Comprehensive review of procedures for total colonic aganglionosis

A comparison of the effectiveness of the Soave and Martin procedures for the treatment of total colonic aganglionosis

“…Because of the advances in surgical techniques, early diagnosis, awareness of enterocolitis, which is the main reason for death with TCA, and extensively improved postoperative care, the mortality rate has decreased greatly in recent years and the overall mortality was 1.9%, as reported by Marquez and his coworkers [9]. Ieiri et al [11] reported that the greater is the extent of aganglionosis, the greater is the morbidity, ranging from 33.5% to 83.3% over 30 years.…”

“…However, in the recent years, the straight endorectal pull-through techniques such as the Soave procedure, which consists of removing the mucosa and submucosa of the rectum and pulling the ganglionic intestine through the aganglionic muscular cuff, as described by Goto et al [18], has a more favorable morbidity profiles compared with that of reservoir procedure. However, a system review by Marquez et al [9] found that no procedures proved to lead to superior outcomes. In our study, the patients in the Soave group experienced a shorter period of postoperative fever and fewer days in hospital, a lower incidence of intraoperative blood transfusion, abdomen wound infection, and anastomotic leakage compared with those in the Martin group.…”

“…Some pull-through procedures initially described by Swenson, Duhamel, and Soave and the modifications of these procedures were preformed for the treatment of TCA half century ago [6][7][8]. Although a marked decrease in the overall mortality rate was reported in recent years after the procedure mentioned above, considering the postoperative complications and function outcomes, there is still no consensus on a superior operation procedure now [9]. Before 2006, the Martin procedure was mainly performed for the treatment of TCA in our hospital.…”

Is complete resection of the aganglionic bowel in extensive total aganglionosis up to the middle ileum always necessary?

“…We have published a review of 55 cases of classic Hirschsprung disease with a mortality of 0% [8], and the mortality rate in our current series of total aganglionosis cases is 13.3%. Marquez et al [1], in a comprehensive review, showed a mortality rate of 1.9% after the definitive operation. Cheung et al [9] published a review of 10 cases in 18 years in which 4 patients were submitted to 1-stage procedure at a mean age of 27 days.…”

“…this patient group, the morbidity and mortality are higher than that in the classic form of Hirschsprung disease [1,[3][4][5].…”