Background/Purpose: The authors' recent experience with the study of t
he presentation, the pathological anatomy, and results of management o
f congenital pouch colon (CPC) malformations is presented. The possibl
e embryogenesis of this condition is discussed. Methods: Between Janua
ry 1991 and June 1997, CPC with anorectal agenesis was diagnosed in 39
patients, 27 boys and 12 girls, who were classified in four groups, b
ased on the length of the normal colon proximal to the distended segme
nt. In 31 patients with little or no normal colon, the choice of prima
ry procedure was based on the condition of the infant and the viabilit
y of the colonic pouch. Definitive surgery has been performed in 15 pa
tients from this group. The eight patients with a suitable length of n
ormal colon had a lower level of termination of the colonic pouch and
a lower fistula. In these, a colostomy was constructed just proximal t
o the pouch, with later definitive surgery in four patients consisting
of excision of the colonic pouch and pull-through of the proximal col
on. Results: Mortality after primary surgery was 13%. Definitive surge
ry was well tolerated in all 19 patients. Conclusion: In patients in w
hom a tubularized segment of the colonic pouch was used, continence wa
s only fair to poor a year later. Copyright (C) 1998 by W.B. Saunders
Company.