Goldenhar 증후군 환자의 마취경험 - 증례보고

Abstract

Goldenhar syndrome is a rare congenital disorder of hemicraniofacial and vertebral defects related to the abnormal development of the first and second branchial arches. In some infants, congenital heart disease, and pulmonary and renal defects are also present. Goldenhar syndrome commonly involves an airway which is difficult to manage. Tracheal intubation may be difficult due to a combination of retrognathia, micrognathia, mandibular hypoplasia, palatal defects and vertebral abnormities. We experienced the anesthetic management of an 8-month-old boy with Goldenhar syndrome who received excision and biopsy for conjunctival dermoid and ureteral reimplantation for vesicoureteral reflux under general anesthesia. Airway management, with thiopental, rocuronium and sevoflurane, and tracheal intubation were accomplished successfully in the patient. Surgery was performed uneventfully and the patient was discharged without complication.