Wunderlich’s Syndrome in Advanced Tuberous Sclerosis Complex: A Rare Case of Perinephric Hematoma

Abstract

Tuberous sclerosis complex (TSC) is a rare, autosomal dominant disease, associated with mutations in either the TSC gene. The incidence of TSC is estimated as 1 in 5,000-10,000 live births and angiomyolipomas (AML) is one of the characteristic findings of tuberous sclerosis. A 36-year-old Caucasian female admitted to the hospital after two episodes of pre-syncope in March 2016. The patient was tachycardic on examination, and laboratory studies revealed acute anemia with hemoglobin of 8.2 g/dL on admission with a decrease to a nadir of 6.2 g/dL. Imaging studies revealed bilateral fat-rich renal masses with distortion of the renal anatomy consistent with angiomyolipomas (AML). Further as a part of treatment embolization of the right renal artery was performed. However, everolimus is also approved for the treatment of AMLs not requiring surgery. Each patient should have an individualized treatment plan based on the clinical situation as determined by the physician.