Current Approaches to Pediatric Polyposis Syndromes

Abstract

Colorectal adenomatous polyposis syndromes encompass a diverse group of disorders with varying modes of inheritance and penetrance. Children may present with overt disease or within screening programs for families at high risk. We provide an overview of the array of pediatric polyposis syndromes, current screening recommendations, and surgical indications and technical considerations. Optimal disease management for these pediatric patients is still evolving and has implications for screening, surveillance, pediatric surgical management, and transition of care gastroenterologic neoplasia physicians and surgeons.

Keywords: IPAA; pediatric polyposis; restorative proctocolectomy.

Fig. 1

Stapled ileoanal anastomosis. ( A ) The anastomosis is undertaken with the circular stapler as indicated. ( B ) Shorter cuff lengths are possible with this technique (©Mayo Clinic).

Fig. 2

Maneuvers to increase mesenteric reach in ileal pouch anal anastomosis. ( A ) Initial mobilization of the ileal mesentery away from the retroperitoneum by optimal dissection to the mesenteric root of the SMA (IC artery, ileocolic artery; RC, right colic artery; –MC, middle colic artery). ( B ) This exposes the aorta (ao), duodenum (D), right ureter (Ur), and gonadal vessels (GV). ( C ) The mesenteric reach can be further extended by scoring the peritoneum stepwise. ( D and E ) Ligation of the ileocolic with preservation of the distal SMA can provide further reach for the ileal J pouch (©Mayo Clinic).