Postoperative outcome and survival in relation to small intestinal involvement of total colonic aganglionosis

Abstract

Background: The prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement.

Methods: Medical records of TCA patients treated in Helsinki University Children's Hospital during 1984-2013 (n=21) were reviewed.

Results: The aganglionosis extended up to cecum or distal ileum (n=12), mid small bowel (n=4), or duodenojejunal flexure (n=5). Patients underwent resection of distal aganglionic bowel with ileoanal (IAA) or jejunoanal anastomosis (JAA) with (n=9) or without J-pouch (n=5), Lester-Martin pull-through (n=1), or were left with an end-jejunostomy (n=6). Further procedures included autologous intestinal reconstruction (n=3) and ITx/listing for ITx (n=2). Compared to distal ileum aganglionosis, patients with more proximal disease required parenteral nutrition (PN) more often (100% vs. 25%) and weaned off PN less frequently (p=0.001). At last follow-up 6.5 (interquartile range 2.5-14.5) years postoperatively, all patients with distal ileum aganglionosis were off PN and alive compared to 78% on PN (p<0.001) and 67% alive (p=0.063) of those with more proximal disease. All had normal plasma bilirubin and patients with preserved intestinal continuity (n=13) were continent.

Conclusions: Outcomes following restorative proctocolectomy for aganglionosis extending up to mid small bowel are promising, whereas long-term outlook in proximal small intestinal disease is dismal without ITx.

Keywords: Autologous intestinal reconstruction procedures; Hirschsprung disease; Intestinal failure; Intestinal transplantation; Short bowel syndrome.