TY - JOUR
T1 - The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease
AU - Hackam, David J.
AU - Reblock, Kim
AU - Barksdale, Edward M.
AU - Redlinger, Richard
AU - Lynch, James
AU - Gaines, Barbara A.
PY - 2003/6/1
Y1 - 2003/6/1
N2 - 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.
AB - 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.
KW - Constipation
KW - Endorectal pull-through
KW - Hirschprung's disease; aganglionosis
KW - Trisomy 21
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U2 - 10.1016/S0022-3468(03)00129-5
DO - 10.1016/S0022-3468(03)00129-5
M3 - Article
C2 - 12778399
AN - SCOPUS:0038657960
SN - 0022-3468
VL - 38
SP - 946
EP - 949
JO - Journal of pediatric surgery
JF - Journal of pediatric surgery
IS - 6
ER -