Congenital chylous ascitis with intestinal lymphangiectasis

Abstract

Background. Chylous ascites is an infrequent entity that is rarely diagnosed in utero. Although intestinal lymphangiectasia is mentioned as one of the causes of chylous ascites, actually it is difficult to detect this relation.

Clinical case. We report a case of an infant diagnosed in utero by ultrasonography of fetal ascites and hydrocele. Physical examination after birth was significant for generalized oedema, ascites, and hydrocele. Aparacentesis yielded liquid of chylous characteristics. Duodenal biopsy showed dilated lymphatics and villous atrophy. Aformula containing most lipids as medium chain triglycerides resulted in a paulatine cessation of ascites and hydrocele.

Conclusions. Primary intestinal lymphangiectasia usually manifests as a protein-losing enteropathy, but it is also considered as part of a congenital disorder of the lymphatic system as a whole. This may explain its presentation as congenital chylous ascites.