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

BackgroundThe prognosis and treatment of total colonic aganglionosis (TCA) vary according to the presence and extent of small bowel involvement.MethodsMedical records of TCA patients treated in Helsinki University Children’s Hospital during 1984–2013 (n = 21) were reviewed.ResultsThe 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.ConclusionsOutcomes 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.