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The colon patch graft procedure for extensive aganglionosis: long-term follow-up
E Nishijima1, K Kimura, C Tsugawa
1Department of Surgery, Kobe Children's Hospital, Sumaku, Japan.
Insights
The colon patch graft (CPG) procedure effectively treats extensive aganglionosis, reducing ileostomy dependence and improving long-term outcomes. Patients show improved weight and bowel habits over time, though anemia monitoring is crucial.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Congenital Disorders
Background:
- Hirschsprung's disease, a form of extensive aganglionosis, requires surgical intervention.
- Long-term management of aganglionosis often involves ostomy procedures and subsequent reconstructive surgery.
Purpose of the Study:
- To evaluate the long-term effectiveness of the colon patch graft (CPG) procedure in treating extensive aganglionosis.
- To assess patient outcomes including nutritional status, bowel function, and overall development after CPG surgery.
Main Methods:
- The study involved 11 patients with extensive aganglionosis treated with an initial ileostomy and subsequent colon patch graft (CPG) procedure.
- A side-to-side ileocolostomy (CPG) was created, followed by a Swenson-type definitive operation in 10 patients.
- Patients were followed for 5 to 17 years, monitoring growth, development, and bowel habits.
Main Results:
- The CPG procedure allowed discontinuation of intravenous nutrition within 1 month, enabling home treatment before definitive surgery.
- After definitive surgery, patients achieved normal body weight for age within 2-4 years.
- Long-term follow-up revealed improved bowel habits over time, with most patients having 1-3 daily bowel movements and no incontinence; however, four patients developed iron deficiency anemia.
Conclusions:
- The colon patch graft (CPG) procedure is effective in managing extensive aganglionosis, significantly reducing "ileostomy diarrhea" and shortening the need for intravenous nutrition.
- Bowel habit patterns and body weight normalize over time post-definitive surgery.
- Long-term surveillance for iron deficiency anemia is essential in patients treated with the CPG procedure.
Purpose:
During the last 17 years, the authors have used the colon patch graft (CPG) procedure to treat 11 patients with extensive aganglionosis. This study evaluates the effectiveness of the CPG procedure in treating patients with this disorder.
Methods:
An initial ileostomy was created during infancy in all 11 patients. At 1 to 21 months (mean, 5.5 months) of age, an 11- to 40-cm (mean, 18 cm) side-to-side ileocolostomy (CPG) was created between the ileum and aganglionic ascending colon, (including aganglionic ileum in three patients). Ten patients later underwent a Swenson-type definitive operation, the CPG segment being brought down to the anus at 9 to 30 months (mean, 19 months) of age. These 10 patients have been followed up for growth, development, and bowel habit patterns for the subsequent 5 to 17 years (mean, 12 years).
Results:
Within 1 month after the CPG was created, intravenous nutrition could be discontinued and the patients treated at home until time for the definitive procedure. During this period, one patient died of aspiration. After the definitive operation, body weight for age returned to a normal range in 2 to 4 years. Currently, seven older patients have one to three bowel movements per day, whereas three younger patients have explosive diarrheal defecations. None are incontinent of stool. Iron deficiency anemia developed in four patients. Two are mentally retarded, but the others are doing well in school.
Conclusions:
(1) The colon patch graft procedure effectively reduces "ileostomy diarrhea," shortening the period of intravenous nutrition to 1 month, (2) bowel habit patterns improve with the advance of time, (3) body weight for age returns to normal 2 to 4 years after the definitive procedure, and (4) during long term follow-up, patients must be evaluated for iron deficiency anemia.