Background: The results of treatment of Hirschsprung's disease (HD) are not
uniformly successful, and the parents' and child's interpretation of the o
utcome may be different from that of the surgeon.
Methods: One hundred seven children treated for HD over 221/2 years were re
viewed retrospectively. Adequate clinical data regarding functional outcome
s were available in 78, of which, an additional questionnaire was completed
by 69. Follow-ups were divided into type of pu I I-th rough (PT), age at t
ime of PT (<4 months and >4 months) and age at last follow-up (<5 years, 5
to 15 years and >15 years). Degrees of constipation and incontinence were d
etermined using standard scoring systems.
Results: The median age at presentation was 9 days (range, 1 day to 9.4 yea
rs), and 41% presented within the first 72 hours of life. Aganglionosis ext
ended to the rectosigmoid region in 75%, proximal to the splenic flexure in
11%, and total colon in 6.5%. Treatments included a Soave procedure in 57,
Duhamel in 31, Swenson in 10, and sphincterotomy-myectomyin two. No surgic
al treatment or only a decompressing enterostomy was performed in two and t
hree children, respectively, and two underwent a PT elsewhere. Complication
s related to the enterostomy occurred in 39 patients. Postoperative enteroc
olitis and bowel obstructions occurred in nine and 13 patients, respectivel
y. Staple-line fusion, anastomotic stenosis, and anastomotic leak occurred
in nine, 17, and two patients, respectively. Seven children died, only one
directly related to the PT. Aside from more constipation associated with th
e Duhamel procedure, functional outcomes were not significantly different a
mong the types of PTs. No differences were found between patients who under
went a Pi at less than 4 months of age and those at greater than 4 months o
f age. Significantly, however, fecal continence was relatively poor in thos
e less than 15 years of age (50% with only fair to poor continence), but im
proved markedly once the child reached later adolescence (8%, P < .0002). C
orrespondingly, the negative impact on the child's social life was much gre
ater in the younger age groups compared with later adolescence (50% of thos
e aged 5 to 15 years v 18% of those >15 years; P = .007). The effect on the
families' lives mirrored this. Finally, only 64% of patients were interpre
ted as having "normal" stooling habits, yet 90% of parents were moderately
or very satisfied with their child's outcome, with no differences found amo
ng the three sets of comparison groups.
Conclusions.. The outcomes for HD are not always as good as surgeons may pe
rceive; long term follow-up is important. With time, most children signific
antly improve with respect to fecal continence, but this may not be until l
ater adolescence. In the meantime, the impacts on their social and family l
ives may be significant. Despite high complication rates, often poor contin
ence, and relative infrequency of normal stooling habits, most parents are
satisfied with their child's outcome and adapt to their functional abnormal
ities along with them. J Pediatr Surg 34:1152-1160. Copyright (C) 1999 by W
.B. Saunders Company.