Invasive Intracranial Electroencephalography Monitoring in the Child with a Bleeding Disorder: Challenges and Considerations.

INTRODUCTION

Hereditary bleeding disorders stem from the absence or insufficient levels of particular clotting proteins, essential for facilitating coagulation in the clotting cascade. Among the most prevalent are hemophilia A (deficiency of factor VIII), hemophilia B (deficiency of factor IX), and von Willebrand disease (VWD). Management of pharmacoresistant epilepsy is more difficult in a patient with bleeding disorder due to increased risk of bleeding during surgery. While patients who have both a bleeding disorder and epilepsy are rare, reporting on the management of these patients who may require intracranial monitoring for pharmacoresistant epilepsy offers valuable insights into the challenges and considerations necessary for safely navigating the complex intersection of bleeding risk and seizure control.

CASE PRESENTATION

Two patients with bleeding disorders (VWD and factor XI deficiency) underwent invasive intracranial monitoring for medical refractory epilepsy followed by epilepsy focus resection surgery. Both patients were found to have a bleeding disorder during their preoperative laboratory work. After abnormal laboratories were reported, both patients were referred to hematology for further evaluation and surgical planning. The first patient was a 10-year-old boy with medically refractory focal epilepsy who was found to have type IIM VWD. He underwent surgery for subdural grid placement followed by resection on postoperative day 6. He required wilate® (human von Willebrand factor/coagulation factor VIII complex) infusions from the day of surgery prior to surgery through postoperative day 14. The second case was a 2-year-old boy with a history of tuberous sclerosis and medically refractory epilepsy who was found to have factor XI deficiency (hemophilia C) who required fresh frozen plasma and platelet transfusions throughout his hospitalization. He underwent surgery for sEEG followed by resection of the tubers. Both patients remained stable throughout their invasive monitoring and completed epilepsy resection surgeries without reported complications. Both patients achieved seizure freedom after surgery since their most recent follow-up of 1 month and 13 months.

CONCLUSION

The two patients successfully underwent invasive neuromonitoring with subdural grids and sEEG for seizure focus identification followed by resective epilepsy surgery without bleeding complications while achieving seizure freedom. While epilepsy patients with a bleeding disorder should not automatically be denied surgery due to the increased risk of hemorrhage, it is crucial that any decision is based on a comprehensive, multidisciplinary evaluation. This case report highlights the potential for future meta-analysis and further conversations regarding improved protocols for patients with bleeding disorders.