Prognostic factors in children with total colonic aganglionosis treated with the Soave procedure: the experience of 43 patients from a single institution

Total colonic aganglionosis (TCA) is an uncommon and severe form of intestinal developmental malformation. The aim of this study is to identify the prognostic factors in children with TCA treated with the Soave procedure. Forty-three children with TCA who were treated with the Soave procedure between February 2008 and August 2013 were included in the study and followed up. Follow-up data were collected regarding growth development, stool frequency and consistency, incontinence, and enterocolitis. Parents of 31 (72.1%) of the 43 patients were followed up. Four patients underwent a primary pull-through procedure, while 27 patients underwent ileostomy and a later definitive operation. The remaining length of the small intestine was 164 ± 53 cm, and pathology revealed 22 cases with mature ganglion cells in the proximal intestine, while there were 9 cases with ecumenical mature ganglion cells in the proximal intestine. Growth development was normal in 19 patients, while mild malnutrition was observed in 8 patients, and moderate malnutrition was observed in 4 patients. Stool consistency was mushy or shaped in 25 patients and mushy or watery in 6 patients. Twenty-eight patients had a frequency of stool ranging between 1-5 bowel movements per day, and 3 patients had more than 6 bowel movements per day. Twenty-five patients suffered from less than 2 bouts of postoperative enterocolitis, and 6 patients suffered from more than 3 episodes of postoperative enterocolitis. Long-term outcomes of children with TCA who are treated with the Soave procedure are satisfactory with good anorectal function and quality of nutrition. The remaining length of the small intestine and the neurodevelopment of the proximal intestine ganglion cells are important prognostic factors.