TürkAnesteziyoloji ve Reanimasyon Derneği 53.Ulusal Kongresi, Antalya, Türkiye, 7 - 10 Kasım 2019, ss.221-222
Goldenhar syndrome (GS), known as oculo-auriculo-vertebral dysplasia is a rare congenital disorder. This syndrome is associated with difficult airway because of facial, oral and vertebral anomalies.
The common features of this syndrome are unilateral maxillary and mandibular hypoplasia, hemifacial microsomia, micrognathia, cleft palate, preauricular skin tags, epibulbar and limbal dermoids, cardiac and vertebral anomalies.
Case reports We report three cases of Goldenhar syndrome who were successfully intubated with direct laryngoscopy using the Machintosh laryngoscope. A 2-year-old, 11 kg, boy presented for anterior vitrectomy and lensectomy under general anesthesia for congenital cataract (Patient 1) (Figure 1A). He suffered from atrophic external auditory canal, left atrophic optic nerve, macroglossia and minimal mandibular retrognathia. A 12-year-old, 49 kg, girl presented for limbal dermoid excision (Patient 2). She had hypoplasia of left maxilla and mandible, her tongue deviated to the left when she opened her mouth. A 6-year-old, 16 kg, boy presented for limbal dermoid excision of the right eye (Patient 3). He had hypoplasia of right maxilla and mandible and preauricular skin tags. Written informed consent of the parents were obtained. Mallampati classification, mouth opening, thyromental distance, sternomental distance, and neck mobility were preoperatively evaluated for the risk factors of difficult airway. Anesthetic induction was provided by sevoflurane 4-8% with slow increments in 80% oxygen-air mixture. After motionlessness was provided, anesthetic gas was decreased to 3-4% in 40% oxygen-air mixture through a face mask. A quick look performed by a Macintosh laryngoscope blade. If the view was satisfactory, tracheal intubation was attempted before giving muscle relaxant under deep sevoflurane anesthesia. After the success of intubation, the relaxation was achieved by rocuronium bromide 0.6mg/kg. The first patient was successfully intubated with 3.5 mm ID endotracheal tube using a Macintosh laryngoscope blade no. 1 on the first attempt. On direct laryngoscopy with a Macintosh laryngoscope blade no. 3, a Cormack- Lehane grade 3 view was obtained and the second patient was intubated with 5.5 mm ID endotracheal tube after two attempts without neuromuscular blocker agent.
The third patient was successfully intubated with 5 mm ID endotracheal tube on the first attempt using a Macintosh blade no.2. The Cormack- Lehane grade 2 was obtained by direct laryngoscopy in the first and third patients. All our patients were successfully intubated with cuffed endotracheal tubes using the Macintosh laryngoscope blades. The Macintosh blade is curved and the tip is inserted into the vallecula. It provides a good view of the oropharynx and hypopharynx (Figure 1B).
Difficult Airway Society indicates the use of videolaryngoscopy as an alternative to direct laryngoscopy in patients with difficult airways (1) . Videolaryngoscope, Airtraq, wire-guided intubation, I-gel supraglottic airway, laryngeal mask airway were reported for difficult intubation in the patients with Goldenhar syndrome (2) .
Macintosh laryngoscope blades and endotracheal tubes were used successfully for ophthalmic procedures in our patients with Goldenhar syndrome.
Preoperative diagnosis and awareness of difficult airway are important in patients with Goldenhar syndrome and the anesthesiologist should be prepared for difficult airway management.