Spontaneous epidural and subdural hematoma in a child with afibrinogenemia and postoperative management

Kilit Y A., Yaman Y., Isguder R., Carti O., Demirag B., Agin H., ...More

BLOOD COAGULATION & FIBRINOLYSIS, vol.25, no.4, pp.398-400, 2014 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Article
  • Volume: 25 Issue: 4
  • Publication Date: 2014
  • Doi Number: 10.1097/mbc.0000000000000049
  • Journal Name: BLOOD COAGULATION & FIBRINOLYSIS
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.398-400
  • Keywords: afibrinogenemia, intracranial hematoma, coagulation disorder, FIBRINOGEN, THROMBOSIS, HEMORRHAGE
  • Dokuz Eylül University Affiliated: No

Abstract

Congenital afibrinogenemia is a rare coagulation disorder that exhibits recessive inheritance. The prevalence of this disease is around 1 per 1 000 000, but it is increased in countries where consanguineous marriages are common. Umbilical cord bleeding during the neonatal period is generally the first manifestation of the disease, but a later age of onset is not uncommon. This disease may also be manifested by gastrointestinal, genitourinary, mucosal, muscular, articular, and intracranial bleeding during childhood. Intracranial hemorrhage is a rare condition, but it is the leading cause of death in patients with afibrinogenemia. In this report, we present the case of a 13-year-old female patient with afibrinogenemia who underwent an operation for spontaneous massive extradural and subdural hematoma.