[Case of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis and angiomyolipomas of the liver and kidney].

Abstract

A 17-year-old woman, who complained of left abdominal pain, was diagnosed as left renal bleeding by ultrasonography at emergency room in Shimane University Hospital. Further ultrasonography revealed numberless round-shaped hyperechoic liver nodules (maximum 16 mm in a diameter) and dissemination of the same type of nodules in both kidneys, which was characterized as angiomyolipomas. The lesions were also detected by abdominal CT and MRI. In addition, the findings of tuberous calcification of the cerebral ventricles and parenchyma, facial angiofibromas and shagreen patches led to the diagnosis of tuberous sclerosis complex. Since two years later, she had recurrent attacks of spontaneous pneumothorax. The histopathological examination found out lymphangioleiomyomatosis from the biopsied lung tissues. Although she became pregnant, she selected a therapeutic abortion due to the high risk for renal failure which would be induced by pressure of the grown uterine. Abdominal ultrasonography was very useful for diagnosis in this case.