Goldenhar Syndrome: Airway and Anesthetic Management – A Case Report

Goldenhar syndrome, also known as oculo-auriculo-vertebral dysplasia, is a rare congenital disorder. This syndrome with malformations of face, eyes and ears was first recorded by the nineteenth-century German physician Carl Ferdinand von Arlt (1812-1887) but was first described by Dr. Maurice Goldenhar in 1952 [1]. The syndrome occurs randomly, with no apparent cause. Most cases of Goldenhar syndrome are not inherited, however few families show unclear inheritance pattern. The male to female ratio is 2:1 and its occurrence is estimated from 1 in every 3,000 to 1 in every 5,000 live births. This syndrome mostly involves face, ears, eyes and vertebrae and is characterized by a wide spectrum of symptoms and physical features that range from mild to severe and is often associated with difficult intubation due to characteristic asymmetrical malformations of the face. Usually children with Goldenhar syndrome have normal life span [1-4].

A 10-year-old female child presented to the ophthalmic outpatient department of our hospital with history of decreased vision and painless swelling of the left eye since birth. On ophthalmic examination, visual acuity in the right eye was 6/6 but best corrected visual acuity in the left eye was 6/60 which did not improve with refraction. There was inferolateral limbal dermoid of about 5×5 mm size with 30 to 35 degree divergent squint of left eye (Figure 1). Fundoscopic examination and intraocular pressure were normal in both eyes but extra-ocular movements of the left eye were restricted. The physical examination showed facial asymmetry due to under development of left maxilla and normally appearing mandible. Airway evaluation showed Mallampatti class II with restricted movements of head and neck, which suggested possibility of difficult laryngoscopy and intubation. On ear examination, the child had multiple preauricular skin tags in front of left ear, lying in line joining tragus and angle of mouth (Figure 2). The external ears were normal in shape and size. Her hearing was normal. Cardiovascular and central nervous system examinations were normal with no other associated congenital anomaly. Lateral view of the cervical spine X-ray showed fused third to fifth cervical vertebral bodies with loss of intervertebral disc (Figure 3). X-ray chest, skull and computed tomography of head were normal. Haemogram, bleeding time, and clotting time were within normal limits. Fenigold and Baun criteria were used to diagnose her as a case of Goldenhar syndrome as she had pre-auricular appendages on left side, infero-lateral limbal dermoid of left eye and vertebral anomalies.

 Airway and Anesthetic Technique

In preanesthetic assessment, she was evaluated for airway by mouth opening, Mallampatti classification which was MP-II, thyromental distance, and neck movement. Nasal patency was checked for both nostrils. The fiberoptic intubation technique under sedation was selected for airway management during surgery. A written informed parental consent was taken after discussing risks and management of difficult airway with parents and ophthalmic surgeon. Local anesthetic, lignocaine sensitivity test was done. The ‘difficult airway cart’ with percutaneous tracheostomy set, was kept ready. The intravenous infusion of ringer lactate was started at 6 to 8 mL kg ^-1 and standard monitors for heart rate, systemic arterial blood pressure, pulse oximetry and oxygen saturation (SpO2), electrocardiography (ECG), capnography and temperature were attached. The topical anesthesia was achieved by nasal packing with lignocaine 4% and xylometazoline 0.1% nasal drops, instilled in both nostrils 15 minutes prior to procedure.  The pharynx was sprayed with 4 to 6 puffs of lignocaine 10% aerosol. She was premedicated with intravenous glycopyrrolate (.01mg kg^-1), fentanyl (1μg kg^-1) and midazolam (.05mg kg^-1). After preoxygenation with 100% oxygen for three minutes, fiberoptic intubation (Pentax –PMS, FI 10P2, Pentex Corporation, Medical instrument division, Japan) was performed under sedation, by nasal route with 5.5 mm portex cuffed endotracheal tube. During procedure the sedation was supplemented with fentanyl. The correct positioning of tracheal tube was confirmed with capnograph and was firmly secured after confirming the equal bilateral air entry by auscultation. Anesthesia was induced with intravenous propofol (1%) in a dose of 2 mg kg^-1,sufficient to abolish the eyelash reflex and was maintained with vecuronium .08 mg kg^-1, halothane 0.5-1% and nitrous oxide 60% in oxygen on controlled ventilation via pediatric Bain’s circuit. The surgical procedure was smooth, uneventful and lasted for 60 minutes. Residual neuromuscular blockade was reversed with neostigmine 0.05mg kg^-1 and glycopyrrolate. She was extubated, when fully awake and breathing spontaneously with adequate tidal volume. The postoperative period was also uneventful.