Léčba Hirschsprungovy choroby na Klinice dětskéchirurgie 2. LF UK a FN Motol v letech 1979–2004

Treatment of the Hirschsprung’s Disease (HD) at the Department ofPediatric 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´sdisease (HD) at the Department of Pediatric Surgery of Charles University, 2nd Faculty of Medicine in Prague from1979 to 2004 is presented.Methods and Results. Paper summarises a twenty-five years long retrospective study of medical records of 274patients who were operated on 1979–2004. 173 (67,3 %) pts had classical rectosigmoid (CRA) and/or long colonicaganglionosis (LCA). 19 (6,4%) pts had total colonic aganlionosiswith small bowel involvement (TCA). 72 (26,3%)pts suffered from ultra-short rectal aganglionosis (URA). 74 pts with CRA and LCA operated on 1979–1991underwent 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 additionalpartial sphincteromyectomy of the internal anal sphincter SFME) had to be done. In period 1991–2004 93 ptsunderwent 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-dopull-through was done. In 2003–2004, 10 pts with CRA underwent a transanal resection of aganglionic segment withSwenson’s colorectoplasty.One pt developed anal stricture. 3 of 19 ptswithTCAoperated on in 1979–1990 underwentclassic Martin’s long side to side ileo-recto-colic anastomosis. In 16 pts of 1991–2004 only short ileo-rectoanastomosis according to Kasai and/or Swenson were done. In 93 pts suffering from URA a Lynn´s SFME wasperformed. Three-stage procedure for the last time was used in 3 pts in 1984. Two- and one-stage procedures arestill used.Conclusions. A number of postoperative complications decreased, a three-stage procedure was abandonned and theoperation of HD is currently performed in newborn. In CRA a TAR replaced the laparatomy and in TCA the shortileo-rectal anastomosis is performed only.

Key words:
Hirschsprung’s disease, intestinal aganglionosis, Swenson’s operation, postoperative enterocolitis,transanal resection.