Case reportA 10-year-old boy with Marfan syndrome exhibiting cerebrovascular abnormalities
Introduction
Marfan syndrome is a congenital disorder of the connective tissue of the entire body. Its main signs and symptoms are skeletal and joint abnormalities, ocular signs and symptoms such as ectopia lentis, and cardiovascular system abnormalities. Anomalies of the heart and great vessels, in particular, often result in sudden death, and observation of the course of cardiovascular anomalies is therefore considered important. It is contradictorily whether Marfan syndrome is associated with an increased prevalence of intracranial aneurysms [1]. In this paper, we describe the cerebrovascular abnormalities revealed by magnetic resonance (MR) and computed tomography (CT) angiography in a 10-year-old boy with Marfan syndrome. His father and his aunts on his paternal side had abnormalities in cerebral vessels and the pulmonary artery.
Section snippets
Case report
The patient was a 10-year-old boy. Signs and symptoms of atopic dermatitis and bronchial asthma appeared when he was about 2 years old, and his course was observed by visits to an out-patient clinic and hospitalization. The patient's father died at the age of 39 from subarachnoid hemorrhage. A review of medical records revealed many cases of cerebral and pulmonary vessel abnormalities in family members on the father's side. The family tree is shown in Fig. 1. The patient's paternal grandmother
Discussion
MR cerebral angiography revealed elongation and tortuosity, but no dilation, of the vertebral and basilar arteries in the present case. If the arteries had also been dilated, they would be what are known as megadolichobasilar arteries [2]. The megadolichobasilar artery in young people has been reported [3] and some congenital factors may contribute to its development. However, to our knowledge, there are no reports of tortuosity of the basilar artery in a patient with Marfan syndrome as young
Acknowledgements
We wish to thank Professor Ishii (Kawasaki Medical School) for significant advice.
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