Title

Vascular involvement in tuberous sclerosis

UMMS Affiliation

Department of Pediatrics

Publication Date

2010-8

Document Type

Article

Subjects

Aneurysm; Aorta; Aortic Aneurysm, Abdominal; Arterial Occlusive Diseases; Blood Vessels; Child, Preschool; Female; Follow-Up Studies; Humans; Hypertension; Infant; Infant, Newborn; Male; Renal Artery Obstruction; Time Factors; Tuberous Sclerosis

Disciplines

Nephrology | Pediatrics

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.

DOI of Published Version

10.1007/s00467-010-1466-5

Source

Pediatr Nephrol. 2010 Aug;25(8):1555-61. Epub 2010 Mar 13. Link to article on publisher's site

Journal/Book/Conference Title

Pediatric nephrology (Berlin, Germany)

Related Resources

Link to Article in PubMed

PubMed ID

20229188

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