Pediatric Nephrology

, Volume 25, Issue 8, pp 1555–1561 | Cite as

Vascular involvement in tuberous sclerosis

  • Ann E. Salerno
  • Olivera Marsenic
  • Kevin E. C. Meyers
  • Bernard S. Kaplan
  • Jeffrey C. Hellinger
Brief Report

Abstract

Vascular involvement in tuberous sclerosis (TS) is rare. Central and peripheral aneurysms and large and medium size arterial stenotic-occlusive disease have been reported in patients with TS. We present here three pediatric patients with TS and severe vascular abnormalities, followed by a review of the literature. The three cases include a 14-month-old girl with polycystic kidneys and cerebral tubers who had a large asymptomatic abdominal aortic aneurysm, a 2-year-old boy with multiple features of TS who had hypertension and was found to have mid-aortic syndrome with bilateral renal artery stenosis, and an 18-year-old girl with abdominal pain and TS features who had greater than 70% celiac artery stenosis. In all cases, noninvasive vascular imaging modalities were utilized for either initial diagnosis, surveillance, or both. These cases highlight the collaborative roles of the pediatric nephrologist and cardiovascular imager in the diagnosis and management of the vascular complications in TS patients. Appropriate care can only be made through a high index of suspicion.

Keywords

Aortic aneurysm Celiac artery Computed tomography angiography Mid-aortic syndrome Magnetic resonance angiography Tuberous sclerosis Vascular Vascular ultrasound 

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Copyright information

© IPNA 2010

Authors and Affiliations

  • Ann E. Salerno
    • 1
  • Olivera Marsenic
    • 1
  • Kevin E. C. Meyers
    • 1
  • Bernard S. Kaplan
    • 1
    • 3
  • Jeffrey C. Hellinger
    • 2
  1. 1.Division of NephrologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Department of RadiologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.Division of Nephrology, Department of PediatricsThe Children’s Hospital of Philadelphia and the University of PennsylvaniaPhiladelphiaUSA

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