Gastroschisis management: an experience in Angau Memorial Hospital.

This paper is the report of a 2 kg baby girl born with a large gastroschisis at the Angau Memorial Hospital. She is the first long-term survivor in Papua New Guinea with this major ventral abdominal wall defect as far as we are aware. In the report important steps in the management of this major congenital defect are highlighted from both the surgical and anaesthetic perspectives. It is imperative that a large gastroschisis be managed by a reduction carried out over two or more stages to prevent catastrophic abdominal compartment syndrome. Postoperative ventilation via an endotracheal tube was required due to respiratory compromise from the raised intra-abdominal pressure. Deflation of the dilated small bowel was shown to be an important step to allow full return of the small bowel into the abdominal cavity. A percutaneous jejunostomy feeding tube was inserted for feeding postoperatively as total parenteral nutrition was not available, and this was crucial for the nutritional management and ultimate survival of the baby. Gastroschisis is a very significant congenital defect with major challenges in its management in a developing country, but if important principles of management are followed, the prognosis can be remarkably improved.

Double bubble, double trouble.

Duodenal atresia (DA) is not uncommon, either as an isolated anomaly or associated with trisomy 21, malrotation, or cardiac anomalies. It may be diagnosed on antenatal ultrasound by a "double-bubble" sign, which typically persists after birth on a plain abdominal radiograph. DA as a familial association is rare but has been reported with or without other associated anomalies. We report DA in two siblings of nonconsanguineous parents, one case occurring with an annular pancreas in association with gestational diabetes. These two cases suggest possible genetic and environmental components in the aetiology of this anomaly.