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? Open in a separate windowRives-Stoppa retromuscular technique: A) polypropylene mesh fixed on the posterior rectus sheath; B) rectus abdominal muscle; C) anterior rectus sheath being sutured  相似文献   

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Neurofibromatosis is a hamartomatous disorder of autonomic peripheral nerve sheaths associated with peripheral nerve sheath tumours. Most tumours are neurofibromas; however, the genitourinary system is rarely involved. We present a rare case of a nerve sheath tumour of the bladder in a young patient, which was discovered to be malignant.A previously healthy 17-year-old woman presented with abdominal pain and recurrent urinary tract infections. She also had intermittent difficulty passing urine and had bilateral loin discomfort. Physical examination was unremarkable aside from fullness in the suprapubic area and skin pigmentation in keeping with her known history of neurofibromatosis 1. Laboratory investigations revealed a mildly raised creatinine of 160 mmol/L and raised inflammatory markers with an erythrocyte sedimentation rate of 80 mm/h. Abdominal ultrasonography revealed moderate bilateral hydronephrosis and a pelvic mass. Computed tomography imaging confirmed a large bladder mass and showed evidence of bilateral hydronephrosis (Fig. 1). The patient subsequently had magnetic resonance imaging of her pelvis, which revealed a very large lobulated mass within the pelvis, lying anterior to the uterus and directly involving the bladder (Fig. 2). The patient underwent surgery with cystectomy and ileal conduit formation. At pathological histology, the mass was shown to be a malignant peripheral nerve sheath tumour of the bladder wall (Fig. 3 and Fig. 4).Open in a separate windowFig. 1Postcontrast axial computed tomography image of the lobulated mass within the bladder.Open in a separate windowFig. 2Axial T1-weighted magnetic resonance imaging demonstrating the heterogenous lobulated bladder mass (A); sagittal T1-weighted image of the pelvis, demonstrating the extent of the heterogenous mass (B).Open in a separate windowFig. 3Corresponding gross specimen of the bladder expanded by a large white variegated and lobulated tumour, primarily based in the bladder wall.Open in a separate windowFig. 4Histological specimen that stains positive for smooth muscle actin confirming the diagnosis of malignant peripheral nerve sheath tumour (original magnification × 20).  相似文献   

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Angiomyolipomas (AMLs) are benign tumours characterized by fat, smooth muscle and vascular components. Epithelioid AML is a recognized variant of AML that is comprised of epithelioid smooth muscle cells. We present a case of a 41-year-old male who presented with light-headedness, dizziness, right-sided abdominal pain and, on subsequent computed tomography, was found to have an enormous right kidney mass characteristic of an AML. The patient underwent preoperative selective arterial embolization followed by a right radical nephrectomy. The pathology revealed a 36-cm AML with focal epithelioid features. Although uncommon, AMLs can present as enormous retroperitoneal masses.A 41-year-old healthy male with a recent history of syncope presented with complaints of persistent light-headedness, dizziness and right-sided abdominal pain. At an outside hospital, he received 2 units of packed red blood cells for his anemia, and a computed tomography (CT) scan of his abdomen and pelvis revealed an enormous right renal mass with multiple vascular and adipose-laden areas characteristic of an angiomyolipoma (AML) (Fig. 1). The patient was subsequently transferred to our hospital for further management.Open in a separate windowFig. 1Mass revealed on computed tomography scan.Upon arrival, the patient’s blood pressure was stable, but he was tachycardic with a heart rate in the 110 range. He had an otherwise unremarkable medical and family history, with no clinical stigmata typical of tuberous sclerosis. On examination, he was moderately obese with a large palpable right-sided abdominal mass. His creatinine was 1.1 mmol/L. His presenting hematocrit of 21% led to an additional transfusion of 1 unit of packed red blood cells; he was immediately sent to interventional radiology for an angiogram and embolization to obtain hemostasis and, by embolizing most of the tumour’s blood supply, to reduce the risk of intraoperative hemorrhage. The angiogram revealed a large hypervascular tumour supplied predominantly from a right superior pole renal artery and its branches; these were all embolized and the post-procedure angiogram showed a significant reduction in the lesion’s vascularity.The patient’s hematocrit continued to drop overnight to 19%; he received an additional 2 units of packed red blood cells and returned to interventional radiology for further evaluation. On angiogram, a 3-cm pseudoaneurysm (Fig. 2) from another branch of the right renal artery was found and embolized proximal to it. In addition, one of the branches of the renal artery, that was embolized the day before, continued to have flow and was re-embolized. The post-embolization angiogram showed stasis of flow both to the pseudoaneurysm and the re-embolized vessel. Subsequently, his hematocrit stabilized at 26%.Open in a separate windowFig. 2Vessels supplying the lesion on angiogram.The following day, the patient underwent a right radical nephrectomy through a thoracoabdominal incision. The tumour did not invade any adjacent organs and weighed 7.6 kg when it was removed (Fig. 3). The pathology revealed a 36 × 25 × 18 cm angiomyolipoma with focal epithelioid areas. The patient was discharged on postoperative day 7, had an unremarkable follow-up appointment 2 weeks later, and is scheduled for a repeat CT scan in 1 year.Open in a separate windowFig. 3Specimen.  相似文献   

