An 18-year experience in total colonic aganglionosis: from staged operations to primary laparoscopic endorectal pull-through.
BACKGROUND
Traditionally, total colonic aganglionosis (TCA) was managed with enterostomy +/- pull-through. Since 1998, primary laparoscopic endorectal pull-through (PLEP) was offered to patients with TCA in our unit. A retrospective study was conducted and early results reviewed.


METHODS
Patient data were collected retrospectively. Before 1998, enterostomy followed by open Duhamel pull-through was performed. From 1998, patients were assessed to receive staged operation or PLEP.


RESULTS
From June 1990 to May 2007, 10 patients (6 males) were treated. All presented in the neonatal period. Seven patients, with transition zone within proximal 15 cm of terminal ileum, had pull-through performed. Three had staged pull-through, whereas 4 had PLEP. Two had extensive small bowel involvement with high output stoma pending reconstruction. One had total intestinal aganglionosis and died. The 3 patients with staged pull-through had normal bowel habit without soiling (mean follow-up, 13 years). For PLEP, all procedures were accomplished laparoscopically without intraoperative complication (mean operative time, 6 hours 50 minutes). The mean bowel motion is 5x per day, but most were too young to evaluate continence (mean follow-up, 3.5 years).


CONCLUSIONS
Primary laparoscopic endorectal pull-through is feasible in TCA with limited small bowel involvement and has the advantage of a single-stage operation, avoiding a stoma and its related complications.
Cheung
Sing-Tak
ST
Department of Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Hong Kong SAR, China. gloriacst@yahoo.com.hk
Tam
Yuk-Him
YH
Chong
Hoi-Man
HM
Chan
Kin-Wai
KW
Mou
Wai-Cheung
WC
Sihoe
Dart-Yin Jennifer
DY
Lee
Kim-Hung
KH
D000714:Anastomosis, Surgical / Q000379:methods; D013505:Digestive System Surgical Procedures / Q000379:methods; D004766:Enterostomy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000601:surgery*; D006801:Humans; D007082:Ileum / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D010535:Laparoscopy / Q000379:methods*; D008137:Longitudinal Studies; D008297:Male; D012007:Rectum / Q000601:surgery; D013536:Suture Techniques; D016896:Treatment Outcome