Abstract
Introduction: Afibrinogenemia is a hereditary type I fibrinogen by quantitative defect with total plasma absence since. The clinical manifestations are varied and may present mild, moderate or severe hemorrhagic manifestations, as well as thrombosis. The diagnosis may be suspected by the changed coagulogram. Treatment will be made with fibrinogen replacement. Case report: A 6-year-old male patient was admitted to the Emergency and Pediatric Emergency Department due to severe headache and increased head circumference following trauma to the parietal region of the head. Minor has hereditary afibrinogenemia diagnosed at 4 months of age. Skull tomography showed an increase of the cutaneous tissue of the scalp in a symmetrical way in all its extension, without presence of bone lesion or intra-cranial affection. Patient was treated with Cryoprecipitate and Fibrinogen and was discharged with improved condition after 6 days of treatment and follow-up. Discussion: Afibrinogenemia is inherited as an autosomal recessive, which means that both parents must carry the defective gene in order to transmit it. The hemorrhagic episodes, although early, are usually post-traumatic, and the affected individuals may remain asymptomatic for long periods. Although potentially fatal intracranial hemorrhage may occur, the prognosis of afibrinogenemia is favorable with early diagnosis and appropriate treatment. Since afibrinogenemia is a disease without cure and with control, it is of fundamental importance the orientation from the diagnosis of the disease so that the accidents can evolve with trauma and cause bleeding that lead the patient to death.