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A 55-year-old man presented to the emergency department with a 12-hour history of severe crampy abdominal pain, nausea, vomiting and obstipation. The patient had a complex medical history, including coronary artery disease, lupus, hypothyroidism, epilepsy, pancreatitis and renal calculi. However, the patient had no history of a hernia or abdominal surgery. Physical examination revealed a temperature of 38.5°C and a soft distended abdomen that was diffusely tender without signs of peritonitis. The rest of the physical examination was unremarkable. Routine laboratory investigations including a complete blood cell count, electrolytes, liver enzymes and amylase were normal, with the exception of a decreased hemoglobin level of 116 g/L. We ordered a plain abdominal radiograph (Fig. 1) and a contrast-enhanced computed tomography (CT) scan of his abdomen. What is your diagnosis?Open in a separate windowFig. 1Plain erect abdominal radiograph of a 55-year-old man presenting with abdominal pain, nausea, vomiting and obstipation.  相似文献   

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Previous coronary artery bypass surgery (CABG) is an independent risk factor for coronary perforation (CP) during percutaneous coronary intervention (PCI) because of complexity of the cases. However in patients with prior CABG surgery, cardiac tamponade is rare because of local or regional pericardial adhesions.Keywords  Post CABG, complex PCI, Coronary perforation, Tamponade

A 70-year-old patient, with a post-coronary artery bypass graft (CABG) surgery in 2010 with left internal mammary artery (LIMA) graft to left anterior descending (LAD) artery, saphenous venous graft to ramus intermedius, and posterior descending artery (PDA), presented with class III angina. He had a concentric left ventricular hypertrophy and normal ejection fraction. Coronary angiography (CAG) showed diffuse left main stenosis of maximum 40%, LAD proximal cut off, left circumflex (LCX) ostio-proximal diffuse, calcific disease, with maximum diameter stenosis of 80% (Fig. 1a), right coronary artery dominant with a proximal chronic total occlusion, patent LIMA and ramus grafts, and blocked PDA graft.Open in a separate windowFig. 1a Right anterior oblique view—left main plaquing with diffusely diseased and calcific left circumflex, maximum 80% stenosis. b 1.25 mm rota burr in left main. c LCx after rota-ablation. d A drug-eluting stent 2.5 × 33 mm was deployed from proximal to distal LCx. Another DES of size 3.5 × 19 mm was deployed from left main to proximal LCx. e Proximal perforation of LCx, as depicted by white arrow. f Progressively increasing perforation. g Extravasated blood seemed to collect in a closed sac surrounding proximal LCx. h Spontaneous and complete closure of the perforationPatient was taken up for percutaneous coronary intervention (PCI) of LCx. Left main was cannulated using an extra backup guide, and coronary wire through a microcatheter was placed in LCx. Microcatheter was placed in distal LCx and wire exchanged with rota wire. A 1.25-mm rota burr was used because of diffuse calcific disease from ostial LCx to distal LCx (Fig. 1b, c).Two drug-eluting stents of size 3.5 × 19 mm and 2.5 × 33 mm were deployed from left main to distal LCx (Fig. 1d). The overlapping stents were post dilated with 3.5 × 19-mm stent balloon at 18 atm. CAG done after dilatation showed Ellis grade IV proximal perforation of LCx, as depicted by white arrow in (Fig. 1e).There was a progressive increase in perforation (Fig. 1f), and extravasated blood seemed to collect in a closed sac surrounding proximal LCx (Fig. 1g). CAG done within few minutes revealed spontaneous and complete closure of the perforation with no hemodynamic compromise (Fig. 1h).  相似文献   

