Treatment of Multiple Oral and Oropharyngeal Venous Malformations in Maffucci's Syndrome with a Combination of Percutaneous Sclerotherapy and Ligation A Case Report

Akan H., Guneren E., Sesen T.

NEURORADIOLOGY JOURNAL, vol.21, no.1, pp.87-92, 2008 (ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 21 Issue: 1
  • Publication Date: 2008
  • Doi Number: 10.1177/197140090802100112
  • Journal Name: NEURORADIOLOGY JOURNAL
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus
  • Page Numbers: pp.87-92
  • Keywords: Maffucci's syndrome, venous malformation, percutaneous sclerotherapy, ethanol, congenital vascular anomalies
  • Ondokuz Mayıs University Affiliated: Yes

Abstract

Maffucci's Syndrome is an osteochondromatous syndrome with congenital vascular malformations. Major ailments involved are habitual fractures, length discrepancy of the extremities, impediment of the affected parts, extremity overgrowth and pain. The vascular lesions, aside from their mass effects, tend to ulcerate and bleed. Endotracheal intubation is also troublesome in these patients. Malignant tranformation of mesodermal tissue is the major lethal factor in this syndrome. This report describes severe respiratory impairment caused by venous malformations in a young girl. Multiple bunched venous malformations within the tongue, oral cavity and oropharynx were treated with percutaneous sclerotherapy and a simple ligation technique. Airway obstruction was cleared following repetitive treatment sessions.