Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings

Vargel I., Cil B., Kiratli P., Akinci D., Erk Y.

BRITISH JOURNAL OF RADIOLOGY, vol.77, no.915, pp.197-203, 2004 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 77 Issue: 915
  • Publication Date: 2004
  • Doi Number: 10.1259/bjr/88438282
  • Journal Name: BRITISH JOURNAL OF RADIOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.197-203
  • Hacettepe University Affiliated: Yes

Abstract

Benign vascular lesions can be classified into two categories depending on clinical behaviour and endothelial cell characteristics: neoplasms (haemangiomas) and vascular malformations. However, intraosseous vascular anomaly, previously called intraosseous haemangioma, is a very rare malformation. In our previous study, we described the first hereditary form of intraosseous vascular malformation of the craniofacial region, vascular malformation osseous (VMOS). Characteristic findings are autosomal recessive inheritance, severe and diffuse intraosseous vascular malformation in all craniofacial bones without soft tissue involvement and associated mid-line abnormalities such as umbilical hernia and supra-umbilical raphe. In this paper, we discuss the imaging findings of this new disorder in detail.