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

Authors : Erol Karaaslan

Pages : 136-139

Doi:10.21673/anadoluklin.1491983

View : 15 | Download : 33

Publication Date : 2025-01-29

Article Type : Other Papers

Abstract :Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal anomaly caused by the deletion of the short arm of chromosome 4. The leading causes of death are aspiration pneumonia, epilepsy, and infection. In this study, we aimed to present our anesthesia experience in a patient with WHS who was scheduled for cleft palate surgery and in whom we performed total intravenous anesthesia (TIVA). In the clinical examination of our 8-year-old patient with WHS, weighing 13 kg, who was planned to undergo cleft palate surgery. Growth retardation, cleft palate, micrognathia, epicanthus and hyptonia were present. Anesthesia induction was performed with 1 mg/kg i.v lidocaine, 2 mg/kg i.v propofol and 1 mg/kg i.v fentanyl, 0.5 mg/kg i.v rocuronium bromide. TIVA (0.5 µg/kg/min remifentanil and 75 µg/kg/min propofol i.v) was used for maintenance of anesthesia. In the perioperative period, the pulse rate was 170 beats/min for a short time. Extubation was achieved 13 minutes after the end of surgery. We think that TIVA (Propofol, remifentanil) and C-MAC videolaryngoscope as intubation device is a reliable method in the anesthetic management of children with WHS syndrome.
Keywords : Anestezi, havayolu yönetimi, laringoskop, Wolf-Hirschhorn sendromu