Anesthesia management of a pediatric patient with Wolf-Hirschhorn Syndrome: A case report

ANESTHETİC MANAGEMENT of A PEDİATRİC PATİENT WİTH WOLF-HİRSCHHORN SYNDROME: A CASE REPORT Assoc. Prof. Erol KARAASLAN Inonu University, Faculty of Medicine Department of Anesthesiology and Reanimation, Malatya, Turkey. E-mail: erkara44@hotmail.com, 05322034830 ORCID NO: 0000-0002-8534-3680 Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal anomaly characterized by deletion of the short arm of the 4th chromosome (1). Clinical findings are usually observed in the early period of life. (2,3). The mortality rate in the first two years is 17-30%(4). Increased incidence of malignant hyperthermia (MH) and congenital anomalies make anesthesia management special. We aimed to present our anesthesia experience in a patient with WHS in whom total intravenous anesthesia (TIVA) was planned for cleft palate surgery. Our 8 year old case, weighing 13 kg, who was followed up with the diagnosis of WHS and planned for cleft palate surgery. Clinical examination revealed growth retardation, cleft palate, micrognathia. His history included, epilepsy, secundum ASD and pneumonia attacks. For induction of anesthesia, 1mg/kg IV lidocaine, 2mg/kg IV propofol and 1mcg/kg IV fentanyl were administered. Rocuronium bromide was administered at a dose of 0.5mg/kg as a muscle relaxant. Intubation was achieved with a C-Mac videolaryngoscope and an endotracheal tube with a size 5.5 cuff and spiral styleon the first attempt. TIVA (0.5 µg/kg/min remifentanil and 75 µg/kg/min propofol) was used for anesthesia maintenance. Perioperative pulse rate was 170 beats/min for a short period of time. There was no other deterioration in hemodynamic data in the perioperative period. The operation lasted a total of 3.5 hours. Extubation was achieved 13 minutes after the end of surgery. We think that TIVA (Propofol, remifentanil) and C-MAC videolaryngoscopy as intubation device is a reliable method in the anesthetic management of children with WHS syndrome.