Surgical implications of congenital chloride diarrhoea.

Congenital chloride diarrhoea (CCD) is an inherited inborn error of metabolism. Hydramnios, premature birth, no passage of meconium and a distended abdomen are the typical features of this disease in the neonate. Loss of Cl- from the intestines leads to urine-like diarrhoea, severe dehydration and alkalosis. Without treatment the mortality rate is high. The abdominal distension may erroneously be ascribed to an intestinal obstruction and thereby lead to unnecessary operations and delay of adequate treatment. There is an overrepresentation of intestinal volvulus in reported cases. Three of the five cases of CCD seen at our departments exemplify these surgical implications.