Splenic lymphangiomatosis: a rare cause of splenomegaly

A case of lymphangiomatosis of the spleen and adrenal in a 10-year-old girl is described. The patient had multiple recurrent lymphangiomata excised from her neck during the first 4 years of life. She presented with a recurrent cystic hygroma of the neck and was found to have an enlarged spleen. This increased in size during 5 months of observation, causing left upper quadrant discomfort, and was excised. The diagnosis, management, and prognosis of this rare disorder are discussed.