Abdominal Wall Defects

Summary
Introduction
Gastroschisis
Exomphalos
Investigation
Management

Summary

the abnormality should be covered with cling wrap
pay careful attention to thermoregulation and fluid management
refer early via NETS to tertiary referral centre with surgical facilities

Introduction

The diagnosis of exomphalos and gastroschisis is often but not invariably made antenatally by ultrasound. These babies will usually be delivered at a tertiary referral centre.

Gastroschisis

small defect of the anterior abdominal wall to the right of the umbilicus through which bowel herniates
occurs in between 1:10,000-30,000 births
no covering sac, the surface of the bowel is usually oedematous and matted
associated anomalies are reported in up to 15% (mainly gastrointestinal)
prematurity and growth restriction are frequent
necrotising enterocolitis and malabsorption may occur
survival rates are about 90%

Exomphalos

a protrusion of intestinal contents through the abdominal wall at the umbilicus
covered by a thin membrane of amnion and peritoneum
herniation of the liver accompanies intestine if a large sac, omentum and intestine are present if a small sac
associated anomalies occur in 45 – 67% (eg trisomies, cardiac defects, G.I. and renal anomalies)
survival rates are mainly dependent on whether other anomalies are present
necrotising enterocolitis and malabsorption are associated complications

Investigation

look for associated problems
- remember exomphalos can be associated with Beckwith-Wiedemann Syndrome (includes macroglossia, pathognomonic horizontal ear crease and hypoglycaemia)
- cardiac malformations
- renal abnormalities
- karyotype infants with exomphalos

Management

wrap abdomen of baby in cling film with gut lying well supported either on the abdomen if a small lesion or supported by a "doughnut". If in one position a length of bowel appears to have impaired blood supply or drainage i.e. looks purple or black, try gentle manipulation of the bowel into other positions to see if the circulation can be improved – the bowel may need to be rotated on its pedicle to achieve a better circulation
cotton wool covering or the use of moist packs is contraindicated. (Cotton wool adheres to the bowel wall, cannot be fully removed and causes peritoneal granulomas; moist packs rapidly become cold and lead to hypothermia)
pass size 8 NG tube, place on continuous low-pressure suction or leave on free drainage and aspirate every 60 minutes
place nil by mouth
start IV infusion - give usual Day 1 fluids
infants with gastroschisis may loose large amounts of fluid into the inflamed gut requiring vigorous fluid replacement. Watch blood pressure
check blood glucose immediately and monitor closely because of the association of Beckwith-Wiedemann syndrome with exomphalos
monitor temperature frequently. Patients with a ruptured exomphalos sac or gastroschisis may have major problems with temperature control due to evaporative heat loss
contact paediatric surgeon and NETS to arrange transfer to surgical centre
give antibiotics: Penicillin, Gentamicin (preferably after blood culture)
take blood for FBE, electrolytes, blood culture, group and hold for cross match of blood

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