Splenic lymphangiomatosis in children

To study the clinical and imaging features of splenic lymphangiomatosis.

The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients.

Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast 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) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later.

Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.