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.
Gastroschisis ; Hospitals ; experience ; Feeding ; Intestines, Small

In the absence of a neonatal intensive care unit (NICU) and total parenteral nutrition (TPN) gastroschisis management is challenging (1). If surgical closure is not done within the first 6 hours post partum, impending complications intervene, which then prevent a good outcome in such infants. The defect near the right side of the umbilicus provides the avenue for the intra-abdominal contents to protrude into the amniotic cavity in intra-uterine life and visceral exposure to the external environment increases the morbidity after delivery. The tight 2.5-5 cm bottleneck diameter provides further complications if not surgically corrected immediately. The prognosis has improved over the years and in well-set-up hospitals more than 95% have survived. Appropriate antenatal diagnosis (2) and early neonatal surgical intervention have improved the survival of these neonates. Blood investigation to assist with the antenatal diagnosis, such as amniotic fluid beta-endorphin analysis (3) and alpha-fetoprotein, assists in anticipating severe complications. Prevention of complications such as mesenteric infarct, fluid and electrolyte imbalance, necrotizing enterocolitis (NEC) and raised intra-abdominal compartment syndrome (RIACS) (2) and providing nutritional support have resulted in the good outcome of these cases. In the absence of accurate radiological diagnosis and back-up support services such as NICU and TPN, it is challenging to manage such infants. In a country where subspecialties are lacking it is very difficult to reassure the parents of a gastroschisis patient. Most of them do not make it. The following two case reports highlight some of these limitations and the alternative measures that can be taken to address the issues.