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Chronic malnutrition and growth failure are features of severe dystrophic epidermolysis bullosa (DEB). Conventional dietetic intervention is of limited benefit. Oesophageal dilatation or reconstruction to alleviate stricture is associated with substantial risks. Surgical placement of a feeding gastrostomy is a comparatively straightforward procedure, provided that specialized anaesthetic and surgical techniques are employed. Gastrostomy insertion was undertaken in 18 children with severe DEB and the effects of this intervention were retrospectively evaluated. The majority received button devices (inserted primarily) and gastrostomy feeding supplemented oral intake. One year postoperatively, the average increase in weight standard deviation scores (SDS) of 13 patients was 0.9 SDS (95% confidence interval 0.44, 1.35) and in height 0.42 SDS (95% confidence interval 0.05, 0.79). One patient developed an incisional hernia and four patients experienced minor leakage around the gastrostomy entry site. Two patients never accepted their gastrostomies, which were therefore removed. Two further patients died for reasons unrelated to the procedure. Our observations suggest that gastrostomy feeding can play a valuable role in severe DEB and is associated with minimal morbidity. Such intervention is best undertaken before growth failure is established, and prior to puberty.

Gastroschisis is traditionally managed by primary closure (PC) or delayed closure after surgical silo placement. Bedside insertion of preformed silos (PFS) and delayed closure has become more widespread, although its benefits remain unclear. To identify differences in outcome of infants managed with PFS compared with traditional closure (TC) techniques. Single-centre retrospective review of 53 consecutive neonates admitted between February 2000 and January 2006. Data expressed as median (range). Non-parametric statistical analysis used with P < 0.05 regarded as significant. Forty infants underwent TC and 13 had PFS and delayed closure. Median ventilation time in both groups was 4 days (P = 0.19) however this was achieved with higher mean airway pressures (MAPs) (day 0, 10 (5-16) versus 8 (5-10) cmH 2 O; P = 0.02) and inspired oxygen (40 (21-100) versus 30 (21-60)%; P = 0.03) in TC group. Urine output on day-1 of life was significantly higher in PFS group (1.1 (0.16-3.07) versus 0.45 (0-2.8) ml/kg/h; P = 0.02). Inotrope support was required in 17/40 (43%) of TC versus 0/13 (0%) in PFS (P < 0.01). After exclusion of infants with short bowel syndrome and/or intestinal atresia (n = 9), there was a shorter time to full enteral feeds in the TC group (22 (12-36) versus 27 (17-45); P = 0.07), although there was no difference in the period of parenteral nutrition (PN) (P = 0.1) or overall hospital stay (P = 0.34). No deaths or episodes of necrotizing enterocolitis occurred. The use of PFS for gastroschisis closure is associated with a reduction in pulmonary barotrauma, better tissue perfusion and improved early renal function, consistent with a reduction in abdominal compartment syndrome.

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