Factor XI deficiency and delayed hemorrhages after resection of choroid plexus papilloma: illustrative case.

BACKGROUND: Factor XI deficiency, also known as hemophilia C, is a rare inherited bleeding disorder that may leave routine coagulation parameters within normal range. Depending on the mutation subtype, prolonged activated partial thromboplastin time may occasionally be found. The disease has an autosomal transmission, with an estimated prevalence in the general population of approximately 1 in 1 million. Heterozygosis accounts for partial deficits, but the tendency to bleed is unrelated to the measured activity of factor XI. Diagnosis usually follows unexpected hemorrhages occurring spontaneously or after trauma or surgical procedures.

OBSERVATIONS: Few cases have been reported in the neurosurgical literature, all occurring spontaneously or after head trauma. Owing to its subtle features, the true incidence of the disease is probably underestimated. The authors report a case of a patient with previously undiagnosed factor XI deficiency who underwent uncomplicated resection of a fourth-ventricle papilloma and experienced delayed, severe hemorrhagic complications.

LESSONS: The known association between choroid plexus tumors and intracranial bleeding raised differential diagnosis issues. This report may serve to help to investigate delayed hemorrhages after cranial surgery.