Fibromuscular dysplasia in children – a single-center experience

Objective: Fibromuscular dysplasia (FMD) is a stenotic disease of the arteries - most often renal, carotid, and cerebral. The disease can occur at any age and is the most common cause of renovascular hypertension in children. The study aimed to present the clinical course of FMD in patients of our Department. Design and method: In 2016-2023, the Department took care of 8 patients with FMD (4 boys, 4 girls). In all patients, we analyzed: age, sex, clinical symptoms, biochemical parameters, renin and aldosterone concentrations, imaging studies (ultrasonography, computed tomography), pharmacological and invasive treatment, and outcome. Results: Age at diagnosis ranged from 10/12 to 15 9/12 years. All patients were referred due to arterial hypertension (7/8 of 2nd degree). Patients presented various symptoms: hypertensive crisis with heart failure (patient 10/12 months), headache (2 patients), vomiting (1 patient), hyperactivity (1 patient), polydipsia and polyuria (1 patient). All patients had normal renal function, 5/8 hypokalemia, 1/8 hyponatremia, and 5/8 metabolic alkalosis; all had hyperreninemia with hyperaldosteronism. Imaging examinations showed critical stenosis of the renal artery trunk in 3 patients, slight stenosis in 1 patient, and stenosis of accessory arteries or branches of main arteries in the remaining patients. Stenosis in other vessels was found in 2 patients (left middle cerebral artery in one patient and abdominal aorta, celiac trunk, superior mesenteric artery, and inferior mesenteric artery in another one). Invasive treatment was performed in 6/8 patients - in 2 patients nephrectomy was necessary; in 2 – embolization of the vessel; in 2 – angioplasty, one patient is waiting for the procedure. Before the procedure, the patients required 3 (max. 5) antihypertensive drugs. After the procedure, antihypertensive treatment was discontinued in 3 patients, and the number and dose of drugs were reduced in the remaining patients. Conclusions: 1. Renal artery stenosis should be excluded in all pediatric hypertensive patients, regardless of age and symptoms. 2.Patients with FMD require an individually selected therapeutic plan. 3.Vessel embolization is an effective method of treating stenoses of the peripheral branches of the renal arteries in the course of FMD in children.