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Duodenal atresia (DA) is a well-described congenital anomaly that usually responds well to surgical correction. Associated defects are common, and these confounding variables often influence outcome. The authors present a case of a newborn female with an unusual constellation of problems including DA with annular pancreas, trisomy 21, and coarctation of the aorta. She developed protracted complications postoperatively and was treated with an innovative surgical strategy.

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Persistent cloaca is characterized by a common vestibule connected to the bladder, uterus, and rectum and a single perineal orifice to the exterior. Treatment can involve vaginal reconstruction with sigmoid/descending colon. We report a case of an 8-year-old girl who, as an infant, underwent vaginoplasty using sigmoid colon for persistent cloaca and developed a fulminant colitis that also affected the colonic neovagina 2 years after her reconstruction.

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PURPOSE: The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure. METHODS: A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006. RESULTS: We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group. CONCLUSION: Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates.

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Congenital pancreatic cysts, particularly solitary cysts, are rare causes of abdominal masses in newborns. The etiology of these cysts is unknown and they can vary in location within the pancreas. We present a novel case of an infant with a solitary congenital cyst of the pancreatic duct, itself, as a result of occlusion of the pancreatic duct. Solitary congenital pancreatic cysts are a rare cause of abdominal masses in infants. We recently evaluated a 7-week-old infant with progressive abdominal distension since birth. This child was found to have a large solitary pancreatic cyst. This cyst resulted from occlusion of the main pancreatic duct at the level of the ampulla resulting in cystic degeneration of the pancreatic duct. Based on our review of the literature we believe this is the first documented case of such a cyst.

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INTRODUCTION: Giant intraabdominal cysts are very rare, and conventional treatment is full midline laparotomy. We present a case of complete laparoscopic extirpation of a giant ovarian cyst. CASE REPORT: A 16-year-old female presented with progressive abdominal distension for 1-year along with early satiety, constipation, and significant weight loss. A CT scan showed a giant multiseptated cystic mass in the abdomen measuring 22.5 x 30 x 40.5 cm with significant mass effect causing intrahepatic ductal dilatation and right hydronephrosis. The mass was decompressed via a mini-laparotomy in a controlled fashion, removing 15 liters of fluid. A laparoscopic left oophorectomy was then performed. The postoperative course was uneventful, and the patient was discharged home on postoperative day 1 with minimal pain and tolerating a regular diet. Pathology examination revealed a mature cystic teratoma. CONCLUSION: Giant ovarian cysts can be managed laparoscopically when a normal tumor marker profile and benign imaging appearance exclude the possibility of malignancy.

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BACKGROUND: Pulmonary hypoplasia and hypertension are the primary causes of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). At present, the origin of CDH and the causes of pulmonary hypoplasia and hypertension are unknown. DATA SOURCES: This article reviews the available published data regarding the origin of CDH and the pathogenesis of the associated pulmonary hypertension and hypoplasia. These investigations have employed human tissues as well as two types of CDH animal models. CONCLUSIONS: Investigations performed to date have not yet provided definitive answers regarding the pathogenesis of CDH. However, they have yielded many new and exciting discoveries and several opportunities for intervention. Ongoing research should open new possibilities to improve the outcome for these unfortunate babies with CDH.

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The authors report a case of an 11-year-old girl with a solitary, congenital pancreatic cyst and review the literature. Such cysts are very rare and typically are diagnosed in childhood. As a neonate, this patient's paternal half brother also had undergone resection of a congenital pancreatic cyst. The authors believe this is the first documented familial incidence of a congenital pancreatic cyst.

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