multicystic pattern is evident. When the cystic lesion is single, the differential diagnosis with the congenital parenchymal cysts and bronchogenic cysts is not possible only on the base of the radiological features;\n2. Type II (microcystic type) represents 20% to 40% of cases and shows multiple tiny cystic structures usually much less than 2 cm in diameter. This type can associate with high frequency to other congenital anomalies, and the prognosis is poor;\n3. Type III (solid type) represents more than 10% of cases and consists of a bulky firm, solid mass with cysts less than 0.5 cm in diameter, mimicking the terminal bronchioles and the alveolar ducts of the pseudoglandular period[,,]; it is now considered a form of pulmonary hyperplasia. The prognosis is usually poor.\nCCAM is most commonly found in the neonatal period and up to 90% of diagnoses are made within the first two years of life [,].\nThe adult form of CCAM shows a wide radiological expression with extreme difficulty of preoperative diagnosis []. Some authors reported very unusual findings like the involvement of an entire lung lobe [] or the presentation as a single large well defined cyst of more than 5 cm in diameter []. The disease may be asymptomatic, diagnosed by means of a routine chest radiograph [], may be a surgical chance finding in the study of an extrapulmonary disease [] or may be revealed by a lung inflammatory process [,]. The histological description lacks in almost all the reported cases in the literature, and the lesion is referred only to Stocker's classification. These previously post-natal and adult CCAM reported cases are enlisted in Table .
A 49-year-old female presented with a 3-week history of sore throat after having received her first dose of the Moderna mRNA vaccine 1 month before. Initially, the symptoms were characterized by headaches and difficulty in concentrating. One week later, she experienced a right cervical sore throat with radiation to the ear. She presented to her primary care physician, who performed an ultrasound on the neck and detected, in addition to thyroid nodules in the left lobe, a newly appeared, large, hypoechoic, and indistinct structure in the right lobe of the thyroid gland. Furthermore, the patient was seen by an otolaryngologist, which revealed no findings other than the sonographic abnormality in the thyroid gland. Therefore, the patient was presented to our thyroid outpatient clinic for further diagnostics and clarification of a possible fast-growing malignant nodule.\nThe patient did not have any past medical history for autoimmune thyroid diseases or recent upper respiratory tract infections. Family history included benign thyroid nodules on the paternal side. The patient's medication consists of a birth control pill and Gynokadin (estrogen for hormone replacement therapy).\nThe thyroid gland presented with mild pressure pain. Sonography showed a normal-sized thyroid (10 ml) with land-map-shaped, hypoechoic, confluent areas with decreased vascularity almost filling the right thyroid lobe (). Laboratory exams revealed an elevated CRP of 21.9 mg/l.\nThyroid function test showed euthyroidism with a TSH in the lower normal range. A complete blood count, especially the leukocyte count was unremarkable ().\nResults pointed to a subacute thyroiditis. However, the patient asked for further clarification of the hypoechoic area in the right thyroid lobe. In order to exclude malignancy and to prove the diagnosis of subacute thyroiditis, fine-needle aspiration cytology was performed and showed lymphocytic infiltrates, macrophages, and multinucleated giant cells consistent with subacute thyroiditis.\nThe patient received symptomatic therapy with 600 mg ibuprofen daily. Due to gastrointestinal intolerance, the patient's private practitioner changed the medication to diclofenac 50 mg/d, and symptoms improved after 2 weeks. However, the patient discontinued the treatment on her own authority after another week and at the 4-week follow-up, thyroid function test showed thyrotoxicosis and positive anti-thyroid peroxidase antibodies and symptoms worsened again. Therefore, the medication was changed to prednisolone 20 mg/d. Her symptoms disappeared within a couple of days. The patient is still being followed-up on treatment with prednisolone during tapering.
A-17-year-old previously healthy female presented to THP with a history of fever for 2 days associated with body aches and nausea. She didn’t have any abdominal pain, bleeding manifestations or postural symptoms. On examination, she was flushed and febrile but was not pale or icteric. She was mildly dehydrated. Blood pressure was 100/70 mmHg, pulse rate 100 beats/min and capillary refilling time (CRFT) was less than 2 s. On abdominal examination, there was no free fluid. Lung fields were clear on respiratory system examination. Other systems examination was normal.\nHer NS1 antigen was positive and serotype was identified as DEN1. She was managed as dengue fever with continuous monitoring. On the 3rd day of fever, she complained of retrosternal chest pain and undue tiredness. At that time her cardiovascular system examination was normal and electrocardiogram (ECG) showed acute T wave inversion in V2-V5 leads. Troponin I was negative and 2D echo showed global left ventricular hypokinesia and mild impairment of LV function. Ejection fraction was 40–45%. She was treated as having dengue fever complicated by myocarditis. Intravenous hydrocortisone 200 mg 8 hourly was administered for 2 days to reduce myocardial inflammation. On the 4rd day following admission, she complained of abdominal pain and ultrasound scanning revealed free fluid in hepato-renal pouch. Blood pressure was 100/70 mmHg, pulse rate 70 bpm, and CRFT was less than 2 s. She was taken to High Dependency Unit (HDU) and was managed as having DHF complicated with myocarditis with continuous monitoring and with careful administration of fluid to avoid fluid overload. She was discharged on day 7 of illness after recovering from critical phase of dengue fever. She was advised on limiting physical activities. During the follow up on day 14 of the illness, ECG showed reversal of T inversions. Echocardiogram showed improvement of left ventricular function with an ejection fraction of 55%.
A 70-year-old woman presented in November 2017 to the Emergency Department at Skåne University Hospital, Sweden, due to the rapid onset of fever, shivers, and a suspected skin infection. She had a previous medical history of left-sided ductal breast cancer with lymph node involvement in 1999, which was treated chronologically with neoadjuvant chemotherapy, partial mastectomy, axillary lymph node dissection, and radiation therapy. In addition, in 2001, a right-sided localised ductal breast cancer in situ was identified and was treated surgically with a partial mastectomy. Secondary to her lymph node dissection, she developed lymphoedema of her left arm, which had been continuously treated with compression stockings. The patient was on treatment with an ACE inhibitor and a beta-blocker due to hypertension, and in addition, she had a known systolic murmur, characterized as physiological, as transthoracic echocardiographs in 2011 and 2017 were normal. Since her surgery in 1999, on a total of six occasions prior to her last and seventh visit, of which the first episode occurred in 2008, she had been treated for erysipelas in her left upper arm. The presentation had always been sudden with spiking fever and erythema spreading in approximately the same localisation. Interestingly, on all three out of the three occasions where a blood culture has been drawn on presentation with erysipelas, the cultures have shown growth of a bacterium belonging to the S. mitis group. These first two isolates also had similar MIC values for penicillin of 0.064 and 0.125 mg/L, for vancomycin of 0.25 and 0.5 mg/L, and for gentamicin of 2 and 2 mg/L (). In addition, they were both sensitive to clindamycin.\nOn the present visit, she once again had a sharply demarcated, warm, swollen, and painful erythema measuring approximately 7 × 15 cm in the lymphoedematous area on her left upper arm. No local portal of bacterial entry was found. Vital parameters showed a temperature of 38.0°C, respiratory rate of 16 breaths/min, O2 saturation of 96% on room air, heart rate of 80 beats/min, and blood pressure of 120/70 mmHg. On physical examination, a grade II systolic murmur was heard with punctum maximum I2 dexter. She had no signs of septic emboli, oral examination showed no signs of infection, and examination of lymph nodes was normal. Possibly due to her quick presentation, that is, less than 6 hours from the onset of symptoms, her laboratory results were normal with a white blood cell count of 8.4 ∗ 109/L, platelets of 263 ∗ 109/L, and hemoglobin of 147 g/L. Her CRP was 12 mg/L. She was clinically diagnosed with erysipelas, and due to previous bacteraemia with the S. mitis group in relation to erysipelas and the presence of a systolic murmur, blood cultures were drawn and she was treated with one dose of intravenous penicillin (3g≈5 million IU) followed by an oral penicillin (1g≈1.6 million IU) three times daily, for seven days. Once again, now for the third time, the two blood cultures showed growth of a bacterium belonging to the S. mitis group. The MIC value for penicillin was 0.125 mg/L, for vancomycin 1 mg/L, and for gentamicin 16 mg/L (). Similar to the two previous isolates, it was also sensitive to clindamycin. Her treatment was prolonged for 10 days, and a follow-up visit was arranged. Repeat blood cultures were drawn 14 days after discontinuation of antibiotics and they were negative. To prevent further infections, she has once again been referred to the lymphoedema outpatient clinic as well as to the dentist office. On follow-up, thereafter, the patient had no sequelae to her infection, and she gave informed consent for this case report to be published.\nThe three blood isolates, one analysed in 2015 and two in 2017 (15 and 8 months apart), were initially subgrouped to S. mitis/S. oralis/S. pseudopneumoniae of the S. mitis group by combining the MALDI-TOF MS results (MALDI Biotyper, Bruker) with the information that the three stains were resistant to optochin. To allow a more detailed comparison, the three stored isolates were reanalysed and now ethanol/formic acid extractions were performed on the strains, and the updated and improved Bruker MALDI Biotyper database (DB-7311 MSP Library) was used for the MALDI Biotyper analysis. In addition to the standard log (score), weighted list (scores) was also calculated []. S. mitis was the best match for both the first and second isolates when both log (score) and list (score) were calculated. For the third isolate, the best match was S. oralis for both types of scores (). Next, the mass spectra of the three isolates were inspected manually. All three strains showed the specific peak 6839.1 m/z which is associated with S mitis and S. oralis strains, but only the third isolate showed the specific peak 5822.5 m/z which is associated with S. oralis () []. In addition, no peak profiles typical for S. pneumoniae and S. pseudopneumoniae could be detected in the three isolates [, ]. These results further support that the first two isolates are S. mitis and the third isolate is S. oralis. Many differences were seen in the mass spectra of the third isolate (S. oralis) compared to the first two (S. mitis). On the other hand, no clear differences in the spectra between the first and second isolate could be seen, and one can therefore not exclude that they belong to the same clone.
A 51-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cancer when she was 48 years old, and lymphedema developed in the left leg soon after the operation. She had one episode of cellulitis. Despite wearing elastic stockings, lymphedema worsened, and she visited our institution at the age of 49 years. She was diagnosed with lymphedema based on lymphoscintigraphic finding. There was a development of collateral lymphatic vessels and dermal backflow in bilateral lower leg (See figure, Supplemental Digital Content 1, which displays lymphoscintigraphic findings. Collateral lymphatic vessels were observed in the bilateral lower legs. Lymphatic function in the left thigh was impaired, ). She had no allergies or other pertinent medical histories.\nLVA was performed at the age of 50 years. The postoperative course was uneventful, and lymphedema improved. However, lymphedema worsened again at 1 year postoperatively after taking a long flight, although she wears elastic stockings daily. A second LVA was planned (Fig. ).\nPreoperative indocyanine green (ICG) lymphography showed a linear pattern in the right leg. Dermal backflow was observed in the left thigh and lower leg. There was no linear pattern in the area (left lower leg) where the lymphatic thrombus was found afterward. Preoperative echography showed 2 hypoechoic circles measuring about 0.5 mm in diameter that did not collapse with pressure from the probe, although the veins collapsed with pressure (Fig. ). Compared with lymphatic vessels, veins usually collapse more easily under pressure, because the inner pressure of the lymphatic vessels is higher than that of the veins. In this case, the 2 circles did not collapse under pressure, and we surmised that the inner pressure prevented collapse. The hypoechoic circles extended proximal and distally and did not have flow with on color Doppler mode.\nDuring LVA, we identified 2 parallel white vessels beneath the superficial fascia. Two vessels were in close contact. We diagnosed these as lymphatic vessels because of the location and appearance and the fact that they ran in parallel, which is not usually observed with other vessels or nerves. When we incised the vessels, white material was extruded (Fig. ). A diagnosis of lymphatic thrombosis was made, and we concluded that the vessels did not collapse with pressure from the probe during echography because of thrombus. Intraoperative echography revealed the same findings, that is, a hypoplastic circle without collapse by probe pressure, 15 cm distal from the incision, which indicated that there was lymphatic thrombus in at least 15 cm. We ligated the lymphatic vessels, closed the wound at this site, and performed LVA at other sites (4 sites in the left and 1 site in the right leg). Though the postoperative course was uneventful, the patient’s lymphedema did not improve postoperatively. This may be partially because the patient gained weight after LVA, and there is another possibility that postoperative thrombus formed within the anastomosis site had harmful effect for lymphedema, although it is difficult to confirm.\nHistopathological examination showed a thickened smooth muscle layer (tunica media) in the lymphatic vessels (See figure, Supplemental Digital Content 2, which displays histopathological findings of the lymphatic vessel and the lymphatic thrombus. (a) The lymphatic vessel (a) and the lymphatic thrombus (b) (×20, H&E). Thickened smooth muscle layer (tunica media) in the lymphatic vessels is observed. (c) The lymphatic vessel (×100, D2-40). The endothelial cells of the vessel were negative for D2-40, ). Fibrous thickening of the tunica intima was observed, and the inner lumen was narrow. The inner layer of the vessel was negative for D2-40, which is a marker to stain the lymphatic endothelial cells. In the thrombus, hyperplasty of fibroblasts and organization were found (Fig. ). We did not observe hyperplasty of the lymphatic endothelial cells, which are positive for D2-40 within the thrombus.
A ten-year-old Qatari boy, of Arab ethnicity, presented to our emergency department with a history of intermittent lower back pain of 12 months’ duration. The pain was reported by the child as severe in nature, intermittent, restricting his daily routine activity, and with partial response to anti-inflammatory medications like 200 mg of ibuprofen. There was no history of joint involvement. The child’s family had sought medical advice repeatedly in the past on an outpatient basis, without any specific work up or diagnosis. The family denied any history of fever, trauma, vigorous excercise, weight loss, or any gastro-intestinal symptoms. There was also a negative history of any raw milk ingestion, chronic cough, or contact with patients known to have tuberculosis. The patient had no previous hospitalizations or blood transfusions. Despite parental consanguinity, there was no family medical history suggestive of sickle cell disease symptoms in any of the first or second degree relatives, nor were the parents aware of any known carrier for Hemoglobin S or thalassemia in the family.\nOn examination, the child was noted to be in pain that was assessed by numerical rating scale as 8/10. General examination did not show any signs of pallor or jaundice. The patient was afebrile and hemodynamically stable with a temperature of 37.5C orally, heart rate of 100 beats per minute, respiratory rate of 20 breaths per minute, and blood pressure of 120 mmHg/85 mmHg.\nThe patient’s height and weight were at the 50th percentile for his age and sex. Muskuloskeletal examination revealed tenderness upon palpation of the entire lumbo-sacral area, with no other external signs of inflammation such as erythema or swelling. He had limited movement of the trunk, with pain elicited during forward and lateral flexion. There was no spine deformity. There was no hepatosplenomegaly or lymphadenopathy, and the rest of the physcial exam was unremarkbale.\nThe patient was admitted to the general pediatric ward for pain management and investigation of his recurrent back pain. The admitting differential diagnosis included chronic osteomyelitis involving the lumbosacral vertebrae, primary and secondary bone neoplams, and possible undiagnosed rheumatological conditions. The patient was started on regular intravenous acetaminophen along with oral ibuprofen to manage his pain, to which he responded rapidly.\nInitial laboratory results were as follows: white blood cells 9,500/uL (neutrophils 90%; lymphocytes 6.1%; monocytes 3.8%; and basophils 0.1%); hemoglobin 12.5 g/dL; platelets 217,000/uL, mean corpuscular volume of 70.8 fL and mean corpuscular hemoglobin of 23.6 picograms/cell, with a normal reticulocyte count. The peripheral smear showed microcyic hyppochromic red blood cells with mild neutrophil leukocytosis, with rare immature granulocytes and some toxic features.\nC-reactive protein was less than 5 mg/L, and erythrocyte sedimentation rate was 9 mm/hour. Serum electrolytes were normal. Urine and blood culture were negative as well as the QuantiFERON test, salmonella, and brucella serology.\nAntero–posterior spine radiograph revealed straightening of the lumbar lordosis with multiple fish mouth-appearing vertebral bodies from L2–L5, diffusely porotic bone with accentuated medullary pattern, and normal intervertebral spaces with slight widening of the sacro-iliac joint space. AP chest radiograph was essentially normal, with no mediastinal mass or hilar lymphadenopathy.\nMagnetic resonance imaging (MRI) of the spine () and pelvis with contrast was requested on the second day post-admission. It showed multiple bone marrow-replacing lesions involving the thoracic, lumbar, and sacral vertebrae extending from T2 vertebral level downwards to the lower sacral segment. These displayed a predominantly low signal intensisty in T1-weighted images and high signal intensity in T12 fluid-attenuated inversion recovery-weighted images, with a patchy homogenous pattern of enhancement. There was an associated reduction of the height of the vertebral bodies, mainly in L3, L4, L5, and S1 levels.\nThe MRI findings prompted a consultation with the pediatric hematology and oncology services to discuss the possibility of malignant inflitration, such as with leukemia and lymphoma.\nA bone marrow biopsy and an aspirate were conducted, both of which were normal.\nMRI of the abdomen was conducted to exclude the possibility of an underlying malignancy. The image showed mild hepatomegaly, with a liver span of 15 cm with moderate splenomegaly, no focal lesions, and no evidence of any abdominal lympahdeopathy or mass lesion in the retroperotenium or adrenal glands.\nOn day 7 post-admission, the result of the hemoglobin electrophoresis that was requested on admission revealed HbF =2.9%, HbA2 =2%, HbA =0%, HbS =66.8%, and HbE =28.3%, which confirmed the diagnosis of sickle cell disease with HbSE component.\nOur patient was discharged with a diagnosis of sickle cell disease (HbSE subtype) on oral folic acid 1 mg once a day and ibuprofen 400 mg per mouth every six hours if needed. In addition, our patient was started on oral penicillin VK 250 mg every 12 hours at the hematologist’s discretion, although there are no specific guidelines clarifying the need for penicillin prophylaxis in HbSE disease. The patient’s parents refused hemoglobin electrophoresis for themselves as well as for the patient’s siblings.
The patient, a 69-year-old male office worker, was incidentally found to have esophageal candidiasis at an annual medical checkup and referred to our hospital 3 years previously. He was an ex-smoker and had a history of moderate alcohol intake. He complained of neither dysphagia nor any other gastrointestinal symptoms. Radiological and endoscopic examinations demonstrated diffuse EIPD involving almost the entire length of the esophagus (Fig. ), esophageal candidiasis, mild GERD, and atrophic gastritis. The orifices of EIPD periodically repeated active opening and closing movements and excreted turbid mucinous material onto the mucosal surface. The esophageal lumen showed mild narrowing, especially in the upper and lower portions, most likely due to submucosal chronic inflammation and fibrosis. Administration of antifungal drugs and proton pump inhibitors was started, and antibiotics for eradication of Helicobacter pylori were also given.\nThereafter, the patient was followed by periodic endoscopic examinations at 3- or 6-month intervals, and candidiasis showed repeated relapses and remissions for about 2 years. When candidiasis relapsed, movements of the orifices of EIPD tended to be reduced, and some of the orifices were covered by white coatings that represented exudates produced by Candida infection. The esophageal mucosa showed diffuse and mild redness and edema. The patient remained almost asymptomatic throughout the clinical course.\nOn endoscopy performed 2 and a half years after the first visit, multiple, small, whitish, scaly flecks were noted on the mucosal surface (Fig. ). They were longitudinally aligned and mainly found along the orifices of EIPD from the middle to lower esophagus. A biopsy specimen taken from one of these flecks showed mild acanthosis, and epithelial cells of the superficial layers had several fine keratohyalin granules in the cytoplasm (Fig. ). No fungal pseudohyphae of Candida species were detected.\nOn endoscopy performed 6 months later, whereas candidiasis was almost cured, multiple, small, whitish flecks slightly increased in size (Fig. ). Biopsy specimens taken from the flecks demonstrated a thick, acellular keratin layer with a basket-weave appearance atop the epithelium showing acanthosis, and a well-developed granular layer with abundant keratohyalin granules was noted beneath the keratin layer (Fig. ). The epithelial cells did not exhibit cellular atypia, and no formation of ectopic sebaceous glands was noted. The lamina propria was not included in the biopsy specimens, and no inflammatory cell infiltration was seen within the squamous epithelium. No fungal pseudohyphae were observed.
A 62-year-old man admitted to outpatient clinic with symptoms of depressive mood, anhedonia, insomnia, decreased appetite, difficulty in concentration, ideas of hopelessness, psychomotor agitation, and abnormal movements of the lips. In his history, his complaints began four months ago as; being followed by strangers, hearing voices that commented and humiliated him, being unable to leave his home, a decrease of self hygiene, increase in irritability, and having some homicidal attempts to family members. The patient had been taken to a psychiatrist without his will and he had been assured to be examined by a psychiatrist.\nAt initial assessment, the patient was assessed with routine laboratory and toxicology examinations, brain magnetic resonance imaging, and electroencephalogram. Both physical and laboratory examination had been resulted unremarkable, thus organic etiology had been excluded. Moreover, he had scored a 26 in the mini mental status examination. The patient had been diagnosed with “brief psychotic disorder” according to Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) by a psychiatrist working in another center. After his psychiatric and physical assessment, the patient had been given a treatment of olanzapine 10 mg/day. During his first outpatient control on the fifteenth day of treatment, it had been noticed that the patient was non adherent to oral treatment and his symptoms persisted. The patient had been given 25 mg of long acting risperidone treatment bimonthly, due to lack of adherence to oral treatment. After the fourth injection of risperidone (at the second month), the psychotic symptoms were reported to be remitted; however, abnormal movements of lips developed. Because of the remission of his initial symptoms and development of abnormal movements, the treatment of 25 mg of long acting risperidone had been stopped. The abnormal movements of the lips were consulted with the neurology department, and the patient’s neurological examination had been reported to be unremarkable. The patient had been treated with biperiden 6 mg/day for 3 weeks, and diazepam 5 mg/day had been added to his treatment for 5 weeks due to lack of response to biperiden treatment in terms of abnormal movements of his lips.\nAfter these interventions, the patient admitted to our outpatient clinic at the fourth month of his treatment. In his psychiatric and physical examination, abnormal movements of lips in a vertical axis, being fine, rapid, and rhythmic, were noted additional to depressive symptoms. Routine laboratory examination, brain magnetic resonance imaging, and other neurological assessments were unremarkable. The patient was diagnosed as major depressive disorder and medication induced movement disorder (Rabbit syndrome) according to DSM-5. Fluvoxamine 100 mg/day was prescribed as his current treatment; whereas, biperiden and diazepam treatments were stopped. The patient was recommended to be followed up bimonthly in our outpatient clinic. In his first assessment, there was a significant improvement in symptoms of Rabbit syndrome and a moderate amelioration in depressive symptoms. The dose of fluvoxamine was increased to 200 mg/day. After the second assessment, the patient was asymptomatic in terms of Rabbit syndrome, and there was significant improvement in depressive symptoms. During the follow-up outpatient visits, the patient was noted to be asymptomatic in terms of both Rabbit syndrome and depressive disorder.
A 13-year-old right-handed male, with no pertinent medical history or prior history of an elbow injury, presented to primary care physician three days after the patient was tackled while playing football and heard a “pop” following hyperextension of his left elbow. He immediately suffered from elbow pain, swelling, and was unable to continue playing. Orthogonal X-ray views of the elbow were obtained, and no evidence for a fracture was appreciated. An elbow sling was recommended with referral to non-operative sports medicine physician for assessment of ligamentous injury of the elbow. Five days after injury, the non-operative sports physician noted moderate swelling at the elbow accompanied by tenderness on palpation at the medial and posterior aspect. Passive range of motion was within normal limits, but painful with the elbow in full flexion. Active range of motion was also within normal limits inflexion but limited to 170° in extension. The patient had 4 of 5 strength on active extension of the elbow and a positive moving valgus stress test. The neurovascular exam was unremarkable. Ultrasound and magnetic resonance imaging (MRI) arthrogram were ordered. One week after injury, ultrasound showed a near complete, partial-thickness, obliquely-oriented tear of the triceps tendon with a bony avulsion-type fracture, approximately 3.0 cm retracted. A portion of the medial aspect of the tendon was still intact. Suspicions of UCL tear from physical exam were confirmed by the MR arthrogram (, , ), which showed a complete tear of the anterior band of the UCL from its humeral attachment. Finally, the MR arthrogram provided further evidence of a high-grade, near complete tear of the triceps tendon with complete avulsion of the lateral and long head portions of the tendon from the olecranon. The patient was referred to be seen by the senior author, an operative sports surgeon,3 weeks after sustaining the injury. Physical examination was notable for palpable tendon gap at the distal triceps insertion, weakness in active extension of the elbow, and extension lag of approximately 15°. There was approximately 15° of valgus laxity with stress testing in slight flexion. Recommendation was made to surgically repair the triceps tendon. Risks and benefits of non-operative and operative treatment were presented to the patient and family. The decision was made to proceed with refixation of the triceps tendon. Since the patient was not a throwing athlete, the UCL was to be treated non operatively with immobilization.
A 59 years old female visited the clinic with ongoing pain located to the right first metatarsophalangeal joint (MTPJ) and medial column. She stated the pain had been ongoing for 2–3 years and has gradually gotten worse recently. Through visual analogue scale, she rated the pain as 7/10. She recently had problems with arthritic changes in her body and felt the same pain in her feet. She stated that the pain in her feet was not significantly bad until recently. The patient has past medical history of thyroid disorder and family history of diabetes and heart disease. Pertinent physical exam revealed some problem in musculoskeletal system. The right lower extremity has pain on palpation to the first MTPJ with a large dorsal prominence as well as pain at the first metatarsal-cuneiform joint with hypermobility. Pain was also elicited throughout the navicular-cuneiform joint. Subluxation and collapse in the medial arch were found out at the navicular-cuneiform joint with subluxed first MTPJ (). Pain was tracked within the first MTPJ with a range of motion in dorsiflexion and plantar flexion of first MTPJ. The left lower extremity had deviated first MTPJ with minimal dorsal medial eminence. No pain was elicited either upon palpation or in range of motion of the joint. Patient ambulated well, however, with significant pain throughout the right lower extremity, especially the medial column of gambrel. Another diagnosis was bilateral hallux valgus arthrosis.\nAlthough the patient understood she had a significant deformity, surgeons informed that they could try conservative measures first if the patient was not ready for reconstructive surgery. The patient stated that she would rather have the surgery now while she was healthy rather than waiting and being too enervated to undergo the surgery. The surgical plan was hence sketched to include a Lapidus procedure with compression fusion of metatarsal-cuneiform joint and navicular-cuneiform joint. To heal the operative wound, patient should be non-weight bearing post operatively for 6–8 weeks.\nBrief operative synopsis was as follows. The surgeons employed mineralized collagen artificial bone repair material products named Bongold® patented by Beijing Allgens Medical Science and Technology Company []. Bongold® is superior to kindred products in its high performance to induce bone regeneration and osteogenesis [, ]. Having high performance in repairing bone defects in orthopedics, Bongold® won the FDA market access license (K141725) in July 2015. With mineralized collagen of BonGold® Bone Sponge and BonGold® Bone Putty, the medial column in the right lower extremity was surgically fused with internally fixation. We began with incision over the metatarsal-cuneiform joint and the navicular-cuneiform joint. Once down through the periosteum the joints were immobilized. Hereafter, the surgeons opened both joints with a Hintermann Style Distractor and then propped these joints. Next, the surgeons saturated BonGold® Bone Sponge with normal saline and inserted them into both of these joints piece by piece (). Then the surgeons released the distractor to allow for proper fitment. The injured medial column was temporary stabilized and then permanently stabilized with an internal fixation plate and screws. Finally, the fusion site firmed up with BonGold® Bone Putty (). The outcome of arthrodesis in metatarsal-cuneiform joint and navicular-cuneiform joint was examined by X-ray shortly after surgical operation (). The curative effect was followed up at the point of 2 weeks, 4 weeks, 6 weeks, 9 weeks and 13 weeks after the surgical treatment (). The medial column was perfectly fused by compression arthrodesis.
