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Pediatric Reports
  • Pediatric Reports is published by MDPI from Volume 12 Issue 3 (2020). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
  • Case Report
  • Open Access

6 August 2020

Renal Angiomyolipomatosis and Bleeding Aneurysms in a Tuberous Sclerosis Context: Selective Artery Embolization in a Girl with End-Stage Renal Failure

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1
Department of Biomedical and Clinical Science ”L Sacco”, University of Milan, Department of Pediatric Surgery, Children's Hospital V. Buzzi", Milan
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Operative Unit of Vascular Radiology and Endovascular Therapy, ARNAS Civico-Di Cristina-Benfratelli, Palermo
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Pediatric Unit, ARNAS Civico-Di Cristina-Benfratelli, Palermo
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Pediatric Nephrology Unit, ARNAS Civico-Di Cristina-Benfratelli, Palermo

Abstract

Recent developments in endovascular radiological techniques and devices have rendered embolization a major therapeutic option prior to surgery in many renal vascular or neoplastic diseases. A 19-yearold female patient, with a diagnosis of tuberous sclerosis complex (TSC) in childhood, was admitted with severe anemia. Polycystic kidney disease in end-stage renal failure appeared four years before and the patient has been undergoing peritoneal dialysis. The patient’s medical history also included bilateral renal angiomyolipomas (AMLs). One year earlier, a unilateral endovascular embolization was performed to repair a bleeding aneurysm at the right renal upper pole. A second bilateral ruptured renal aneurysm was diagnosed at admission. To continue with peritoneal dialysis and prevent intrarenal hemorrhage and intraperitonal bleeding, an urgent bilateral renal AE was performed. Two months later she underwent a bilateral retroperitoneal nephrectomy. The posterior surgical approach, preserved the peritoneal surface area and adequate conditions to continue dialysis. At histology, bilateral AMLs were confirmed and a renal cell carcinoma of the right kidney was concurrently discovered. She undergoes continuous peritoneal dialysis. Urgent selective renal AE represents a feasible treatment for bilateral AML bleeding. It is safe and feasible before performing nephrectomy in patients with end-stage renal failure.

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