Proctocolectomy and ileal J-pouch anastomosis for familial adenomatous polyposis in a paediatric patient

Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, which presents with thousands of adenomas. Polyps appear in childhood or adolescence. Colectomy is the treatment of choice to prevent colorectal cancer. Decision on the timing for colectomy should be determined by polyp burden and characteristics of colonic adenomas in the context of social, personal, and educational factors. A panproctocolectomy and Ileal J-pouch-anal anastomosis for FAP in a 9-year-old girl was done jointly by the adult and paediatric colorectal team. The national multidisciplinary meeting recommended to proceed with a laparoscopic proctocolectomy and J-pouch reconstruction. The aim was to perform the procedure jointly by the adult and paediatric surgery team in a high-volume centre, to cover the pouch with a temporary ileostomy and do a hand-sewn anastomosis. Laparoscopically, the colon was mobilised and the proctectomy was performed. In lithotomy position, the incision was made on the dentate, a 5 cm circular dissection of a mucosal cuff, and a full thickness dissection of the rectum was performed. The pouch was created, a hand-sewn ileoanal anastomosis was performed. A joint paediatric-adult MDT approach is essential for any decision making, providing expertise-sharing cornerstone of the best outcome for the patient.