Hemophilia is generally considered a contraindication to deep brain stimulation (DBS) and other elective intracranial surgery due to the elevated risk of perioperative hemorrhage. Recent case reports of patients with Parkinsonian tremor have suggested, however, that DBS may be safe in patients with hemophilia who undergo appropriate factor replacement. Here, we describe the first case of DBS surgery for medically-refractory essential tremor (ET) in a patient with hemophilia A.
A 68-year-old right-handed man with mild hemophilia A presented for a ten-year history of bilateral (right greater than left), medically-refractory ET. The patient was considered an appropriate candidate for DBS by a multidisciplinary movement disorders conference, and hematology consultation was obtained. Baseline preoperative labs showed a quantitative factor VIII (FVIII) level of 38%. Perioperative management consisted of daily intravenous doses of recombinant FVIII from the morning of surgery to postoperative day ten. The patient underwent uncomplicated unilateral DBS placement in the left ventralis intermedius thalamus. Intraoperative and postoperative imaging showed no hemorrhage. His postoperative course was uncomplicated except for a single self-limited episode of hematuria requiring no intervention.
DBS placement for ET may be safe in patients with coexisting hemophilia A if appropriate FVIII replacement is given, which may be delivered as bolus infusions rather than continuous infusion.
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