[Total colonic form of Hirschsprung disease. Apropos of 6 cases]

6 patients with total colonic aganglionosis without small bowel involvement (HCT) have been observed during a 13-years period. From this material and a review of literature, the authors stress the diagnostic and therapeutic particularities of HCT, which justify a separate analysis and a comparative study with the more typical forms of Hirschsprung's diseases. The percent of HCT out of all types of Hirschsprung's disease is 7.9%. Male to female ratio is 2:1 and familial occurrence is much higher (15 to 20%) than that seen in the classical form of Hirschsprung's diseases. A diagnosis of HCT is more difficult because of lack of specificity in clinical picture, radiologic findings and manometric evaluation. However, age at time of revelation is the neonatal period in 83% and enterocolitis is present in 25% with a higher incidence than in shorter types of Hirschsprung's disease. Therefore in HCT, laparotomy with appendicectomy and open frozen biopsies is always required for correct diagnosis. Ileostomy is mandatory. It must be done early and adequate in situation and technic. This kind of diversion has its own morbidity and mortality with a higher rate than that observed after colostomy. Definitive operation must be ideally performed at age 2. The principal of preserving and utilizing a segment of colon in a side-to-side anastomosis to the ganglionated small-bowel, followed by a pull-through ileoproctostomy has been established as a reasonable treatment for HCT since Martin's first description. 5 cases underwent a Martin repair and 1 a total colectomy.(ABSTRACT TRUNCATED AT 250 WORDS)