Abstract

Background: Emanuel syndrome (ES) is a rare hereditary disorder characterized by growth and mental retardation, congenital malformations, and craniofacial anomalies. There is limited data on the anesthetic management of this syndrome, all of which come from case reports. Report: A 7-year-old girl presented with urinary incontinence caused by tethered cord syndrome. Laminoplasty plus transection of the tight filum terminale to release the tethered conus medullaris under total intravenous anesthesia was performed. Discussion: Anesthesia management of patients with ES requires special attention due to the airway difficulties associated with micrognathia and palate anomalies and accompanying congenital malformations that primarily affect cardiovascular and neurologic systems. Monitoring the cardiac parameters and neurological signs during the surgery is of great importance for the patient safety. Conclusion: This case report is the first demonstrating total intravenous anesthesia in patients with ES, and may help to guide anesthesiologists in the anesthetic management of these patients.

Key words: Airway management, total intravenous anesthesia, difficult intubation, Emanuel syndrome