GASTROSTOMY AND GROWTH IN DYSTROPHIC EPIDERMOLYSIS-BULLOSA

Citation
L. Haynes et al., GASTROSTOMY AND GROWTH IN DYSTROPHIC EPIDERMOLYSIS-BULLOSA, British journal of dermatology, 134(5), 1996, pp. 872-879
Citations number
29
Categorie Soggetti
Dermatology & Venereal Diseases
ISSN journal
00070963
Volume
134
Issue
5
Year of publication
1996
Pages
872 - 879
Database
ISI
SICI code
0007-0963(1996)134:5<872:GAGIDE>2.0.ZU;2-B
Abstract
Chronic malnutrition and growth failure are features of severe dystrop hic epidermolysis bullosa (DEB), Conventional dietetic intervention is of limited benefit. Oesophageal dilatation or reconstruction to allev iate stricture is associated with substantial risks, Surgical placemen t of a feeding gastrostomy is a comparatively straightforward procedur e, provided that specialized anaesthetic and surgical techniques are e mployed. Gastrostomy insertion was undertaken in 18 children with seve re DEB and the effects of this intervention were retrospectively evalu ated, The majority received button devices (inserted primarily) and ga strostomy feeding supplemented oral intake. One year postoperatively, the average increase in weight standard deviation scores (SDS) of 13 p atients 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 aroun d the gastrostomy entry site, Two patients never accepted their gastro stomies, which were therefore removed, Two further patients died for r easons unrelated to the procedure. Our observations suggest that gastr ostomy feeding can play a valuable role in severe DEB and is associate d with minimal morbidity. Such intervention is best undertaken before growth failure is established, and prior to puberty.