Vascular complications (splenic and hepatic artery aneurysms) in the occipital horn syndrome: report of a patient and review of the literature

We report an 18-year-old boy with occipital horn syndrome who developed ane urysms of the splenic and hepatic arteries. Occipital horn syndrome, also c alled X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is chara cterised by a skeletal dysplasia which includes occipital horns, broad clav icles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified lo ng bones and coxa valga. Distinctive features common to all patients are un usual facial appearance, hypermobility of finger joints, limitation of exte nsion of elbows, chronic diarrhoea and genitourinary abnormalities. Tn this case report we describe the difficulties encountered in the diagnostic man agement of patients with EDS-related vascular lesions.