Gastrostomy and growth in dystrophic epidermolysis bullosa.
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.
Haynes
L
L
Department of Dermatology, Great Ormond Street Hospital for Children NHS Trust, London, U.K.
Atherton
D J
DJ
Ade-Ajayi
N
N
Wheeler
R
R
Kiely
E M
EM
D000293:Adolescent; D000886:Anthropometry; D001827:Body Height; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D003248:Constipation / Q000178:diet therapy; D004032:Diet; D004043:Dietary Fiber / Q000627:therapeutic use; D004750:Enteral Nutrition; D016108:Epidermolysis Bullosa Dystrophica / Q000503:physiopathology / Q000628:therapy*; D005260:Female; D005774:Gastrostomy / Q000009:adverse effects; D006128:Growth; D006801:Humans; D007223:Infant; D008297:Male; D015596:Nutrition Assessment; D012189:Retrospective Studies