An uncommon variant of Hirschsprung disease is total colonic aganglionosis, including the small intestines. For a study, researchers intended to examine long-term results, digestive autonomy, and problems in order to make preventative and treatment suggestions. All patients who were followed at the center between 2000 and 2015 were included retrospectively. They examined the duration of aganglionosis, surgical operations, growth, parenteral nutrition (PN) duration, enterocolitis, liver disease, and intestine transplantation.

A total of 25 patients were monitored for an average of 10.9 years. The median length of the ganglionic small bowel (SB) in 15 cases was 20 cm. Ten individuals had ganglionic sB more than 80 cm in length, with a median of 115 cm. The median PN duration was 0.9 years for patients with more than 80 cm compared to 7.5 years for those with less than 80 cm (P<0.001). Except for one patient who had intestine transplantation, no patient with less than 80 cm was weaned off PN. About 10 patients with less than 80 cm develop enterocolitis on the omitted segment, necessitating emergency enterocolitis surgery in five. Patients with fewer than 80 cm were likelier to have a liver illness (11 vs. 0). Three patients required a combination liver-intestine transplant, whereas two required an isolated intestinal transplant. Most individuals with more than 80 cm of ganglionic SB had digestive autonomy. Enterocolitis was the most serious complication. Without a transplant, long-term survival was hampered by liver illness.

Reference:journals.lww.com/jpgn/Abstract/2022/05000/Outcome_of_Total_Colonic_Aganglionosis_Involving.6.aspx