A seven and half year-old boy visited the outpatient clinic of Pediatric Dentistry Department, Faculty of Dentistry, Cairo University in June 2015 with a chief complaint of pain on the lower right molar area. The patient’s mother stated that the pain was at times throbbing in nature, and child is not able to chew on this side.\nClinical examination showed a badly decayed, lower second primary molar with related localized intraoral abscess, where the lower first primary molar was intact. The patient had poor oral hygiene; he had not received any professional dental care, and was very apprehensive.\nRadiographic examination revealed root resorption and bone rarefaction related to lower second primary molar. The interesting finding was a considerable amount of root resorption of the distal root of the adjacent lower first primary molar (\n).\nThe case was managed by performing pulpectomy\n to the lower second primary molar, with root canals filled with calcium hydroxide paste with iodoform (Metapex, Meta Biomed, Republic of Korea). The tooth was then restored with high viscosity glass ionomer (GC Fuji IX GP capsule, GC corporation, Tokyo, Japan) (\n). The lower first primary molar was not touched and instead monitored. No antibiotics or analgesics was prescribed.\nUnfortunately, the patient’s mother did not want follow-up appointments in person, however, she was contacted on the phone, after 2 weeks, 3 months and 6 months, and she said everything was fine and there was no swelling or pain.\nAt about 8 months from the treatment appointment, the patient’s mother visited the outpatient clinic with the patient for other reasons, and decided to pass by the Pediatric Dentistry Department for patient follow-up. Clinical examination showed no signs or symptoms, occlusal restoration was intact, and radiographic examination revealed arrested root resorption, on both molars, and an increase in the density of bone although this was not at a normal level yet (\n).\nshows the patient’s timeline of symptoms, treatment and follow-up.
A 64-year-old Caucasian woman presented to her local district general hospital with a subacute progression of ataxia, initially diagnosed in the community as labyrinthitis. Her past medical history was only notable for a thyroidectomy in 1993 for a papillary carcinoma, which was considered cured at subsequent follow-up. Her only regular medication was thyroxin and she had no allergies, occupational exposure to hazardous materials, or family history of note. She neither smoked nor drank alcohol.\nUpon transfer to our hospital, clinical examination demonstrated marked confusion, ataxia, and horizontal nystagmus; power, muscle tone, and deep tendon reflexes were normal. There were no significant chest, abdominal, or other physical findings and temperature was normal. An MRI of her brain showed bilateral parenchymal oedema and leptomeningeal/cortical enhancement associated with the cingulate and superior sagittal gyrus (). A lumbar puncture revealed opening CSF pressure >34 cm H2O (the maximum value on the manometers used) with reactive cytology (although no white blood cells were detected upon automatic counting, cytospin analysis revealed lymphocytes and plasma cells together with macrophages and an occasional eosinophil; no neoplastic cells were observed) and raised CSF protein (742 mg/L). A staging CT revealed a mass in the right upper lobe of the patient's lung with a satellite nodule (image not shown) and a plan for CT guided biopsy was made. Serum angiotensin converting enzyme was normal and an electroencephalogram showed no epileptiform activity.\nDetailed questioning by the rheumatologist elicited a history of two years of joint pain responsive to NSAIDs, two episodes of an inflammatory eye disease (likely episcleritis) treated successfully with topical steroids, and intermittent nose bleeds, though none recent. A provisional diagnosis of GPA was suggested, with differential diagnoses including infection (particularly tuberculosis) and cancer, possibly with a paraneoplastic syndrome. However, strongly positive c-ANCA specific for PR3 suggested a vasculitic condition and the patient was commenced on intravenous methylprednisolone, with an early good clinical response in terms of both limb power and improved higher cognitive function. In terms of differential diagnosis, the absence of elevated ACE and calcium, typical lung parenchymal changes, cranial neuropathies or seizures, signs of myelopathy, radiculopathy, peripheral neuropathy, or neuroendocrine dysfunction (hypothyroidism was due to previous thyroidectomy) did not support a diagnosis of sarcoidosis. Cerebrospinal fluid analysis did not show either acid-fast bacilli or growth of Mycobacteria; the lung abnormalities were not suggestive of tuberculosis. Meningeal carcinosis was unlikely in the absence of detectable systemic primary tumours, in particular, breast and melanoma (negative clinical examination) and lung (parenchymal lesions not suggestive of lung cancer). In addition, cerebrospinal fluid cytology did not show any cancerous cells.\nFollowing the third dose of IV steroids, the patient became extremely confused and complained of chest pain, with clinical findings of tachycardia, tachypnea, hypotension, and desaturation on room air. Serial electrocardiograms demonstrated anterior ST depression and T wave inversion extending inferiorly. Troponin I was transiently elevated to 0.110 μg/L. An arterial blood gas showed type 1 respiratory failure. The patient was transferred from the neurology ward to the coronary care unit, though the cardiology team were unconvinced that the ECG changes represented acute coronary syndrome. An urgent CTPA did not identify a pulmonary embolus, and coronary arteries were patent. However, interval cavitation in the right upper lobe mass lesion was demonstrated. A subsequent echocardiogram was also normal. These features in association with positivity for c-ANCA and the presence of anti-proteinase 3 antibodies at high titre (39 U/mL; reference 0–5 U/mL), alongside negative cultures for microorganisms of the patient's CSF, blood, urine, and other potential sources of infection, confirmed the diagnosis of GPA [, ].\nThe patient was commenced on aspirin and clopidogrel alongside IV heparin. The patient's confusion and immobility had persisted to this point, while her ECGs had returned to normal as had her heart rate and respiratory function. Since she lacked capacity to consent at the time, after discussion with her family, it was decided that more aggressive immunosuppressive treatment would be in her best interest.\nA rapid clinical response to the intravenous cyclophosphamide therapy was observed [] and radiological intracranial features demonstrated improvement (). Within days of starting cyclophosphamide therapy the patient had a remarkable recovery of cognitive function. She was discharged to outpatient care under rheumatology and neurology with a referral to her local hospital for rehabilitation and physiotherapy. With regard to nonspecific markers of inflammation, initial peripheral blood values were CRP 53 mg/L (peak value 106 mg/L), WBC counts 14.2 × 109/L (peak value 23.5 × 109/L), and neutrophils 10.7 × 109/L (peak value 20.7 × 109/L); haemoglobin remained in the normal range.\nTreatment was given with intravenous cyclophosphamide pulses (at 15 mg/kg, initially every 2 and then every 4 weeks, without dose alterations as kidney function was normal) with a plan to switch treatment to oral methotrexate after the 10th infusion. Repeat imaging 9 months after presentation was performed shortly after discontinuation of cyclophosphamide and a few weeks before methotrexate could be initiated, a delay caused by a respiratory infection, treated with cotrimoxazole, which interacts with methotrexate. At the time, there was mild cognitive deterioration and imaging demonstrated radiological progression of the underlying disease process (). However, other clinical and objective measures of disease activity (including inflammatory markers and BVAS scores []) remained stable. Therefore, methotrexate was introduced as planned. Further imaging 12 months after presentation and after 3 months of treatment with methotrexate showed partial improvement in the extent of T2 abnormality and oedema (). Further improvement was documented 6 months after the scan presented in (not shown).
A 24-year-old pregnant woman (G2P1) was referred to us due to suspected bilateral ovarian cysts at 8 weeks of gestation. She had undergone ovarian cystectomy twice under open surgery: left and right ovarian cystectomy for mature cystic teratoma and mucinous cystadenoma, respectively. She had no additional medical history or familial medical history. Transvaginal ultrasound and magnetic resonance imaging (MRI) (Figures and ) revealed two pelvic cysts. The left-sided unilocular cyst was 9 cm in diameter. The right-sided multilocular cyst was 5 cm in diameter. We diagnosed this condition as bilateral ovarian cysts.\nAlthough the serum levels of tumor markers (CA125, CA19-9, and CEA) were normal for a pregnant woman, considering the large size of the cyst, cyst resection was attempted at 14 weeks; however, it was converted to probe laparotomy. Marked adhesion around the cysts, posterior uterus, and Douglas' pouch made cyst resection impossible as extensive adhesiolysis may cause uterine damage and also uterine contractions after surgery. Gross examinations revealed no metastatic lesions or lymph node swelling. Abdominal fluid cytology revealed no malignant cells.\nAt 32 weeks of gestation, MRI revealed that the left-sided cyst size had increased to 27 cm in diameter (Figures and ), although she was asymptomatic. As shown in , the right-sided multilocular cyst became very close to the left monocytic cyst. At this stage, the left large monocytic cyst appeared to merge with the smaller right multilocular cyst, forming a large cyst occupying the entire pelvic cavity, which was later confirmed by laparoscopic findings.\nThis large cyst showed no solid-part or papillary growth. The serum levels of tumor markers remained normal. Malignant ovarian tumor could not be ruled out but was considered less likely. We weighed merits and demerits between relaparotomy for tumor resection during pregnancy and a wait-and-see approach for several weeks; the former is likely to require extensive adhesiolysis and may cause preterm delivery. We decided on the latter strategy, since resection should be performed in the event of a size increase or images indicative of malignancy. The fetus normally developed without fetal growth restriction.\nCesarean section and tumor resection were performed at 37+4 weeks of gestation, yielding 3,012-g male infant with Apgar score 8/9 at 1/5 minutes, respectively. The infant did not have congenital abnormalities. After the completion of cesarean section, we ruptured the wall of this large cyst, with care to avoid the cyst content entering into the abdominal cavity. A large amount of serous fluid was drained. This large cyst was a multicystic cyst (5 cm), considered to be the right multicystic ovarian cyst that had been observed from the first trimester. The wall of the large cyst showed marked adhesion to the peripheral peritoneal cavity. We resected it as widely as possible together with right salpingo-oophorectomy (Figures and ). The left ovary was macroscopically normal, and thus there was no evidence of the left ovarian tumor. The resected tumor consisted of a large unilocular cyst with serous fluid and a mucinous cystadenoma (Figures and ). In the former, lining epithelium was absent in many parts () and mucinous epithelium was occasionally found in continuity with the cyst wall of the latter (right ovarian cystadenoma). No malignant cells were found in the resected specimen. Immunohistochemistry revealed focally positive staining for estrogen and progesterone receptors on the resected cyst wall (Figures and ). At 12 months after the delivery, left ovary remained normal and the retention cyst did not recur. An informed consent for this reporting was obtained from this patient.
A 61 years old Egyptian gentleman presented with a 6 months history of progressive exertional dyspnoea associated with chest tightness to the Emergency Department. He had been treated 2 months earlier for a Non-ST elevation myocardial infarction, when he presented with similar symptoms and had a troponin rise from 28 to 81 and a computerised tomographic coronary angiogram showed moderate to severe right coronary artery and mild left anterior descending artery disease. Other symptoms on this occasion included a productive cough and a significant unintentional weight loss over the past 6 months. He did not have any other previous medical problems, and he did not have any occupational exposures of note. His height was 165cm with a weight of 74 kg. He was afebrile on admission with a heart rate of 99 beats per minute, blood pressure of 126/77 mmHg and oxygen saturation of 93% on air with a respiratory rate of 23. He had bilateral temporal muscle wasting but no clubbing, palpable lymphadenopathy or peripheral oedema. Chest auscultation revealed bilateral coarse crackles up to mid-zones. His blood work showed a white blood cell count of 4.0 × 103/μl, haemoglobin 11.3 gm/dl, MCV 80fl, alkaline phosphatase of 306 U/L with normal calcium, renal and liver function tests. A chest X-ray revealed airspace opacities bilaterally in the mid and lower zones and diffuse sclerotic changes in the visualised bones. Sputum was negative for acid-fast bacilli and general microbiology on smear and cultures.\nComputed tomography (CT) of the chest showed diffuse reticulonodular and ground-glass opacities involving both lung fields associated with interlobular septal thickening but no apparent EM [a,b]. The lung changes suggested a chronic pneumonitis type picture, nevertheless, the possibility of metastatic nodules/disease could not be excluded. Diagnostic bronchoscopy showed multiple tiny endotracheal and bronchial nodules in the anterior wall of the distal trachea and major bronchi [, a and b]. Histology of the endobronchial nodules on biopsies showed bronchial mucosa heavily infiltrated by a tumour characterized by cords, vague nests and vague acini consisting of small-sized tumour cells, some having hyperchromatic nuclei with prominent nucleoli [, a].\nImmunohistochemistry was strongly positive for CK AE1/AE3 and PSA confirming metastatic prostatic adenocarcinoma [b]. CT of abdomen and pelvis revealed a diffusely heterogeneous and enlarged prostate with postero-lateral bladder wall thickening and prominence of lymph nodes within the abdomen and pelvis. Coupled with this were extensive sclerotic bony lesions. His total prostate-specific antigen (PSA) was more than 5000 ng/ml. A bone scan showed multiple deposits involving the ribs, vertebrae, sternum, clavicles, humeri, femora, skull and the bony pelvis. Subsequently, he was started on hormonal treatment by the Uro-oncology team with his last PSA dropping to 62 ng/ml. He was offered chemotherapy as well with Docetaxel which he declined.
A 25-year-old female patient reported to the Department of Periodontics, CSI College of dental Science, Madurai, with a chief complaint of the mobile upper front teeth. The patient also complains of spacing between upper and lower anteriors that keep increasing over a period of 6 months. All teeth were present with root stump in relation to 16 and 26. Her oral hygiene status was fair. No familial history could be elicited.\nHer gingival examination revealed reddish discoloration in the upper and lower anterior region, with rounded margins in the anteriors. The gingiva in the anterior region was soft and edematous, with loss of stippling. There was generalized bleeding on probing evident. The gingival margin was apically placed in relation to the upper and lower anteriors.\nA full-mouth periodontal charting revealed generalized periodontal pockets and clinical attachment loss. Pocket depths were deeper in the molar and incisor region with recession evident in the upper and lower incisor region. Clinical attachment loss ranged from 7 to 10 mm in the incisor region and 5–7 mm in the molar region. Most of the teeth were Grade I mobile, with Grade II mobility in 12, 22, 24, 31, 32, 41, 42 and Grade III mobile 11, 21.\nAn orthopantomogram and a full-mouth intraoral radiograph were taken. Radiographic findings revealed generalized horizontal bone loss with severe bone loss in the anterior region. There was a typical arch-shaped bone loss in relation to the first molar region []. A routine blood investigation revealed no abnormal findings.\nBased on the patient's history, clinical findings, and radiographic findings, it was diagnosed as generalized aggressive periodontitis, according to AAP 1999 classification.\nA through supragingival scaling was done, and oral hygiene instruction was given to the patient. Modified bass method was advised and chlorhexidine mouthwash was prescribed. Amoxicillin 500 mg and metronidazole 400 mg were prescribed for 7 days, and the patient was recalled after 14 days for review and subgingival scaling.\nAt the second visit subgingival scaling was completed. The patient was advised to continue chlorhexidine mouthwash, and oral hygiene maintenance was reinforced, with additional use of interdental cleaning with an interdental brush. Two weeks after subgingival scaling, there was marked a reduction in pocket depth and no bleeding on probing was present.\nOverall treatment was planned with a multidisciplinary approach, with consultations from the Department of Orthodontics, Department of Endodontics, Department of Oral Surgery, and Department of Prosthodontics for complete rehabilitation of the patient. Teeth with hopeless prognosis were identified. Implants were planned for the upper anteriors.\nQuadrant-wise full-mouth flap surgery was done, except on the upper anterior region. Kirkland flap was raised; with respective bone surgery was done. Extraction of root stumps in relation to 16 and 26 was done. Interrupted silk sutures were placed and co-pak was given.\nSutures were removed after 10 days. After suture removal impression with alginate impression material was made for immediate denture in the upper anterior region. After 1 week, extraction of 11, 12, 21, and 22 was done [], and the areas were curetted thoroughly before freshly prepared platelet-rich fibrin was placed in the socket, for socket preservation and sutured [Figures -]. A passively fitting immediate denture was inserted. The patient was instructed not to disturb the surgical area for 1 week. Betadine gargle was prescribed. Sutures were removed after 1 week. The patient was recalled after 45 days for implant placement.\nAfter 45 days, implant placement was planned for the replacement of 11, 12, 21, and 22. A full-thickness mucoperiosteal flap was raised in the upper anterior region. Bone quality was poor (Type 3). Furthermore, there was reduced vertical dimension. Sequential drilling was done, up to 2.8 mm final twist drill. Three implants of 3.75 mm × 11.5 mm were placed. The implant in relation to 12 was angulated more palatally because of lack of buccal bone. Interrupted sutures were placed and radiograph was taken to check the implant position []. Second-stage surgery was planned after 8 months. Sutures were removed after 1 week.\nAfter 1 week, sutures were removed and the RPD was adjusted before fitting. The patient was under review every month for the next 8 months. After 8 months, second-stage implant surgery was done, healing abutments were connected, and sutures placed. Sutures were removed after 10 days and an open tray impression was taken with rubber base impression material, after that the impression coping was splinted with pattern resin. The impression was send to the laboratory for processing.\nGig trial was checked after 10 days, and Cementable final denture was delivered in 15 days. The patient was recalled after 1 week for checkup. After which, the patient is reviewed every 6 months, for the past 2 years.
Mr. C, a 42-year-old African American man with a past psychiatric history of schizophrenia and past medical history of childhood asthma, hypothyroidism, and hyperlipidemia, presented to a large, academically-affiliated medical center with acute, severe epigastric pain accompanied by several episodes of bilious emesis. On arrival to the emergency room, the patient was afebrile and hemodynamically stable. Computed tomography of the abdomen demonstrated extensive acute pancreatitis, complicated by extensive necrosis, and a multiloculated fluid collection replacing most of the body and proximal tail of the pancreas, measuring 20 cm by 3.2 cm. Initial laboratory testing was notable for lactic acidosis. During the hospitalization, the patient was found to have P. anaerobius bacteremia, developed acute hypoxic respiratory failure, and was transferred to the intensive care unit (ICU). The patient subsequently required multiple hospitalizations for endoscopic drainage of the fluid collection and pancreatic stent placement.\nPrior to the development of acute necrotizing pancreatitis, the patient had longstanding and complicated history of psychopharmacology for the treatment of schizophrenia. His psychotic illness was refractory to the treatment with multiple typical and atypical antipsychotics, including a trial of clozapine that was ceased due to the development of neutropenia. After a series of failed antipsychotic trials, the patient was eventually stabilized for twelve years on a regimen of haloperidol 10 mg daily, haloperidol decanoate 100 mg every four weeks, and olanzapine 20 mg daily. The patient developed hypertriglyceridemia eight years prior to the development of pancreatitis and was treated with 500 mg docosahexaenoic acid (DHA) and 500 mg eicosapentaenoic acid (EPA) with subsequent improvement in his triglyceride levels. Of note, eight months prior to the development of pancreatitis, the patient's triglyceride level was 162 mg/dL (greater than 200 mg/dL is considered high). On repeat evaluation, one month prior to admission, the patient's triglyceride level was 185 mg/dL. The patient had persistently elevated hemoglobin A1c levels (HbA1c) in the prediabetic range, between 5.7% and 6.6% for the past ten years (4%-5.6% is considered normal). On initial evaluation, during the above hospitalization, his HbA1c remained in that range at 6.2%, reflective of continued pancreatic dysfunction potentially due to atypical antipsychotic treatment.\nCommon etiologies of acute pancreatitis were considered, though the patient did not have overt triglyceride dysfunction, acute change in HbA1c, gallstones, or recent alcohol or other substance use. An infectious etiology was ruled out as the patient neither showed signs of infection nor met the Systemic Inflammatory Response Syndrome (SIRS) criteria []. The lactic acidosis on admission was believed to be secondary to pancreatic damage and has been shown to be a predictor of severity of disease []. While the patient had Peptostreptococcus bacteremia, this was later in the hospital course and thus determined to be a consequence, rather than a cause, of pancreatitis. The patient also did not have any recent abdominal instrumentation or reported family history of pancreatitis. Given the absence of other significant risks factors for pancreatitis, iatrogenic causes were considered.\nOf the patient's medications, olanzapine was identified as the most likely causative agent for the development of pancreatitis. Thus, olanzapine was discontinued and his dose of haloperidol decanoate was increased to 200 mg every four weeks. On follow-up, the patient had developed insulin-dependent diabetes mellitus, though the pancreatitis itself had not progressed further. Ten months after the initial event, the patient's HbA1c was 12.5% and he was hospitalized several times for complications of hyperglycemia. Repeat abdominal imaging demonstrated extensive loss of pancreatic tissue, replaced by fluid collections and chronic inflammation.
A 22-year-old woman presented to the Neurosurgery clinic in Nobel Teaching Hospital with a history of slow progressive but painless swelling over her left neck, ongoing for the last 6 months. She was also experiencing some difficulty while swallowing. She denied history of trauma, any episodes suggestive of transient ischemic attacks or paroxysmal episodes of severe headache, flushing or chest pain. She did not have major surgery or medical illnesses in the past, or any significant family history.\nLocal examination revealed a pulsatile swelling on her left neck. There was no audible bruit over the swelling. The patient then underwent a CT angiography, which revealed a well-defined large (> 8 cm) heterogeneous and hyperdense soft tissue lesion; showing intense arterial enhancement at the level of carotid bifurcation (\n). It was causing significant compression and displacement of a long segment of the internal carotid artery (ICA) and external carotid artery (ECA), encasing a broad area of the ICA and ECA ( Shamblin Grade 3) (\n). The lesion was getting vascular supply from both the ICA and ECA branches (\n). These findings were all highly suggestive of a carotid body tumor.\nUltrasound imaging of the abdomen to assess adrenal glands was normal. 24-hour urine vanillylmandelic acid (VMA) and plasma metanephrines were within normal range.\nThe patient was thoroughly counseled regarding her condition and the need for operative management. The risks associated with the surgery, including intra-operative uncontrolled bleeding, lower cranial nerve palsy, ischemic stroke and even death, were detailed. Anaesthetic precautionary measures were implemented to reduce the risk of hypertensive crisis (during tumor manipulation) and hypotensive episodes (following tumor removal), by administering antihypertensive drugs and fluid support, respectively. After ensuring temporary carotid control with vascular loops, sub-adventitial dissection of the lesion was carried out starting from the common carotid caudally and then progressing cranially towards the bifurcation and its branches (\n). The major vascular supplies were sequentially isolated, ligated and divided. A venous graft from the long saphenous vein was prepared for repair in case of inadvertent tears within the carotid or any of its branches. The internal jugular vein and the vagus nerve within the carotid sheath, and the hypoglossal nerve were all selectively isolated and well preserved. The lesion was completely excised and sent for histopathological analysis. There was only one instance of temporary bradycardia throughout the procedure. Patient made an uneventful recovery with no lower cranial nerve deficits or any vascular insults. The histology from the lesion revealed a characteristic Zellballen pattern, highly suggestive of a Paraganglioma (\n). The patient has been for regular follow-ups in the last 4 months, with complete resolution of her previous symptoms. She has been advised for lifelong periodic visits.
A 30-year-old gravida 1, para 1 presented because of bilateral buttocks and low back pain that started 10 days postpartum. The pain was dull in nature, was more severe on the right side, and was progressively increasing in intensity resulting in a limping gait. Pain worsened with walking, bending, and minor activity and was partially relieved with rest. Patient self-prescribed analgesics and non-steroidal anti-inflammatory drugs (NSAIDs) which used to relief pain transiently.\nThe patient is otherwise healthy with no history of metabolic bone disease, menstrual irregularities, previous fractures, eating disorders, or strenuous athletic activity. No family history of osteoporosis was reported. The course of pregnancy was smooth with no remarkable back, hip, or buttocks pain reported. The total weight gained during pregnancy was 14 kg. She was maintained on supplemental iron, calcium (1000 mg daily), and vitamin D (800 international units (IU)) daily as of the second trimester. At term, she presented in labor and received epidural anesthesia. The first stage of labor was 7 h and second stage was 100 min. She had an uneventful, spontaneous, normal vaginal delivery without the need for forceps or vacuum application. The baby was healthy and weighed 3350 g.\nTwo weeks after onset of her symptoms, the patient sought medical attention because of persisting pain and abnormal gait. On physical exam, the patient was of average body habitus with height 164 cm and weight 60 kg. Mobility of the lumbosacral spine was within normal limits. Tenderness around the upper gluteal region and sacrum was noted bilaterally; more evident on the right side.\nPelvic magnetic resonance imaging (MRI), performed to rule out soft tissue lesions, demonstrated moderate-to-severe bone marrow edema involving the right sacrum along its entire length with a non-displaced fracture line. Mild edema was also seen involving the left sacrum. Results of the MRI are displayed in Fig. and .\nDual-energy X-ray absorptiometry was performed to rule out pregnancy-associated osteoporosis. It revealed normal lumbar spine and left forearm mineral densities with low normal values at the femur bone (Table ). Basic metabolic workup including complete blood count, thyroid stimulating hormone, parathyroid hormone, vitamin D, serum calcium, and urinary calcium levels were normal.\nPatient was treated with relative rest, analgesics, and NSAIDs. Empirically, and based on no solid evidence, she was also given oral vitamin D (50,000 IU per week) and calcium (1 g daily) supplementation to facilitate and fasten bone healing. Breastfeeding was not interrupted. Vitamin D was continued for a total of 4 months. One month later, patient symptoms started to improve gradually requiring less pain medications. Pain completely resolved and gait was restored around 6 months postpartum. No follow up MRI was performed and the patient was considered to have a healed fracture based on resolution of her symptoms.\nTo note, after 5 months of the diagnosis of sacral fractures, the patient underwent a follow up dual-energy X-ray absorptiometry. This study was performed as a follow up after improvement of symptoms and as a baseline before a planned second pregnancy. The results are outlined in Table .\nThis article presents a case of postpartum spontaneous sacral fracture in the absence of documented osteoporosis. Risk factors for sacral stress fractures during pregnancy or in the first weeks postpartum include vaginal delivery of large for gestational age infants, increased lumbar lordosis, excessive weight gain and a rapid vaginal delivery (precipitous labor) []. Other risk factors include vitamin D deficiency, anticoagulant therapy with heparin and transient osteoporosis associated with pregnancy and lactation. Our patient did not suffer from any of those risk factors. Since all the workup done including dual-energy X-ray absorptiometry was normal; the fracture can be classified as a sacral fatigue fracture. However, there is a high probability that we might have been dealing with a case of pregnancy-associated osteoporosis due to two reasons. The first one is the result of the first dual-energy X-ray absorptiometry that was performed 5 weeks postpartum. The International Society for Clinical Densitometry recommends the use of BMD Z-scores in premenopausal women, where a Z-score of lower than −2.0 should be interpreted as “below the expected range for age” []. Thus, despite the fact that the results do not fulfill criteria of low bone density (Z score < −2) in premenopausal women, it is evident that the bone density is borderline low, especially at the femur where Z score was - 1.9. Thus, there might be a possibility that these numbers were lower during pregnancy and the immediate postpartum period and have already started to correct by the time the study was done. Another reason is that the follow up dual-energy X-ray absorptiometry that was performed 5 months later revealed an increase in the bone density at the spine and femur. Densitometry measurement of the forearm was not repeated. Another reason why this case can be regarded as a complication of pregnancy-associated osteoporosis is the timing of the event, which occurred in the immediate postpartum period; the affected skeletal region being the spine; and the lack of other reasons or risk factors for fractures. However, the possibility of pregnancy-associated osteoporosis remains a hypothesis that cannot be fully proven in this case.
