title: [Colonic aganglionosis with ileal involvement. Ileocolic plasty].
abstract: During 1994 through 1999, we have treated five patients (3 boys, 2 girls) with total colonic aganglionosis (TCA) and ileal involvement. In three of them we performed a diverting ileostomy in the neonatal period and at the age of four and five months respectively in the remaining two patients, due to intestinal obstruction. In these two last patients a diagnosis of Hirschsprung's disease was made by anorectal manometry and rectal biopsies in the neonatal period. During laparotomy, a cutaneous ileostomy was created in all patients at the distal end of normal ileum, which was 30 to 110 cm (mean = 71 +/- 37 cm) from the ileocecal valve. After operation, a short bowel syndrome developed in three patients causing fluid and nutritional problems that required prolonged total parenteral nutrition (TPN). The definitive operative repair is performed at 4.5 to 14 months (mean = 8 +/- 3.7 months) by a 12 to 20 cm side-to-side ileocolostomy created between the ileum and aganglionic ascending color (Boley procedure) and ileorectal primary anastomosis (Rehbein procedure) using a circular stapler. Rectal dilatation, irrigation of the colon with saline, loperamide hydrochloride and resincholestyramine were begun in all patients postoperatively. Oral antibiotic prophylaxis, was given to the three patients who suffered from SBS. Oral feedings with semielemental diet were tolerated once stools were semiformed and TPN was discontinued at 8 to 34 days (mean = 21 +/- 1.7 days). postoperatively. After the definitive operation, enterocolitis developed in two patients, requiring one of them a short hospitalization during the episode. A possible explanation for the low incidence of enterocolitis in this series is the systematic postoperative use of irrigations of the colon with saline in all patients. These five patients have been followed-up for growth, development, bowel habit and continence. Follow-up has ranged from 15 to 62 months (mean = 32.2 +/- 19.2 months). Presently, all patients in this series have full enteral feeding and one to three bowel movements per day, with formed or semiformed stools. No patients is incontinent of stool. The patient's body weights (74 to 93%) and heights (89 to 92%) for their age were below average (four patients) or within normal range (one patient). Hemoglobin levels are within normal range in all patients. One patient has iron deficiency and another one has serum ferritin concentration below normal. Three patients have folic acid deficiency. Vitamin B12 absorption is normal in all patients. Although we can not conclude this is a better procedure than others, with is use we have obtained satisfactory results, with an excellent survival, scanty morbidity, a rapid return of bowel function and continence, and an acceptable physical development. During long-term follow-up, patients mus be evaluated for iron and folic acid deficiency.
authors.affiliation: Servicio de Cirugía Pediátrica, Hospital Universitario Materno-Infantil Infanta Cristina, Badajoz.
authors.forename: R
authors.initials: R
authors.lastname: Núñez
authors.forename: R
authors.initials: R
authors.lastname: Cabrera
authors.forename: E
authors.initials: E
authors.lastname: Agulla
authors.forename: C
authors.initials: C
authors.lastname: Moreno
authors.forename: A
authors.initials: A
authors.lastname: Serrano
authors.forename: C
authors.initials: C
authors.lastname: Ortiz
authors.forename: E
authors.initials: E
authors.lastname: Blesa
mesh_terms: D002648:Child; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications* / Q000601:surgery*; D006801:Humans; D007077:Ileal Diseases / Q000209:etiology* / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male