[Retroperitoneal lymphangiodysplasia with exudative enteropathy. A case of cystic dilated cisterna chyli and splenic cysts].

In a 16 years old girl we found the syndrome of exsudative enteropathy with intestinal lymphangiectasy which differs from previous reported cases by several malformations of the lymphatic system. Besides changes in the intestinal lymphatics there was a retroperitoneal lymphangiodysplasia, a reduction and hypoplasia of the lymph vessels in the legs and cysts in the spleen. By substitution of albumin followed by a diet with middle chained triglycerids (MCT), serum protein was stablised but a relapse could not be prevented. Surgical treatment was not possible because the changes were too extensive. The value of diagnostic measures for differential diagnosis of the intestinal lymphangiectasy is presented.