We report a case of true trilineage hematopoiesis in an excised area of HO from an enucleated blind painful eye.\nA 70-year-old Caucasian man presented with a blind painful right eye to our ophthalmology department to evaluate enucleation bulbi. He had a history of a blunt trauma injury to his right eye that occurred 49 years ago, and he subsequently experienced hyphema, vitreous hemorrhages, and traumatic cataract. Over the past 10 years, he experienced episodes of ocular pain phthisis bulbi associated with trigeminal neuralgia. A slit lamp examination of his right eye showed band keratopathy and seclusion pupillae, with a brunescent traumatic cataract. A funduscopic examination of his right eye was not executable due to media opacity. His left eye was normal without any kind of lesions and had an uncorrected visual acuity (UCVA) of 20/20. A right eye ultrasound B-scan examination showed a phthisic eye, with complete retinal detachment, and hyperechogenic tissues with an acoustic shadowing due to calcification projected into the orbital tissue. For that reason, the clinicians decided to perform the enucleation of his right eye. His past medical history was positive for asymptomatic lymphoplasmacytic lymphoma associated with immunoglobulin M (IgM) monoclonal peak diagnosed 12 years before enucleation. At the onset, neither lymphadenopathies nor hepatosplenomegaly were described, and 20% bone marrow infiltration was documented. Our patient was periodically followed up without any treatment with evidence of a slow progressive disease, which did not require treatment up until now. Prior to the enucleation, magnetic resonance imaging (MRI) of his brain was performed to study a possible trigeminal neuralgia and was normal without any cerebral lesions.\nThe eyeball was removed and marked fibrosis with places of osseous metamorphism was noticed macroscopically. A histopathologic examination revealed diffusive fibrotic and calcified uveo-scleral flaps, with widespread bone metaplasia with hematopoietic marrow, mixed with retinal fragments with atrophic areas alternating with hyperplastic aspects and blood clots (Fig. a).\nA further typing showed the presence of intertrabecular infiltration in the metaplastic bone marrow of non-Hodgkin B lymphoma, with small lymphocytes, with similar characteristics to the already known disease. Immunostaining was positive for CD20, CD3, CD10, CD43, CD23, CD138, S100 GFAP, MelanA, and Ki-67 (Fig. b-c).
A 42-year-old male complained of recurrent episodes of melena and dizziness, fatigue and reduced exercise capacity for more than 2 mo.\nThe patient's symptoms started 2 mo ago with recurrent episodes of melena and he frequently felt fatigued. He was diagnosed with a gastric ulcer and anemia after undergoing gastroduodenoscopy, colonoscopy and laboratory blood tests. Then he took oral proton-pump inhibitors and iron for 1.5 mo, but these therapies did not ameliorate the symptoms.\nThe patient had no previous medical history.\nOn examination, anemic face and upper abdominal tenderness were noted. The vital signs were normal with a respiratory rate of 18/min, heart rate of 96/min and blood pressure of 102/62 mmHg.\nBlood analysis revealed severe iron-deficiency anemia with hemoglobin of 53 g/dL, and fecal occult blood was positive. Blood biochemistry, tumor biomarkers, other blood tests as well as urine analysis were normal. Electrocardiogram and chest X-ray were also normal.\nWhen the patient presented in our hospital, two units of blood were transfused. The gastroduodenoscopy was performed again. A sealed ulcer without any signs of bleeding sign were found in the antrum. We then performed a CE. Bleeding was found in the jejunum after running the capsule for 97 min. The total running time in the small bowel was about 300 min. The CE cannot determine the cause of bleeding due to the short stay around the lesion and the influence of the blood. An anterograde BAE was performed, and a protruded lesion was revealed in the jejunum at about 150 cm distal to the ligament of Treitz. It filled half of the intestinal cavity. The tumor was lobulated with white patches on the mucosal surface with blood oozing in the fundus (Figure ). Multiple biopsies were taken, and pathological findings further revealed that hyperplastic thin-walled lymphatic and venous with luminal dilation presented in the submucosal area (Figure ).
A 61-year-old lady presented with complaints of acute headache with nausea and vomiting. Physical examinations revealed ataxia and right hemiparesis. She also revealed that she had undergone an abdominal and thoracic surgery to remove tumours from the duodenum as well as the right lung 3 months earlier. The duodenal lesion was presumed to be the primary lesion, while the lung was thought to be the metastatic site. Histopathological findings were consistent with PEComa for both sites.\nDuring current admission, a contrast-enhanced computed tomography (CT) of the brain revealed two lesions – one right cerebellum (4 × 4 cm2) and another left temporal lobe (2 × 2 cm2) with avid contrast uptake (). Magnetic resonance imaging (MRI) revealed two distinct lesions with low signal intensity on T1-weighted sequence and avid enhancement post gadolinium administration (). T2-weighted sequence demonstrated blooming artefact in the right cerebellar lesion suggestive of intratumoural haemorrhage and necrosis.\nAn initial diagnosis of metastatic PEComa to the brain was made. The patient underwent excision of both lesions via left posterior temporal and right cerebellar craniotomy using intraoperative CT image guidance in a single setting. The right temporal tumour was greyish, vascular and friable, contained within a loosely adherent pseudocapsule, excised en bloc. The cerebellar tumour was excised en bloc in a similar manner. Intraoperatively, no macroscopic parenchymal invasion was noted and clear excision margin confirmed with intraoperative CT scans.\nHistopathological sections of the specimens showed sheets, clusters and haphazardly dispersed spindled and epithelioid tumour cells (). These cells exhibited pleomorphic vesicular nucleus with occasional prominent nucleolus, as well as moderate to fairly abundant clear to faintly granular cytoplasm. In certain areas, the tumour cells were seen closely aggregated around blood vessels (). Mitotic figures were easily seen. Foci of necrosis were present. There was no evidence of keratinization or gland formation.\nImmunohistochemistry revealed focal positivity for HMB45 () and showed weak smooth muscle actin positivity (). Ki67 proliferative marker was more than 30% (). These tumour cells were negative for epithelial membrane antigen (EMA), S100, CD10, CD34 and CD117.\nThese findings were compatible with metastatic PEComa.
A 58 year old woman presented to the emergency department with a bleeding mass on the medial aspect of her lower left leg. Three years prior, she sustained a burn to that area of her leg resulting in a scar. One year ago, she sustained minor trauma to that region leading to a small painless bump that slowly progressed in size. She noticed bleeding from the mass, which prompted her to seek medical attention. Upon presentation to the emergency department, the bleeding had stopped.\nThe patient denied constitutional symptoms of fever, chills and night sweats or recent weight loss. She had no numbness, tingling or localized weakness. There was no history of cancer in the family. The patient was married and worked as a sales clerk. She was a non-smoker and did not drink alcohol.\nExamination showed a multiloculated and fungating soft tissue mass measuring 15 cm × 18 cm × 5 cm, with interspersed areas grossly consistent with necrosis. Neurologic function was normal distal to the mass with intact ability to flex and extend both the ankle and toes painlessly. A 6–8 cm non-fungating, palpable mass was also noted in the left thigh near the groin. Both masses were non-tender. In addition, right supraclavicular and bilateral axillary lymphadenopathy was appreciated.\nThe mass was suspected to be a soft tissue sarcoma. Staging abdominal, pelvic, and chest CT was performed and the patient underwent surgical amputation of the left leg below the knee, as well as excision of masses in the following locations: left groin (16 cm), right supraclavicular (3 cm), left anterior shoulder (3 cm), right wrist, left mid-back, right posterior axilla, and right lateral breast. The patient tolerated the lengthy procedure well. Biopsy of the mass showed sheets and nests of epithelioid and spindle tumor cells within the superficial and deep dermis and subcutis, with the epidermis uninvolved. On permanent section, tumor cells showed no evidence of melanin pigment on Fontana Masson stain (not shown), but S-100 protein, HMB-45, MART-1, and MITF were all positive in the tumor (Figure ), which resulted in a histopathological diagnosis of malignant melanoma. At the request of the patient’s oncologist, additional fluorescence-in-situ hybridization (FISH) for Ewing Sarcoma Breakpoint Region (EWSR) was performed and was negative for a rearrangement. In addition, molecular testing for BRAF exon 15, was negative for the V600E mutation.\nAlthough the primary site could not be discerned with certainty, assuming that the leg mass is the primary tumor yielded a stage of T4bN3M1a. Following discharge, the patient had monthly follow-ups with orthopedics, plastic surgery, and prosthetics. Six months after the original diagnosis, follow-up PET scan showed small lesions in the liver, spleen, retro-peritoneal lymph nodes and left external iliac nodes with increased activity. The patient began treatment with ipilimumab at that time, and was responsive to therapy.
A 39-year-old female patient initially presented to the emergency department after a fall at home. Computed tomography (CT) imaging of the abdomen and pelvis at the emergency department did not reveal any major pathology. She visited the clinic a month later for persistent tenderness at the right lower quadrant of the abdomen. She denied any exacerbating or relieving factors. The patient additionally denied any associated symptoms, including fever, chest pain, shortness of breath, palpitations, dysuria, or known exposure to coronavirus disease 2019 (COVID-19). The patient has a past surgical history of an appendectomy, three cesarean sections with bilateral salpingo-oophorectomy, and abdominoplasty. Her family history was only remarkable for unspecified heart disease.\nAt the clinic, physical examination revealed right lower quadrant tenderness and nodularity 2 cm above her abdominoplasty scar that was potentially overlapping the site of a previous appendectomy incision. The nodularity could not be easily reduced into the abdominal cavity. On closer review of the previous CT scan from her emergency room visit, there was a newly appreciated area of hypervascularity near the palpable nodularity (Figure ).\nGiven the likelihood of a hematoma or a fascial defect at the site of the incision along with the concern that this suspected hernia was not reducible, urgent imaging was warranted to rule out incarceration or strangulation. Subsequent preoperative CT imaging revealed a 1 cm by 1 cm hypovascular pocket arising from the cecum and protruding into the anterior abdominal wall without any inflammatory changes (Figure ). Such findings confirmed the initial suspicion of a fascial defect as the cause of the nodularity, and strongly suggested incarceration of a cecal hernia.\nShortly after, the patient appropriately underwent an exploratory laparoscopy. The abdomen was entered in the left upper quadrant and left lower quadrant with three optical trocars (Figure ). It was appreciated that both omentum and cecum were adhered to the fascial defect of her previous appendectomy site. Lysis of adhesions was completed with scissors, and extensive dissection revealed an incarcerated Richter’s hernia of the cecum. The hernia was non-obstructing as there was only a knuckle of cecum herniating into a 1 cm defect. There was no evidence of bowel ischemia. We were unable to reduce the hernia because of dense adhesions. After upsizing a trocar site, an endovascular stapler was used to transect the cecum tangentially. This allowed us to pull on and dissect the herniated portion of the cecum. This component was sent as a specimen to the pathology laboratory. Incisional hernia repair was then performed in a primary fashion due to the small defect size and contaminated nature of the case. The fascia was closed with two layers of running #2 PDS (polydioxanone suture) Quill sutures (Westwood, MA: Surgical Specialties Corporation).\nThe patient did well postoperatively and was discharged home from the recovery unit. The pathology laboratory reported the patient’s specimen as demonstrating benign disease with nonspecific chronic inflammation. A repeat CT of the abdomen and pelvis demonstrated full resolution of the incisional Richter’s hernia with lack of pathology at the surgical sites (Figure ).
The patient was a 42-year-old Turkish White man presenting with nausea, vomiting, fatigue and abdominal pain. Laboratory data revealed no abnormalities in blood chemistry or tumor markers. An enzyme-linked immunosorbent assay disclosed positivity for HBsAg. No abnormal findings were observed in the chest X-ray. Computed tomography revealed thickening at the second and third portions of the duodenal wall and a polypoid contrast-enhanced area. Because a gastroduodenoscopy showed no abnormalities, an endoscopic retrograde cholangiopancreatography (ERCP) was performed. The ERCP revealed a duodenal mass. Biopsy specimens derived by ERCP were highly suspicious for malignancy. Subsequent color Doppler examination performed for abdominal aorta and portal vein invasion did not reveal any abnormalities. The plan was to perform a pancreaticoduodenectomy, and 10 days later the patient underwent surgery. A mobile, polypoid, palpable tumor was further uncovered in the third portion of the duodenum, distal to the duodenal papilla. Pancreas, papilla and duodenal wall except the third portion seemed to be free from the polypoid mass. Considering the possibility of a NET tumor, the relatively invasive procedure was postponed, complete tumoral tissue was excised locally, and the specimen sent for frozen section examination. Pathological examination revealed that resected tumor margins were free of malignancy. However, owing to the proximity of the tumor to the ampulla of Vater, a sphincteroplasty was conducted to prevent potential obstruction. In addition, cholecystectomy and choledochotomy procedures were performed, and a T-tube inserted into the choledochus. Finally, all the enlarged lymph nodes were resected. Pathological examination with paraffin embedding and staining disclosed a 1.7cm well-differentiated, Grade 1 neuroendocrine carcinoma (NEC) (Figure ). Tumor cells were detected in the muscularis propria of the sphincter of Oddi. Lymphovascular invasion of tumor cells (a total of eight lymph nodes) was positive. According to the TNM staging system, the tumor was classified as pT2. The tumor consisted of uniform cells formed in glandular patterns. Tumor cells were stained for chromogranin A, synaptophysin and somatostatin. The final diagnosis was made by the pathology unit as primary duodenal somatostatinoma. The proliferation index was high and because of the lymphovascular invasion, the tumor was thought to be malignant. Because the patient had been operated on for papillary thyroid cancer 6 years earlier, the possibility of multiple endocrine neoplasia was excluded. The level of neuron-specific enolase was 14.4ng/mL at the second postoperative week. The patient was discharged from the hospital with no complications on postoperative day 22.
The patient was a 26-year-old woman with schizophrenia who was admitted to our hospital’s department of psychiatry in a catatonic state. The schizophrenia had been diagnosed 3 years before her presentation to us. Her medical history included a lumbar vertebral fracture that occurred at the time of schizophrenia onset; she had tried to jump during a state of confusion. After the diagnosis, she visited psychiatric departments at numerous hospitals, but she loathed the weight gain that occurred with the use of antipsychotic drugs, so she did not return for follow-up examinations or take her prescribed medications regularly. A few weeks before admission to our hospital, she began displaying stereotyped behaviors, such as suddenly lying down and getting up again repeatedly. Shortly thereafter, she became unresponsive when addressed and fell into a catatonic stupor.\nOnce admitted to our department, the patient was prescribed oral quetiapine at 400 mg per day, lorazepam at 3 mg per day, and eszopiclone at 1 mg per day. Blood tests performed soon after admission revealed no electrolyte abnormalities (Na 139 mEq/L, K3.4 mEq/L, and Cl 100 mEq/L), and brain computed tomography (CT) showed no organic abnormality despite a slight difference in size between the left and right cerebral ventricles. The catatonic stupor improved after admission, but the patient became delusional, expressing a belief that “war is about to break out.” This resulted in extreme anxiety, and from the evening of hospital day 3 to the morning of hospital day 4, the patient impulsively drank an excessive amount of water, attested to by the empty PET bottles, which together would have contained 4500 mL, and by the fact that the patient readily obtained water from other sources. She complained of a headache and vomited on the morning of hospital day 4. Over the next several hours, her level of consciousness decreased to Glasgow Coma Scale (GCS) 3 (E1, V1, M1), and she became unresponsive to speech. Her vital signs remained stable, with blood pressure of 128/89 mmHg and body temperature of 36.4°C. Both pupils measured 5 mm, and both were sluggishly responsive to light. No seizure was observed. Her blood counts were normal, and blood tests revealed the following: Na 116 mEq/L, K 3.6 mEq/L, and Cl 82 mEq/L. Her blood gas levels were normal. Brain CT was once again performed, this time revealing narrowing of the cerebral ventricles and globally indistinct cerebral sulci, findings suggestive of cerebral edema (). A brain surgeon suspected subarachnoid hemorrhage on the basis of the CT images, but 2 days later, the part that appeared to be bleeding had disappeared, and no aneurysm was evident on the 3D image, so subarachnoid hemorrhage was ruled out. CSF examination revealed no abnormality. T2-weighted, fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted brain magnetic resonance (MR) imaging were also performed, revealing areas of high signal intensity, primarily in the cortex and subcortical white matter of the occipital and parietal lobes. In addition, an apparent diffusion coefficient (ADC) map of these areas, obtained on hospital day 7, showed decreased signal intensity (). PRES was suspected on the basis of the imaging findings.\nCerebrospinal fluid (CSF) analysis was performed, but no abnormalities were detected. Autoimmune encephalitis was ruled out on the basis of the CSF test results and results of blood tests for antinuclear antibodies, rheumatoid factors, and complement proteins. We did not treat the hyponatremia by means of aggressive sodium supplementation but rather balanced the extracellular fluid by continuous infusion of isotonic electrolyte replacement fluid. The serum sodium concentration increased to 138 mEq/L within 2 days. Despite rapid correction of the hyponatremia, none of the magnetic resonance (MR) images obtained over the next 5 days suggested central pontine myelinolysis. The patient opened her eyes in response to speech on hospital day 6, with her level of consciousness improving to GCS 14 (E4, V4, M6) by day 8. However, she scored 20 points on the Hasegawa Dementia Scale-Revised (HDS-R) on day 23, a score indicative of decreased cognitive function. (HDS-R scores are known to be correlated with MMSE scores.) Her level of consciousness continued to improve, but she suffered from a persistent mild attention disorder and decreased visual memory. Brain T2-weighted FLAIR MR imaging performed on hospital day 85 showed the high signal intensity regions in the occipital subcortical white matter to be gradually disappearing, and we believe this was part of the healing process (). She was discharged on day 141 after admission. Upon brain MR imaging performed 1 year after her presentation to us, we noted that the high density area had disappeared. We also noted absence of residual executive function deficits.
A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis (Fig. , ). The major findings of EPS, such as peritoneal thickening and calcification, were not noted on CT.\nOn physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction (Fig. ). As we suspected that the peritonitis might be associated with bacterial translocation from the dilated intestine, he was advised to stop eating and was switched from CAPD to hemodialysis. Additionally, he was treated with intravenous vancomycin and cephtazidime. The PD catheter was flushed once a day to prevent catheter obstruction with fibrin, and the characteristics of the peritoneal fluid were monitored. His abdominal pain was resolved and peritoneal fluid cell count decreased to < 30/mL, and thus, he resumed oral intake on day 8.\nAfter resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells (Fig. ). Although he stopped eating again, his abdominal pain did not improve, and fecal material with foul smell was identified from the PD catheter on day 23 (Fig. ). Culture of peritoneal dialysate on admission was negative; however, culture of peritoneal dialysate on hospital day 23 was positive for Enterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. ). Considering these facts, intestinal perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. Although intra-abdominal adhesion was severe, fibrinous encapsulation of the bowel, which would suggest EPS, was not detected macroscopically during surgery (Fig. ). As indicators of EPS were not evident, the PD catheter was removed. The perforation site was located at the adhesive intestine. The tip of the peritoneal catheter was located in Douglas’ pouch, and it did not injure the adhesive intestine. Pathological examination of the resected specimen revealed inflammatory cells associatet with the peritonitis in the intestinal wall. Intestinal fibrosis, arterial alteration, and tissue calcification were not evident pathologically (Fig. , ). Although his serum beta-2 microglobulin (B2M) level was high (41.05 mg/L), amyloidosis and deposition of B2M were not observed (Fig. -). The postoperative course was uneventful and left arteriovenous fistula surgery was performed on day 42. Since then, he has been on maintenance hemodialysis with no recurrence of peritonitis.
A two-month-old female child presented with the complaints of passing stools from normal anal opening and a fistulous opening in the vestibule. A provisional diagnosis of perineal canal was made. Abdominal sonography, echocardiography and spinal radiography were normal, ruling out associated urinary tract, cardiac and vertebral anomalies. Examination under anaesthesia revealed a normal anal opening. The vestibule had 3 openings, which were a normal urethral and vaginal opening and a rectovestibular fistula () with no communication with the normal anorectum. Considering this to be a complex anomaly, a diverting stoma was planned. On exploration of the abdomen for the stoma, tubular duplication of the sigmoid colon was noted (). A high sigmoid loop colostomy was done which resulted in four stomal lumens. Later, for better delineation of the anatomy of the anomaly, a proximal and distal colostomogram was performed (). It revealed duplicated colon with separate lumens from the transverse colon downwards with two anal openings. The duplicated colonic segment towards the mesenteric side terminated as a rectovestibular fistula (). At 6 months of age, the patient was taken up for definitive surgical management. An abdominoperineal approach was used, in which initially the colostomy was mobilized. For the distal duplicated colonic segment, the mucosal cuff of the colon leading to the vestibular fistula was dissected like a Soave's endorectal pullthrough from the distal stoma to the vestibular fistula and excised (). The remaining muscular cuff was plicated and closed.\nThe proximal duplicated colonic segment shared the vascular supply with the native colon, hence resection was not possible. The duplicated segment extended proximally till the ascending colon and common wall comprised of two layers of mucosa. Therefore, the intervening mucosal septum between the duplicated colon, upto the non-duplicated ascending colon was divided (). A colo-colic anastomosis between the proximal unified colon with the distal native colon leading to the normal anus was done. In view of the significant disparity in the proximal and distal colonic luminar diameter, a covering ileostomy was also done. The postoperative course was uneventful and the ileostomy was closed after 3 months. At follow up of 6 months post-surgery, the opening in the vestibule had closed, with no bowel complaints.
A 5-year-old Caucasian male with Fitzpatrick skin type 2 was referred for evaluation of a lesion along the midline of his upper back. The lesion was first noted at 2 years of age. It later developed as a 6 mm brown papule, which was diagnosed as a nevus, with a peripheral halo of hypopigmentation and central thickening (). There was no family history of melanoma.\nAn excisional biopsy (9 mm by 6 mm by 3 mm) was performed. The junctional component of the nevus was relatively symmetric and contained large melanocytes with abundant cytoplasm and nucleoli. In the epidermis, melanocytes were distributed primarily as nests at the tips and sides of the rete ridges with some clefting from the overlying epidermis and discohesion among melanocytes within the nests (). Nests appeared to predominate over single cells, without a significant upwards scatter (). Single cells were plump epithelioid cells with amphophilic to eosinophilic cytoplasm and nuclei. Some had prominent nucleoli, and others had marked nuclear hyperchromasia and smudging. In the dermis, similar melanocytes were present with poor maturation upon descent. Kamino bodies were not observable. The dermal component also revealed prominent nuclear atypia with scattered mitotic figures (). Asymmetrical melanin pigmentation of the dermal component and a mild, asymmetric lymphocytic infiltrate were noted. A small cluster of melanocytes with cytomorphology suggestive of a conventional nevus component was also present.\nImmunohistochemistry showed a loss of p16 throughout the lesion, except in the region of the conventional nevus component. HMB45 revealed faint positivity throughout the thickness of the lesion. MelanA/Ki67 was unable to be interpreted due to poor MelanA staining quality.\nDue to the atypical features, the specimen was sent out for further molecular investigations. aCGH demonstrated gain in chromosome 7p, subthreshold gain in chromosome 7q, loss in chromosome 9, and subthreshold loss in 8p (). The loss of chromosome 9 was the most prominent molecular result and directly correlated with the loss of p16 expression demonstrated by immunohistochemistry ().\nThe patient then underwent wide local excision with 1 cm margins. Pathology of the wide local excision tissue showed a focal area of dermal fibrosis at the presumed previous biopsy site and some reactive, overlying melanocytic cells. However, there were no definitive atypical melanocytic cells and no evidence of residual malignant melanoma. Sentinel lymph node biopsy (SLNB) showed no evidence of metastatic malignant melanoma. The final diagnosis was an invasive melanoma, non-ulcerated, 1.2 mm Breslow thickness, mitotic index 1/mm2, Clark’s level unspecified, and Stage IB. The patient remains well 20 months after diagnosis and receives regular follow-up.
A-5-year-old boy with 3-week mouth ulcers on both cheeks visited our children's dental clinic with his parents. He had been examined and treated with systemic antibiotics at another clinic for 1 week yet there had not been any recovery. A doctor at the clinic asked him for biopsy to make an initial diagnosis, which made patient's parents worried, resulting in their decision to have him transferred to our dental clinic.\nAt our place, patient's body was normal and showed no signs of fever. He was not aware of the habitual cheek biting during the day. The patient's face was swollen and suffered from deformity. The ulcers (Figure ) on the mucous membrane of both cheeks, with a white implant about 3 × 2 mm on each side. The lesions were strong, not sticky, but painful if being touched. The patient's saliva had normal color and glands; lower jaw nodes were portable, painful, and 5 mm in size. The patient could bite normally and had no crowded teeth.\nWe noticed that the child sucked his cheeks frequently while he was playing. Even when he was reminded, he continued sucking the cheeks.\nUlcers of the mucosa on both cheeks were due to unknown reason of cheek biting.\nTreatments of local lesions including the reduction of pain and the risks of subsequent infections associated with drug use, the prevention of any further trauma in cheek mucosa, as well as the promotion of wound healing process being planned with the orthodontic support.\nTreatment of local lesions: Oral gingivitis (2% chlorhexidine) and gingival hygiene (metronidazole 10%), three times a day after meals. Biting correction: Orthodontic support was used to remind the patient and protect him against further damages. We have used the EFStart Evolution function of Orthopedics—France in this case; it was cut and modified in the second molar area to avoid being touched and stimulating injuries. The patient was followed up within the first 7-10 days during the period of local treatment and biting correction. Then, orthodontic support and on-site treatment were maintained, and the patient was re-checked after 1 and 2 months. After 4 days of treatment, the injured right cheek mucosa was almost completely healed, and ulcer on the left cheek was almost reduced (Figure A). After 10 days of treatment, the lesion on the right cheek was completely healed, and the one on the left cheek was almost healed (Figure B). After 30 days of treatment, the lesions on both sides of the cheeks were completely healed (Figure C). Treatment finished after 6 months follow-up without recurrence.
A 74-year-old female was admitted to the Emergency Department of Sekghoma Memorial Hospital, Serowe village, Botswana, past midnight with progressive swelling of her face, tongue and breathing difficulty for about 8 h. The family gave a history of change of antihypertensive medication recently. She has been started on tablet enalapril, 20 mg, once daily, orally, 2 days back. On examination, she was conscious, coherent but very anxious. Her heart rate was 123/min, regular. Blood pressures were high and reading was 180/96 mmHg. Her room air oxygen saturation was 91% (SpO2). There was no stridor. On auscultation of chest, air entry was good bilaterally and there were some conducted sounds from pharynx due to excessive secretions. The tongue was grossly swollen, hard in consistency, it was wedged between the teeth and she was not able to close her mouth []. Lot of secretions were pooling in and dribbling from the mouth. There was no space in oral cavity for oral intubation. Only possible airway managements were a blind nasal intubation or surgical airway. It was not possible to transfer this case to a tertiary care hospital by helicopter as there were landing issues in darkness. Transfer by road was risky for patient as it would have taken 5 hours. It was decided to go ahead with management locally. The patient was started on oxygen support at the rate of 5 l/min by facemask for building up her oxygen reserves in case of airway emergency. Lateral view X-ray of neck was done which showed airway patency as fine []. Elective blind nasal intubation as a proactive airway management was risky due to less chances of success and more risk of further airway compromise. There were no ear, nose, and throat (ENT) specialists available for either evaluation or surgical airway management. She was injected with 100 mg of hydrocortisone, intravenously and 0.5 ml of injection adrenaline (1:1000) subcutaneously. Injection pheniramine maleate, 45.5 mg, was injected intravenously. She was positioned in sitting position. Intermittent atraumatic oral suction was advised. She was observed for about 30 min in which she gradually became worse and swelling of face and tongue increased. Blood pressures were shooting up. Provisional diagnosis was enalapril-induced angioedema. We decided to go ahead with fresh-frozen plasma infusion under intravenous beta-blocker antihypertensive coverage. Her blood group was O-positive and hospital being a peripheral center; we had only blood storage facility where O-positive fresh-frozen plasma was not available. Blood bank was 3 h away. We decided to go ahead with transfusion of the only pint of O-negative fresh-frozen plasma available at our storage facility. Injection metoprolol, 5 mg, intravenously was instituted and then we started with O-negative fresh-frozen plasma infusion intravenously, 220 ml over next 30 min. On post-fresh-frozen plasma infusion, patient's condition started improving []. Blood pressures remained stable around 150/90 mmHg after metoprolol injection. Over next 2½ hours, she recovered grossly and after 6 h she was able to close her mouth completely. We shifted her to wards after counseling about ACEI and ARB. We explained in detail the need to avoid enalapril.[]
An 8-year-old healthy male child reported to the Department of Oral and Maxillofacial Pathology of our institute with a chief complaint of pain and mild swelling in the left body of the mandible which had been increasing in size for the past 2 months. On clinical examination, no extraoral swelling was present in lower one-third of the face. Mild intraoral swelling with obliteration of the vestibular space was associated with deciduous mandibular left first molar. The swelling was diffuse and hard in consistency, with expansion of buccal cortex. Tenderness on palpation was noticed. Overlying mucosa was normal with no ulceration or purulent discharge. No carious teeth were seen in the region.\nRadiological examination using cone beam computed tomography revealed a localized mixed radio-opaque–radiolucent lesion in the buccal aspect extending from the distal aspect of 32 to the mesial aspect of developing 34 [Figure and ]. The lesion was surrounded by a thin, uniform radiolucent line []. It was involving the periapices of 74 and was in continuity with the roots of the same []. It extended inferiorly up to the middle third level of the coronal portion of the developing 33. The approximate maximum dimensions of the lesion were 11.9 mm × 13.8 mm × 16 mm []. Considering the clinical and radiographical findings, differential diagnosis of the lesion included odontogenic tumor, fibro-osseous lesion or hypercementosis. An excisional biopsy was performed for final diagnosis.\nThe gross specimen included multiple bits of hard tissues and deciduous mandibular first molar. Hematoxylin and eosin stained sections showed sheets of cementum-like tissue with prominent reversal lines []. Areas of fibrovascular connective tissue interspersed between cementum like masses []. At the periphery of the lesion, radiating columns of cellular unmineralized cementoid tissue was evident []. Multinucleated giant cells and plump cementoblasts were also seen []. Prominent and numerous basophilic reversal lines were appreciable []. On basis of clinical, radiological and histopathological correlation, a diagnosis of cementoblastoma was given. The patient is on follow up since last 6 months and is free of disease.