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A 15-year-old Caucasian boy with a diagnosis of confluent and reticulated papillomatosis who had received numerous treatments with minimal responses cleared with a 20-week course of 13-cis-retinoic acid and has remained in remission. It is important to consider the use of oral retinoids in the treatment of this stubborn, unsightly, and psychologically upsetting disease.This case describes a 15-year-old Caucasian boy who presented with a pruritic, brownish eruption of a few years'' duration that was treated with diphenhydramine hydrochloride cream and moisturizers. His past history is unremarkable except for being premature due to abruptio placenta. He is overweight and plays football. His prior skin diagnoses include eczema, for which he was given topical steroids, and tinea versicolor, which responded minimally to oral and topical ketoconazole.On physical examination, the patient had brownish-tan sessile, velvety, hyperkeratotic, coalescing papules on the chest, back, nape of neck, and extensor surfaces of upper arms (Figures 1 and and2).2). Scraping produced a powdery scale.Open in a separate windowFigure 1Velvety, brown, hyperkeratotic lesions on the backOpen in a separate windowFigure 2Lesions on the abdomenA biopsy showed horn formation and was difficult to assess (Figure 3). Asteatotic eczema, pityriasis rubra pilaris, acanthosis, and fungus were considered and dismissed. In view of the deeper sections and the history, a diagnosis of confluent and reticulated papillomatosis of Gougerot et Carteaud (also known as Gougerot-Carteaud syndrome) was made.Open in a separate windowFigure 320x magnificationTreatment began with minocycline 100mg daily for three months. The dosage was tapered to 50mg daily over the next three months as the lesions faded and the patient''s skin felt smooth. Unfortunately, the lesions began to return, and isotretinoin was started at 1mg/kg or 60mg twice daily with food for 20 weeks. The patient''s triglycerides were monitored and fenofibrate was added in the third month of the regimen. The patient had an excellent response—the unsightly lesions were eliminated and his skin became smooth (Figures 4 and and55).Open in a separate windowFigure 4The patient''s back post-treatment—smooth skinOpen in a separate windowFigure 5The patient''s chest post-treatment  相似文献   

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Cutaneous reactions to tattoos have been increasing in conjunction with the rise in popularity of tattoos. While localized lichenoid reactions to tattoo inks are fairly common, generalized lichenoid reactions are relatively rare. Herein the authors present a case of a generalized lichenoid reaction to a tattoo containing only black ink. They also present a brief discussion of tattoo reactions and treatment options.A 17-year-old boy with no past medical history presented five weeks after obtaining a professional tattoo with black ink on the left arm. Two weeks after the tattoo, he noted the onset of a generalized, pruritic rash. Examination revealed an African-American male teenager with numerous 1 to 4mm, flat-topped, skin-colored to hyperpigmented papules with a fine white scale, concentrated at the site of the tattoo, but also scattered over the trunk, genitals, and all four extremities, including the dorsal hands and feet (Figure 1--3).3). The oral mucosa was spared. A skin biopsy from the right forearm revealed a lichenoid infiltrate with few eosinophils (Figure 4 and and5).5). Liver function tests were within normal limits, and hepatitis C antibody was negative. He was treated with triamcinolone acetonide 0.1% cream applied topically twice daily and a two-week oral prednisone taper starting at 40mg. Unfortunately, despite multiple attempts to contact the patient, he was lost to follow-up. Open in a separate windowFigure 2Disseminated lichenoid papules Open in a separate windowFigure 1Lichenoid papules at the site of the tattooOpen in a separate windowFigure 3Disseminated lichenoid papules with koebnerizationOpen in a separate windowFigure 4Histopathologic findings demonstrating a lichenoidOpen in a separate windowFigure 5Histopathologic findings demonstrating eosinophils pattern  相似文献   

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