[Treatment of the Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine, from 1979 to 2004].

BACKGROUND

Survey of surgical techniques, strategy and results of the treatment of patients with Hirschsprung's disease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from 1979 to 2004 is presented.

METHODS AND RESULTS

Paper summarises a twenty-five years long retrospective study of medical records of 274 patients who were operated on 1979-2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonic aganglionosis (LCA). 19 (6,4 %) pts had total colonic aganlionosis with small bowel involvement (TCA). 72 (26,3 %) pts suffered from ultra-short rectal aganglionosis (URA). 74 pts with CRA and LCA operated on 1979-1991 underwent Kasai's colorectoplasty. 8 (10,8 %) of them had anastomotic leak, 7 (9,5 %) pts had anastomotic stricture, 2 (2,7 %) pts had postoperative enterocolitis (PEC) and 10 (13,5 %) had chronic constipation. In 6 pts an additional partial sphincteromyectomy of the internal anal sphincter SFME) had to be done. In period 1991-2004 93 pts underwent Swenson's procedure with (SFME). Anastomotic leak occurred in 3 (3,2 %) pts. Anastomotic stricture, PEC and/or chronic constipation were not registered. In 6 pts with anastomotic leak from both groups Soave re-do pull-through was done. In 2003-2004, 10 pts with CRA underwent a transanal resection of aganglionic segment with Swenson's colorectoplasty. One pt developed anal stricture. 3 of 19 pts with TCA operated on in 1979-1990 underwent classic Martin's long side to side ileo-recto-colic anastomosis. In 16 pts of 1991-2004 only short ileo-recto anastomosis according to Kasai and/or Swenson were done. In 93 pts suffering from URA a Lynn's SFME was performed. Three-stage procedure for the last time was used in 3 pts in 1984. Two- and one-stage procedures are still used.

CONCLUSIONS

A number of postoperative complications decreased, a three-stage procedure was abandonned and the operation of HD is currently performed in newborn. In CRA a TAR replaced the laparatomy and in TCA the short ileo-rectal anastomosis is performed only.