Gastroschisis and the risk of short bowel syndrome: outcomes and counselling.

BACKGROUND

Gastroschisis can be associated with short bowel syndrome (SBS). We present 4 cases with significant bowel shortening with very different ante- and postnatal presentations.

CASES: Three of the cases demonstrated 'vanishing' gastroschisis with progressively worsening antenatal intra-abdominal bowel dilatation with no defect present at birth. The fourth case followed an uneventful course antenatally but developed SBS considered secondary to gastroschisis-related atresia and necrosis. All 4 cases were classified as having SBS and were enrolled within the paediatric Intestinal Rehabilitation Programme. This involves autologous gastrointestinal reconstruction following a period of bowel expansion with concomitant parenteral and enteral nutrition.

DISCUSSION: These cases demonstrate that the serious complication of significant bowel loss cannot always be anticipated. Early multidisciplinary discussion with the parents enables a shared understanding of potential outcomes. It can aid the recognition of 'vanishing' gastroschisis enabling expedient investigations and early intervention, minimising morbidity and maximising bowel length.