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General anesthesia for a child with Goldenhar syndrome

SIR—Goldenhar, in 1952, described the classical triad of epibulbar dermoids, preaurical tags and blind fistulas and vertebral anomalies (1). Difficulty in tracheal intubation may result from a combination of mandibular hypoplasia, craniovertebral anomalies, and hemifacial microsomia. Radiological evaluation for mandibular deformity and craniovertebral anomalies is recommended (2). We describe the anesthetic management of a child with Goldenhar syndrome. A 4.5-year-old, 18 kg, girl previously diagnosed with Goldenhar’s Syndrome was scheduled for ectropion surgery. The manifestation of Goldenhar’s syndrome were right sided maxillofacial hypoplasia, mandibular hypoplasia, right microtia, right epibulbar demoid. She had no vertebral abnormalities or cardiac disease. Blood tests and chest radiography were normal. The patient had been operated 2 years previously because of right epibulbar dermoids. There was no information about difficulty in tracheal intubation in this operation. Mouth opening was limited because of the mandibular hypoplasia. Because we suspected that tracheal intubation would be difficult, we decided to anesthetize and intubate the patient without muscle relaxants using propofol and remifentanil. Special attention was paid to the possibility of difficulties in airway management. After preoxygenation, a continuous infusion of remifentanil 1 lÆkgÆmin was started. Anesthesia was induced with propofol 2.0 mgÆkg and maintained with sevoflurane 1% in oxygen. Lung ventilation was achieved via a facemask. Laryngoscopy was performed using a Macintosh 2 blade. First attempt at visualization of the larynx and oral intubation of the trachea was unsuccessful. After the first attempt, it was decided to exchange the curved blade for straight (Miller) blade and use a stylet for a blind technique. A 4.5 mm tracheal tube with a curved stylet was guided in the presumed direction of the glottis. The tube slid off the stylet but would not pass into the trachea. We exchanged the tube for 4.0 mm and the intubation was performed successfully at the next attempt, confirmed by auscultation and capnometry. Anesthesia was maintained with sevoflurane 1 and 50% O2/N2O and the lungs ventilated with volume controlled mechanical ventilation (Cicero, Draeger, Lübeck, Germany). The remifentanil infusion was maintained at 0.15 lÆkgÆmin. Muscle relaxants were not required. Anesthesia, which was uneventful, lasted for 160 min, after which the patient was extubated. The postoperative course was uneventful. Anesthesiologists should be aware of the possibility of difficulty in airway management, cardiovascular and renal disorders and neuromusculer problems in Goldenhar’s syndrome. Evaluation of upper and lower airway obstruction, pulmonary function, laryngeal, renal, cardiac and neurological function is recommended (2). There are previous reports of anesthesia in patient with Goldenhar’s syndrome pointing out difficulties with airway management. A retrograde intubation method was used successfully in a 5-month-old infant with Goldenhar’s syndrome by Cooper and Murray–Wilson (3). Bahk et al. described four different cases of difficult airway including one with Goldenhar’s syndrome. They reported use of a laryngeal mask airway and fiberoptic intubation via the laryngeal mask (4). Sculerati et al. assessed airway management in children with major craniofacial anomalies in a retrospective study (5). They found craniofacial synostosis patients (Crouzon, Pfeifer, or Apert syndrome) had the highest rate of tracheostomy (48%), mandibulofacial dysostoses patients (Treacher collins or Nager syndrome) had the next highest rate (41%) but patients with oculo-auriculovertebral sequence were less likely to undergo tracheostomy (22%). Before anesthetizing this patient, we anticipated that tracheal intubation might be difficult, avoided using muscle relaxants and expected a rapid return of spontaneous ventilation if intubation had failed. Awakening time from remifentanil based anesthesia is significantly faster than inhalational anesthesia alone (6). This method could be considered if there is possibility of difficulty in airway management. Fatis Altintas Ozlem S. Cakmakkaya Department of Anaesthesiology and Reanimation, Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey (email: sercakmakkaya@hotmail.com or serpilcakmakkaya@yahoo.com)