A 37-year old female patient reported to the department of prosthodontics with a request to discuss the options for prosthetic replacement of mandibular right first molar which was extracted 6 months back. She was given an option of implant-supported crown. The patient agreed to proceed with an implant-supported crown with 36. Clinical and radiological assessment showed an adequate bone support with 36. The benefits and the risks of the planned treatment were explained to the patient and a signature for consent was obtained.\nRecently, a Canadian company, Stomatotech, came up with a simple idea to retract the gingival tissue using a disposable plastic collar that is inserted on the apical end of the abutment before the abutment is engaged to the implant. Following the abutment's engagement to the implant, the plastic collar is found between the apical part of the abutment and the gingival soft tissue. Shortly after the removal of the impression from the mouth, the plastic collar is pulled out and removed permanently. The plastic collar creates a perfect gingival retraction with a valve factor preventing the liquids from contaminating the area of the finish line of the abutment.\nThe patient was called after three months for prosthodontic intervention. The healing abutment was removed () and the gingival tissue surrounding the implant was assessed clinically (). The G-Cuff (Stomatotech, Canada) () was planned to temporarily support the gingival surrounding the implant before making a direct-abutment level impression. The prepared abutment was checked in the special G-cuff measuring tool (). After determining the size of the cuff, the correct one G-Cuff collar was picked from the kit. The G-Cuff was placed, and the abutment was screwed back to the implant fixture (). The screw driver was fastened to the torque of 20 Ncm. The extra part of the collar was cut () and a periapical radiograph was obtained (). Then the direct PVS impression was taken () and sent to the lab. A metal-ceramic crown was fabricated (). The definitive crown was placed, and only minor occlusal adjustments were needed (Figures and ). The patient was called for regular check-up and a periapical radiograph was obtained after six months (). To show the comparison between impressions with G-Cuff and without G-Cuff, one more impression was made with open tray technique. A square impression coping was screwed to the implant fixture () and the top of screw was blocked with wax (). After that, a suitable stock plastic tray was selected and a window was created over the region of coping (). Then a PVS impression was obtained as described in open tray technique (Figures , , and ). The comparison between both impressions shows that G-Cuff records peri-implant tissues in a similar way as that of conventional method with gingival retraction.
A 24-year-old pregnant woman (G2P1) was referred to us due to suspected bilateral ovarian cysts at 8 weeks of gestation. She had undergone ovarian cystectomy twice under open surgery: left and right ovarian cystectomy for mature cystic teratoma and mucinous cystadenoma, respectively. She had no additional medical history or familial medical history. Transvaginal ultrasound and magnetic resonance imaging (MRI) (Figures and ) revealed two pelvic cysts. The left-sided unilocular cyst was 9 cm in diameter. The right-sided multilocular cyst was 5 cm in diameter. We diagnosed this condition as bilateral ovarian cysts.\nAlthough the serum levels of tumor markers (CA125, CA19-9, and CEA) were normal for a pregnant woman, considering the large size of the cyst, cyst resection was attempted at 14 weeks; however, it was converted to probe laparotomy. Marked adhesion around the cysts, posterior uterus, and Douglas' pouch made cyst resection impossible as extensive adhesiolysis may cause uterine damage and also uterine contractions after surgery. Gross examinations revealed no metastatic lesions or lymph node swelling. Abdominal fluid cytology revealed no malignant cells.\nAt 32 weeks of gestation, MRI revealed that the left-sided cyst size had increased to 27 cm in diameter (Figures and ), although she was asymptomatic. As shown in , the right-sided multilocular cyst became very close to the left monocytic cyst. At this stage, the left large monocytic cyst appeared to merge with the smaller right multilocular cyst, forming a large cyst occupying the entire pelvic cavity, which was later confirmed by laparoscopic findings.\nThis large cyst showed no solid-part or papillary growth. The serum levels of tumor markers remained normal. Malignant ovarian tumor could not be ruled out but was considered less likely. We weighed merits and demerits between relaparotomy for tumor resection during pregnancy and a wait-and-see approach for several weeks; the former is likely to require extensive adhesiolysis and may cause preterm delivery. We decided on the latter strategy, since resection should be performed in the event of a size increase or images indicative of malignancy. The fetus normally developed without fetal growth restriction.\nCesarean section and tumor resection were performed at 37+4 weeks of gestation, yielding 3,012-g male infant with Apgar score 8/9 at 1/5 minutes, respectively. The infant did not have congenital abnormalities. After the completion of cesarean section, we ruptured the wall of this large cyst, with care to avoid the cyst content entering into the abdominal cavity. A large amount of serous fluid was drained. This large cyst was a multicystic cyst (5 cm), considered to be the right multicystic ovarian cyst that had been observed from the first trimester. The wall of the large cyst showed marked adhesion to the peripheral peritoneal cavity. We resected it as widely as possible together with right salpingo-oophorectomy (Figures and ). The left ovary was macroscopically normal, and thus there was no evidence of the left ovarian tumor. The resected tumor consisted of a large unilocular cyst with serous fluid and a mucinous cystadenoma (Figures and ). In the former, lining epithelium was absent in many parts () and mucinous epithelium was occasionally found in continuity with the cyst wall of the latter (right ovarian cystadenoma). No malignant cells were found in the resected specimen. Immunohistochemistry revealed focally positive staining for estrogen and progesterone receptors on the resected cyst wall (Figures and ). At 12 months after the delivery, left ovary remained normal and the retention cyst did not recur. An informed consent for this reporting was obtained from this patient.
A 45-year-old Chinese male patient with cirrhosis of the liver due to hepatitis B virus presented one focal hepatic lesions with a cytologic and histologic diagnosis of hepatocellular carcinoma (Edmondson II) in October 2015. The computed tomography (CT) showed a 4 cm HCC located in the right hepatic lobe (Fig. ). The patient was placed on a waiting list for LT. His pretransplantation serum alpha-fetoprotein level is 1210 ng/mL. The pretransplant work up for LT included a CT scan of the abdomen and chest that showed no evidence of distant metastasis. A nuclear bone scan was also negative for bone metastasis. According to Hang-Zhou Criteria of China, he received an orthotopic liver transplantation on December 17, 2015. One day after liver transplant, the patient was given immunosuppressive therapy based on a standard rapid-taper regimen of corticosteroids and low-dose tacrolimus monotherapy (1 mg every 12 h) to the currently recommended target level. The patient had at 3-month intervals regular follow-up visits to the liver transplant clinic and had a normal alpha-fetoprotein (97 ng/mL), chest X-ray, and ultrasound (US) of the liver until March 2017.\nIn May 2017, he suffered from severe headache, and head contrast enhanced CT scan did not show clues for brain or skulls metastasis. Then, he developed hoarseness and dysphagia. Magnetic resonance imaging (MRI) showed that there was a mass on the posterior wall of the nasopharynx and multiple bone destruction in the basal part of the occipital bone (Fig. ). Examination with a nasopharyngoscope was performed, which revealed a neoplasm on the posterior wall of the nasopharynx (Fig. ). A biopsy of the neoplasm was conducted, indicating the nasopharyngeal cancer which was proven to be the metastatic tumor from liver histologically by biopsy (Figure ). This patient underwent radiotherapy (RT) of the metastatic nasopharyngeal tumor, and there was significant symptomatic relief. Finally, the patient died 3 months after nasopharyngeal metastasis was diagnosed.
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.
A healthy immunocompetent 44-year-old man had a 10-year history of a recurrent, occasionally itchy, skin rash on his upper back and abdomen with no improvement on moisturizing creams or topical antibiotic ointment that had been prescribed by his previous physicians. He currently presented for a total body skin check. Examination showed individual and grouped follicular non-inflammatory papules; the clinical differential diagnosis included follicular eczema and follicular contact dermatitis for the back and abdomen lesions, respectively. Triamcinolone acetonide 0.1% cream, twice daily for seven days, resulted in significant improvement of the dermatosis; however, the condition would promptly recur after treatment.\nThe patient subsequently presented for evaluation of his skin condition after recently completing a week of topical therapy with the corticosteroid cream. Cutaneous examination demonstrated localized areas of flattening or completely resolved follicular papules not only on the posterior neck and upper back (Figure ), but also on the mid abdomen above his umbilicus (Figure ); the individual hair follicles in the areas were also prominent. In addition, there was also brown darkening of the surrounding skin at prior sites of the condition on his back, consistent with post-inflammatory hyperpigmentation.\nSimilar to his initial presentation, the differential diagnosis included treated follicular eczema (on the upper back) and follicular contact dermatitis (on the abdomen). However, since the prior treatment with topical corticosteroids may have partially resolved the inflammatory component of the patient’s condition, the possibility of a primary folliculitis (either associated with bacteria, eosinophils, or yeast) was also considered. Therefore, punch biopsies from the upper back and the abdomen were performed. Both biopsies showed similar pathologic changes.\nMicroscopic examination of the biopsy specimen taken from the abdomen and stained with hematoxylin and eosin showed a prominent hair follicle with a perifollicular infiltrate of neutrophils and lymphocytes in the dermis (Figure ). Within the upper portion of the hair follicle, the infundibulum, there is a collection of neutrophils and numerous yeast. A periodic acid-Schiff stain highlights the fungal elements within the follicle as round purple organisms (Figure ).\nThe pathologic changes observed are those of Malassezia folliculitis. The topical application of the corticosteroid cream accounted for the atypical morphologic appearance of the skin condition. Therefore, correlation of the history, clinical presentation and pathology findings established the diagnosis of Malassezia (Pityrosporum) folliculitis incognito.\nThe patient was treated for four weeks with ketoconazole 2% shampoo to the affected areas in the shower for five minutes each day and ketoconazole 2% cream twice daily to the skin lesions. There was complete resolution of the condition (with residual post-inflammatory hyperpigmentation) on follow-up. He will continue to use the topical antifungal therapies two to three times weekly to prevent recurrence.
A 74-year-old man was admitted to our hospital for the examination of IPMN in the pancreas head. A cystic lesion, 6 mm in diameter, in the pancreas head was initially revealed when he underwent ultrasonography for a routine checkup for fatty liver 10 years prior. Magnetic resonance imaging revealed the cystic lesion and a slightly dilated main pancreas duct (6 mm). The patient was diagnosed with mixed-type (both main and branch pancreatic ducts involved) IPMN and has been carefully followed-up by magnetic resonance imaging every six months for the last 10 years. The most recent magnetic resonance images revealed an increase, relative to the second most recent images, in the size of the IPMN (that is, an increase from 8 mm to 12 mm) in the pancreatic head, while the dilation (6 mm) of the main pancreatic duct showed minimal change (axial image in Figure A and cholangiopancreatography in Figure B). The findings in the branch duct IPMN showed discrepant results with different imaging modalities. While contrast-enhanced computed tomography (Figure C) and magnetic resonance imaging did not show any nodules in the IPMN, endoscopic ultrasound revealed a suspected nodule (6 mm). The tumor markers carcinoembryonic antigen (CEA), carbohydrate antigen 19–9 (CA19-9), and DUPAN-2 were all within normal limits. Because there was an increase in the size of the branch duct IPMN, and a preoperative endoscopic ultrasound suggested the existence of a nodule in the IPMN, a malignancy in the branch duct IPMN was suspected and we performed pylorus-preserving pancreatoduodenectomy (PpPD) with lymphadenectomy for diagnostic and therapeutic purposes. The postoperative course was uneventful and the patient was discharged on post-operative Day 29 and has been alive for 18 months.\nIn the resected specimen, a dilated main pancreas duct, 6 mm in size, and a cluster of multiple dilated branch pancreas ducts were seen (Figure A). Histological examination revealed that both the main and branch pancreas ducts were composed of intraductal papillary mucinous adenoma with mild atypia (Figure B, C). Therefore, we diagnosed this patient as having mixed-type IPMN. Incidentally, a 3-mm nodule of endocrine cells was found in the IPMN lesion in the main pancreas duct (Figure B, D). Since the cells demonstrated positive staining for chromogranin A and synaptophysin (Figure E, F), the nodule was diagnosed as a PEN. The cells also showed positive staining for glucagon (Figure G), and negative staining for insulin and somatostatin. The plasma levels of glucagon, insulin and somatostatin had not been examined preoperatively. Since the mitotic count per 10 high-power fields was less than 2 and the Ki-67 index was less than 2% (Figure H), the PEN was pathologically classified as a well-differentiated neuroendocrine tumor and was low-grade (G1) according to the 2000 and 2010 WHO criteria, respectively [,]. We retrospectively assessed the preoperative images but did not find a nodule corresponding to the PEN lesion. Regarding the nodule in the branch duct IPMN, we also retrospectively assessed the preoperative images and resected specimen but did not find evidence of a nodule in the cluster of multiple dilated branch pancreas ducts corresponding to the endoscopic ultrasound finding. Thus, we recognize that such nodules can be misdiagnosed or over-diagnosed (that is, generate a false positive result) by endoscopic ultrasound.
A 42-year-old woman with biliary colic was assessed for an elective LC. Medical history was positive for factor V Leiden coagulopathy, recurrent deep vein thromboses and pulmonary embolisms (treated with lifelong anticoagulation).\nIntraoperatively, severe inflammation of the gallbladder required conversion to an open cholecystectomy. Due to the occurrence of an intraoperative common bile duct injury, a stent was inserted manually under visual control via the cystic duct in an anterograde direction. The injury was located at the insertion of the cystic duct into the common bile duct, was an anterior, partial transection with loss of substance of 7–10 mm, resulting in a Type D or E1 iatrogenic bile duct injury in Strasberg classification. Because of the loss of substance, the surgeon preferred to place a stent rather than a T-tube. There was no arterial injury. A drain was left in place in contact with the cystic stump. On postoperative day 8, a bile leak occurred and was treated by laparoscopic washout. The patient was subsequently discharged.\nSix weeks later, she was referred to a tertiary academic centre with severe right upper quadrant pain. On examination, she was found to be afebrile and normotensive, with a distended abdomen and right upper quadrant tenderness. On admission, blood tests showed: white blood cell count, 14.4 g/L, normal serum amylase, aspartate transaminase 26 IU/L, alanine transaminase 179 IU/L, bilirubin 4 µmol/L, albumin 24 IU/L, globulin 42 g/L. An abdominal computed tomography (CT) scan revealed no significant intra-abdominal collection, but the common bile duct stent was shown to be malpositioned, perforating the duodenum and lying in the retroperitoneal cavity (). ERCP confirmed a malpositioned biliary stent, running parallel but extra-anatomically to the common bile duct, which was dilated to 3 cm. The stent could not be accessed and removed by endoscopic means (). It was surgically removed using an open approach and T-tube enabled biliary drainage. Postoperatively, a resurgent leak of the cystic duct was successfully managed with endoscopic insertion of a biliary stent. The patient recovered completely following the procedure.
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.
An 80-year-old Caucasian male with history of coronary artery bypass graft (CABG) was brought to emergency room (ER) after being found unconscious at home. Detailed examination showed that left radial pulse was markedly diminished compared to right side and blood pressure on left arm was 60/44. There was numbness and weakness of left hand with hand exercise but did not result in any overt mental status changes or cardiovascular manifestations. No bruit was appreciated over the subclavian artery; however, carotid bruit was appreciated bilaterally. Electrocardiogram showed sinus rhythm with RSR pattern in V1, Q waves in V2, V3, with T wave inversions in AVL, which were new compared to his old EKG. Troponin I was 22.75 ng/mL, and diagnosis of NSTEMI was made.\nEptifibatide was started per cardiac and renal protocol. The patient had chronic kidney disease with acute worsening due to dehydration. Echocardiogram showed anterior wall, distal septal, and apical hypokinesia with ejection fraction around 50%, grade II diastolic dysfunction, moderate mitral and tricuspid regurgitation, and severe pulmonary hypertension. Difference in blood pressure in two arms was indicative of underlying left subclavian artery stenosis which was probably the cause of his syncope. Cardiac catheterization was planned, but patient was at considerable risk for contrast induced nephropathy given his renal insufficiency.\nPatient had left heart catheterization after stabilization of renal insufficiency. This showed that left main coronary artery bifurcated into the left anterior descending and left circumflex artery. The left main coronary artery was normal. The left anterior descending coronary artery (LAD) showed an ostial 90–95% stenosis. The proximal LAD was diffusely diseased, and mid LAD was 100% occluded. There was evidence of flow into the left internal mammary artery (LIMA) anastomosis to LAD, distal to mid LAD occlusion. There was significant back filling of the LIMA graft with minimal competitive flow (Figures and ). The first obtuse marginal artery (OM1) was 100% occluded. The right coronary artery was proximally occluded 100%. The saphenous vein graft (SVG) to the right coronary artery was patent. The SVG to the OM1 was widely patent and provided grade III collaterals to the LAD system. The LIMA to the LAD was not cannulated; however, the graft itself appeared to be healthy based on back filling with left coronary injection. Pressure in the LIMA appeared low.\nSelective injection into the left subclavian artery demonstrated high-grade stenosis of 99% or greater in the ostial left subclavian artery (). Diagnosis of coronary subclavian steal syndrome (CSSS) was made.\nAngioplasty confirmed 2.5 cm long calcified occlusion of the left subclavian artery. It extended from the aortic arch to the near vertebral artery. A 6 French R4 guide was advanced and engaged into the ostium of the left subclavian artery. An attempt was made to cross this stenosis with a Terumo Glide followed by a stiff Terumo Glide. 4000 units of heparin were given. The obstruction was approached distally using a 5 French R4 guide and a Terumo Glide followed by a stiff Terumo Glide. Attempts were unsuccessful to cross this lesion. The procedure was aborted due to severe calcification and length of stenosis.\nCardiothoracic surgery was consulted for possibility of bypass surgery to ameliorate the stenosed section of subclavian artery. Carotid duplex was obtained which showed evidence of bilateral carotid artery stenosis of 70–90% on the right and 50–69% on the left. Given the bilateral carotid artery disease, carotid subclavian bypass was deferred.\nLater on, the patient had magnetic resonance angiography () which delineated his coronary and subclavian anatomy appropriately, and he finally underwent a bypass to his proximal left subclavian artery stenosis from the right subclavian artery (subclavian-subclavian bypass).\nSurgery was performed using propaten graft to bridge the patent portion of left SCA with right SCA. Completion angiogram showed that the graft was widely patent, that the left subclavian artery did show flow from the right side, and that there was an antegrade flow reestablished into the vertebral artery (Figures and ).\nThis was a daring attempt on the part of cardiothoracic surgeon which resulted in complete amelioration of his symptoms and normalized blood pressure in both arms thus decreasing potential of further cardiovascular or cerebrovascular adverse events. His postoperative course was uneventful.
Three years before initial presentation, a 19 year-old female experienced the first of seven spontaneous pneumothorax events. By that time, three first trimester miscarriages had occurred (etiology unknown) and she had already undergone an uncomplicated term vaginal delivery of a healthy male infant. The patient did not use alcohol but had a four pack-year smoking history; she had stopped smoking 10d before her first pneumothorax. In the sixth week of her fifth pregnancy, she "felt sharp right-sided chest pain" radiating posteriorly accompanied by right arm paraesthesia and dyspnea. This developed while driving her car home. There was no history of trauma, coughing or sneezing episodes. She was brought to the emergency room where she was admitted with the diagnosis of right pneumothorax and a chest tube was placed. Good re-expansion was noted on chest x-ray and the patient was discharged home 2d later.\nApproximately two weeks later, a second pneumothorax occurred, this time following a prolonged "vomiting spell" secondary to hyperemesis of pregnancy. Evaluation in the emergency room identified a recurrent right pneumothorax. The patient's pulmonary symptoms were less pronounced and it was not considered large enough on chest x-ray to warrant treatment with a chest tube.\nA third small right pneumothorax developed at the 10th gestational week that responded well to conservative management and, again, no chest tube was required.\nHowever at ~19 weeks' gestation, the patient sustained a fourth pneumothorax with symptoms similar to that of the first. Chest x-ray revealed approximately 50% collapse of the right lung; she agreed to referral for definitive surgical management. Thoracoscopic excision of right apical lung blebs and mechanical pleurodesis proceeded without complication. The remainder of pregnancy was unremarkable and she was delivered (vaginally) of a viable female infant at term. Of note, the infant was diagnosed with postaxial polydactyly of both hands and one foot. Speech therapy identified "limited vocabulary for ag" and developmental delay was diagnosed. A normal karyotype was confirmed in the infant.\nAt age 21 the patient presented for prenatal care of her sixth pregnancy. She had resumed smoking following her penultimate delivery, but had discontinued cigarettes seven months before this conception. Based on her miscarriage history, additional testing was undertaken during antenatal screening and the patient was found to have protein S deficiency and be heterozygous both for MTHFR and prothrombin G20210A genes. Perinatal consultation determined that anticoagulation therapy was unnecessary based on these findings. Other prenatal laboratory tests and cervical cytology were normal. At approximately five weeks' gestation, the patient experienced "sharp left chest pain" and a spontaneous left pneumothorax was diagnosed by chest radiograph. Her previous four pneumothoraces involving the contralateral (right) hemithorax were noted. With supportive care her condition improved and lung re-expansion was achieved without chest tube placement. The patient was discharged home after three days.\nFor her sixth pneumothorax, she was readmitted to hospital one week later with similar symptoms and left pneumothorax was again identified on chest x-ray. Seven days of supportive care was followed by clinical improvement and radiographically confirmed resolution of the pneumothorax; the patient was discharged from hospital in good condition.\nThirteen days later, the patient described another episode of "extreme left-sided chest pain". Left pneumothorax was found on chest x-ray (50% collapse of the left lung). A 20 French chest tube was placed under intravenous sedation (midazolam HCl 2 mg) and lung re-expansion to <5% pneumothorax was achieved. Her hospital course during this seventh pneumothorax was unremarkable except for a mild intercurrent pneumonia (Tmax38.2°C) that responded well to intravenous antibiotics. The patient was discharged home in good condition 19 days later.\nAt 16 weeks' gestation the patient was admitted to hospital for mini-thoracotomy and thoracoscopically directed mechanical pleurodesis. Apical bleb disease was not prominent, and no obvious source of leak was identified. Recovery was uneventful and complete re-expansion of the lung was achieved.\nShe experienced no further chest pain and the remainder of her pregnancy was uncomplicated. At 34 5/7 weeks' gestation the patient underwent a spontaneous vaginal delivery of a viable male infant (weight 2336 g, Apgars 9/9). Given her tendency to develop pneumothoraces in pregnancy, the patient requested permanent surgical sterilization and laparoscopic bilateral tubal ligation was performed 10 weeks later. The progression of recurrent pneumothoraces and their treatment during the two pregnancies is depicted in Figure . Significant chest x-ray findings observed during the two pregnancies are summarized in Table .
A 60-year-old male was diagnosed with histologically confirmed glomerulonephritis secondary to anti-neutrophil cytoplasmic antibody -positive microscopic polyangiitis, following investigations for elevated creatinine on routine blood test. He was treated in hospital with intravenous methylprednisolone (500 mg daily) and oral cyclophosphamide (100 mg daily) for three days. He was discharged home on a weaning course of prednisolone and cyclophosphamide, with normalisation of his renal function.\nTwo weeks following this admission, the patient was admitted to a regional hospital with a two day history of nausea, vomiting and diarrhea with intolerance of oral intake. His wife had had similar symptoms recently. The patient developed large volume watery diarrhea, up to eight liters per day. He required transfer to a tertiary hospital intensive care unit where he received hemofiltration for hypovolemic acute kidney injury. Cyclophosphamide was initially reduced to 50 mg daily and then ceased in setting of potential infectious pathology. The patient had received approximately one month of cyclophosphamide, up to 2.1 g of total dose. Empiric antimicrobial therapy was commenced including tazobactam and piperacillin, intravenous metronidazole and ganciclovir. His stool specimen showed secretory diarrhoea with no infective agents identified. Vasoactive intestinal polypeptide and chromogranin levels were also non-diagnostic.\nSerial computed tomographs of his abdomen revealed diffuse mural thickening of the small and large bowel. Upper and lower endoscopic evaluations demonstrated denuded and erythematous mucosa in the duodenum, as well as from sigmoid colon to terminal ileum with no significant interval change in macroscopic appearance (Figure ). The rectum was relatively spared. Histopathology showed full thickness mucosal ulceration and inflammation throughout the terminal ileum and large bowel (Figure ). There were no features of inflammatory bowel disease, vasculitis or viral inclusions.\nHis diarrhea persisted up to six liters daily despite empirical treatment with maximal doses of antidiarrheals, octreotide and cholestyramine. Repeat imaging, stool specimens, endoscopic evaluation and histopathology failed to reveal an infectious, neuroendocrine, inflammatory or neoplastic etiology. Repeat colonic biopsies showed regenerative mucosal changes. In particular, viral polymerase chain reaction (PCR) and bacterial and fungal cultures were negative on repeated testing. He required continuous intravenous therapy, electrolyte replacements and total parenteral nutrition for severe hypoalbuminemia. Infliximab was administered as empirical therapy without significant clinical or endoscopic improvement.\nThe patient subsequently developed septic complications with Enterobacter and Candida glabrata bacteremia. He returned to intensive care and subsequently died from severe acute respiratory distress syndrome.\nPost-mortem examination showed multiple areas of hemorrhagic ulceration in the small bowel. Histology of the ulcerated areas in small bowel showed minimal residual mucosa. Similar changes, but less severe were seen in sections of ascending and transverse colon. A small amount of retained mucosa was seen in the descending colon with relative sparing of the rectum (Figure ). No evidence of vasculitis or thromboemboli was seen in multiple sections of the bowel wall and mesentery. No definite infectious etiology was identified in histological sections (no bacteria or fungal organisms seen on PAS or Gram stains, immunohistochemistry for cytomegalovirus, herpes simplex virus-1 and -2 were negative). Viral PCR from the small and large bowel tissue detected herpes simplex virus-1 DNA, but the clinical significance of this was uncertain in the absence of consistent immunohistochemistry and previously negative PCR.\nSections from all lobes of the lungs revealed changes of diffuse alveolar damage (shock lung) and metastatic pulmonary calcification. Culture detected Enterobacter faecium, Candida krusei and Pneumocystis jiroveci. Candida krusei was also cultured in small and large bowel.
