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

Abstract:

:We report an 18-year-old boy with occipital horn syndrome who developed aneurysms of the splenic and hepatic arteries. Occipital horn syndrome, also called X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is characterised by a skeletal dysplasia which includes occipital horns, broad clavicles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified long bones and coxa valga. Distinctive features common to all patients are unusual facial appearance, hypermobility of finger joints, limitation of extension of elbows, chronic diarrhoea and genitourinary abnormalities. In this case report we describe the difficulties encountered in the diagnostic management of patients with EDS-related vascular lesions.

journal_name

Pediatr Radiol

journal_title

Pediatric radiology

authors

Mentzel HJ,Seidel J,Vogt S,Vogt L,Kaiser WA

doi

10.1007/s002470050526

subject

Has Abstract

pub_date

1999-01-01 00:00:00

pages

19-22

issue

1

eissn

0301-0449

issn

1432-1998

journal_volume

29

pub_type

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