[Ileocaecal valve incompetence. A new syndrome (author's transl)]

Out of series of 237 colonic aganglionisms (1965--1977) 32 cases (13%) showed a clincial, radiological and manometric syndrome of "incompetent ileocaecal valve". Symptoms were vomiting, constipation with bouts of diarrhea, incomplete intestinal obstruction and failure to thrive. Sixteen cases were under P-3 weight percentiles. Initial diagnosis were aganglionism (10 cases), hiatal hernia (4 cases) and N.E.C. (1 case). Seventeen patients were explored through an iliac incision in the first three months of life. Ileocaecal continence was minimal (less than 25 cm. of water pressure). Ilocaecoplication was performed and completed with internal sphincterectomy (10 cases), colostomy (1 case) and caecostomy (3 cases). This new syndrome has been experimentally studied, but its etiology remains obscure. It introduces a bad prognostic component in aganglionism. Ileocaecoplication, a very simple operation, can be a lifesaving procedure.