A 42 years old woman was admitted to our hospital due to gross hematuria and right flank pain. The patient had a history of epilepsy, delayed development, and learning difficulties during childhood. The Patient had multiple hyper-pigmented angiofibromas on her face, since her childhood. Her mother died a few years ago from chronic renal failure as a consequence of TSC.\nPhysical examination revealed hypotension, tachycardia, hematuria and a palpable mass at the level of the right side. The laboratory blood analyzes revealed anemia (Hb 4.9 g/dl), leukocytosis (WBC 19.100/μl), creatinine 6.07 mg/dl, azotemia 216 mg/dl, CRP 292 mg/L.\nDuring RBC concentrates transfusion, the patient performed an abdominal CT without contrast medium that showed on the right kidney a coarse formation (17 cm × 14 cm x 9 cm) with not homogeneous density as for possible bleeding areas in the context, that entirely occupies the renal lodge and extends along the entire right flank till the right iliac fossa in the absence of safe cleavage planes with the minor structures. In addition, multiple exophytic cortical alterations were reported in the right and left kidney, some hypodense and others hyperdense, hypervascular and with adipose component in the context of dimensions ranging from a few millimeters to about 3 cm, to be referred to AMLs.\nThe patient was diagnosed with giant bleeding renal AML. We performed blood and clots evacuation in the bladder and bladder irrigation with three-ways Foley catheter.\nAfter nephrological preparation, the patient underwent a new CT with contrast medium which confirmed the information provided by the previous CT examination, highlighting a central hypervascular core borne by the known voluminous formation on the right kidney ().\nSubsequently, the patient underwent selective catheterization of the right renal artery which showed active high-flow diffusion of contrast medium in one of the lower third division branches of the renal artery itself ().\nSelective embolization of the hemorrhagic branch was therefore performed through metal spirals from 5 to 3 mm, distal and proximal to the blood spill, by performing a luminal trapping. The final angiographic balance showed an arrest of active blood shedding (Video 1).\nSupplementary video related to this article can be found at\nThe following is/are the supplementary data related to this article:\nThe CT control performed 18 days after the procedure showed the hematoma in the right kidney substantially unchanged in size, currently less hyperdense in relation to the presence of blood component in different stages of organization.\nThe patient underwent a total of 6 units RBC concentrates transfusion in the first 3 days of hospitalization. No blood transfusions in the next 15 days of hospitalization, with stable hemoglobin values.
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.
The patient is a 57-year-old nulligravid female who presented to clinic with symptoms of urinary urgency incontinence. She had a past medical history of endometriosis, autoimmune hepatitis, cirrhosis, and denied any pertinent mental/psychological history or trauma. Her initial symptoms included urgency, voiding up to 5 times an hour, and nocturia up to five times per night. She had no previous vaginal surgeries. Baseline sexual function evaluation was completed at intake with the Female Sexual Function Index (FSFI) questionnaire where the arousal domain equaled 0.9 (range 0-6, with 6 indicating maximal arousal) (). Pelvic examination was significant for vaginal atrophy but no notable clitoral or labial abnormalities were visualized. There were no signs of prolapse or pelvic floor musculature hypertonicity. She was asked to keep a voiding diary and then advised to attempt timed voiding upon its completion. She was also started on vaginal estrogen and a generic anticholinergic agent (oxybutynin 10 XL daily). After 4 weeks of treatment, she experienced significant worsening of anticholinergic side-effects and stopped the medication on her own. She was then started on a beta sympathomimetic (mirabegron 25mg daily). After 6 weeks of this therapy, she did not have any subjective improvement in her symptoms. The dose was increased to 50 mg daily and continued for an additional 4 weeks. Again, she did not have an adequate reduction in symptoms and was counseled on third tier treatment options. Ultimately the decision was made to proceed with SNM.\nThe sacral neuromodulator was implanted per manufacturer instructions after undergoing a peripheral nerve evaluation with >50% improvement in her urgency symptoms. At one-week follow-up, her incisional pain was minimal. She did not require oral analgesics and had reported marked improvement in urinary symptoms consistent with the test phase.\nHowever, near the six-month follow-up appointment, she expressed concerns about persistent arousal symptoms in the vaginal area overall with new onset hypersensitivity localized to the clitoris. She did not have these symptoms prior to or immediately postimplant, but she reported gradual development of arousal symptoms postoperatively over the six-month period. She had not initiated the use of any new medications or therapies during the same time period.\nPelvic examination did not demonstrate engorgement of the clitoris, change from the intake examination, or evidence of hypertonic pelvic floor muscle dysfunction based on digital assessment. To manage her arousal, the four programs that were programmed into the system were alternated with cycling activated. Behavioral modifications were suggested including loose clothing. Upon no change in symptoms, device deactivation was performed. This resulted in no notable improvement of the manifest arousal symptoms. However, her urgency symptoms immediately recurred upon the deactivation. At this point, the plan was to trial a new set of programs to see if her arousal symptoms could be eliminated using different settings. The patient agreed to trial all four new programs, each over at least a 10-day period and assess which one was associated with fewer arousal symptoms. The programming was done at sensory levels. Alternation of pulse width and frequency was performed as well. The patient was instructed to complete a 4-week diary indicating arousal and bladder activity. At follow-up, she had trialed each program and continued to experience sexual arousal symptoms. Her symptoms were present even when the device was turned off and intensified when the machine was on. The symptoms were also present without any clitoral contact by undergarments. It was becoming so bothersome that it was difficult for her to stay asleep at night and creating anxiety. Complete testing of the neuromodulator unit was reperformed with normal values noted for impedance. Reprogramming of the unit with a new set of programs was performed again with subsensory levels used at this point. Additionally, she was offered sexual counseling but it was declined.\nOne year after placement, she elected for removal of the generator and lead. The generator and lead, intact with tip, were successfully removed with no complications. At the 6-week follow-up after explant, she had recurrence of urinary urgency symptoms with persistence of hyper-arousability. One year later, she continues to be sexually active with mild discomfort due to vaginal atrophy (as noted on the pain domain in the FSFI). She reports that the clitoris remains hypersensitive with persistence of the arousal symptoms, although moderately improved from prior to SNM. At the follow-up visits, no changes in medications or new medical diagnoses were reported when compared to prior visits. Furthermore, she denied any new stressors or change in her personal life regarding the relationship with her husband.
63-year-old woman who had suffered from the right medial knee pain for 5 years and was not responsive to conservative treatment was admitted to our clinics. 30° varus-valgus stress X-ray indicated that the patient had an intact MCL and LCL. After the detailed physical examination and reviewing of X-ray images, it was decided that UKR would be the most suitable option for the patient with anteromedial knee osteoarthritis. After spinal anesthesia application and sedation, the UKR surgery was performed with a standard minimal invasive midline vertical incision and a medial parapatellar approach; the patella was removed laterally but not dislocated or everted. The patient received a medial partial knee implant with a mobile-bearing insert (medium size with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). Following the UKR surgery (), weight bearing was allowed as tolerated by the patient and a standard postoperative physiotherapy was started on the first postoperative day. The patient was discharged at postop 2nd day when she met the following criteria: independent ability to get dressed, to get in and out of the bed, and to sit and rise from a chair/toilet; independence in personal care; and mobilization with crutches. After discharge, a home-based exercise program was given to the patient. At postoperative follow-up, our patient acquired a full knee RoM in the postop 1st month and returned to independent daily activities without any external support in the postop 3rd month.\nAt postoperative 1st year after first UKR application, the patient fell down while getting on a public bus; this caused that the right knee of the patient was exposed to the valgus force vector. After that moment, the patient heard a pop sound and felt an incredible pain that prohibited the flexion and/or extension of the medial side of the right knee. And then she was admitted to our emergency department. The first evaluation was performed, and the patient was diagnosed with a grade 3 MCL rupture and the UKR insert dislocation (). Having completed the preoperative preparations, the patient was operated on the same day. After anesthetic administration, a surgery with a standard minimal invasive midline vertical incision and a medial parapatellar approach (to a previous incision site) was performed to change the mobile-bearing insert with the same size (medium-sized mobile-bearing insert with 4 mm thickness; Oxford®, Zimmer Biomet Inc., Warsaw, IN, USA). After having changed the mobile-bearing insert, the MCL structures were repaired and anchored to its femoral origin with a 5 mm titanium anchor. Following the surgery, weight bearing and full RoM with a hinged knee brace were allowed as tolerated by patient and a standard postoperative physiotherapy was started on the first postoperative day. Crutches were recommended for 2 to 3 weeks to enable the patient to regain a normal gait. The brace was used continuously for 4 weeks and thereafter during the day for 2 weeks. After the physiotherapy program administration, the patient was discharged at postop 1st day.\nThe patients were evaluated regarding pain intensity (Numeric Pain Rating Scale (NPRS)), active range of motion (RoM), and quality of life (Short-Form 12 Health Survey (SF-12 Health Survey)). Functional capacity was evaluated using the Iowa Level of Assistance Scale (ILAS), Iowa Ambulation Velocity Scale (IAVS), Hospital for Special Surgery (HSS) knee score, and Timed Up and Go (TUG) test. Rehabilitation program and outcome evaluation were conducted by one clinical physiotherapist at preoperative period (before the first UKR application), at discharge (postop 2nd day after the first UKR surgery), and at postop 2nd year (after 2 years from the MCL repair and the insert change). The evaluation results are shown in .
A 40-year-old male patient born to nonconsanguineous parents presented to our hospital for more than 15 episodes of nonbloody, nonmucoid diarrhea associated with severe diffuse abdominal pain and vomiting of 30 days duration. The patient concurrently complained of severe weight loss, visual problems, progressive bilateral hearing loss, and progressive distal muscle weakness more prominent in the lower extremities of 2 years duration.\nHe recalls neurological symptoms beginning at the age of 27 years with episodes of intermittent bilateral blurry vision which would later resolve on their own. At the age of 32 years, the visual symptoms reemerged with worsened blurry vision and diplopia. After presenting to an ophthalmologist, a brain MRI was ordered with no significant findings at that time.\nAged 40 years, the patient presented to our hospital for the aforementioned complaints. He reported a significant weight loss of 18.5 kg representing a 34.4% decrease of his initial weight (∼55.4 kg) over the course of 2 years despite normal food intake. He also described postprandial episodes of nonbloody, nonbilious vomiting associated with diffuse abdominal pain for the last 8 months. The patient denied any symptoms of body dysmorphia or laxative abuse. On physical examination, the patient looked cachectic with a current body mass index (BMI) of 13.06 kg/m2. The patient had mild bilateral ptosis and ophthalmoplegia along with decreased sensations and proprioception in the lower extremities. The patient was also experiencing mild-to-moderate weakness of the upper and lower extremities and showed hyporeflexia in the upper extremities, while the reflexes were absent in the lower extremities. The motor weakness was more prominent in the lower extremities, leading to the patient's inability to ambulate without the assistance of a cane. Moreover, no weakness was to be reported upon flexion and extension of the neck, with absent pathological reflexes. The abdominal examination was negative for any tenderness or organomegaly.\nOn laboratory workup, the patient was found to have a mild increase in the hepatic enzyme AST with a value of 91 IU/mL. ALT was 33 IU/mL, and GGT was 341 IU/mL. The lipid panel revealed cholesterol levels of 201 mg/dL and triglyceride levels of 401 mg/dL.\nThe patient was found to have elevated levels of blood lactate (4.82 mmol/L) and elevated blood pyruvate levels (0.28 mmol/L). The complete blood count, thyroid function tests, arterial blood gases, metabolic tests (urine organic acids and blood-urine amino acids), and creatine kinase levels all returned normal. The results of the pulmonary function test, done to assess the function of the patient's lungs, were in favor of a mild restrictive lung disease. An audiogram was also performed to assess the patient's complaints of hearing loss, and it showed bilateral sensorineural hearing loss, while the electromyography showed severe neuropathy with demyelination suggestive of associated myopathy.\nFor the evaluation of the GI symptoms, the patient underwent stool analysis and an esophagogastroduodenoscopy with gastric, duodenal, and ileal biopsies, all of which returned with negative results with the biopsies showing no evidence of active inflammation, mucosal changes of chronicity, specific granulomas, or parasite. Moreover, a colonoscopy with superficial colonic biopsies was performed showing no evidence of thickening of the subepithelial basement membrane or evidence of intraepithelial lymphocytosis. The patient underwent a computed tomography scan of the abdominopelvic area revealing diffuse dilated fluid filling the small bowel loops without any evidence of bowel obstruction. Due to persistent and severe GI symptoms, the patient underwent a second colonoscopy with mucosectomy for deeper tissue studies. The pathology sections showed colonic tissue with normal mucosa, intact muscularis mucosa, and mildly cauterized submucosa. Ganglion cells were seen in the submucosal plexus showing features of intracytoplasmic eosinophilic inclusions consistent with megamitochondria (Fig. ). The appearances were consistent with mitochondrial inclusions. Clinical and genetic studies were advised for the diagnosis.\nAlthough no previous brain MRIs were available for comparison, the brain MR images demonstrated T2 FLAIR hyperintensity within the periventricular white matter, more so posteriorly, with minimal faint extension to the subcortical areas in both frontal lobes and in both temporoparietal areas (Fig. ). There were no findings of brainstem or posterior fossa involvement. The ventricles and the subarachnoid spaces were normal.\nTaking into consideration the clinical presentation and the workup findings, subsequent metabolic evaluation showed elevated plasma and urine thymidine and deoxyuridine; these findings are consistent with TP deficiency. Genetic testing was performed revealing 2 heterozygous TYMP variants. The 1st variant was c.401C>A p.(Ala134Glu), which was reported by Garone et al. []. The 2nd variant was c.1142T>G p.Val256Phe, which was reported by Etienne et al. []. Thus, the diagnosis of MNGIE was confirmed.
A 10-year-old male was referred to our office after being seen by a pediatric orthopedic surgeon for a distal left forearm mass with block in pronation and supination. The patient's past medical history is significant for a distal radius fracture 2 years prior to his aforementioned office visit. Follow-up radiographs after fracture union at that time showed no evidence of disease (Fig. ). Radiographs taken at the time of presentation 2 years after fracture healing revealed a 6.5 cm long lesion in the distal syndesmosis arising from the radius. The lesion resulted in pressure erosions upon the distal radius and ulna, causing a slight splaying of the bones (Fig. ).\nThe differential diagnosis included primary benign and malignant bone tumors, and thus preoperative staging studies were performed. A triple-phase bone scan was administered and demonstrated increased uptake in the distal shaft of the left radius and left ulna, as well as in the distal physis of the left radius (Fig. ). Subsequently, acquired SPECT images demonstrated similar findings of increased uptake.\nA MRI images revealed a large mass at the level of the distal radial and ulnar metadiaphyses, measuring ∼6 cm craniocaudad, 3.7 cm AP and 3 cm transverse (Fig. ).\nAfter initial imaging, a fluoroscopy-guided biopsy was performed. Positive B-Catenin nuclear immunostaining, along with histology showing fibrous tissue, supported a diagnosis of desmoplastic fibroma.\nUpon confirmation of desmoplastic fibroma, a discussion with the patient's mother was held, explaining the rarity of the tumor, and the likelihood that the patient would require radical resection to decrease risk of local recurrence, which would lead to a vast reduction in wrist motion. Additionally, as the tumor extended to the distal radius, resection of the joint surface would be necessary. Subsequently, a conversation about reconstructive options was held, including options for use of the fibula, allograft and allo-arthrodesis of the wrist joint. Ultimately, the patient's mother decided to pursue allo-arthrodesis with allograft, with plans to pursue future reconstruction with use of the fibula if this surgery was unsuccessful.\nUpon removal of the mass, the distal halves of the right ulna and radius were resected. Dorsal plate fixation was used to stabilize the allograft, which extended from the native proximal radius to the proximal carpal row, effectively creating a single-bone forearm (Fig. ).\nPost-operatively, the patient began occupational therapy 1 week after surgery, completing 13 sessions over several months, with a progressive increase in strength, range of motion and coordination of the left upper extremity. At 8 weeks, the patient was able to employ pincer grasp to pick up small marbles and make a fist at 10 lbs of force, with minimal pain on palpation as well as during therapy exercises. At the request of the patient's family, they were given a hand-strengthening routine to continue at home. At 12 months follow-up, the patient has exhibited excellent recovery, including normal range of motion at the shoulder and elbow and ability to perform all activities of daily livings despite reduced grip strength. On all follow-up imaging, the allograft has fully incorporated, with no hardware failure or loosening.
A 59-year-old female patient presented to a plastic surgery clinic with a lump on her right medial thigh. She noticed the lump eight months ago and it was gradually increasing in size. It was painless initially; however, the lump gradually became tender. The patient was otherwise fit and well with no co-existing morbidities. Closer examination of the right thigh revealed a high consistency mass, with reduced mobility. There was no right inguinal lymphadenopathy or lymph nodes enlargement.\nLaboratory blood tests were unremarkable. An MRI scan was requested to further assess the mass, which was irregular but well-defined in the medial compartment of the thigh, measuring 8.2 x 6.6 x 4.3 cm in size. The mass showed an intermediate signal on both T1 and T2-weighted imaging, appearing hyperintense compared to the adjacent muscular tissue, which also persisted on fat-suppressed imaging. The mass invaded the intramuscular fat planes and caused compression and displacement of the adductor muscles. There was no evidence of intra-muscular invasion. Normal cortical outline and medullary signal intensity of the femur were seen in the right thigh. There was no evidence of bone contusion, marrow oedema, fracture line, or cortical discontinuity. Other muscles of the thigh had a normal outline and signal intensity with no evidence of focal or diffuse oedema. Neurovascular structures were unremarkable. Overall, MRI findings were suggestive of soft tissue neoplasm, with a possibility of a neurogenic tumour. A wide excisional biopsy was then performed to further assess the tissue histologically by the pathologist and confirm the diagnosis. The removed mass measuring 8.5 x 6.5 x 5.4 cm and is shown in Figure . The mass had a smooth outer surface and a soft consistency. The cut surface showed a homogenous tan-white appearance with some slit-like spaces and occasional haemorrhagic spots. Multiple sections were processed for histopathological examination from different planes.\nHistopathology of the mass revealed a tumour arranged in sheets and fascicles composed of round to oval cells along with variably sized adipocytes (Figure ). Numerous interspersed lipoblasts with indented nuclei were also seen. Moreover, severe nuclear atypia was noted including bizarre cells (Figure and Figure ). An area of necrosis was seen with a few areas, which showed malignant and fibrous histiocytoma-like features. Furthermore, a few thin-walled dilated and congested blood vessels were also noted. Also, brisk mitosis was noted (35-38 per 10 HPF) (Figure ). Immunohistochemical staining (IHC) was also performed and the tumour cells were diffusely positive for vimentin and focally positive for S-100; while negative for creatine kinase (CK), smooth muscle antigen (SMA), desmin, CD34 and MyoD1. Overall, the pathological findings are all suggestive features of a high-grade sarcoma, favouring the epithelioid variant of PLS.\nThe patient was also referred to the Department of Nuclear Medicine & Molecular Imaging for post-surgical restaging and assessment for possible metastases using 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET-CT) scan. The abdominopelvic section showed the liver measuring within normal limits; however, the scan revealed an ill-defined hypermetabolic hypodense lesion seen involving segment VI measuring approximately 2.8 x 1.8 cm suggestive of hepatic metastases with concurrent fatty liver. The scan was also remarkable for the musculoskeletal system, revealing multiple hypermetabolic osseous lesions involving the skull base, right iliac bone, and few vertebrae (predominantly T7-L1); which was suggestive of skeletal metastases. A non-hypermetabolic pulmonary nodule in the right lung field was also detected; it was considered to be likely benign, but interval scanning is suggested to monitor further progress. Finally, there was also evidence of diffuse subtle hypermetabolism at the site of surgery which is suggestive of postsurgical inflammatory sequelae; however, microscopic residual disease cannot be completely ruled out. These multi-organ lesions were due to be biopsied to examine their immunohistochemical findings to confirm metastatic lesions of primary pleiomorphic liposarcoma. However, the patient did not attend the follow-up appointments.
A 47-year-old female, P4, who resided at Ado in Karu, FCT, presented to a private clinic in Abuja on the 7th of January, 2018 with vomiting, fever, sub-acute and progressive dizziness of about 8 hours duration which later progressed to generalized body weakness, dysphagia and odynophagia, dysarthria, left partial ptosis, sudden blurring vision, diplopia. Preliminary laboratory investigations conducted (full blood count, and electrolyte, Urea and Creatinine) were within normal range. Electorcardiogram investigation revealed sinus arrhythmia with intraventricular conduction block, and right atrial overload. Based on these findings initial diagnosis of ischaemic heart disease was made with oesophageal stricture and central retinal vein thrombosis. Within 24 hours of presentation her neurological symptoms worsened rapidly with bilateral ptosis and inability to open her eyes, and respiratory muscle paralyses followed by complete respiratory failure with progressive loss of consciousness. She died on the 8th of January, 2018 (, ).\nThe husband of case 1 presented to the same hospital on the same day with nausea, dizziness, vomiting, progressive dysarthria, odynophagia and partial ptosis of about 8 hours prior to presentation on the 7th of January 2018. There was history of consumption of fish pepper soup prepared by case 1 the night prior to presentation, with ingredients sourced at the local market (see details 72 hours dietary recall in ). Further information on the source of the fish, other ingredients and the method of preparation could not be ascertained. The meal was consumed by all members of the household. There was no history of consumption of canned foods or recreational drugs. Food recall could only be determined from Friday 5th of January 2018. He spent the day at a symposium with his office colleagues during which there was a buffet at the office from which all his colleagues ate. The specific items he ate could not be ascertained, there was no ingestion of other snacks or in-between meals. He is a known hypertensive on treatment; not diabetic on any other long-term medication or suffering from other chronic illness. He was afebrile (axillary temperature (T) of 36.4°C), acyanosed, not jaundiced, with partial ptosis, pulse rate (PR) of 90 beats per minute (bpm), blood pressure (BP) of 150/98mmHg.\nExcept for serial reduction in platelet count (from 271x109/L on the 8th January to 83 x109/L on the 9th January 2018), full blood count, serum electorlyte, urea & creatinine, C reactive proteins, erythrocyte sedimentation rate, liver function tests, Urine toxicology for opiates, morphine, amphetamine, cocaine and other illicit drugs were normal. He had serial plasmapheresis and enema, was placed on polyvalent immunoglobulin (botulinum-specific immunoglobulin was unavailable), pyridostigmine, glycopyrolate, amlodipine, crystalline penicillin, and hydrocortisone. He deteriorated with complete ptosis, dysarthria and respiratory failure (SPO2=90%) within 8 hours of presentation. He was managed at the intensive care unit, where he was intubated, had percutaneous endoscopic gastrostomy (PEG), and placed on ventilatory support. He remained stable until day 5 when he developed tachycardia (PR of 120bpm) and uncontrolled hypertension (systolic BP of 180 to 220mmHg systolic and diastolic BP of 120 to 150 mmHg). He developed sudden cardiac arrest and died on the 7th day on admission.\nThe daughter of the other two cases presented two days after the presentation of the first two cases with colicky abdominal pain, nausea, diplopia, dizziness and partial ptosis with intermittent headache. She was afebrile (T of 37.1°C), PR of 88 bpm, respiratory rate (RR) of 22 cycles per minute (cpm), BP of 113/61mmHg, and normal heart sounds. Similar investigations as done for case two turned out normal. She received polyvalent immunoglobulin (botulinum-specific immune The details of the food recall revealed that the family globulin was unavailable), repeated doses of activated charcoal and enema, and was placed on supportive therapy. Trivalent anti-toxin containing Type A, B and E ordered from Bangkok arrived on day seven after the onset. She had a stat dose of 250 ml slowly administered, followed 6 hours later with a continuous drip infusion of 250 ml. She was subsequently discharged after one week, and followed up for another one week. Apart from complains of mild dizziness, she reported no further symptoms post discharge.\nThe details of the food recall revealed that the family of six (parents and four children) had eaten a home-cooked meal of pounded yam with fresh fish pepper soup, a local delicacy, 36 hours prior to onset of symptoms in the first patient. The third case claimed eating only a small portion. The other three children were followed up closely but did not develop any symptoms.\nLaboratory diagnosis: we collected blood from all the three cases for laboratory testing. Food samples from remnant fresh fish soup and locally made drink from the family home were also collected and sent to the laboratory for culture. The samples collected in this case series were transported in a geostyle box on ice packs, to the National Veterinary Research Institute (NVRI) Jos in Plateau State, Nigeria for preliminary testing. Duplicate samples were sent to the Centres for Disease Control and Prevention (CDC) Botulism Laboratory in Atlanta, USA. Samples were plated on enhanced medium and incubated anaerobically at 37°C for 24 hours for possible isolation of clostridium species. Colonial morphology and the gram stain reaction were not consistent with clostridia. The food and blood samples were used for toxins by intra-peritoneal injection of mice (the mouse lethality assay) diluted in phosphate buffer which were also negative after 72 hours.
A 68-year-old woman visited our clinic in February 2015 complaining of nonproductive cough exacerbated by talking for about 2 months. Poor appetite with a weight loss of 2–3 kg in 1 month was also noted. Hemoptysis, hoarseness, fever, chillness, chest pain, shortness of breath, acid regurgitation, and bone pain were denied. As a common housewife with hypertension on regular medical control, she did not smoke cigarettes, nor indulge in alcohol drinking.\nDespite no significant findings on physical examination, her chest X-ray film revealed a mass shadow in the right middle lung field just adjacent to the hilum. A CT scan of the chest disclosed a right hilar tumor with obstructive pneumonitis in the right middle and upper lobes. Enlarged mediastinal lymph nodes, lymphangitis carcinomatosis of the whole right lung field, and a ground glass nodule, most likely a metastatic lesion, in the right lower lobe were also identified. A bone scan showed multiple metastases involving the skull, spine, ribs, sternum, pelvic bones, bilateral proximal femurs, and the medial aspect of the right knee.\nErythematous change and tumor infiltration were detected in the right bronchial trees on bronchoscope examination. A biopsy from the intermediate bronchiole of the right middle lobe led to a pathologic diagnosis of adenocarcinoma. A Leu858Arg (L858R) mutation of the EGFR gene was found by DNA study of the tumor.\nThe patient went to a medical center in northern Taiwan for a second opinion and there received daily oral treatment with 40 mg afatinib since March 20, 2015. Her devastating disease status resolved quickly, and a stable condition was well maintained until April 27, 2016. At that time, she was admitted to the neurology ward of that hospital for impairment of memory, attention, calculation ability, as well as gait and movement disturbance. Frequent episodes of dizziness, vertigo, and falling were also notable. These symptoms had developed progressively in the previous 3 months according to her family. Evident paranoid delusion and depressed mood were detected during hospitalization. However, there was no headache, vomiting, cranial nerve palsy, decreased muscle power, seizure, visual or auditory hallucination.\nA lumbar puncture was done. Cerebrospinal fluid (CSF) analysis showed a glucose level of 61 mg/dl, a total protein level of 49 mg/dl, and a white cell count of 8/μl. All the white cells were lymphocytes. CSF cytospin cytology, however, disclosed atypical cells of undetermined significance as reported by the pathologist. Thus, a diagnosis of LC was made.\nBecause a positron emission tomography-CT scan performed 1 month prior to admission demonstrated only 3 small hot spots on the right pulmonary hilum, right middle lobe, and right iliac bone, afatinib treatment was kept on while the patient received whole-brain radiotherapy with 3,000 cGy in 10 fractions from May 23 to June 3, 2016. She was brought home after radiotherapy, with afatinib taken irregularly due to a progressive downhill consciousness level.\nBy the time her family pushed her in a wheelchair into our outpatient clinic on June 28, 2016, she had already become unable to speak or eat, had no response to verbal order, and kept her eyes closed all the time. Acne, paronychia, and diarrhea were identified as probable adverse effects of afatinib at admission to the ward. Intravenous fluid supplementation and nasogastric tube feeding were established. A brain MRI showed diffuse abnormal leptomeningeal enhancement at bilateral cerebral and cerebellar hemispheres, compatible with LC (fig ). On account of the uncooperative and irritable status, a lumbar puncture was not done.\nAfter reviewing her previous medical record retrieved by her family, we decided to hold afatinib and prescribed mashed high-dose, pulsatile erlotinib 600 mg every 4 days through a nasogastric tube for rescue. In keeping with our expectation, the patient's consciousness level improved, as evidenced by opening her eyes to verbal stimulation after 2 doses of erlotinib. She could recognize her husband, smile, say simple sentences, and sit up on the bed with support after 4 doses. A second brain MRI revealed that the leptomeningeal lesions almost completely disappeared 17 days after the treatment had begun (fig ). She was able to walk several steps to the toilet under supervision when she returned home and kept on erlotinib therapy. Her acne, paronychia, and diarrhea also resolved soon after afatinib was discontinued.
