Renal manifestation of tuberous sclerosis (Bournevilles disease) : case presentation

Background: Tuberous sclerosis is a phacomatosis-type disease that causes the growt of non-malignant tumors in the brain as well as in other vital organs such as kidneys, heart, eyes, lungs, liver and skin. Abdominal symptoms usually include kidney disorders that can precede the appearance of symptoms from other organs. Kidney failure is also the main cause of deaths in adults over the age of 30. One of the most characteristic findings in diagnostic imaging of patients with tuberous sclerosis are multiple angiomyolipoma tumors and cysts of the kidneys. Patients with TSC are also more likely to develop clear cell renal cell carcinoma. Case Report: Presentation of a clinical case of a patient with tuberous sclerosis complicated by bleeding from the right kidney tumor treated by embolization. Conclusions: Diagnostic imaging (including ultrasonography, CT scans and MRI) plays a crucial role in diagnosis and management of visceral lesions in patients with TSC. Regular check-ups to manage the development of the disease are necessary, because tumors can be multiple and bilaterally located; they also tend to grow very rapidly and reach very large sizes. Retroperitoneal hemorrhage caused by tumor's rupture, also known as the Wunderlich syndrome, is a life-threatening complication of TSC.