Management of Anesthesia in Goldenhar Syndrome: Case-Series Study


1 Department of Anesthesiology and Intensive Care, Labaffinejad Hospital,

2 Department of Anesthesiology, Anesthesiology Research Center, Shahid Beheshti University M.C., TEHRAN-IRAN


Goldenhar syndrome or oculo-auriculo-vertebral dysplasia (OAVD) is characterized by a wide range of congenital anomalies including ocular, auricular, facial, cranial, vertebral and cardiac abnormalities. It is associated with the anomalous development of the first and second branchial arches. Patients with this syndrome usually suffer from unilateral maxillary and mandibular hypoplasia and vertebral anomalies which often result in limitation of neck movement. For this reason, intubation is very difficult in these patients and anesthesiologists usually face difficulty in airway management. Newborns with this syndrome often have premature internal organs, low birth weight and airway disorders. As a result, a safe anesthesia in such patients requires a complete knowledge regarding the metabolism and side effects of anesthetic drugs. Our first case was a preterm 28-day old female with a fetal age of 28 weeks, birth weight of 1,200 g and gestational age of 32 weeks. At the time of admission, she weighed 1,500 g. She developed jaundice shortly after birth for which she underwent exchange transfusion. She was hospitalized in NICU for 14 days. Our second case was a 2 kg, 20-day old newborn with a fetal age of 37 weeks. Our third case was a full term 10-month old infant weighing 8 kg and our forth case was a 14 kg, 29- month old child who was a candidate for emergency surgery of dermoid cyst and bilateral upper lid coloboma. Anesthesia induction was performed by inhalation anesthesia with N2O/O2= 50% and sevoflurane (0.5-3.5%); and in BIS (Bispectral Index) = 43, the appropriate size LMA (laryngeal mask airway) was inserted. Anesthesia was maintained by using N2O/O2=50% and sevoflurane (1-2.5%). The BIS value was maintained in the range of 42-47. The surgical operations lasted for about 60-150 minutes. Patients regained consciousness 5 minutes after the completion of surgery and were transferred to the recovery room with stable vital signs. They were transferred to the ward 90 minutes later. Patients were discharged from the hospital the next morning with no post-up complications. (Tanaffos 2009; 8(4): 43-50)