Colo(ileo)rectoplasty in the treatment of Hirschsprung's disease and congenital neural malformations of the distal intestines

The objective of the presented paper is to define the characteristics of colo(ileo)rectoanastomosis for the treatment of Hirschsprung's disease (H. d.) and other congenital malformations in the innervation of the distal gut (CMDI). During 1979-1994 at the Clinic of Paediatric Surgery of the Second Medical Faculty in Prague-Motol 137 patients (100 boys and 37 girls), aged 5 months to 18 years with H. d. and CMDI were operated. In 124 patients Kasai's colorectoplasty was used, in 40 of them supplemented by partial sphincteromyectomy of the internal sphincter of the anus (SPME) and Swenson's transanal colorectal anastomosis. In 10 patients with total aganglionosis of the colon (TCA) in three instances ileorectoplasty and Martin's anastomosis was used, in seven instances only ileorectoplasty. In three patients the authors used Soave's endorectal pull-through. 85 patients (62.1%) had no postoperative complications. Early infection was recorded in 12 patients (8.6%), dehiscence of the surgical wound in seven patients (5.1%). Dehiscence of the colo(ileo)rectorectal anastomosis occurred in 13 patients (9.5%), stricture in 10 patients (7.2%). Postoperative obstruction of the gut was recorded in 7 (5.1%) patients, postoperative enterocolitis in three patients (2.2%). There were no deaths. Regular opening of the bowels after 1-2-day intervals was achieved in 110 patients (84.1%). Patients after surgery of TCA have on average 2-5 stools per day. Sixteen patients developed chronic constipation and subileous conditions. Incontinence of faeces was found in two patients with Down's disease. Colo(ileo)rectoplasty in H. d. and other CMDI should meet the following conditions: maximal resection of the affected portion--creation of a satisfactory anastomosis with a minimal occurrence of strictures and dehiscences--preservation of satisfactory continence--elimination of anorectal sphincteroachalasia. Modified Kasai's rectoplasty or Swenson's procedure meet these conditions. In case of TCA a 10-15 cm ileorectocolic anastomosis is quite sufficient. For reoperations of strictures and inflammatory complications after previous colo(ileo)rectoplasty Soave's technique is probably the best choice.