According to an independent forensic report compiled a year after the events for which CP was charged in November 2001, CP was a 12-year-old, 5'2”, 95-lb boy with a family background involving considerable social dislocation. Despite the difficulties of his social situation, he had no record of treatment for nervous disorders or of violence or behavioural disturbance. Following an argument with his father at the end of October 2001, he was admitted to a behavioural centre for six days where he was started on paroxetine. His behaviour worsened daily on paroxetine. He was discharged against medical advice to the care of his grandparents, who, when his paroxetine ran out, took him to their primary-care physician who prescribed sertraline 50 mg, increasing this to 100 mg two days before the killings for which CP was charged. The duration of sertraline treatment was three weeks.\nAfter the prescription of sertraline, CP was involved in a number of aggressive incidents at school, the first on record for him, and was reported by family members and church members to be restless and talking unusually volubly. Relatives noted a series of risky behaviours. On the day of the killings, his grandparents had told him that he could not take the school bus following an episode of aggression toward one of the other children on the bus. Later that evening he attended choir practice with his grandparents, who in response to escalating difficulties had warned him he might have to be returned to his father.\nThe independent forensic report on the case notes CP as saying that that night: “something told me to shoot them”. He had initially reported this to be hallucinations and then said he thought it was his own thoughts. When asked to specifically describe what the experience was like, he said it was “like echoes in my head saying ‘kill, kill’, like someone shouting in a cave”. According to the forensic report, “He reported this began happening after he went to bed…He reported he had never considered harming his grandparents before and this was unlike anything he had previously experienced. He reported that the voices were coming from inside his head and they bothered him so much that he got up. He reported that the voices continued until he killed his grandparents. He reported that he couldn't control himself and reported the echoes stopped after he shot his grandparents. He set fire to the house but could not explain these actions saying the thoughts just popped up”. He then took a vehicle and began driving but reported that he had no idea where he was going and that it all felt like a dream. He recalled asking the police about his grandparents after he was picked up because he was not sure if it had really happened or not.\nThese events and CP's overall behaviour and history led an independent forensic child psychiatrist to diagnose substance-induced mania and psychotic disorder. The charges of double murder and arson were heard by jury trial in an adult rather than juvenile court. In the process of jury selection, 32 of 75 prospective jurors declared that they or someone related to them were on or had been on an antidepressant. Court TV covered the trial in its entirety. Both prosecution and defence from the outset accepted that CP had shot his grandparents. Media coverage focused heavily on the question of “‘Evil’ or ‘chemically compelled’?”\nIn February 2005, after a two-week trial, a jury found CP guilty of murder and he was sentenced to 30 years in prison []. Questioned by the media afterwards, “Steven Platt, a 26-year-old accounting clerk for an electrical supply wholesaler, said the group believed that Christopher exhibited side effects from Zoloft but did not feel it was severe enough to let him escape criminal responsibility” []. Summing up some of the points at issue, the judge Daniel Pieper stated: “There is no case in South Carolina that addresses involuntary intoxication by prescription drugs…It seems to turn the whole medical system on its side if you can't rely on the medication your doctor prescribes. It could potentially force you into a situation of lifetime commitment if that drug induces an effect of which you're not aware… There's something disconcerting about that, albeit probably something of a legal nature that is troubling me” []. The verdict is currently under appeal in the South Carolina Supreme Court.
A 67-year-old man was admitted to our hospital because of liver dysfunction during a screening examination. Enhanced abdominal computed tomography (CT) revealed a hypervascular mass of 35 mm in diameter in the descending portion of the duodenum (Fig. ), and the left three sections of the liver were occupied by multiple cystic tumors with contrast enhancement of the cystic wall, 13 cm in diameter (Fig. ). A duodenal tumor was identified on gastrointestinal endoscopy (Fig. ), and a biopsy revealed a NET. The serum levels of insulin, gastrin, and glucagon were within normal ranges. CT did not initially reveal evidence of pancreatic invasion between the tumor and the pancreas; however, irregularities of the duodenal wall and swelling of the lymph nodes around the pancreatic parenchyma were observed. Thus, the patient was diagnosed with non-functional duodenal NET with multiple liver metastases, T2N1M1 stage IV (UICC 8th). In addition, CT revealed the anatomical variation of the CHA, which branched from the SMA and ran fully through the head of the pancreatic parenchyma (Fig. , Additional file Figure S1). The CHA branches into the left hepatic artery (LHA), the middle hepatic artery (MHA), and the right hepatic artery (RHA) (Fig. a, b). Furthermore, a developed gastric arterial arcade, 4 mm in diameter, was found between the left gastric artery (LGA) and the right gastric artery (RGA). The RGA was branched from a distal portion at a distance of 10 mm from the root of the LHA (Fig. ). Incidentally, we did not observe stenosis of the celiac axis due to compression by the median arcuate ligament. We planned PD and left trisectionectomy with caudate lobectomy combined resection of the tp-CHA with the preservation of the gastric arterial arcade in order to maintain arterial flow of the remnant liver, preserving the route of the celiac artery to the right posterior hepatic artery (RPHA) via the gastric arterial arcade from the LGA to the RGA, LHA, and RHA. If the hepatic arterial flow could not be maintained by this route, the preservation of the tp-CHA by separating from pancreatic parenchyma or arterial reconstruction using radial artery graft between CHA and RHA was planned. Four weeks after percutaneous transhepatic portal embolization, surgery was carried out.\nAfter laparotomy, the gastric arterial arcade was exposed and encircled, and the LHA, RHA, and proper hepatic artery (PHA) were encircled (Fig. ). The LHA was divided at the distal side of the origin of the RGA. The MHA and the right anterior hepatic artery (RAHA) were also divided. The left portal branch and the right anterior portal branch were divided (Fig. ). The liver was transected, and the left hepatic duct and right anterior hepatic duct were divided. The left trisections and caudate lobe were anatomically resected. After clamping the PHA, the hepatic arterial signals of the RPHA via the gastric arterial arcade were confirmed by intraoperative Doppler ultrasonography (Fig. ). After trisectionectomy and caudate lobectomy, PD was performed. The pancreatic head was dissected from the SMA after the upper jejunum was divided. The pancreas was divided in front of the SMV. Finally, the specimen was only connected by the tp-CHA and the common hepatic duct (CHD) (Fig. ). The hepatic arterial signals of the RPHA was maintained after clamping the PHA. The PHA and the origin of CHA were divided, and the tp-CHA was taken out with the pancreatic head (Fig. ). The CHD was divided, and the specimen was removed (Fig. ). Reconstruction was performed via modified Child’s method. The operative time was 1072 min and the intraoperative blood loss was 3052 ml, and red blood cell transfusion was performed (1680 ml).\nPostoperatively, the patient developed pancreatic fistula (Clavien-Dindo IIIa) and biliary leak (Clavien-Dindo IIIa), and these complications were treated conservatively. There were no signs of hepatic ischemia. The patient was discharged on postoperative day 39. The pathological diagnosis was duodenal neuroendocrine tumor G2 with multiple liver metastases. The Ki-67 labeling index was < 20%, and staining for chromogranin A and synaptophysin were positive. There was no evidence of invasion of the pancreatic parenchyma; however, the duodenal tumor was confined to the MP layer, and one of the 25 examined lymph nodes was positive, and moderate lymphovascular invasion was observed. The final diagnosis was pMP, med, INFa, ly1, v2, pPM0, pDM0, and pEM0. The patient has shown no recurrence in the 22 months since the operation. Enhanced abdominal CT at 4 months after surgery revealed the blood flow of the RPHA via the gastric arcade (Fig. ).\nOver the years, several authors have described variations in the hepatic arterial anatomy; a CHA arising from the SMA—called the hepatomesenteric type—is a rare clinical entity. Yang et al. and Hiatt et al. reported that this condition was observed in only 31 of 1324 patients and 15 of 1000 patients, respectively [, ]. A CHA passing through the pancreatic head parenchyma, tp-CHA, is even rarer; Yang et al. [] reported that among 31 patients with the hepatomesenteric type, only 3 had this condition.\nWhen PD is scheduled in such patients with tp-CHA, it is important to maintain the arterial supply to the liver. Surgeons should preoperatively determine whether to preserve or perform combined resection of the tp-CHA. Tp-CHA preservation was selected in several previous reports [, , ]. This surgical procedure is technically feasible; however, there is a risk of a positive surgical margin or insufficient lymph node dissection and a tendency for increased intraoperative blood loss during the separation of the pancreatic parenchyma. If the tp-CHA is resected, reconstruction is usually necessary in order to maintain the hepatic arterial flow. Previous reports [, , ] have described successful arterial reconstruction after CHA resection during PD; however, such procedures are associated with an increased risk of thromboembolism, which can lead to a fatal outcome, especially in HPD []. In contrast, when collateral circulation develops, surgeons can perform combined resection of the tp-CHA, preserving the collateral circulation without arterial reconstruction. Several reports have recommended preoperative embolization of CHA in order to maintain the hepatic arterial flow through enlarged collateral arteries []. Although preoperative embolization can increase the liver arterial flow through collateral arteries, it is not routinely recommended because of the risk of complications, which includes the migration of embolic material [, ].\nA developed gastric arcade or pancreaticoduodenal arcade is frequently seen in patients with the stenosis of the CHA due to factors such as compression by the median arcuate ligament []. There are only a few cases in which the hepatomesenteric trunk and the tp-CHA and the association between the tp-CHA and the development of a gastric arterial arcade have not been reported. On the other hand, Miyamoto et al. reported the case of a patient with pancreatic head cancer with a CHA arising from the SMA who underwent radical PD combined with the resection of the CHA, in which the hepatic arterial flow was maintained via the gastric arterial arcade []. In this report, the patient did not have a developed gastric arterial arcade; however, the hepatic arterial flow via the gastric arterial arcade was sufficient and hepatic ischemia was not detected after the operation. Considering this case, even if the patients with tp-CHA do not have a developed gastric arterial arcade, surgeons may be able to preserve hepatic arterial flow via the gastric arterial arcade alone. If the hepatic arterial flow via the gastric arterial arcade alone is adequate after clamping the PHA, the combined resection of the tp-CHA can be considered, even if the gastric arcade is not developed before surgery. In cases in which the hepatic arterial flow is not adequate, the preservation of the tp-CHA or arterial reconstruction should be considered.\nWhen performing HPD, a PD-first procedure before hepatectomy is generally performed, as this approach is anatomically rational []. However, in the present case, performing hepatectomy after PD carried a risk of the arterial supply to the liver being reduced during hepatectomy. Had we chosen a PD-first procedure and the hepatic arterial flow not been maintained after CHA resection, it would have been necessary to perform arterial reconstruction before liver transection. This method is associated with a risk of injury to the reconstructed artery and thrombosis during liver transection. Given the above, we opted to perform hepatectomy before PD in our patient with a tp-CHA undergoing HPD.\nIn the procedure for separating the tp-CHA from the pancreatic parenchyma entirely, the surgeon should be concerned about the increasing rate of hemorrhage, surgery time, and the risk of injury to the tp-CHA. The surgical reconstruction of the hepatic artery when performing HPD is also associated with a high degree of risk. The association between tp-CHA and gastric arterial arcade was recognized on preoperative CT scans; the development of this collateral circulation may have the potential to prevent ischemia-related liver complications. From these points of view, the preoperative identification of the developed arcade of the gastric arteries helps in planning an appropriate operative procedure, and this procedure seems to be a viable and simple option. To our knowledge, this is the first report of PD combined with resection of a tp-CHA without preoperative embolization. Furthermore, this is also the first report of HPD for a patient with a tp-CHA. The preoperative identification of the developed arcade of the gastric arteries helps in planning the appropriate operative procedure when PD is scheduled for patients with a tp-CHA.
A 66-year-old man with familial dilated cardiomyopathy and a CRT-D device (Resonate X4; Boston Scientific, Marlborough, MA) visited our outpatient clinic owing to transient faintness and palpitations. He had been diagnosed with juvenile sick sinus syndrome and was implanted with a permanent pacemaker at the age of 25. Furthermore, his son had been diagnosed with juvenile dilated cardiomyopathy and died at the age of 15. The pulse rate was 90 beats per minute and systolic blood pressure 102 mm Hg. The 12-lead electrocardiogram (ECG) revealed a regular wide QRS rhythm with a QRS duration of 200 ms and right bundle branch block configuration with a marked left axis deviation in the absence of obvious P waves (A). The device interrogation demonstrated ventricular sensing without CRT pacing at a V-V interval of 700 ms, which was faster than the right atrial (RA) regular rhythm (B). The ventricular rhythm was not diagnosed as a tachycardia by the CRT-D detection algorithm because the detection zone for ventricular tachyarrhythmias for defibrillation or antitachycardia pacing had been programmed to over 150 beats per minute. From those observations, our initial speculation was that it was an accelerated idioventricular rhythm (AIVR) with VA dissociation. Then, we decided to attempt a catheter ablation procedure because the AIVR inhibited effective CRT pacing.\nAt the beginning of the procedure, a 10-polar catheter and a 20-polar catheter were placed into the coronary vein (CV) and on the RA free wall, respectively. A 4-polar catheter was also placed in the right ventricular apex. The intracardiac electrograms revealed different A-A intervals between the right atrium (970 ms) and CV (350 ms) with a V-V interval of 700 ms (A). Overdrive pacing at 320 ms from the distal CV induced a transient AV prolongation with biventricular pacing and then shortened the V-V interval to 640 ms. The RA rhythm was not affected by the CV pacing (B). Our final rhythm diagnoses were a left atrial tachycardia (AT) with simultaneous sinus rhythm observed in the RA. Interatrial dissociation (LA-to-RA conduction block) with 2:1 LA-to-V conduction, right bundle branch block, and marked left axis deviation of the conducted QRS complex were observed. Three-dimensional electroanatomical mapping of the LA during the AT was performed using a Rhythmia® system (Boston Scientific, Marlborough, MN). Activation mapping revealed a reentrant tachycardia pattern with a head-meets-tail-style activation at the center of the LA roof (A) and voltage mapping revealed low-voltage areas around the anterior wall and roof of the LA (B). A radiofrequency ablation catheter was inserted into the LA by the trans–atrial septal approach, and the left AT was successfully eliminated by a single radiofrequency application at a left high posterior LA site where diastolic fragmented potentials were recorded. The CRT pacing was restored immediately after the termination of the left AT. Then, we examined the interatrial conduction by a programmed stimulation method from the LA appendage and tricuspid annulus. The LA-to-RA conduction blocked at a pacing cycle length of 740 ms (C) and 1:1 RA-to-LA conduction was observed up to a pacing cycle length longer than 440 ms; however, Wenckebach-type block occurred at 430 ms. The activation sequence of the coronary sinus was “distal to proximal,” which suggested that the RA-to-LA conduction was dominant through the Bachmann bundle region rather than the mid–atrial septum or coronary sinus region (D). A single extrastimulation with a basic pacing cycle of 700 ms from the high RA resulted in RA-to-LA conduction block at 350 ms, and stimulation from the LA appendage exhibited LA-to-RA block at 440 ms. These findings suggested rate-dependent, bidirectional interatrial conduction block with a greater impaired conduction in the LA-to-RA direction than RA-to-LA direction, resulting in interatrial dissociation only during the LA tachycardia. After the successful ablation of the left AT, regular CRT pacing resumed immediately and was maintained during 20 months of follow-up.
A 53-year-old Sinhalese woman from Chilaw in the North Western Province of Sri Lanka, presented with intermittent high fever with chills for one month and a painful left inguinal mass for two weeks. She had diabetes.\nShe had undergone an incision and drainage of a left deep thigh abscess three months previously and had been treated with a short course of antibiotics. There was no record that abscess material was tested microbiologically.\nOn examination, she was febrile with the temperature of 103°F. She had a tender, fluctuant mass in the left inguinal region. The rest of the examination was unremarkable.\nThere was a raised erythrocyte sedimentation rate (ESR) of 80 mm in the first hour and a very high C-reactive protein (CRP) level of 208 mg/dl. The white cell count was 19 × 109/L with 75% neutrophils. Liver function and renal function were normal. Ultrasound scan revealed a left inguinal abscess. Excision biopsy of the abscess was done and samples were sent for histology, bacterial culture and antibiotic sensitivity testing, microscopy for acid fast bacilli and culture for tuberculosis.\nHistology revealed chronic lymphadenitis with perinodal abscess formation. There were no acid fast bacilli in the direct smear. Initial culture results showed a scanty growth of Pseudomonas sp. and E. coli which was sensitive to ceftazidime and treatment commenced according to the sensitivity pattern.\nHowever, since the Pseudomonas isolate was resistant to gentamicin and the patient had a history of recurrent abscess formation, melioidosis was considered in the differential diagnosis and the bacterial isolates were sent to the Department of Microbiology, Faculty of Medicine, University of Colombo for identification.\nBacterial colonies on blood agar were pin point in size after overnight incubation developing into 1-2 mm, white, umbonate colonies after 48 hours. On MacConkey agar the isolate was salmon pink, which may account for the erroneous conclusion that the “Pseudomonas” isolate was mixed with E.coli. The colonies had the characteristic musty, earthy odour of B. pseudomallei and were slowly oxidase positive. Gram stain appearance revealed the typical safety pin appearance. The isolate was resistant to gentamicin, polymyxin and colisitin and sensitive to co-amoxyclav. Subcultures of the isolate were couriered to a reference laboratory where they were confirmed as B. pseudomallei by polymerase chain reaction (PCR). Serum antibodies to melioidin antigen using an in-house indirect haemagglutination (IHA) test based on that described by Alexander et al. with antigen prepared from local strains of B.pseudomallei[] were positive at a titre of 1:160.\nAfter confirmation of the diagnosis of melioidosis, it was decided to treat her with intravenous ceftazidime for four weeks. There was excellent clinical improvement with normalization of markers of inflammation. Eradication therapy with oral co-trimoxazole and doxycycline was started on the third week, overlapping with the intravenous ceftazidime for one week. The patient tolerated the medications well.\nThe patient was discharged after completion of four weeks of intravenous ceftazidime with a plan to continue oral antibiotics for 12 weeks. However she presented to the outpatient follow up 2 weeks after discharge with an itchy rash which was presumed to be an adverse effect of co-trimoxazole. The rash improved after co-trimoxazole was replaced by co-amoxyclav. The patient was able to tolerate the co-amoxyclav/doxycycline combination and completed 12 weeks of eradication therapy. At the time of this writing she was well without evidence of recurrence or relapse.
A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).\nPostoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.\nTwo months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.\nHe resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma.
A 25-year-old female, who had been using disposable soft contact lens for several years, was assessed for a sensation of pain in her left eye. A sty was diagnosed, and medical therapy with tobramycin eye drops was prescribed.\nAfter 10 days, despite topical antibiotic therapy for the persistence of pain, the patient referred a reduction of visual acuity to 6/15, and because of the appearance of diffuse edema at the epithelial level, Acanthamoeba keratitis was suspected; a swab was taken and analyzed together with her contact lenses for a microbiological examination. Other than therapy with tobramycin drops every 2 h, Polihexanide 0.02% (PHMB) eye drops were also administered (2 drops × 6 v/die). The patient's condition subsequently deteriorated rapidly, and 3 days later, she was hospitalized. On local examination, corneal abscess with a collection of purulent inflammatory exudate of ~1 mm in size was observed (). No view of the lens or fundus was possible, and vision was impaired. Routine blood tests were normal, and the search for Acanthamoeba from the swab and contact lenses was negative. Therapy with vancomycin intravenous 1 g daily and ceftazidime 1 g every 12 h daily was started. Atropine drops 3 per day, levofloxacin and tobramycin drops every 2 h, and PHMB (2 drops × 6 v/die) were also administered. Corneal scraping was performed 4 days later and sent to the Clinical Pathology Service that reported the presence of yeast cells on Gram stain, and that there was a fungal culture in progress. Considering the suspicion of probable Candida infection, vancomycin was changed to topical fluconazole (200 mg/100 ml) 1 drop every hour, and caspofungin intravenous (50 mg/die) was also administered, whereas the PHMB eye drops and tobramycin drops were stopped. After 6 days, the anterior chamber was washed with fluconazole (200 mg/100 ml), and fibrinous tissue was taken and reported as fibrin-leukocyte exudate. After a slight improvement in symptoms, the patient's condition worsened, and after a further anterior chamber washing with fluconazole, a corneal transplant was performed with removal of the membrane that covers the corneal iris angle and the crystalline lens, followed by intraocular lens (IOL) implantation. The patient was discharged from the hospital after 10 days. On the 26th day after the transplant, she was hospitalized again due to a worsening of her clinical condition ().\nAfter 2 days, a new corneal transplant was performed. The explanted corneal flap and the purulent material of the anterior chamber were sent to the Mycology Laboratory of the University Hospital of Catania, Sicily, Italy. For the mycological examination, conventional and molecular diagnostic methods by microscopic, fungal culture in Sabouraud Dextrose Agar (SDA) medium supplemented with 1% chloramphenicol and gentamycin and real-time quantitative PCR (qPCR) assay for the detection of Fusarium and Aspergillus were carried out.\nThe qPCR assay was used for the detection of Fusarium spp. using the primers and probes as described by Koo et al. (), whereas the AsperGenius® multiplex PCR (PathoNostics, Maastricht, Netherlands) was used to detect the most clinically relevant Aspergillus species. DNA was extracted by using the GenoXtract instrument (Hain Lifescience, Nehren, Germany) following the manufacturer's instructions. qPCR was performed adding 5 μl of DNA extract to the PCR mix, and a Rotor-Gene Q (Qiagen) was used for amplification and melting curve analysis. A positive control (Fusarium falciforme ATCC® MYA3636™) and a negative template control (NTC) were included in each PCR run. The AsperGenius® multiplex PCR was performed according to the manufacturer's instructions.\nThe direct microscopic examination with 15% potassium hydroxide (KOH) showed several hyaline septate hyphae. Fusarium spp. was detected by qPCR, whereas Aspergillus DNA was negative. After 4 days of incubation at 30°C, the colonies were clearly visible on SDA medium. The colonies were wooly, cottony, flat, and white. Identification of the isolate was performed by standard phenotypic methods based on the macroscopic and microscopic morphological studies. In particular, the microscopic morphological study showed long monophialidic conidiogenous cells, moderately curved macroconidia and cylindrical to oval microconidia with thick walls, and single-celled to two-celled. The pathogen was identified as F. solani complex. The results of the mycological examinations are shown in .\nMatrix-assisted laser desorption ionization time of flight mass spectrometry (MALDI-TOF MS) on a Microflex LT (Bruker Daltonics, Bremen, Germany) platform after ethanol–formic acid extraction identified the isolate as F. solani (score: 1.646). Susceptibility to fluconazole, itraconazole, voriconazole, posaconazole, caspofungin, anidulafungin, micafungin, flucytosine, and amphotericin B was evaluated. Minimum inhibitory concentration (MIC) values of >256 μg/ml for fluconazole, >16 μg/ml for itraconazole, 0.256 μg/ml for voriconazole, >8 μg/ml for posaconazole, >8 μg/ml for caspofungin, >8 μg/ml for anidulafungin, >8 μg/ml for micafungin, >64 μg/ml for flucytosine, and 1 μg/ml for amphotericin B were obtained. The patient was put on topical voriconazole eye drops every h and voriconazole 200 mg i.v. twice a day, and there was an almost immediate improvement of her eye condition (). The systemic therapy with voriconazole 200 mg per os twice a day was continued for at least 3 months postoperatively.\nAt 3 months, despite the control of her fungal infection, the cornea is opaque due to a fibrinous reaction that also affects the IOL. After controlling the infection, the visual function can probably be restored with a new cornea transplant and replacement of the IOL.
A 66-year-old female patient with a history of bilateral lower limb lymphedema reported the aggravation of the condition over the years, reaching stage III (elephantiasis). The patient was sent to the Godoy Clinic and reported having undergone several treatments throughout her life as well as several episodes of erysipelas. She did not marry due to the lymphedema and complained of the frequent occurrence of strangers staring at her leg, which upset her. The physical examination confirmed elephantiasis. The circumference of the left lower limb was 106 cm. Her body weight was 106 kilograms, height was 160 cm, and the body mass index (BMI) was 41.6 kg/m2 ().\nThe patient was submitted to intensive treatment for three weeks, which led to a 21 kg reduction in weight and 66 cm reduction in leg circumference ().\nIntensive treatment with the Godoy Method consisted of eight hours per day of mechanical lymphatic drainage, 15 minutes of simultaneous cervical lymphatic therapy, and hand-crafted compression stockings made from grosgrain fabric. Mechanical lymphatic therapy consisted of an electromechanical device that performs plantar flexion and extension. After three weeks of intensive therapy, the patient continued treatment at home using the compression stockings. At the follow-up evaluation, the patient was submitted to electrical bioimpedance analysis as well as circumference measurements and volumetry. The bioimpedance analysis revealed a pattern of normality, with the reduction in lymphedema. Ten years after treatment, the patient has maintained the results with the compression stockings. In occupational therapy throughout this period, the patient has been encouraged to perform activities that she has always wanted to do to improve her wellbeing. She took up belly dancing, followed by tap dancing. She reports that these activities changed her life and she is very happy for being able to realize her dream of dancing, which is an activity that she began at the age of 76 years. The study was approving Ethical Committee of Medicine School of Sao Jose do Rio Preto# 2.929.115.
A 21-year old man with history of 8 year palpitation was referred for electrophysiologic study and possible radiofrequency ablation. Physical examination and transthoracic echocardiographic study did not disclose any abnormality. Baseline ECG showed normal sinus rhythm with normal PR and QRS intervals and no evidence of preexcitation. Antiarrhythmic drugs (propranolol and verapamil) were discontinued two days before the procedure. Baseline intervals in sinus rhythm were as follows: sinus cycle length=690 msec, AH=74 msec, HV=37 msec, QRS=90 msec, PR=133 msec. The minimal pacing cycle length maintaining 1:1 antegrade conduction (AVWP) was 320 msec and the minimal pacing cycle length maintaining 1:1 retrograde conduction (VAWP) was 400 msec. Single extrastimulus testing in the right atrium and the right ventricular apex leaded to a sustained narrow complex tachycardia. The arrhythmia was a short PR- long RP tachycardia with following characteristics: cycle length=376 msec, AH=141 msec, HV=42 msec, VA=200 msec, HA (HRA) =236 msec, HA (His) =243 msec and eccentric atrial activation during the arrhythmia (). The arrhythmia was easily reproducible with stable hemodynamic.\nA ventricular extrastimulus delivered at RV septum in the inflow region synchronous with His activation during the arrhythmia neither advanced nor delayed the subsequent atrial activation. Earlier ventricular premature beat at the RV septum terminated the arrhythmia (the His was not refractory) without conduction to the atrium. Right ventricular apical pacing at a cycle length just shorter than the tachycardia cycle length showed the same retrograde atrial activation sequence as during the arrhythmia.\nSubsequently, the right atrioventricular annulus was mapped in the tachycardia and the shortest VA interval was noted at the posterolateral of tricuspid annulus. The radiofrequency current was delivered to this site at the ventricular aspect of tricuspid annulus and resulted in termination of the arrhythmia within 5 seconds. The successful ablation site had a VA interval of 109 msec ().\nFollowing this ablation, programmed atrial and ventricular stimulation failed to induce any arrhythmia and retrograde atrial activity sequence during burst ventricular pacing was concentric. Comparing retrograde atrial activity sequence before ablation with retrograde atrial activity sequence after ablation in the same pacing cycle length showed a significant decrease in VA interval after ablation of the accessory pathway (). What is the mechanism?
