Angiomyolipoma of the tunica dartos of the scrotum in infancy

AbstractA 12-month-old boy presented with left scrotal swelling. The mass was irregular, soft, fleshy, and nontender. It adhered to the scrotal skin and gradually enlarged. Operative findings revealed a mass fixed to the scrotal fundus and diagnosed as angiomyolipoma. This is apparently the first report of scrotal angiomyolipoma in infancy

Congenital Diaphragmatic hernia – a review

Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes. Delivery of infants with CDH is recommended close to term gestation. Immediate management at birth includes bowel decompression, avoidance of mask ventilation and endotracheal tube placement if required. The main focus of management includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary hypertension followed by surgery. Although inhaled nitric oxide is not approved by FDA for the treatment of PPHN induced by CDH, it is commonly used. Extracorporeal membrane oxygenation (ECMO) is typically considered after failure of conventional medical management for infants ≥ 34 weeks’ gestation or with weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors such as prematurity, associated abnormalities, severity of PPHN, type of repair and need for ECMO can affect the survival of an infant with CDH. With advances in the management of CDH, the overall survival has improved and has been reported to be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO

Undifferentiated sarcoma developing 14 years after colocystoplasty: Our experience and literature review

A boy with myelomeningocele who had sigmoidocolocystoplasty and ureteric reimplantation when 2-years old and normal annual cystoscopies, developed hematuria and abdominal pain with liver dysfunction 14 years postoperatively. Computed tomography showed a tumor on the left side of the augmented bladder, a large lymph node, and large multiple probable metastases in the liver. Cystoscopy 3 months earlier had been normal, but when repeated showed a tumor originating from the augmented sigmoid colon. Biopsy showed undifferentiated sarcoma. Despite chemotherapy, he died 3 months later. The diagnosis at autopsy was undifferentiated sarcoma originating from the sigmoid colon. We report the first case of undifferentiated sarcoma developing 14 years after sigmoidocolocystoplasty for meningomyelocele, and also review the 55 cases of post-bladder augmentation malignancy in the literature

A case of tubular adenoma developing after bladder augmentation: Case report and literature review

AbstractIntroductionWe encountered a rare case of tubular adenoma developing after bladder augmentation. We here report our case as well as summarize reports in the literature on adenomas developing after bladder augmentation.Presentation of caseA 23-year-old man came to our hospital for routine surveillance cystoscopy. He was born with a lipomyelomeningocele and neurogenic bladder with low bladder compliance, and hence his bladder was routinely emptied by clean intermittent catheterization. He was also treated with anticholinergic agents. However, because the patient’s neurogenic bladder was unstable, he underwent sigmoidocolocystoplasty when he was 8-years old. After the bladder augmentation, he was examined annually by surveillance cystoscopy.On cystoscopy, a 5-mm pedunculated polyp was found on the front side of the sigmoid colon cap. Therefore, we performed snare polypectomy together with electrocoagulation under cystoscopy. The patient’s final diagnosis was tubular adenoma (mild atypia) with no malignancy, as assessed by histopathology. There has been no evidence of recurrence after the polypectomy on routine surveillance cystoscopy.DiscussionTo the best of our knowledge, there have been 11 cases of adenoma occurring after bladder augmentation reported in the literature, including our present case. There are several carcinogenic pathways associated with colorectal oncogenesis. Adenomas that are larger than 1.0cm in diameter with a marked villous component have a high risk of oncogenesis.ConclusionWe believe that the early detection of carcinoma or adenoma and their treatment at an early stage is crucial. Therefore, we recommend routine surveillance cystoscopy for patients after bladder augmentation

Endoscopic retrieval of a gastric trichobezoar

A 9-year-old girl presented with a chief complaint of abdominal pain. Esophagogastroduodenal endoscopy (EGD) identified a long and large gastric trichobezoar extending into the duodenum. We attempted endoscopic retrieval after informed consent was obtained from the patient’s mother. Initially, a gasper with 5-prolongs, commonly used for retrieval of endoscopically excised polyps, failed to remove the whole trichobezoar. When a net was used instead, it proved impossible to remove the trichobezoar completely. Therefore, we withdrew the scope from the mouth, leaving the net grasping the tricobezoar firmly in the stomach. Subsequently, we were able to retrieve about 70% of the trichobezoar manually by grasping the snare part of the net directly. A second pass found no deep laceration or perforation endoscopically. The remaining trichobezoar was completely retrieved with the net. The procedure was completed within 15 min. The retrieved specimens were 34 cm in length and 100 g in weight. The patient was discharged uneventfully 5 d thereafter. She was advised to visit a psychiatrist to avoid suffering from a relapse. Follow-up EGD showed no trichobezoar, and the patient’s frontal hair grew back

Initial Oxygenation Response To Inhaled Nitric Oxide Predicts Improved Outcome In Congenital Diaphragmatic Hernia

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