Case series with literature review: Surgical approach to megarectum and/or megasigmoid in children with unremitting constipation

BACKGROUND: The role of surgery in treating children with functional constipation (FC) is controversial, because of the efficacy of bowel management programs. This case series is comprised of failures: 43 children, spanning 25 years' practice, who had megarectosigmoid (MRS) and unremitting constipation. PURPOSE: To determine whether these children were helped by surgery, and to contribute to formulating a standard of care for children with megarectum (MR) and/or redundancy of the sigmoid colon (MS) who fail medical management. METHOD: We describe our selection criteria and the procedures we utilized – mucosal proctectomy and endorectal pull-through (MP) or sigmoidectomy (SE) with colorectal anastomosis at the peritoneal reflection. The internet (social media) allowed us to contact most of these patients and obtain extremely long follow-up data. RESULTS: 30/43 patients had MP and 13/43 had SE. Follow-up was obtained in 83% MP and 70% SE patients. 60% of MP and 78% of SE patients reported regular evacuations and no soiling. 20% MP patients had occasional urgency or soiling or episodic constipation. 12% MP and 22% SE patients required antegrade continence enemas (ACE) or scheduled cathartics and/or stool softeners. 4% MP had no appreciable benefit, frequent loose stools and soiling, presumably from encopresis. CONCLUSION: MR is characterized by diminished sensation, poor compliance and defective contractility. Patients with MR do better with MP, which effectively removes the entire rectum versus SE, where normal caliber colon is anastomosed to MR at the peritoneal reflection; furthermore, MP reliably preserves continence; whereas total proctectomy (trans-anal or trans-abdominal) may cause incontinence.