The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease.
BACKGROUND
Children with Down's syndrome (DS) have a reportedly poorer outcome after treatment of Hirschsprung's disease (HD) compared with control children. Because of overall improvements in their management, the authors hypothesized that the diagnosis of DS would not influence outcome after the management of HD.


METHODS
Consecutive children with HD (1995 through 2002) were collected prospectively then divided retrospectively into those with DS and controls (C). Patients who underwent surgery at another institution and those with total colonic aganglionosis were excluded.


RESULTS
Of 66 patients, 9 had DS. Mean age at diagnosis, gender, racial distribution, gestational age, and proximity to our center were similar between groups. Presenting symptoms, location of the transition zone, and type of initial operation were similar. Patients with DS had significantly more comorbidities than controls, which generated significantly greater treatment costs and a higher mortality rate. However, with an average of 22 months of follow-up, the overall outcome including postoperative complications, enterocolitis, and constipation was similar.


CONCLUSIONS
These data suggest that in contrast to earlier reports, DS has minimal influence on surgical outcome of patients with HD. Although the overall cost of treating patients with DS is greater, this mainly reflects the impact of managing comorbidities.
Hackam
David J
DJ
Division of Pediatric Surgery, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
Reblock
Kim
K
Barksdale
Edward M
EM
Redlinger
Richard
R
Lynch
James
J
Gaines
Barbara A
BA
D000714:Anastomosis, Surgical / Q000191:economics / Q000379:methods; D015897:Comorbidity / Q000639:trends; D003937:Diagnosis, Differential; D004314:Down Syndrome / Q000150:complications / Q000503:physiopathology*; D004760:Enterocolitis / Q000175:diagnosis / Q000191:economics / Q000601:surgery; D005260:Female; D006627:Hirschsprung Disease / Q000150:complications / Q000175:diagnosis / Q000191:economics / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000191:economics; D011446:Prospective Studies; D012189:Retrospective Studies; D016896:Treatment Outcome