A rare presentation of tuberous sclerosis complex: Complicated renal cyst leads to sepsis

Abstract

Introduction: Tuberous sclerosis complex is a rare autosomal dominant genetic disease, having multi systemic involvement. Κidneys are affected up to 80% of patients, in the form of renal angiomyolipomas, renal cysts or renal cell carcinoma. Renal cysts are presented as single or multiple lesions that are infrequently symptomatic. Rarely, tuberous sclerosis coexists with polycystic kidney disease with poor prognosis through renal failure or hematuria. The complication of the renal cysts with the form of abscess is extremely rare. Here we present a case of tuberous sclerosis in which complicated renal cyst led to sepsis. Case Report: A 30-year-old female, with a history of tuberous sclerosis, presented with a week-long fever and a left pleuritic chest pain. Chest radiograph disclosed a left costophrenic angle blunting. Thoracic ultrasound followed, surprisingly revealed a big subdiaphragmatic renal cystic formation pressing against left hemidiaphragm, not previously known. While the patient was septic with no reply to the empirical antibiotic therapy and with a gradually worsening dyspnea, the cystic drainage was decided, resulted in a fast clinical improvement. Proteus mirabilis was cultured from cystic content. Conclusion: Renal disease is the most common cause of death in population affected by tuberous sclerosis. Thus, monitoring and serial radiologic examination are necessary to improve the prognosis of kidney damage. Abscess following simple renal cyst infection constitute another rare but potentially life-threatening renal complication should be taken under consideration. (This page in not part of the published article.) International Journal of Case Reports and Images, Vol. 7 No. 2, February 2016. ISSN – [0976-3198] Int J Case Rep Images 2016;7(2):106–110. www.ijcasereportsandimages.com Kotsiou et al. 106 CASE REPORT OPEN ACCESS A rare presentation of tuberous sclerosis complex: Complicated renal cyst leads to sepsis Ourania S. Kotsiou, Konstantinos I. Gourgoulianis