Clinical Outcomes After Staged and Primary Laparotomy Soave Procedure for Total Colonic Aganglionosis: a Single-Center Experience from 2007 to 2017

Purpose To compare the clinical outcomes of the staged laparotomy Soave procedure (SLSP) and primary laparotomy Soave procedure (PLSP) for patients with total colonic aganglionosis (TCA), including some patients who underwent surgical treatment after the age of 1 year. Methods A retrospective, comparative study of TCA patients who underwent SLSP or PLSP at Beijing Children Hospital from 2007 to 2017 was conducted. The primary outcomes were height-for-age (HFA), weight-for-age (WFA), and bowel function score (BFS); the major types of postoperative complications were also analyzed. Results Thirty-five patients (SLSP = 14, PLSP = 21) were included. The patients in the SLSP group underwent an enterostomy at a median age of 47 (14, 104) days. The median age at the time of the Soave procedure showed no significant difference between the two groups ([181 (131,346) vs 55 (29, 343)] days,p = 0.072). No significant differences between groups were noted in terms of presenting symptoms, perioperative results, and postoperative outcomes. Twenty-three patients (SLSP = 8, PLSP = 15) were followed for more than 1 year. Both the SLSP and PLSP groups showed similar operative mortality ([1/14, 7.1%] vs [3/21, 14.3%],p = 0.635) and complication rates ([postoperative enterocolitis, (5/8, 62.5%) vs (5/15, 33.3%),p = 0.221] [perianal excoriation, (7/8, 87.5%) vs (10/15, 66.6%),p = 0.369]). Nineteen patients (19/23, 82.6%) had normal growth according to the median percentage of HFA or WFA. Mean BFS was 15.9 +/- 3.3 in the 15 patients aged older than 4 years who were followed up. Six patients (SLSP = 1, PLSP = 5) who underwent surgical treatment aged older than 1 year were all alive with good outcomes. Conclusion PLSP and SLSP had equivalent clinical outcomes, but enterocolitis and perianal excoriation after the Soave procedure need to be managed carefully. PLSP is a feasible option for older TCA patients after conservative treatment.