New perspective for the management of near-total or total intestinal aganglionosis in infants.
OBJECTIVE
Extensive intestinal aganglionosis is rare. The diagnosis and treatment are known to be difficult and it had been considered to be fatal. The aim of this study was to review our experience with children with extensive intestinal aganglionosis.


METHODS
Retrospective analysis was conducted in patients referred to the intestinal transplantation unit since 1993. Presentation and outcome were analysed looking at 2 groups who had either undergone previous subtotal intestinal resection (group I) or no or limited resection (group II).


RESULTS
Eight children were selected (3 patients in group I and 5 in group II). Group I was remarkable in that patients all were referred early in age with progressing liver failure. Parents of one patient refused to accept transplantation as treatment, and he died one month later. Two noncirrhotic patients were maintained in the parenteral nutrition programme and currently progress well with enteral feedings. The other 5 patients underwent transplant, and 4 of 5 are alive after transplantation with a mean follow-up of 22.2 months (range 0.4 to 63.6).


CONCLUSIONS
Subtotal resection of intestine at the time of diagnosis must be avoided. Conservative management with parenteral nutrition may be associated with long-term good outcome. Small bowel transplant may open new perspective in the management of patients with end-stage liver disease.
Sharif
K
K
Liver Unit at Birmingham Children's Hospital, and Liver Unit at Queen Elizabeth Hospital, Birmingham, England.
Beath
S V
SV
Kelly
D A
DA
McKiernan
P
P
van Mourik
I
I
Mirza
D
D
Mayer
A D
AD
Buckels
J A C
JA
de Ville de Goyet
J
J
D005260:Female; D006627:Hirschsprung Disease / Q000178:diet therapy* / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007421:Intestine, Small / Q000637:transplantation*; D017093:Liver Failure / Q000209:etiology / Q000628:therapy; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D010289:Parenteral Nutrition, Total / Q000379:methods; D012189:Retrospective Studies