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- Title
Gastrostomy and growth in dystrophic epidermolysis bullosa.
- Authors
L.Haynes; Atherton, D. J.; Ade-Ajayi, N.; Wheeler, R.; Kiely, E. M.
- Abstract
Chronic malnutrition and growth failture 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% contidence 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.
- Subjects
EPIDERMOLYSIS bullosa; MALNUTRITION; GASTROSTOMY; DILATATION &; extraction abortion; ANESTHETICS; OPERATIVE surgery; STATISTICAL hypothesis testing
- Publication
British Journal of Dermatology, 1996, Vol 134, Issue 5, p872
- ISSN
0007-0963
- Publication type
Article
- DOI
10.1111/j.1365-2133.1996.tb06318.x