Hirschsprung's Disease: Evaluation of Mortality and Long-term Function in 260 Cases

Frederick J. Rescorla, Albert M. Morrison, Drew Engles, Karen W. West, Jay L. Grosfeld

Research output: Contribution to journalArticle

168 Citations (Scopus)

Abstract

This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82%) and 47 girls (18%). Age at diagnosis was younger than 30 days in 106 patients (41%), 1 month to 1 year in 90 patients (35%), and older than 1 year in 64 patients (25%). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67%), the left colon in 38 patients (15%), and the proximal colon in 23 patients (9%); 25 patients (9%) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18%). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95%) (modified Duhamel in 185, Soave in25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8% (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65%) had normal bowel function, 28 (27%) occasionally used enemas or stool softeners, and eight (8%) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58% of patients at less than 5 years of follow-up and in 88% of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow- up is an important component of patient care.

Original languageEnglish (US)
Pages (from-to)934-942
Number of pages9
JournalArchives of Surgery
Volume127
Issue number8
DOIs
StatePublished - Aug 1992

Fingerprint

Hirschsprung Disease
Mortality
Enterocolitis
Colon
Ileostomy
Colostomy
Enema
Constipation
Down Syndrome
Rectum
Habits
Patient Care

ASJC Scopus subject areas

  • Surgery

Cite this

Hirschsprung's Disease : Evaluation of Mortality and Long-term Function in 260 Cases. / Rescorla, Frederick J.; Morrison, Albert M.; Engles, Drew; West, Karen W.; Grosfeld, Jay L.

In: Archives of Surgery, Vol. 127, No. 8, 08.1992, p. 934-942.

Research output: Contribution to journalArticle

Rescorla, Frederick J. ; Morrison, Albert M. ; Engles, Drew ; West, Karen W. ; Grosfeld, Jay L. / Hirschsprung's Disease : Evaluation of Mortality and Long-term Function in 260 Cases. In: Archives of Surgery. 1992 ; Vol. 127, No. 8. pp. 934-942.
@article{24667e6e455c46f1974f8879d83dfa6e,
title = "Hirschsprung's Disease: Evaluation of Mortality and Long-term Function in 260 Cases",
abstract = "This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82{\%}) and 47 girls (18{\%}). Age at diagnosis was younger than 30 days in 106 patients (41{\%}), 1 month to 1 year in 90 patients (35{\%}), and older than 1 year in 64 patients (25{\%}). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67{\%}), the left colon in 38 patients (15{\%}), and the proximal colon in 23 patients (9{\%}); 25 patients (9{\%}) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18{\%}). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95{\%}) (modified Duhamel in 185, Soave in25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8{\%} (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65{\%}) had normal bowel function, 28 (27{\%}) occasionally used enemas or stool softeners, and eight (8{\%}) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58{\%} of patients at less than 5 years of follow-up and in 88{\%} of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow- up is an important component of patient care.",
author = "Rescorla, {Frederick J.} and Morrison, {Albert M.} and Drew Engles and West, {Karen W.} and Grosfeld, {Jay L.}",
year = "1992",
month = "8",
doi = "10.1001/archsurg.1992.01420080068011",
language = "English (US)",
volume = "127",
pages = "934--942",
journal = "JAMA Surgery",
issn = "2168-6254",
publisher = "American Medical Association",
number = "8",

}

TY - JOUR

T1 - Hirschsprung's Disease

T2 - Evaluation of Mortality and Long-term Function in 260 Cases

AU - Rescorla, Frederick J.

AU - Morrison, Albert M.

AU - Engles, Drew

AU - West, Karen W.

AU - Grosfeld, Jay L.

PY - 1992/8

Y1 - 1992/8

N2 - This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82%) and 47 girls (18%). Age at diagnosis was younger than 30 days in 106 patients (41%), 1 month to 1 year in 90 patients (35%), and older than 1 year in 64 patients (25%). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67%), the left colon in 38 patients (15%), and the proximal colon in 23 patients (9%); 25 patients (9%) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18%). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95%) (modified Duhamel in 185, Soave in25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8% (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65%) had normal bowel function, 28 (27%) occasionally used enemas or stool softeners, and eight (8%) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58% of patients at less than 5 years of follow-up and in 88% of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow- up is an important component of patient care.

AB - This report describes 260 patients treated for Hirschsprung's disease. There were 213 boys (82%) and 47 girls (18%). Age at diagnosis was younger than 30 days in 106 patients (41%), 1 month to 1 year in 90 patients (35%), and older than 1 year in 64 patients (25%). Diagnosis was achieved with barium enema and rectal biopsy. Aganglionosis involved the rectum or rectosigmoid in 174 patients (67%), the left colon in 38 patients (15%), and the proximal colon in 23 patients (9%); 25 patients (9%) had total colonic aganglionosis. Enterocolitis occurred in 47 cases (18%). Following an initial colostomy or ileostomy, a definitive pull-through procedure was performed in 247 patients (95%) (modified Duhamel in 185, Soave in25, Swenson procedure in 15, and anomyectomy/sphincterotomy in 22); the overall survival rate was 93.8% (244 of 260 patients). An increased mortality was associated with Down syndrome, total colonic aganglionosis, and enterocolitis. Long-term follow-up (mean, 6 years 10 months) was available in 103 patients who underwent a Duhamel procedure. Sixty-seven (65%) had normal bowel function, 28 (27%) occasionally used enemas or stool softeners, and eight (8%) had severe constipation or soiling. Bowel habits improved with time and were considered normal in 58% of patients at less than 5 years of follow-up and in 88% of patients at more than 15 years of follow-up. The Duhamel operation is a very effective definitive procedure for Hirschsprung's disease. Long-term follow- up is an important component of patient care.

UR - http://www.scopus.com/inward/record.url?scp=0026756410&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0026756410&partnerID=8YFLogxK

U2 - 10.1001/archsurg.1992.01420080068011

DO - 10.1001/archsurg.1992.01420080068011

M3 - Article

C2 - 1642536

AN - SCOPUS:0026756410

VL - 127

SP - 934

EP - 942

JO - JAMA Surgery

JF - JAMA Surgery

SN - 2168-6254

IS - 8

ER -