A 36-year-old male engineer engaged in parts manufacturing and the decoration industry was referred to our hospital on September 12, 2012. He complained of dry and scaly skin across his entire body, which caused inconvenience in his daily life. He had been experiencing discomfort for 8 years without any obvious itching, burning, pain, or other discomfort. He denied any systemic symptoms, including fever, chills, nausea, vomiting, weight loss, night sweats, and abdominal pain. He denied a history of medical diseases such as diabetes, coronary heart disease, and infectious diseases. His vaccination status was unknown. He also denied any food and drug allergies. The patient reported no poor life habits and denied family history of hereditary disease. He applied moisturizer himself (details unknown) but the symptoms were not alleviated. He had been diagnosed with dermatitis, for which topical urea cream and oral vitamin C, vitamin E, and vitamin A were prescribed. However, his condition did not improve significantly. The patient had received intermittent treatment for 8 years and his condition had not been well controlled. In general, the disease condition was more severe in autumn/winter and milder in spring/summer.\nPhysical examination showed dry skin, diffused distribution of dark erythema, and various small white scales particularly on the neck, chest, and both upper limbs. There was mild erythema on the face and neck with unclear boundaries. The patient had a dry mouth, no sweat, and normal urine and bowel movements. The patient's tongue was red and covered with a thin and greasy coating, and pulse was fine and rapid.\nSince the onset of the disease, the patient had no fever, chills, joint pain, swollen lymph nodes, weight loss, or history of trauma, foreign body contact, and suspicious drug use. The patient conveyed that he had undergone allergen tests, sex hormone tests, and tumor marker tests in the other hospital, but no abnormalities were detected. The patient's clinical symptoms of dry and scaly skin are shown in Figure .\nBased on the clinical manifestations of dry and scaly skin, we could not easily make a clear diagnosis of the disease. The skin lesions of the patient were nonspecific and could be easily confused with diseases such as psoriasis and pityriasis rosea. To make a clear diagnosis, we took a biopsy of the skin from the arm and abdomen during examination, and the results were obtained within a week. Given the character of the skin lesions, we temporarily suspected vice psoriasis and CM diagnosis of “Yifeng Sores.” Tongue coating, pulse, and character of the skin lesions were consistent with blood-heat syndrome. We considered blood-heat and Yang floating as the main pathogenesis in CM theory. The patient was treated with herbal medicine orally for “cooling blood and restraining Yang” twice daily, 30 minutes after meals. Previous research conducted by our team showed that cooling blood and Yang restraining herbs had a positive clinical effect in the treatment of skin diseases such as blood-heat syndrome, eczema, and psoriasis vulgaris.[ In addition, experiments have shown that the drug for cooling blood and retraining Yang may function by upregulating the expression of PD-1 mRNA, PD-L2 mRNA, and their proteins in patients with psoriasis vulgaris, which is characterized by blood-heat syndrome.[ The specific composition, dosage, and medicinal parts of herbal components are shown in Table . In addition to oral herbal medicine, the patient was treated with a ceramide-linoleic acid -containing moisturizer (YuZe Skin Barrier Recovery Body Lotion, produced by Shanghai Jahwa United Company, China); the moisturizer was to be applied on the entire body after showering once daily.[\nThe dryness of the skin was alleviated, scales improved, and sweat was reduced by September 26, 2012. The patient's tongue was red with a thin and greasy coating, and pulse was fine and rapid. Microscopic examination revealed hyperkeratosis, parakeratosis, and mild acanthosis of the epidermis. Many atypical lymphocytes with medium-sized hyperchromatic nuclei were observed in the epidermis. Epidermotropism is characterized by lymphocyte colonization of the basal layer of the epidermis in a linear configuration. The superficial dermal infiltrate was band-like and scant (Fig. . A and B). Immunohistochemical staining showed that the atypical cells were positive for CD45RO, CD3, CD4, CD5, and lithocholic acid, weakly positive for CD7, CD8, and CD30, and negative for CD79a, CD56, CD68, CD20, granzyme-B, Perforin-T, and S-100. Some cells were positive for TIA-1. Ki-67 proliferative index was less 10% within the infiltrate. (Fig. . C, D and Fig. , Supplemental Digital Content (Figure S1)) In addition, gene rearrangement of T-cell receptor (TCR) γ was partially positive for V1J and weakly positive for V2J. TCR β showed V1J, V2J, and DJ were weakly positive, and TCR δ showed VJ, DD, and DJ were negative in clonal rearrangement of TCR genes. In summary, the final diagnosis was MF.\nThe previous misdiagnosis was corrected in time; however, the main composition of cooling blood and restrain Yang herbal medicine remained unchanged. The dose of drugs was adjusted appropriately, and the patient continued the use of the previously-described moisturizer on his skin.\nThe patient was followed up twice, on October 23, 2012 and on November 6, 2012. After taking the herbal medicine and using the moisturizer, the patient's dry skin and scales were alleviated and his sweating was significantly improved. The tongue was still red with the thin and greasy coating and the pulse fine and rapid; hence, the composition of herbal medicine was adjusted according to the clinical symptoms. After taking herbal medicine for 1 year, the symptoms improved greatly. The patient stopped taking oral herbal medicine in September 2013 in accordance with the advice of the doctor.\nAt presentation, the patient showed only dry and scaly skin, and the lesions did not involve internal organs or lymph nodes. Throughout the treatment and follow-up period, the patient showed good adherence and tolerance to the CM therapy. The patient's condition was well controlled and quality of life was improved. The patient has been regularly followed up in the clinic with no complaints of discomfort or adverse events occurring during the treatment. In 5-year follow up, the dry and scaly skin and erythema in neck were notably improved compared to the pre-treatment condition. The patient's skin lesions before and after treatment are shown in Figure . Full timeline of the diagnosis, medical treatment process, physical examination, clinical characteristics, and laboratory findings of the patient are shown in Figure .
A 30-year-old primigravida booked for antenatal care at 9-week gestational age. Her booking parameters were all normal. Pelvic ultrasonography showed a single intrauterine live fetus at 9 weeks and a solitary right lateral wall myoma measuring 17.4 cm × 11.2 cm. Her antenatal period was essentially uneventful. During her visit at 36 weeks, her abdomen was asymmetrically distended with increased prominence of the right half of the abdomen. Obstetric examination revealed a live fetus in oblique lie and breech presentation. Obstetric scan done confirmed a live fetus in breech presentation and a large myoma and tortuous dilated vessels on the lower myometrium on Doppler flow study.\nShe was counselled and scheduled for an elective caesarean section at 38-week gestation due to breech presentation in a primigravida with coexisting leiomyoma. Her preoperative investigations were normal.\nAt surgery, she was diagnosed with uterine torsion (120-degree dextrorotation) due to the right position of the left round ligament, fallopian tube and ovary. The torsion was corrected and a huge (about 40 cm × 40 cm) myoma was observed to occupy the entire anterior wall of the gravid uterus (). There was no obvious uterine anomaly and the fallopian tubes and ovaries were grossly normal. The spinal anaesthesia was converted to a general anaesthesia with endotracheal intubation. The subumbilical skin incision was extended cephalad, and the gravid uterus and leiomyoma were exteriorized before a deliberate midline vertical incision was made on the posterior wall of the uterus and a healthy male baby was delivered by breech extraction. After delivery of the baby, she was given intravenous carbetocin 100 μg and tranexamic acid 1 g. The uterine incision was repaired in layers using 1–0 polyglactin suture and haemostasis was achieved as shown in . The anterior abdominal wall incision was closed in layers with Nylon 2 suture to the rectus sheath. Her post-operative vital signs remained satisfactory, and she was discharged of fourth post-operative day.
The patient filled in an informed consent for participating to the study. The study presents the case of a 43-year-old female patient, who was first hospitalized in 2011 in the Cardiology Clinic of Emergency County Hospital of Craiova for palpitations. We noted a positive familial history for restrictive cardiomyopathy in her mother’s sister, deceased at the age of 60 years old, who was diagnosed only by echocardiography. However, there were no genetic tests performed in her case. Moreover, our patient related a history of cardiovascular disease in her mother and maternal grandfather. However, they are no longer alive, and we have no medical documents concerning their pathology. Furthermore, we decided to perform echocardiography to patient’s 20-years-old asymptomatic son and there were no pathological changes found. Even though we recommended genetic testing in his case, it has not been performed yet. She could not mention any other medical history. The ECG revealed atrial fibrillation, with QRS axis at 90 degrees, Q wave in DIII, negative T wave in DI, DII, DIII, aVF, V1-V6. Echocardiography revealed normal dimensions of aortic root and ascending aorta, normal diameter of left ventricle, left ventricular volume slightly reduced, normal LVEF (55%), mild mitral regurgitation, severe left atrial enlargement, normal right ventricular diameter and slight right ventricular longitudinal systolic dysfunction, severe right atrial enlargement, mild tricuspid regurgitation, moderate pulmonary hypertension (). We started oral anticoagulation and we decided to attempt electric conversion to sinus rhythm, which was successful. At that point, the diagnosis of restrictive cardiomyopathy has already been put in discussion and the next investigation that we recommended was a cardiac MRI, which showed reduced left ventricular volume, normal systolic function (LVEF=62%), normal left ventricular wall thickness, without any pathological changes of regional contractility. Certain trabeculations were described in the left ventricle, without meeting the criteria for non-compaction cardiomyopathy. Moreover, we did not identify abnormal
The proband is a 19-year-old male of Northern European extraction, the first child of non-consanguineous parents. Born full term at 3550 g, he was noted to have a two-vessel cord at birth and a murmur due to a bicuspid aortic valve. CT scan showed an absent inferior vena cava and a continuous dominant hemiazygos vein that drained into the azygos vein at the level of the T9 vertebral body. The azygos vein drained the right kidney. At his most recent cardiac evaluation at 18 years of age, he had stable dilation of his aorta without significant aortic stenosis or insufficiency. He has also had two episodes of symptomatic pericarditis.\nA small area of hypertrichosis and hyperpigmentation in the lumbar area of his back was noted as an infant. This patch increased in size and extended to his anterior thighs and shins as he grew older.\nHe was noted to have localized swelling in his scrotum during his first year of life. Over time, the scrotum and mons pubis became indurated and tense. Ultrasound at age 17 months revealed normal appearing testes with echogenic material filling the scrotum and lining the wall, particularly the right epididymis. The findings were felt to be consistent with calcified meconium from meconium peritonitis. The scrotal mass increased in size until the whole scrotum was noticeably distended and by 4 years of age, the swelling also involved the suprapubic area. CT of his pelvis showed diffuse increased attenuation involving the subcutaneous fat from the level of the iliac crests to the upper thighs. An MRI at age 6 revealed bilateral inguinal lymphadenopathy.\nA deep skin punch biopsy from patient 2 showed mild epidermal hyperplasia with relatively mild basal hyperpigmentation and a striking infiltrate in the deep dermis and subcutis accompanied by edema and rare hemosiderin deposition. Cellular aggregates seen at low power (Fig. ) included a predominance of plasma cells with prominent perivascular cuffing (Fig. ), in addition to other mononuclear cells. Immunohistochemical stains for kappa and lambda showed a polyclonal phenotype, although with inversion of the expected kappa:lambda ratio (not shown). The plasma cells did not show strong immunoreactivity for IgG4 (not shown). Immunohistochemical staining for histiocytic markers showed diffuse staining of cells for CD68 and CD163, which were often present between the plasmacytic aggregates (Fig. ). However, these histiocytes were not markedly enlarged, did not show significant emperipolesis, and were not highlighted by immunohistochemical staining for S-100 protein (Fig. ).\nThe patient has always been short for his age. At 4 months old, his height was at the 10-20th percentile, which fell to <5th percentile by 9 months and to <3rd percentile by 5 years, at which time he was diagnosed with growth hormone deficiency. He had mildly delayed bone age throughout childhood and adolescence with no pituitary mass seen on imaging. A DEXA scan at 9 years of age showed normal bone mineral density.\nHe had a normal hearing screening at birth but developed significant hearing loss by 14 months of age and eventually had bilateral cochlear implants placed. He had enlargement of the lateral and third ventricles as well as the bifrontal and bitemporal extra-axial fluid spaces on brain MRI at 18 months of age consistent with cerebral volume loss. Arcus senilis was noted at 4 years of age. Two years later, he was described by a dysmorphologist to have a prominent forehead with a normal nasal root (OFC 50th percentile), mild hypertelorism and ptosis, prominence to his eyes, and a cowlick with a whorl on the right side of his forehead. External ears were slightly posteriorly rotated, but otherwise normal. At age 7, his hearing worsened and by a year later, he had the first of his now bilateral cochlear implants.\nHe had episodes of abdominal pain and loose stools throughout childhood. Upper endoscopy and colonoscopy performed at age 11 showed partial lactase deficiency in his duodenal mucosa and lymphoid hyperplasia throughout the colon with a normal terminal ileum biopsy. CT enteroclysis of the abdomen and pelvis at age 15 was unremarkable.\nAt age 15, he developed pain and stiffness in multiple joints, especially ankles and knees. He had proximal interphalangeal joint contractures in his 5th digits bilaterally and loss of range of motion in both ankles and knees. Left hand films showed normal mineralization and no erosions or joint space narrowing.
A 67-year-old woman with a 1-month history of epigastric pain was referred to our hospital with suspected pancreatic head cancer. Endoscopic ultrasonography and fine-needle biopsy of the tumor revealed adenocarcinoma of the pancreatic head. Computed tomography (CT) showed a 17 × 12 mm hypovascular tumor without arterial or portal venous invasion in the pancreatic head, and the CHA formed a common trunk with the LGA, coursing within the lesser omentum (Fig. ). In addition, preoperative three-dimensional CT angiography revealed that the celiac artery branched into the splenic artery and the common trunk, and the usual CHA was absent in the supra-pancreatic area. The CHA formed a common trunk with the LGA and arched in a cranial direction within the lesser omentum immediately adjacent to the liver. No other arterial supply to the liver was identified (Fig. ). With the preoperative diagnosis of resectable pancreatic head cancer and clinical staging of T2N0M0 (UICC 8th edition), pancreatoduodenectomy was planned.\nIntraoperatively, the CHA was identified in the lesser omentum, forming a common trunk with the LGA as shown on preoperative CT. As the first step of lymph node dissection in the hepatoduodenal ligament, the gastroduodenal artery was identified and taped carefully in the supra-pancreatic area. Second, the gastroduodenal artery was skeletonized cranially, with taping of the right anterior hepatic artery, the right posterior hepatic artery, the left hepatic artery, and the CHA in turn. Third, after the anterior surface of the portal vein was exposed in the supra-pancreatic area in the absence of the CHA, the tissue and lymph nodes along the portal vein were completely skeletonized toward the hepatic hilum. Before dividing the vessel, we clamped the gastroduodenal artery and confirmed blood flow in the intrahepatic arteries using ultrasonography. During skeletonization, we were careful to avoid vascular injury to both the portal vein and common trunk coursing within the lesser omentum. Finally, the common bile duct was divided at the level of the junction of the cystic duct, and pericholedochal tissue and the lymph nodes of the bile duct were dissected. No arterial vestiges of the usual CHA were found in the supra-pancreatic area (Fig. ). The pancreatoduodenectomy procedure including lymph node dissection was completed as usual, without injury to any hepatic arteries (Fig. ).\nThe postoperative course was largely uneventful except for mild delayed gastric emptying. The patient was discharged on postoperative day 19. The microscopic findings revealed invasive ductal carcinoma of the pancreas with lymph node metastasis. The patient received adjuvant chemotherapy and developed recurrence of lymph nodes.
A 32-year-old man injured his dominant right hand by striking a punching bag machine with a clenched fist. Clinical examination revealed that his right hand was markedly deformed and swollen. The range of motion was limited due to pain. He had small bulla formation on the dorsal aspect of the hand. There was no neurovascular deficit.\nPlain radiography and computed tomography revealed dorsal dislocations of the index, middle, ring, and little fingers at the CMC joints and simultaneous fractures of the dorsal aspect of the trapezoid and hamate []. An initial attempt of closed reduction under conscious sedation in the emergency room was unsuccessful.\nThe surgery was performed through the dorsal aspect of carpus in a zigzag fashion from proximal part of the wrist to the middle part of the metacarpal bone. An interposed dorsal joint capsule in the CMC joints was found which might prevent the initial closed reduction of these joints. After removing the interposed joint capsule, CMC joint reduction was easily achieved under fluoroscopy.\nHowever, we could not achieve stable reduction without fixation. The Kirschner wire fixation was performed while the CMC joints reduced. Under fluoroscopic vision, the first Kirschner wire was inserted retrograde from the base of the fifth metacarpal bone to the hamate. The second Kirschner wire was inserted antegrade from the hamate to the fourth metacarpal bone. The third Kirschner wire was inserted antegrade from the trapezoid toward the second metacarpal bone. The fourth Kirschner wire was inserted retrograde from the third metacarpal bone to the capitate. Finally, dorsal fractures of the trapezoid and hamate bone were reduced and each fracture was fixed with a mini screw (1.5-mm cortical screw, Synthes, West Chester, PA, USA).\nAfter bony fixation, the ruptured dorsal capsule and ligaments of the CMC joint were repaired with absorbable sutures. Postoperatively, the patient remained in a volar above elbow splint in the neutral position for 2 weeks, followed by a below elbow cast for 4 weeks. The Kirschner wires were removed 6 weeks after the operation. Gentle passive range of motion exercise and isometric grip-strengthening exercise were started immediately after the Kirschner wire removal.\nThe patient had full range of motion in the fingers and wrist with minimal pain 3 months postoperatively. Grip strength (81% of unaffected hand) and pinch strength (85% of unaffected hand) were slightly lower than that of the unaffected hand. He returned to sport activities 6 months postoperatively. One year after the operation, plain radiography showed a well-preserved “tram-line” and no evidence of posttraumatic arthritic changes. The patient and his family were informed that data from the case would be submitted for publication and they gave their consent.
An 8-month old female child with motor delay presented with a history of left-sided weakness following a fall from the walker at the ground level. There were no other signs of traumatic brain injury such as loss of consciousness, vomiting, convulsions etc. She was first born to a non consanguineously married couple with normal birth history. However, she had attained neck control at 5 months of age and was still not sitting with support and cognitive as well as language milestones were normal. At admission, she was conscious, oriented to mother and surroundings, had normal vitals and anthropometry with no external injury. She had a paucity of movements of left upper and lower limbs with variable tone and brisk deep tendon reflexes on the same side. She had intermittent dystonic posturing of the left lower limb. There was no cranial nerve palsy. There were no neurocutaneous markers and fundus was normal. A workup of pediatric stroke was conducted. Her complete hemogram, bleeding time, PT, Aptt, INR, serum calcium, serum homocysteine, protein C, protein S, antithrombin III, HB electrophoresis, paratharmone, lipid profile, ABG, lactate, ammonia, plasma tandem mass spectroscopy and urine gas chromatography and mass spectroscopy echocardiogram were normal and HIV, TORCH titres, lupus anticoagulant were negative. MRI of the brain [] showed calcification of right basal ganglia and no evidence of infarct and MR angiogram of the brain was normal. CT of the brain [] showed multiple linear calcifications in bilateral basal ganglia predominantly involving caudate nuclei. This was a rare case of mineralizing angiopathy with developmental delay and basal ganglia stroke. The child showed improvement in her movements during her hospital stay. She was discharged with physiotherapy and is being followed up in our out patient department. Presently, she is using both upper limbs equally, but the paucity of movement in the left lower limb is still present.
This patient is a 43-year-old woman with an underlying history of right SWM with intraorbital extension, first diagnosed in 2014. She underwent a right craniotomy and tumour excision (Simpson II) in the same year but repeated imaging showed residual tumour over right sphenoid and orbital wall. Initial histopathological findings confirmed the diagnosis of meningothelial meningioma WHO Grade 1. Due to tumour progression, she had a severe right eye (RE) proptosis and a right orbital decompression was done by the Neurosurgical team in 2015. Subsequently, in 2016 her tumour continued to progress with involvement of right posterior ethmoid and sphenoid sinuses and she underwent radiotherapy. She was referred for eye assessment and noted to have RE proptosis, restricted extraocular movement and vision was perception to light. She subsequently defaulted follow-up. The surgical team continued to monitor her tumour progression with a six-monthly imaging until 2019 when her MRI brain showed evidence of increased size of residual mass with new extension into right temporal region, as well as another new right temporal mass. A third surgery involving craniectomy and debulking was done. Two months later, she was treated with radiotherapy to a total dose of 60Gy given in 30 fractions (Figure ).\nThree weeks after completing radiotherapy she complained of worsening of RE proptosis for one-week duration, associated with pain with a score of 3/10. She had no fever or any other symptoms. On examination, her RE vision was non-perception to light (NPL), positive relative afferent pupillary defect and no ocular motility. She had corneal epithelial defect and lagophthalmos due to proptosis, conjunctival hyperemia and chemosis. Her fundus examination showed a pale disc but not swollen. Left eye (LE) vision was 6/7.5 and ocular findings were unremarkable. Neurological examination and review of general systems showed normal findings. She was diagnosed with exposure keratopathy and managed conservatively with close monitoring of her proptosis. A week later, the patient returned with fever and rapid worsening of RE proptosis, redness and pain, as can be seen in Figure .\nAn urgent CT orbit and brain was done (Figure -) and there was evidence of increasing size of the residual mass along with two hypodense regions with rim enhancement within the right orbital extended mass. There was also thickening of the right optic nerve and increasing degree of intraorbital fat stranding suggesting possible infection and/or tumour lysis syndrome. Baseline blood investigations were performed and full blood count showed leucocytosis with neutrophilia. Blood culture however reported the absence of microorganisms. The patient also refused surgical biopsy which was offered to her.\nShe was treated as right orbital cellulitis and was immediately commenced on intravenous (IV) metronidazole 500mg TDS and IV ceftriaxone 2 grams OD. Two days later, her right eye proptosis and other symptoms were markedly reduced. IV dexamethasone 8mg TDS was commenced on the third day following improvement of symptoms and completed for three days. The IV antibiotics therapy was continued for a total of two weeks. Final review on day 14 showed remarkable improvement. Patient was able to fully close her right eye and pain-free. Patient was subsequently discharged however her RE vision remained NPL.
A 38-year-old Caucasian woman, 35 weeks into her first pregnancy, presented to the emergency department for acute right-sided hip pain which precluded weight-bearing. Her right leg was shortened and externally rotated - there was no bruising or evidence of trauma.\nThe patient’s history was significant for hereditary thrombophilia (Factor V Leiden) and secondary anemia. Hip radiography revealed an unstable, displaced, right-sided femoral neck fracture with no evidence of osteonecrosis (Figure ). The decision to administer radiography, in this case, was based on the American College of Radiology guidelines, which cite an absence of in-utero deterministic effects of ionizing radiation effects after 27 weeks of gestation. Unfortunately, it was not possible to evaluate the symptoms of the patient with MRI at this time due to the coronavirus disease pandemic-induced stress on the healthcare system of our country.\nThe patient denied falls or trauma during the pregnancy, nor was there any history of smoking, alcohol abuse, use of glucocorticoids, or presence of rheumatologic/oncologic disease. Additionally, the patient was not malnourished, she underwent routine antenatal care, and took multivitamins. Serologic tests for inflammatory markers, as lab tests for serum calcium, phosphate, alkaline phosphatase, parathyroid hormone, vitamin D, and D-dimer returned normal.\nDuring multidisciplinary rounds, it was decided that delaying surgery was the best course of action out of fear of causing either mechanical or fluoroscopy-induced damage to the fetus during total hip arthroplasty. Five days later the patient experienced premature rupture of membranes, which was managed with emergency cesarean section (C-section) - no complications were encountered and a healthy 2300 g female was successfully delivered. Three days later the patient was transferred to our orthopedic surgery department for the treatment of the fracture. The significant degree of displacement (grade IV) of the fracture lasting over one week precluded open reduction with internal fixation due to fears of femoral head necrosis. During our literature review, we encountered a similar case of femoral neck fracture with grade IV displacement that was treated with open reduction internal fixation - despite restoration of blood flow to the femoral head within 15 hours, the authors still encountered femoral head necrosis with collapse six months later []. Given the considerable delay between symptom presentation and treatment, we decided the case warrants total hip arthroplasty instead of native hip salvage. Hemiarthroplasty was considered but was ultimately discarded as the conversion rate to total hip arthroplasty in young patients remain relatively high and the fracture was subsequently treated with a total uncemented prosthesis (Figure ), consisting of a 50 mm cup with 32 mm ultra-high-molecular-weight polyethylene insert and a 32 mm head with a 4 mm ceramic insert (Link Inc., Hamburg, Germany). Postoperative radiography confirmed prosthesis placement (Figure ); antibiotic and anticoagulant prophylaxis was initiated with ampicillin/sulbactam and enoxaparin sodium, respectively.\nThree days after the intervention the patient developed moderate abdominal pain without fever and accelerated intestinal transit, which both worsened over the next four days. Given the clinical presentation, there was a high index of suspicion for infection with Clostridium difficile (C. diff), although the diagnosis was ambiguous as the enzyme immunoassay (EIA) for the C. diff-specific antigen glutamate dehydrogenase was positive, while EIA for exotoxin A and B were negative. Due to exacerbation of symptoms and development of moderate hypokalemia (2.9 mEq/L), empiric treatment with metronidazole was began and marked rapid improvement. The patient was discharged five days later.
A 55kg, 11-year-old male underwent a right craniotomy and frontal lobectomy for resection of a seizure focus identified by prior intraoperative subdural EEG electrode grid application four days prior. The patient’s previous history was significant for pansinusitis complicated by right holohemispheric subdural empyema and thrombosis of the anterior third of the superior sagittal sinus in January 2019. This was treated with antibiotics and a right frontal craniotomy. In April of 2020, he developed seizures that were fulminant in presentation and rapidly became intractable. Seizure semiology included poor attention at school, body stiffening episodes, and periods of “spacing out.” The increase in seizure frequency had resulted in worsening academic performance (A to C student), blunting of effect, inability to continue with soccer, and impaired peer relationships. He was scheduled for right frontal lobe resection for focal, lesional epilepsy.\nOn the morning of surgery, he received his scheduled valproic acid and levetiracetam but experienced breakthrough seizure activity immediately prior to surgery. Therefore, he was transferred from the Pediatric Intensive Care Unit to the operative suite urgently. The patient was induced with rocuronium and propofol for endotracheal intubation. Maintenance included total intravenous anesthesia (TIVA) with propofol and remifentanil infusions in order to accommodate the need for intraoperative neuromonitoring. The case proceeded uneventfully for 12 hours, and approximately one hour prior to the conclusion of surgery, the propofol and remifentanil infusions were discontinued. The total propofol dose throughout the case was approximately 2500 mg. Sevoflurane was initiated at 0.5 MAC in order to expedite emergence. Of note, these actions correlated with the conclusion of neuromonitoring and the beginning of dural closure. During the closure, the patient exhibited gross signs of full-body rigors. In the absence of neuromonitoring, seizure activity could not be confirmed or refuted by that diagnostic modality. At the request of the neurosurgeon, 2 mg of midazolam and 500 mg levetiracetam were given. Despite the cessation of all anesthetics for almost one hour, the patient failed to exhibit spontaneous respiratory effort or response to oral and tracheal suctioning. It also appeared that the patient had a downward gaze of his eyes, but pupillary reflexes were intact. He was brought directly from the operating room to CT to identify a possible post-surgical cause for his delayed emergence.\nCT revealed left to right midline shift into the surgical bed with diffuse loss of grey-white differentiation thought to reflect cerebral and cerebellar edema. The surgeon performed a bi-frontal craniotomy for reexploration based on these findings, which did not reveal a definitive cause. After the surgery, the skull fragment was not replaced in order to accommodate for swelling. The patient’s neurologist was consulted in the OR, and a loading dose of 1000 mg of intravenous fosphenytoin was recommended and administered. The patient remained hemodynamically stable throughout both anesthetics. The patient was transferred to the PICU with plans to maintain deep sedation, ICP monitoring, and continued aggressive seizure prophylaxis for at least 48 hours or until brain edema decreased. Results of an MRI without contrast obtained later that evening included “extensive cerebral and cerebellar edema without evidence for cytotoxic edema. The possibility of toxic or metabolic etiology is favored, florid posterior reversible encephalopathy syndrome (PRES) could also be considered”. The patient had an uneventful ICU course; no observed seizure activity, continuous negative EEG, normal neurologic exams, and was extubated on a postoperative day four after sedation with fentanyl and midazolam infusions weaned, and extubation criteria met. Upon discharge, a non-focal neurologic exam was elicited. The patient exhibited no neurologic sequelae at subsequent outpatient follow-up visits with his neurologist with a significant improvement from his baseline symptoms and was free to resume all activities.
