Outcome of Total Colonic Aganglionosis Involving the Small Bowel Depends on Bowel Length, Liver Disease, and Enterocolitis

Objectives: Total colonic aganglionosis involving the small bowel is a rare form of Hirschsprung disease. We aim to analyse the long-term outcomes, digestive autonomy, and complications, to suggest recommendations for prevention and treatment. Methods: All patients born between 2000 and 2015 followed in our centre were retrospectively included. We analysed the length of aganglionosis, surgical procedures, growth, duration of parenteral nutrition (PN), enterocolitis, liver disease, intestinal transplantation. Results: Twenty-five patients were followed for a median of 10.9 years. Fifteen patients had less than 80 cm of ganglionic small bowel (SB) with a median of 20 cm. Ten patients had more than 80 cm of ganglionic sB with a median of 115 cm. The median PN duration was significantly shorter for patients with more than 80 cm: 0.9 versus 7.5 years in those with less than 80 cm (P < 0.001). No patient with less than 80 cm was weaned off PN, except 1 who underwent intestinal transplantation. Ten patients with less than 80 cm develop enterocolitis on the excluded segment, leading to emergency entero-colectomy in 5. Liver disease was more frequent in patients with less than 80 cm (11 vs 0). Three patients required combined liver-intestine transplantation; 2 underwent an isolated intestinal transplantation. Conclusions: Digestive autonomy was possible in most patients with more than 80 cm of ganglionic SB. The more severe complication was enterocolitis. Liver disease compromised long-term survival without transplantation. Both complications should be prevented by early diversion and enterectomy of the whole aganglionic segment. Follow-up in or together with a multidisciplinary intestinal rehabilitation centre is suggested.