title: Management of Hirschsprung's disease in children with trisomy 21.
abstract: Thirteen infants and children with trisomy 21 have been treated for Hirschsprung's disease since 1975. Clinical presentation of Hirschsprung's disease included constipation (five); neonatal intestinal obstruction (four); enterocolitis (three); and meconium plug syndrome (one). Additional associated congenital anomalies occurred in 10 patients, of which complex cardiac disease accounted for 25% of the defects. Seven children underwent definitive operation: Duhamel pull-through (four); Soave pull-through (two); and anal myectomy (one). Satisfactory continence occurred in all but one child. Enterocolitis developed in seven patients (54%): two at diagnosis of Hirschsprung's disease; three after colostomy; and two after pull-through. Five children died (38%): one from enterocolitis, two from cardiorespiratory failure after recovery from enterocolitis, and two from end-stage cardiac disease. Children with trisomy 21 can safely undergo definitive operation for Hirschsprung's disease but are at high risk for developing enterocolitis and complications of associated cardiac disease.
authors: Division of Pediatric Surgery, Ohio State University College of Medicine, Columbus.
authors: D A
authors: DA
authors: Caniano
authors: D H
authors: DH
authors: Teitelbaum
authors: S J
authors: SJ
authors: Qualman
mesh_terms: D002648:Child; D002675:Child, Preschool; D003125:Colostomy / Q000379:methods; D004314:Down Syndrome / Q000150:complications*; D004760:Enterocolitis / Q000209:etiology / Q000401:mortality; D005069:Evaluation Studies as Topic; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications; D006627:Hirschsprung Disease / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality; D011379:Prognosis; D012189:Retrospective Studies