Vascular involvement in tuberous sclerosis.
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.
Division of Nephrology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Ann E
AE
Salerno
Olivera
O
Marsenic
Kevin E C
KE
Meyers
Bernard S
BS
Kaplan
Jeffrey C
JC
Hellinger
D000783:Aneurysm / Q000150:complications; D001011:Aorta; D017544:Aortic Aneurysm, Abdominal / Q000150:complications*; D001157:Arterial Occlusive Diseases / Q000150:complications*; D001808:Blood Vessels; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006973:Hypertension / Q000150:complications; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012078:Renal Artery Obstruction / Q000150:complications*; D013997:Time Factors; D014402:Tuberous Sclerosis / Q000150:complications*