Abstract
Background: Congenital pouch colon (CPC) is a rare, complex anorectal malformation often associated with various anomalies. Timely diagnosis and recognition of anatomical variations are crucial for effective management. Late referrals and complications result from limited awareness of this condition.

Aims and Objectives: The present study was planned with the objective to evaluate the patients presenting with CPC in terms of – initial presentation; associated anomalies; surgical management – single/staged surgery; and outcomes in terms of complications and continence.

Materials and Methods: A study on 54 patients diagnosed with CPC found that most undergo staged surgical management, starting with a pouch ostomy followed by a definitive surgery like abdomino-PSARP or abdominoperineal pull through. Window colostomy is preferred due to its ease and less anesthesia exposure. Follow-ups are conducted 3 weeks after surgery, with regular washouts and uroprophylaxis. Patients with adequate weight, good general condition, and no signs of sepsis underwent definitive surgery, including laparotomy, fistula ligation with pouch excision, and normal bowel pull through. The second stage of surgery was performed after achieving adequate weight gain and good pouch volume. Follow-ups are conducted 3–4-week postsurgery, and patients undergo stoma closure 6–8 weeks after surgery.

Results: Presentation typically occurred on the 2nd day of life in male patients (56.66%), while females predominantly presented in the neonatal period (75%). Abdominal distention was the most common presentation (83.33%). Due to delayed presentation with features of sepsis, most patients underwent staged surgeries, with 85.18% receiving a diversion procedure, mainly window colostomy. Definitive surgery was typically planned after the age of 10–12 months, based on weight, general condition, and pouch volume. Colporrhaphy was performed to achieve an adequate length of colon, with 55.55% of patients undergoing colporrhaphy with abdominalperineal (AP) pull-through and 33.33% undergoing posterior sagittal anorectoplasty with colporrhaphy and AP pullthrough. The most common complications included pouch ostomy stenosis (11.11%) and perineal excoriation (9.25%), both showing improvement over time. Continence was evaluated using Kelly’s score, revealing good continence in 35.3% of patients and fair continence with occasional soiling in 64.7% of patients.

Conclusion: CPC is a challenging condition requiring multidisciplinary care and staged surgeries. Early diagnosis is vital to prevent complications. The prognosis varies by CPC type, with Types III and IV CPC showing better long-term outcomes due to a normal colon with better absorption capacity. Proper surgical approaches can lead to fair continence, and long-term follow-up is necessary to address complications and fertility concerns in female patients with genital abnormalities.

Key words: Congenital Pouch Colon; Clinical Presentation; Surgical Evaluation; Complications