SPLENIC LYMPHANGIOMATOSIS IN CHILDREN

PURPOSE: To study the clinical and imaging features of splenic lymphan giomatosis. MATERIALS AND METHODS: The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectivel y reviewed. The modalities used in the study included computed tomogra phy (CT) (10 patients), sonography (five patients), and magnetic reson ance (MR) imaging (two patients). Pathologic confirmation of lymphangi omatosis was obtained in nine patients. RESULTS: Splenic lymphangiomat osis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic dise ase as well. Only two children had clinical splenomegaly. Sonograms an d MR images showed multiple, well-defined cysts. Multiple, low-attenua tion lesions that did not enhance with intravenous administration of c ontrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) i mage obtained after administration of a bolus of contrast material. On e child underwent a splenectomy; one child underwent therapeutic embol ization. Eight patients remained asymptomatic with respect to the sple en 1-20 years later. CONCLUSION: Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imagi ng appearance. The recognition of this appearance helps in diagnosis o f this disease and may prevent the need for further invasive procedure s. Splenic changes can be isolated or can coexist with bone or soft-ti ssue lymphangiomas.