Giant angiomyolipoma with Wunderlich’s syndrome in a patient with tuberous sclerosis

Abstract

A 25-year-old male presented with right loin pain of two weeks duration. On examination, he had adenoma sebaceum over face (Figure 1), gross pallor, severe tenderness in right lumbar region and a right loin lump extending into hypochondriac and iliac regions. Contrast enhanced computed tomography (CT) of the abdomen revealed right side enlarged kidney with massive subcapsular right renal haematoma (Figure 2) and multicentric nodules with of fat densities (< 20 Hounsfield units) within both the renal parenchyma, suggestive of bilateral renal angiomyolipomas. CT of the brain showed subependymal nodules (Figure 3). This pattern of cerebral ependymal nodules together with adenoma sebaceum was diagnostic of tuberous sclerosis. Patient was resuscitated and planned right sided renal artery embolization but the procedure failed due to altered renal hilar anatomy due to displacement by haematoma.