Résumé
Congenital factor Vll deficiency is a rare bleeding disorder with an estimated incidence of 1 in 500,000. It is inherited as an autosomal recessive pattern with variable expression and high penetrance. In severely affected patients, repeated hemarthroses, chronic crippling hemarthropathy, and dangerous hematomas can occur. Other types of hemorrhage include epistaxis, menorrhagia, hematuria, gastrointestinal and gingival bleeding. Fetal cerebral hemorrhage has been reported, although less frequently than in severe hemophilia A or B. It is characterized by normal partial thromboplastin time and prolonged prothrombin time. Definitive diagnosis rests on a specific assay for factor Vll clotting activity. Replacement therapy is necessary to control the hemorrhage. Conventional prophylaxis and therapy in this disorder have consisted of fresh frozen plasma (FFP) or prothrombin complex concentrate. We experienced a case of intraventricular hemorrhage and hydrocephalus in a 4-year-old girl who had been diagnosed with congenital factor Vll deficiency during her neonatal period. She presented with episodes of frontal headache, frequent vomiting and malnutrition. We report this case with a brief review and related literatures.