How commonly do children with complex cerebral arteriopathy have renovascular disease?

To describe the frequency of renovascular abnormalities and hypertension in an opportunistic cohort of children with complex cerebrovascular disease from a single tertiary/quaternary referral centre.

This was a retrospective case note and imaging review of children who had had cerebral and renal angiography, with a diagnosis of moyamoya or other occlusive cerebrovascular disease (OCVD). Hypertension was defined as at least three systolic blood pressure readings of the 95th centile or above.

Of 34 children (12 males, 22 females; median age 5y 11mo, range 2mo-15y 3mo; 20 with moyamoya, 14 with OCVD), primary presentation was neurological in 29 (arterial ischaemic stroke, transient ischaemic attack, or headache) and with hypertension in five. Renovascular abnormalities were identified in 17, of whom 10 had main renal artery stenosis. Renovascular involvement was not predictable according to arteriopathy diagnosis. Blood pressure was rarely plotted on centile charts. Using the 50th height centile for blood pressure, and based on a median of five systolic blood pressure readings per patient, 20 out of 34 met the definition for hypertension (15/29 patients with primary neurological presentation).

Renovascular abnormalities were common in this group of children with complex cerebrovascular disease. Blood pressure was frequently abnormal but rarely measured and infrequently plotted on centile charts. Neurologists should be alert to potential systemic vascular involvement and its sequelae in children with complex cerebrovascular disease.