Staged laparoscopic-assisted endorectal pull-through for long segment Hirschprung's disease and total colonic aganglionosis.

AIM

Recently laparoscopic endorectal pull-through (LERPT) has been widely performed for treatment of Hirschsprung's disease (HD) as a one stage procedure. In long segment aganglionosis (LSA) and in case of total colonic aganglionosis (TCA) a staged procedure can be preferred. The authors report their experience in the staged LERPT for LSA and TCA.

METHODS

In the last five years we treated 4 infants (3 male, 1 female) with LSA and TCA. The mean age at presentation was 40 days (2-110 days). In 3 patients we performed in the first step rectal biopsies, multiple laparoscopic biopsies and stoma. In one case ‑ presenting as small bowel obstruction - we performed an emergency laparotomy for ileostomy and biopsies of the bowel. Histology showed 2 left colon aganglionosis (LCA) and 2 TCA. The LERPT was performed at a main age of 10 months. After stoma closure the LERPT was performed according to Georgeson's technique.

RESULTS

There were no intraoperative complications. The patient with previous laparotomy needed conversion because of the abdominal adhesions. The two infants with LCA had preoperative and recurrent postoperative enterocolitis. In one case a successful posterior anorectal myectomy was performed. At follow up the children present constipation. The patient with TCA undergoing to LERPT presents a good frequency of defecation.

CONCLUSION

Our experience, although on limited series, shows that staged minimally invasive surgical treatment can be safely performed in the LSA and TCA, but conversion can be necessary especially in case of previous laparotomy.