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.