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

Abstract

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. Management of pharmacoresistant epilepsy is more difficult in a patient with bleeding disorder due to increased risk of bleeding during surgery. There are no reported instances of invasive intracranial monitoring being conducted on patients with both a bleeding disorder and medically refractory epilepsy.

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 lab work. After abnormal labs 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 2M von Willebrand disease. He underwent surgery for subdural grid placement followed by resection on post-operative day 6. He required Wilate® (antihemophilic and von Willebrand factor complex) infusions from preoperative day 0 through post operative 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 FFP 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 with surgery.

Conclusion: The two patients successfully underwent invasive neuro monitoring with subdural grids and sEEG for seizure focus identification followed by resective epilepsy surgery without bleeding complications while achieving seizure freedom. Epilepsy patients with a bleeding disorder should not be denied surgery due to increased risk of hemorrhage, however they should undergo a thorough multi-disciplinary evaluation for the patient to safely undergo an invasive procedure.