Reversible String of Pearls-Like Spasm of the Right Iliac Artery in a Young Woman with Williams–Beuren’s Syndrome after Receiving Contrast Agent during Cardiac Catheterization

Objectives: Williams–Beuren’s syndrome is a contiguous gene syndrome as a result of a deletion in chromosome 7q11.23 leading to an elastin arteriopathy which can cause stenosis or other alteration in all arteries. Patients with this syndrome have been reported to be at high risk for anesthesia-related adverse events in medical interventions. However, to date, the reasons for this effect are not finally known.

Case Report: We describe a 25-year-old woman with Williams–Beuren’s syndrome suffering from abdominal angina due to severe stenosis of the superior mesenteric artery visualized by computed tomography. Cardiac catheterization was performed for further clarification and therapy planning. Apart from severe long segment stenosis of superior mesenteric artery minimal supravalvular aortic stenosis, this examination showed mild mid-aortic syndrome and moderate stenosis of the truncus celiacus and left renal artery.

There was a sudden drop in blood pressure recorded from the femoral line. Otherwise, the patient remained stable. Evaluating the cause of blood pressure drop showed no stenosis of the coronary arteries and normal ventricular function. Blood pressure in the descending aorta was normal. But injection of contrast agent into the right femoral artery had led to a string of pearls-like spasm of right iliac artery, which was nearly reversible following the administration xylocaine 1% in the artery.

Conclusion: After receiving contrast agent, this patient with Williams–Beuren’s syndrome displayed a string of pearls-like spasm in an otherwise morphologically normal right iliac artery. Such vascular spasms related to medical interventions in patients with this syndrome can lead to fatal outcome if they affect coronary arteries.