Two Cases of Congenital Deficiency of Coagulation Factor XIII Causing Intracerebral Hemorrhage

Abstract

Two cases of intracranial hemorrhage due to the congenital deficiency of coagulation factor XIII were experienced.
The first case was a 25-year-old man with an intracerebral hemorrhage in the right parieto-occipital region and was treated conservatively by hematological replacement therapy. The second case was a 30-year-old woman with an intracerebral hemorrhage in the parietal region which was surgically treated. Both cases were completely cured.
Since Duckert described the first case of the congenital deficiency of coagulation factor XIII in 1960, 93 cases have been reported in the literature. The clinical picture of the intracranial hemorrhage based upon factor XIII dificiency is characterized by a tendency of spontaneous rebleeding 48 hours to several days after temporary cessation of bleeding. Therefore, no case of surgical treatment has been reported yet.
The second case showed unconsciousness and intracranial hypertension and was treated by craniotomy and removal of the intracerebral hematoma. Replacement therapy with a factor XIII preparation was given before, during and after the operation. No side effects of the factor XIII replacement therapy were noted.
Surgical therapy for the congenital deficiency of coagulation factor XIII might be safe if the patient is controlled by hematological replacement therapy.