High prevalence of extrarenal artery involvement in children with fibromuscular dysplasia - a single-center experience.

Abstract

Although the clinical presentation of fibromuscular dysplasia (FMD) and its generalized character has been previously described in adults, data on FMD in children are limited. In this study, we aimed to assess visceral artery involvement in pediatric FMD patients with documented renal artery stenosis (RAS) and renovascular hypertension (RVH) in comparison with healthy individuals. We retrospectively analyzed the results of angiographic studies of 16 patients with a median age of 13.9 years (10 girls) in comparison with 16 age- and sex-matched healthy controls. Out of the 16 FMD patients, 10 (63%) had stenotic lesions identifiable only in renal arteries, whereas six (37%) had additional stenoses in other vascular beds - in the celiac trunk (four patients), superior mesenteric artery (four patients), inferior mesenteric artery (one patient), splenic artery (one patient), common hepatic artery (three patients), and abdominal aorta (one patient). The comparison of ostial diameters of vessels, in which no periostial narrowing, stents, or occlusions were found, revealed that patients with FMD had a significantly smaller diameter of the celiac trunk (P = 0.017), splenic arteries (P = 0.007), and common hepatic artery (P = 0.026) than their age- and sex-matched healthy peers. We found that 69% of children with RVH caused by FMD had clinically silent stenoses or tortuosity of visceral arteries. The results of our study suggest that pediatric FMD is a generalized arterial condition, and the patients may need screening for both renal as well as nonrenal manifestations of the disease.