PURPOSE: In this study, functional results with regard to fecal continence levels and other parameters were studied in 22 patients with congenital pouch colon associated with anorectal agenesis (CPC) more than 3 years old who had undergone definitive pull-through surgery 1 to 13 years earlier. An attempt was made to formulate treatment protocols for management of fecal incontinence and other problems associated with CPC. METHODS: The study sample consisted of 14 males and 8 females. Three of the 8 female patients had had a cloacal malformation. The medical records of the patients were scrutinized and they were classified into 4 subtypes based on the length of normal colon proximal to the colonic pouch. The patients were further categorized into 3 groups based on the terminal bowel that had been pulled-through, namely, the ileum or colon proximal to the colonic pouch or a tubularized segment of the colonic pouch. The somatic growth of the patients was studied. Clinical assessment of fecal continence was performed by the Kelly and the Kiesewetter and Chang scoring systems. A computed tomographic scan of the pelvis with a barium enema was performed to assess the terminal bowel and its placement as well as the bony and muscular anatomy of the pelvis. The urinary system was assessed by a clinical history as well as by abdominal ultrasound and a micturating cystourethrogram. Various treatment modalities including dietary modifications, drugs, and enemas were instituted in patients with poor continence levels, and the response to treatment studied. RESULTS: Thirteen patients (59.2%), all with an ileal pull-through, had height and weight less than 50% of that expected for their ages. Overall fecal continence was "poor" in 17 patients and "fair" in only 5 patients. Patients with pull-through of either ileum or normal colon often had very frequent passage of liquid or semisolid stools, whereas the 4 patients with pull-through of tubularized colon had infrequent passage of semisolid stools with abdominal distension and bloating. One of these 4 patients had massive colonic redilatation necessitating surgical correction. Mucosal prolapse and perineal excoriations were frequent findings. Ultrasonography and micturating cystourethrogram showed hydroureteronephrosis and vesicoureteric reflux in 5 patients. Radiologic assessment revealed that there were no significant sacral abnormalities and the striated sphincteric musculature was well developed, although the levator ani was thinner than normal in 15 patients (68%). The bowel was very well placed in the sphincteric complex in 19 patients (86%). In 7 of the 13 patients who had pull-through of normal ileum or colon, some improvement in continence levels was seen 3 to 6 months after institution of dietary measures, loperamide, and saline-water enemas. Two of 3 patients with pull-through of tubularized colon improved to some extent with colonic washouts alone. Overall, quality of life was poor in the 22 patients. CONCLUSIONS: Despite the fact that the sacrum is usually normal, the sphincteric musculature well developed, and the terminal bowel well placed without any anal strictures, long-term prognosis with regard to fecal continence, growth and development, and quality of life appears to be dismal for all subtypes of CPC, irrespective of the type of definitive surgery performed. Corrective measures also appear to be of limited value. Various newer management modalities for management of fecal incontinence may be considered, but in several patients a permanent abdominal stoma may be a more practical solution.
PURPOSE: In this study, functional results with regard to fecal continence levels and other parameters were studied in 22 patients with congenital pouch colon associated with anorectal agenesis (CPC) more than 3 years old who had undergone definitive pull-through surgery 1 to 13 years earlier. An attempt was made to formulate treatment protocols for management of fecal incontinence and other problems associated with CPC. METHODS: The study sample consisted of 14 males and 8 females. Three of the 8 female patients had had a cloacal malformation. The medical records of the patients were scrutinized and they were classified into 4 subtypes based on the length of normal colon proximal to the colonic pouch. The patients were further categorized into 3 groups based on the terminal bowel that had been pulled-through, namely, the ileum or colon proximal to the colonic pouch or a tubularized segment of the colonic pouch. The somatic growth of the patients was studied. Clinical assessment of fecal continence was performed by the Kelly and the Kiesewetter and Chang scoring systems. A computed tomographic scan of the pelvis with a barium enema was performed to assess the terminal bowel and its placement as well as the bony and muscular anatomy of the pelvis. The urinary system was assessed by a clinical history as well as by abdominal ultrasound and a micturating cystourethrogram. Various treatment modalities including dietary modifications, drugs, and enemas were instituted in patients with poor continence levels, and the response to treatment studied. RESULTS: Thirteen patients (59.2%), all with an ileal pull-through, had height and weight less than 50% of that expected for their ages. Overall fecal continence was "poor" in 17 patients and "fair" in only 5 patients. Patients with pull-through of either ileum or normal colon often had very frequent passage of liquid or semisolid stools, whereas the 4 patients with pull-through of tubularized colon had infrequent passage of semisolid stools with abdominal distension and bloating. One of these 4 patients had massive colonic redilatation necessitating surgical correction. Mucosal prolapse and perineal excoriations were frequent findings. Ultrasonography and micturating cystourethrogram showed hydroureteronephrosis and vesicoureteric reflux in 5 patients. Radiologic assessment revealed that there were no significant sacral abnormalities and the striated sphincteric musculature was well developed, although the levator ani was thinner than normal in 15 patients (68%). The bowel was very well placed in the sphincteric complex in 19 patients (86%). In 7 of the 13 patients who had pull-through of normal ileum or colon, some improvement in continence levels was seen 3 to 6 months after institution of dietary measures, loperamide, and saline-water enemas. Two of 3 patients with pull-through of tubularized colon improved to some extent with colonic washouts alone. Overall, quality of life was poor in the 22 patients. CONCLUSIONS: Despite the fact that the sacrum is usually normal, the sphincteric musculature well developed, and the terminal bowel well placed without any anal strictures, long-term prognosis with regard to fecal continence, growth and development, and quality of life appears to be dismal for all subtypes of CPC, irrespective of the type of definitive surgery performed. Corrective measures also appear to be of limited value. Various newer management modalities for management of fecal incontinence may be considered, but in several patients a permanent abdominal stoma may be a more practical solution.