Abstract
Hemophilia A is an X-linked hereditary hemorrhagic disorder caused by a defect or deficiency in blood clotting factor Ⅷ. Its main treatment involves periodic replacement of the clotting factor ; however, inhibitors can diminish the effects of treatment. Patients with hemophilia, particularly those receiving inhibitors, must be monitored for bleeding during the perioperative period. Here, we report the anesthetic management of a patient with autism spectrum disorder (ASD) and hemophilia A who was receiving inhibitors.
The patient was an 8-year-old boy. He was being treated with replacement therapy using bypassing agents thrice weekly. Dental treatment was scheduled to be performed under general anesthesia.
We continued to administer a bypassing agent (FEIBA) to manage the bleeding tendency. During the perioperative period, we administered two bypassing agents (FEIBA and Novoseven), carefully intubated the patient, and recommended that the operator use tight sutures. To enable the patient to adapt to the ward and staff, we admitted him to the hospital one day before the scheduled operation.
Prior to the induction of the general anesthesia, we administered FEIBA. Anesthesia was induced using thiopental, remifentanil, atropine, and rocuronium and was maintained using propofol and remifentanil. Additional replacement using Novoseven was provided 6 hours after the administration of FEIBA. On postoperative day 1, FEIBA was periodically administered.
Since patients with ASD have impairments in interpersonal and environmental adaptability, keeping them rested following an operation can be challenging. Hence, patient information should be shared with the involved physicians and staff members to ensure a comfortable and less stressful experience for the patient.
