TY - JOUR
T1 - Long-term clinical outcome in patients with total colonic aganglionosis
T2 - a 31-year review
AU - Menezes, Maria
AU - Prato, Alessio Pini
AU - Jasonni, Vincenzo
AU - Puri, Prem
PY - 2008/9
Y1 - 2008/9
N2 - Background: The purpose of this study is to review the long-term clinical outcome in patients surgically treated for total colonic aganglionosis (TCA). Methods: The hospital records of 58 patients with TCA from 2 centers were retrospectively analyzed. These patients were then followed up by personal or telephonic interviews using a standardized questionnaire. Results: Of the 58 patients, 36 were male and 22 were female. Thirty-eight patients presented in the neonatal period. A total of 13 (22.4%) presented with enterocolitis, 43 (74.1%) with intestinal obstruction, and 2 (3.5%) with severe constipation. All patients had disease involving the entire colon up to at least the terminal ileum. Two patients had total intestinal aganglionosis and died after ileostomy. Various pull-through procedures performed in 56 patients included Soave in 16, Swenson in 5, and Duhamel in 35 patients. Postoperatively, 31 (55.4%) patients had enterocolitis. At long-term follow-up (2-31 years), 3 patients had opted for permanent ileostomies because of intractable incontinenece or recurrent enterocolitis. Eleven patients were too young to assess bowel function or unavailable for follow-up. Of the remaining 42 patients, 22 had normal bowel control and 20 were soiling. At 5 years, patients had an average of 5.2 bowel movements per day, which reduced to a mean of 3.4 per day at the age of 15 years. Conclusion: Patients with TCA continue to have long-term problems with bowel control, although many improve with time.
AB - Background: The purpose of this study is to review the long-term clinical outcome in patients surgically treated for total colonic aganglionosis (TCA). Methods: The hospital records of 58 patients with TCA from 2 centers were retrospectively analyzed. These patients were then followed up by personal or telephonic interviews using a standardized questionnaire. Results: Of the 58 patients, 36 were male and 22 were female. Thirty-eight patients presented in the neonatal period. A total of 13 (22.4%) presented with enterocolitis, 43 (74.1%) with intestinal obstruction, and 2 (3.5%) with severe constipation. All patients had disease involving the entire colon up to at least the terminal ileum. Two patients had total intestinal aganglionosis and died after ileostomy. Various pull-through procedures performed in 56 patients included Soave in 16, Swenson in 5, and Duhamel in 35 patients. Postoperatively, 31 (55.4%) patients had enterocolitis. At long-term follow-up (2-31 years), 3 patients had opted for permanent ileostomies because of intractable incontinenece or recurrent enterocolitis. Eleven patients were too young to assess bowel function or unavailable for follow-up. Of the remaining 42 patients, 22 had normal bowel control and 20 were soiling. At 5 years, patients had an average of 5.2 bowel movements per day, which reduced to a mean of 3.4 per day at the age of 15 years. Conclusion: Patients with TCA continue to have long-term problems with bowel control, although many improve with time.
KW - Long-term bowel function
KW - Total colonic aganglionosis
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U2 - 10.1016/j.jpedsurg.2008.01.072
DO - 10.1016/j.jpedsurg.2008.01.072
M3 - Article
C2 - 18779009
AN - SCOPUS:50849120426
VL - 43
SP - 1696
EP - 1699
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 9
ER -