Splenic Lymphangioma

Abstract

Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from increases in the size and number of thin-walled lymphatic vessels that are abnormally interconnected and dilated. The condition may be restricted to the spleen, but in most cases it involves multiple organs (systemic lymphangiomatosis). The clinical picture is variable; small lesions are often incidentally detected through imaging studies, while larger lesions can result in compression of organs, causing pain or rupture even after minor trauma. Therefore, splenic lymphangiomas should be considered in the differential diagnosis of splenomegaly or left upper quadrant pain even among adults and should be immediately treated with splenectomy; delay in the therapeutic intervention can lead to life-threatening complications.

Isolated Splenic Lymphangiomas Presenting in an Infant with Isolated Anaemia

“…Spleen with lymphangiomas can be of normal size or enlarged, and the radiological findings are not specific; lymphangiomas are usually well defined and found as hypoechoic or anechoic cysts with septa or calcifications [1,2]. Having calcifications in the peripheral wall on imaging is indicative of lymphangiomas [1]. ey are also seen on CT scans as thin-walled cysts with low attenuation or contrast enhancement.…”

“…ey are also seen on CT scans as thin-walled cysts with low attenuation or contrast enhancement. Lymphangiomas are usually found in the subcapsular region but may be found in the parenchyma when the tumours are large [1,2]. Splenic cysts are presented on the CT scan as spherical and well-defined lesions that have an attenuation close to water with thin walls that have no enhancement [6].…”

“…Splenic lymphangiomas are mostly benign, and malignance transformation can rarely occur [1]. Total splenectomy is the treatment of choice for isolated splenic lymphangiomas as other options are not as effective [1,5] so that severe complications will be avoided such as splenic rupture and haemorrhage [2]. Most symptoms are nonspecific and depend on the size as the enlarged spleen can compress the adjacent tissue such as the renal tissue or the…”

“…Lymphangiomas are benign tumours of the lymphatic vessels. ey are typically at the neck and axilla in 95% of the cases [1]. ey mainly occur in children, but are rarely located in the spleen.…”

“…ey frequently affect many organs simultaneously in a context of a syndrome called "lymphangiomatosis". Patients hardly ever have isolated lymphangiomas of the spleen as only few cases were reported in the literature [1,2]. Lymphangiomas of the spleen are often asymptomatic and diagnosed incidentally by imaging [2].…”