A 19-year-old woman complained of pain in the plantar aspect of the foot for eighteen months. The pain was moderate and tolerable in the initial stage, and often worse at night, and even much worse with compression and low temperature. The patient visited the department of radiology due to aggravation of the local pain for the last four months. Physical examination revealed three prominent soft tissue nodules with swelling and tenderness, in the plantar aspect of the left foot, but without adhesion to the surrounding tissue. Neurological examination showed numbness in the medial aspect of the distal plantar aspect of the left foot. The movement and strength of both toes and foot were normal. The patient denied any history of trauma. The family history of neurofibromatosis type 2 (NF-2) was also negative.The MRI examination was performed to characterize the nodules detected during physical examination. On MR imaging, a linear nodular distribution of the lesions, located in the mid part of the plantar aspect of the left foot was observed (Figures and ). The lesions were homogeneously isointense to skeletal muscle on T1-weighted images (Figure A) and hyperintense on T2-weighted fat-suppressed images (Figures B, and ). No evident cystic degeneration was observed on all the MR images. The rim of the lesion appeared as hyperintensity on T2-weighted fat-suppressed images (Figure A). A small extension of high signal arising from the lateral aspect of the lesion seemed like a mouse-tail (Figure B). Two of three nodules were located in the flexor digitorum brevis below the plantar aponeurosis (Figures A and ) and one nodule was in the superficial fascia of the third metatarsophalangeal joint (Figure B). These nodules were not scattered and they had a peculiar linear distribution originating from the branches of the common plantar digital nerves. No invasion of the surrounding tissue was observed. Based on the clinical and imaging findings, they were initially thought to be glomus tumor, neurofibroma or schwannoma. Multiple PSs were suspected and surgical excision was recommended.During the operation, there were three round nodules located in the subcutaneous tissue, between the skin and deep fascia, with the sizes of 0.5 × 0.5 × 0.8 cm, 0.7 × 1.0 × 1.2 cm and 1.0 × 1.1 × 1.5 cm, respectively. The nodules were elastic and movable. These nodules were not well demarcated from surrounding peripheral nerve tissue. Intraoperative frozen sections of the nodules were performed and the examination indicated PS. Therefore excision of the lesions was performed. Histopathological examination with the use of the hematoxylin-eosin staining demonstrated that the specimen was composed of uniform spindle cells. Characteristic cellular Antoni-A areas with nuclear palisading and Verocay bodies were observed (Figure A). The tumor cells stained positive for S-100 protein and exhibited strong immunoreactivity for S-100 protein (Figure B). The pathological findings give the diagnosis of PS in the plantar aspect of the foot.\nAfter operation, the patient reported hypoesthesia in the distant third and fourth toes. The patient was followed up for two and a half years postoperatively, and there was no evidence of local recurrence or new lesions at other parts of the body.
A 17-year-old male (weight 62 kg) was admitted in our referral poisoning emergency hospital because of acute organophosphate (OP) poisoning. He had ingested about 50 mL of poison in a suicidal attempt. The patient had nausea and vomiting 10 min after ingestion and his brother brought him to the hospital. At the time of hospitalization, he had a low level of consciousness. He had no history of previous suicide, psychological problems, hypertension, diabetes mellitus, and cardiac or respiratory disease. In the patient's family, his mother had diabetes mellitus, and his father had a history of benign prostatic hypertrophy. There was no history of any noteworthy disease in patient's siblings. There was also no identified genetic disease in the patient's family. On admission, his vital signs revealed a pulse rate of 137/min, blood pressure of 110/70 mmHg, respiratory rate of 18/min, afebrile, and O2 saturation of 93%. Neurological examination revealed Glasgow Coma Scale of 12/15. He had cold and wet skin, sialorrhea, and myotic pupils. He had poison odor in his breath. Auscultation of the lungs was normal. Gastric evacuation and administration of activated charcoal via nasogastric tube were performed. Laboratory studies include complete blood count, blood urea nitrogen and creatinine, liver enzymes, blood sugar, coagulation tests, venous blood gas, electrocardiogram, and chest X-ray, which were normal on admission. Fluid therapy was started, and because of central nervous system depression and muscarinic manifestations, atropine therapy was administered. After intravenous (IV) injection of 2 mg atropine, cutaneous signs of hypersensitivity to atropine were observed on his body. The patient had erythema and urticaria with temperature elevation. He did not have pulse rate elevation, hypotension, or respiratory distress. Atropine injection was stopped at once and IV hydrocortisone 100 mg and IV ranitidine 50 mg and chlorpheniramine 10 mg were given. The general condition of the patient was improved. Oral cetirizine was also administered to him after he was conscious. To improve the clinical manifestations of OP poisoning, pralidoxime infusion was started for the patient. After 2 days of hospitalization, he discharged from the hospital with stable and good condition.
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.
A 30-year-old-male patient was admitted to another hospital complaining of sudden fierce headache and severe vomiting without antecedent trauma. The brain MRI was performed due to the suspicion of acute stroke. On his cerebral MRI, neither acute ischemic nor hemorrhagic stroke was detected. However, a large aneurysm of the basilar tip artery was revealed with the largest diameter of 22mm. The aneurysm exerted a critical mass effect on the pons (). He was transferred to our hospital for further evaluation on the same day.\nInitial neurological examination on admission noted that he was conscious and without neurological deficit. His medical history was unremarkable. Subsequent diagnostic cerebral angiogram confirmed the present of a fusio-sacciform dissecting aneurysm in the distal segment of the basilar artery. The aneurysm had partial thrombosis, measuring 13 mm in width and 15mm in height, with an undefined neck. Its location in the trunk was just above the pontine perforators. In particular, the dissecting part involved in both P1 segments of posterior cerebral artery (PCA), causing severe stenosis of right P1 and occlusion of left P1. Regarding to the collateral circulation, the bilateral PCA was supplied by bilateral posterior communicating artery (PComA). The diameter of right and left PComA were 2mm and 2.5mm respectively ().\nAfter taking the clinical symptoms, the aneurysm characteristics, and patient’s age into account, treatment of the aneurysm by endovascular approach was considered appropriate in order to prevent rupture and to release the mass effect on the brainstem. This proposal was accepted by the patient and his family.\nThe procedure was performed under general anesthesia via bilateral femoral artery. Bilateral vertebral artery was selected with Chaperon 6F guiding catheter (MicroVention, Aliso Viejo, California, USA). Angiography confirmed the presence of unchanged dissecting aneurysm in the distal segment of the basilar artery. Working projections were selected to show the aneurysm sac, the proximal part of the dissecting lesion, and the origin of the pontine perforators. Via the left VA, a PX Slim microcatheter (Penumbra, Alameda, California, USA) with half round loop was placed along the posterior wall of the sac. The proximal part of the aneurysm was selected by a Headway 17 microcatheter (Microvention, Aliso Viejo, California, USA) via the right VA. Through the PX Slim microcatheter, six Ruby conventional 18 coils (Penumbra, Alameda, California, USA) were deployed into the aneurysm sac. Then, eleven Hydrocoils (Microvention, Aliso Viejo, California, USA) were placed in the proximal part of the aneurysm via the Headway microcatheter. The angiographic control was performed to evaluate the occlusion of the aneurysm. The final DSA showed the disappearance of blood flow in left VA upper of the PICA, the normal blood flow in right VA, and a significant reduction of blood flow in the aneurysm (). Because the aneurysm was unruptured, we decided to not occlude completely and to stop the coiling. The procedure was completed with the compression of bilateral femoral artery. Anticoagulant therapy with 3000 IU (50 IU/kg) unfractionated heparin was administered with an IV bolus after the first coil deployed.\nThe patient recovered post-procedure without any neurological symptoms or femoral access site complications. An emergent non-enhanced CT scan of brain was performed at night of the intervention day due to his worsening headache symptoms. Neither ischemic nor hemorrhagic stroke was noted (). The brain MRI on the next day showed an edema in the pons without hyperintensity on DWI. The aneurysm sac was occlusion. The solumedrol 40 mg was administered for the next 5 days. The dual antiplatelet therapy with clopidogrel 75 mg and aspirin 81 mg per day was given for 3 months post intervention. The patient was discharged after 7 days without any neurological deficit.\nThe first angiographic follow-up after 3 months of the intervention revealed a complete occlusion of basilar tip as well as the aneurysm sac with a hypoplasia of the basilar trunk. The aspirin 81 mg per day was maintained up to 1 year. The DSA follow-up after 6 months showed a stable occlusion of the aneurysm with a minor recurrence in the left remnant P1 segment. According to the size of recurrent aneurysm and the difficult anatomy, we decided to continue follow up. The most recent routine clinical follow-up after treatment revealed that the patient was still clinical intact (mRS 0).
An 18-year-old male presented to our institution in 2014 with headaches and vomiting for two weeks. A magnetic resonance image (MRI) of his brain showed a complex extra-axial dumbbell-shaped lesion with the epicenter in the left Meckel’s cave, extending anteriorly to the cavernous sinus and the cerebellopontine (CP) angle posteriorly with mass effect over the brainstem. He underwent a left retromastoid craniectomy and decompression of left CP angle lesion elsewhere. The histopathology was suggestive of malignant melanoma, and he was referred to our institution for further management.\nHis general physical examination revealed a deep gray-blue nevus over the left upper eyelid, extending to the frontal and temporal region. He had dysarthria, left upper motor neuron facial nerve palsy, and right hemiparesis with Grade 4 power in his right upper and lower limbs. The biopsy of the nevus over his left eyelid was reported as superficial, and the deep dermal dendritic melanocytosis with histological features was suggestive of a blue nevus.\nThe MRI of his brain showed a large contrast-enhanced extra-axial mass with solid and cystic components measuring 4 cm x 2 cm x 3 cm in the left cavernous sinus extending through the Meckel’s cave into the posterior fossa (Figure ). A whole-body positron emission tomography-computed tomography (PET-CT) scan confirmed no extracranial disease. He underwent a left temporal craniotomy and zygomatic osteotomy, and we took an interdural middle cranial fossa approach for the radical excision of the tumor.\nPerioperatively, we noted the blue nevus on the left side of the forehead in the ophthalmic distribution of the trigeminal nerve. The pigmentation extended into the subcutaneous tissue and galea. The diploe of the temporal bone was also pigmented. The entire temporal dural convexity was pigmented completely black as were the dural root sleeves of the trigeminal nerve and the lateral and medial walls of the cavernous sinus. The tumor was localized in the cavernous sinus and had a well-defined capsule surrounding the divisions of the fifth cranial nerve. It extended into the posterior fossa through Meckel’s cave. The tumor was completely removed via the cavernous sinus through an interdural approach.\nThe surgical specimen revealed a tumor composed of sheets of moderately large polygonal cells with markedly pleomorphic nuclei with evidence of mitotic activity with foci of necrosis, and occasional cells with intracytoplasmic melanin (Figure ). We also saw small segments of nerve containing ganglion cells with perineural deposits of melanin. The tumor cells showed diffuse positivity for S100 protein and Melan A. Occasional cells were positive for human melanin black (HMB)-45. The ki67/mib-1 was 20% to 25%.\nGiven the coexistent skin lesion reported as a blue nevus, neurocutaneous melanoma was considered as the provisional diagnosis. The postoperative hyperacute MRI showed no residual tumor. His symptoms gradually resolved after surgery. He received postoperative intensity modulated radiotherapy to the tumor bed (4950 cGy in 22 fractions to the planning target volume with a biologically effective dose [BED] of 60.39 Gy and an equivalent dose in 2 Gy fractions [EQD2] of 50.32). He was closely monitored during the follow-up period.\nHe presented again about 20 months later with recurrent symptoms of headaches for two months and diplopia and vomiting for two days. On examination, sensations over the V1, V2, and V3 dermatomes were reduced on the left side. There was masseter and temporalis muscle wasting on the left side with impaired blinking in both eyes. His spino-motor system and higher mental functions were found to be normal.\nA lobulated heterogenous signal intensity mass lesion was seen in the left CP angle and Meckel’s cave. The mass is predominantly isointense and weighted T1, and T2 images were hypointense (Figure ). Various other similar intensity lesions were seen along the left tentorium suggestive of recurrent meningeal carcinomatosis.\nThe brain MRI with contrast showed a recurrent mass in the left Meckel’s cave extending into the CP angle compressing the brainstem. Another lesion of a similar nature was seen in the lateral aspect of the cerebellum. There was evidence of thickening and abnormal enhancement of the adjacent pachymeninges and leptomeninges suggesting disease recurrence with leptomeningeal spread. He was further evaluated with a CT of his thorax and abdomen which ruled out extracranial disease.\nHe underwent a left retromastoid suboccipital re-exploration and subtotal excision of the tumor. A postoperative CT scan of his brain showed no residual disease. However, the patient died five months after the second surgery.
A 55-year-old male presented with grouped, fluid-filled lesions over the right side of the neck, shoulder and upper chest since 3 months. The lesions started on the nape of neck. They increased in size and number to form larger lesions resembling cluster of grapes and further spread to involve the right upper chest and shoulder in a month. The patient developed severe pain, redness and swelling over the affected area and swelling over the right side of the face with dysphagia and odynophagia since 15 days. This was associated with eruptions of few fluid-filled lesions on the neck and infra-axillary area. The patient gave history of loss of weight, loss of appetite and frequent upper respiratory tract infections for the past 1 year.\nOn examination, bilateral cervical, submandibular and supraclavicular lymph nodes were enlarged, tender and firm to hard in consistency. On cutaneous examination, a large ill-defined tender, erythematous and indurated plaque studded with grouped papulovesicular lesions resembling a bunch of grapes varying in size from 0.5 × 1 to 5 × 3 cm was present in a zosteriform fashion extending from the neck to the upper chest including the shoulder on the right side in C3, C4 and C5 dermatomes [Figures and ]. Few discrete vesicles on an erythematous base were present on right side of neck and right infra-axillary area. A single firm to hard tender subcutaneous nodule measuring 2 × 3 cms was present on the right upper back.\nA differential diagnosis of adult onset lymphangioma circumscriptum superimposed with herpes zoster and zosteriform cutaneous metastasis was considered.\nComplete hemogram was normal except for an ESR of 32 mm at the end of 1 hour. Blood sugars, liver function tests, renal function tests, and serum electrolytes were normal. VDRL was non-reactive and HIV test was negative. Tzanck smear from vesicles did not show any multinucleated giant cells.\nA skin biopsy from papulovesicular lesions and from vesicles showed clusters of large, atypical round to oval cells having a high nuclear cytoplasmic (N/C) ratio with vesicular, hyperchromatic nuclei and prominent nucleoli []. They were arranged in sheets along with other inflammatory cells suggesting metastatic deposits of poorly differentiated carcinoma. Vascular and lymphatic invasion by tumor emboli was seen []. An ultrasound scan of the neck showed bilateral lymph nodal masses.\nMRI of the head and neck showed bilateral thyroidomegaly, multiple enlarged submandibular, cervical and supraclavicular lymph nodes on both sides of the neck. Enlarged sub-carinal lymph nodes were seen. A solid focal lesion at nape of neck was seen indicative of a tumor deposit.\nBased on these findings, a diagnosis of zosteriform cutaneous metastasis to an unknown primary was made. Immunohistochemistry with a panel of antigens was done from the biopsy specimen to look for the unknown primary malignancy. HMWCK, Pan CK, p63, and desmoglein markers were positive and HMB 45, S 100, CEA, and Napsin markers were negative. Immunohistochemistry markers favored a diagnosis of squamous cell carcinoma of pulmonary origin.\nUnfortunately, the patient expired in 1 week after admission, so a detailed work up for the primary malignancy was not possible.
A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).\nPostoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.\nTwo months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.\nHe resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma.
A 54-year-old male presented to the Accident and Emergency Department with inability to pass urine and a painful, foul-smelling, and swollen penis for 3 days. He had no known medical problems and was not diabetic. On admission, the skin of the penis was partially necrotic with progressing gangrene, it was malodourous, and the abdominal wall and scrotum appeared normal.\nUltrasound, retrograde urethrogram, intravenous urogram, and urethroscopy were done within 24 hours. These confirmed a normal upper urinary tract and bladder and showed obstruction of the penile urethra secondary to multiple calculi; however, the calculi were embedded and the tissues were grossly inflamed and ulcerated. He was started on intravenous ampicillin, gentamicin, and metronidazole which were continued for 5 days during his hospital stay followed by amoxicillin/clavulanic acid upon discharge for a further week.\nThe patient consented for extraction of the urethral stones as well as aggressive debridement of the penile tissues with the risk of amputation. The exploration was done under general anaesthesia and an incision was done on the ventral (urethral) surface of the penis. Through a ventral urethrotomy, three urethral stones were successfully removed and the soft tissues of the penis aggressively debrided. A suprapubic cystostomy was also done and a catheter was inserted at this level (see Figures , , , and ). The ventral penile urethrotomy was closed primarily over a long-term catheter and the penile wounds were dressed and later closed with a scrotal skin advancement flap. Circumcision was not done as the skin though swollen was viable, and having already lost some penile skin to gangrene, all the intact remaining skin was conserved. The preputial oedema settled within 5 days of debridement. The urethral catheter was removed at 4 weeks and the suprapubic catheter was removed at 6 weeks. On a 12-month follow-up he had no evidence of erectile dysfunction or urethral stricture.
A 26-year-old high-myopic man with a history of wearing soft extended-wear hydro-gel contact lenses was referred for evaluation with complaints of decreased vision, photophobia, and pain in the left eye of 3 days’ duration as well as a history of using topical steroids and antibiotics prior to referral. The patient provided written informed consent for the case details and images to be published. The Ethics Committee of Tehran University of Medical Science approved the publication of the case details.\nThe patient’s ocular and systemic history was unremarkable. On ocular examination, the visual acuity was hand motion in the left eye and 2/10 in the right eye. The conjunctiva in the left eye was 3+ injected with a marked limbal flush. The corneal examination revealed an 8 mm inferior paracentral ulcer with 4 mm of central corneal thinning. The surrounding cornea was noted to have a marked stromal infiltration and a significant purulent discharge. These clinical features were most probably compatible with fulminant bacterial keratitis ( and ).\nThe smear was Gram-negative bacilli, and culture revealed Pseudomonas aeruginosa. A few hours later, severe thinning of the descemetocele turned into a perforation. Based on the large affected area in the cornea by keratitis and severe infections, a large limbus to limbus tectonic graft was implemented with lateral tarsorrhaphy in the same setting ( and ).\nLateral tarsorrhaphy was performed to enhance reepithelialization after the limbus to limbus transplant. The patient was discharged from the hospital with close follow-up monitoring. One month later, uncorrected visual acuity increased to 4/10 in the involved eye. The graft was clear with no signs of early rejection ().\nOn subsequent follow-up, the patient developed a corneal epithelial defect increasing in size from the central cornea to the periphery ( and ).\nThe patient was instructed to use copious lubrication, instill a lubricating ointment frequently, and tape the lids closed to minimize eyelid friction during the night. Cefazolin (50 mg/mL, 5%) eye drops and 2% fortified amikacin eye drops were prescribed every 1 hour. He was prescribed close follow-up. Three months later, the patient developed a total epithelial defect involving whole parts of the cornea. He traveled long distances to get to our hospital. Unfortunately, the patient was lost to follow-up for subsequent visits. Six months later, he returned with visual loss and perforation of the donor cornea as shown in . The patient was admitted, and a concomitant corneal patch graft and a bipedicular conjunctiva flap were implanted. Subsequently, the patient experienced three episodes of graft rejection that were managed by topical and systemic steroids.
A previously healthy 10-year-old girl presented to our emergency department with a history of back pain, fever, and night sweats for 4-month duration associated with progressive walking difficulty and back deformity over the last 2 months. Physical examination revealed middle back swelling with kyphotic (gibbus) deformity. Neurological examination revealed grade 4/5 motor weakness in both lower extremities with normal deep tendon reflexes and sensations. There was no evidence of cranial nerve involvement, meningeal irritation, or cerebellar dysfunction. Laboratory tests showed a normal complete blood count, elevated C-reactive protein of 81 mg/dl, and erythrocyte sedimentation rate of 111 mm/h. Serology testing for HIV was negative. Her Mantoux test was positive of 25 mm although there was no history suggestive of tuberculosis in the family. Chest X-ray was normal; however, spine X-ray demonstrated destruction of the thoracic vertebra (T11) body and inferior end plate of the T10 vertebra with loss of disk spaces between T10, T11, and T12 (). The contrasted magnetic resonance imaging (MRI) of her spine showed kyphotic destruction of thoracic vertebrae T10, T11, and T12 with abnormal high signal on T2 and low signal on T1 in addition to a large heterogeneous multiloculated prevertabral and paraspinal abscess extending from T7 through T12 with a peripheral thick wall that had low T2 and high T1 signal with intense enhancement. These are associated with an epidural collection and retropulsed collapsed T11, which result in complete attenuation of the anterior subarachnoid spaces and compression of the distal cord (). She underwent open surgical drainage of the paravertebral abscess and posterior spine fixation from T6 to L2 with pedicle screws. During surgery, a massive purulent fluid was discharged, and biopsy from the bone and soft tissue was taken and sent for microbiological examination and cultures including Mycobacterium tuberculosis. Both acid-fast staining and mycobacterial polymerase chain reaction were negative. Histopathology examination revealed caseating granulomatous inflammation consistent with tuberculosis. Based on radiological and histopathological findings, Pott's disease was highly suspected; therefore, the decision was made to start the patient on four anti-TB drug therapy of isoniazid, rifampicin, pyrazinamide, and ethambutol) while waiting for the final result of the mycobacterial culture. Ophthalmology team was consulted for baseline eye examination before starting ethambutol, and incidentally, bilateral papilledema was detected during fundus examination. Consequently, MRI of the brain showed multiple brain lesions in the left cerebellar hemisphere, left superior temporal gyrus, and right occipital lobe (). The lesions show a ring enhancement with a hypointense center mildly surrounded by a hyperintense area, most likely represent vasogenic edema which is highly suggestive of brain tuberculomas. There was no leptomeningeal enhancement suggestive of meningitis. After four weeks, her tissue culture grew Mycobacterium tuberculosis. The patient was treated with four antituberculous drug regimen for 2-month duration, followed by additional 10 months of isoniazid and rifampicin. Oral prednisone was given initially at a dose of 2 mg/kg/day for 4 weeks and tapped gradually over the next 6 weeks. In a three-year follow-up, the patient had normal neurological examination, and her brain MRI showed complete resolution of the tuberculomas.
A 49-year-old man, hypertensive with type 2 diabetes, came with a complaint of diffuse insidious, sporadic, abdominal pain associated with recurrent vomiting and early satiety for 3 months, with inconclusive family history. General examination was unremarkable. On abdominal examination, there were palpable bilateral retroperitoneal masses. Contrast-enhanced computerized tomography (CECT) abdomen [] showed retroperitoneal heterogeneously enhancing lesion with hemorrhagic foci in the suprarenal region with a predominance of fatty tissue with -26 to 17 HU density, measuring 30 × 18 × 13 cm in the left suprarenal region and 8 × 5 × 6.7 cm in the right suprarenal region. The differential diagnoses considered were adrenal myelolipoma, exophytic angiomyolipoma, and retroperitoneal sarcoma.\nOther findings were bilateral renal cortical cysts (Bosniak type 3) and hepatic cyst in the IVa Couinaud segment. There were no abnormalities in laboratory tests, as well as in the hormonal investigations (24 h urinary metanephrines and creatinine to exclude pheochromocytoma, 1 mg overnight dexamethasone test to exclude Cushing's syndrome, and plasma aldosterone renin ratio to exclude Conn's Syndrome as advised by the endocrinologist.) for adrenal masses (nonfunctional tumor). In view of the large size of the tumor and the compressive symptoms, the patient was advised for left adrenalectomy. After informed consent, the patient was taken up for left cortical sparing adrenalectomy under general anesthesia. Postoperative period was uneventful. Histopathology report of the adrenalectomy specimen showed myelolipoma, and the patient was kept under regular follow-up. The patient was asked to follow up with annual CT imaging. He was advised of the possible need for right adrenalectomy in case of persistent symptoms, new onset of symptoms, or significant change in imaging or laboratory investigations. Three years later the patient presented with right-sided loin pain for 15 days for which a CT scan of abdomen was performed, which showed bilateral nephrolithiasis with no increase in the size of the right adrenal tumor. The patient underwent right double J (DJ) stenting under spinal anesthesia on 25/12/19. Six months later, the patient complained of dull aching pain in the right loin, for a period of 1 month. In view of the similar presentation and laboratory investigations like the left-sided lesion, he was advised right adrenalectomy after necessary clearances were obtained from the relevant departments.\nPreoperative urology consultation was taken for intraoperative assistance for right renal calculi. After informed consent, the patient was taken up for right adrenalectomy with right nephrolithotomy with adhesiolysis under general anesthesia on 20/6/2020. Postoperatively, the patient was shifted to the Intensive Treatment Unit. Steroid replacement was started as advised by the endocrinologist. The patient improved symptomatically. Histopathology report of the resected tumor showed myelolipoma.\nThe steroid dose was reduced and on postoperative day 9, the patient was switched to oral steroid replacement. The patient was discharged on hydrocortisone and fludrocortisone supplement and was asked to follow up regularly with the endocrinology department to consider tapering the steroid dose.
A 65-year-old male patient visited the Department of Urology with a history of dysuria, supra-pubic pain, urinary frequency associated with macroscopic hematuria. His past history was remarkable for gastro-esophageal reflux and hypertension. The ultrasound of the urinary tract suggested the presence of a sessile bladder wall’s tumor approximately 3 cm in size engaging anterior wall and part of the bladder neck. These imaging were confirmed performing a cystoscopy. In October 2012 the patient underwent a transurethral resection of the bladder tumor (TURB). Histological examination showed a high-grade papillary TCC of the bladder with muscle invasion (pT2). The computed tomography (CT) of the abdomen-pelvis and chest X-ray was negative for metastases. A month later the patient underwent a RC and urinary diversion to an ileal conduit. The final pathology report confirmed the result of the TURB. A high-grade TCC with invasion of the bladder muscle layer was found, and TNM staging was pT2a N0M0. The prostate, the distal ureters, the proximal urethra and lymph nodes were free from tumor involvement. Patient did not receive chemotherapy in a neoadjuvant or adjuvant setting. The patient 14 months after RC presented a dull persistent pain in left groin that increases with body movements and walking. Subsequently, a full body CT and magnetic resonance imaging (MRI) of pelvis were performed and showed a lesion about 8 cm in diameter in the left obturator muscle with myositis ossificans aspect, and infiltrating branches ileum and ischio-pubic left (). The patient did not present any other abnormalities or metastatic lesions. Transrectal ultrasonography-guided biopsy of the mass lesion in the left obturator muscle was performed. The pathological analysis of the biopsy specimen revealed poorly differentiated metastatic urothelial carcinoma with malignant myositis ossificans aspects and positive to the CK7 marker. We recommended additional surgical resection of the mass to prevent the disease development. However, the patient refused additional surgery, receiving subsequently systemic chemotherapy and radiotherapy at the site of the lesion. More than 6 months after treatment, the patient has a good performance status with a partial reduction of the mass and negative imaging for metastases in the follow-up.

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