Lymphangioleiomyomatosis Presenting with Perirenal Hemorrhage

Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder of unknown etiology, primarily affecting women. The disease mainly affects the lung and is characterized histologically by a diffuse proliferation of smooth muscle cells in the walls of the airways, venules and lymphatic vessels. Proliferation of these cells leads to vascular and airway obstruction, cyst formation and a progressive decline in respiratory function. The most frequent extrapulmonary manifestations include retroperitoneal lymphadenopathies, lymphangioleiomyomas, renal angiomyolipomas and chylous ascites. A symptomatic extrapulmonary manifestation of LAM, preceding pulmonary symptoms and/or diagnosis, is extremely rare. In this paper we describe a patient in whom LAM was incidentally discovered when she presented with spontaneous perirenal hemorrhage (Wunderlich syndrome) from a ruptured renal angiomyolipoma.