The influence of trisomy 21 on outcome in children with Hirschsprung's disease

FMJ Quinn, R Surana, P Puri - Journal of pediatric surgery, 1994 - Elsevier
The association of Hirschsprung's disease (HD) and trisomy 21 has been well recognised.
Seventeen (13%) of 135 patients presenting with HD between 1975 and 1992 had trisomy
21. Nine (53%) presented in the neonatal period, with intestinal obstruction (5), enterocolitis
(2), or perforation of the colon (2). Eight patients presented after the neonatal period, with
constipation. Pathological involvement included rectosigmoid (12), long segment (4), and
total colonic aganglionosis (1). Definitive surgery was performed in 14 patients. At the mean …