[Initial manifestation of primary intestinal lymphangiectasis as acute abdomen]

A 32-year-old man had ten years previously undergone several laparotomies for recurrent ileus of the small intestine. They revealed severe intestinal oedema and histology showed hyperplasia of the lymphatic system but the aetiology was unclear. After a 10-year interval free of symptoms he presented with marked hypoproteinaemic oedema and exudative enteropathy the cause of which was to be clarified by exploratory laparotomy with excision of lymph nodes and a small section of small intestine.

Histology revealed intestinal lymphangiectasis with partly hyperplastic lymphoid tissue. Lymphangiography demonstrated several lymph nodes in the region of the aortic bifurcation and renal vessels with a central filling defect. It is thought likely that obstruction to lymphatic flow in this region resulted in oedema of the intestinal wall which caused the recurrent episodes of ileus.

Retrospectively it is assumed that primary intestinal lymphangiectasis was responsible for the initial manifestation of an acute abdomen. For treatment of hypoproteiaemia with human albuminea prot system was implanted. The course was complicated by recurrent inflammation and thrombosis of the port catheter. Because of immune deficiency risk of carcinoma is high in primary intestinal lymphangiectasis.

Primary intestinal lymphangiectasis, even though a rare condition, should be considered in the differential diagnosis of otherwise unclear acute abdomen.