Lymphangioma circumscriptum associated with paravesical cystic retroperitoneal lymphangioma.

A boy with long-standing congenital cutaneous lymphangioma circumscriptum (LC), presented at the age of 6 years with an episode of painless haematuria, subsequently attributed to bladder wall involvement of a paravesical cystic retroperitoneal lymphangioma. Magnetic resonance imaging demonstrated this lymphangioma to be in communication with the cutaneous lesions. Deep lymphatic and visceral involvement is an extremely unusual occurrence in association with LC. This is the first report of an association between LC and cystic retroperitoneal lymphangioma. LC occasionally heralds a deeper, more clinically significant lymphangioma which may not become apparent until many years after the initial dermatological presentation. We report this case to raise awareness of this potential association and to demonstrate the usefulness of magnetic resonance imaging in the assessment of the deep component of cutaneous lymphangiomas.