A Case of Extraction Surgery for Supernumerary Teeth under General Anesthesia Management with Gillespie Syndrome

Abstract

Gillespie syndrome is a congenital neurological disorder characterized by nonprogressive cerebellar ataxia, partial aniridia, and intellectual disability. It is an extremely rare disease, with fewer than 30 individuals affected globally. We report a case of a 9-year-old male patient with Gillespie syndrome to whom general anesthesia was administered in order to perform a tooth extraction for a maxillary impacted supernumerary tooth. The patient presented with symptoms of Gillespie syndrome, including cerebellar hypoplasia, partial aniridia, cervical hypoplasia, and intellectual disability. General anesthesia was slowly induced with sevoflurane. The intubation dose of muscle relaxants was reduced considering the decreased muscle tone from cerebellar ataxia, and the effect was equivalent to that of the standard dose. Tracheal intubation was performed using a video laryngoscope due to cervical hypoplasia. In addition, patients with Gillespie syndrome are prone to aspiration due to dysphagia caused by cerebellar ataxia, so intraoral suctioning was performed immediately before extubation, and EEG monitoring confirmed that the patient had awoken from anesthesia before extubation. After leaving the operating room, the patient was hospitalized for observation to prevent aspiration, and safe perioperative management was ensured. The key considerations for general anesthesia management in patients with Gillespie syndrome are：there is a risk of prolonged effect of muscle relaxants due to decreased muscle tone caused by cerebellar ataxia；there is a risk of aspiration during the perioperative period due to dysphagia and the effects of anesthesia；and some patients may have a variety of clinical symptoms in addition to the triad of signs. Patients with Gillespie syndrome should be thoroughly examined preoperatively, monitored and evaluated for muscle relaxants and sedation levels, and managed postoperatively on an inpatient basis to avoid aspiration.