Document Type

Article

Publication Title

Cureus

Abstract

Goldenhar syndrome, also known as the oculo-auriculo-vertebral (OAV) spectrum, is a rare congenital disorder characterized by craniofacial and vertebral anomalies due to abnormal development of the first and second branchial arches. These structural abnormalities often necessitate frequent surgical interventions in patients with Goldenhar syndrome and may also impair airway visualization, thereby complicating effective tracheal intubation. Despite recognition of the airway risks associated with Goldenhar syndrome, evidence guiding anesthetic and airway management remains limited and unexplored. Current literature consists predominantly of isolated case reports, with each portraying a unique and patient-specific airway scenario. A systematic review of the literature was conducted to evaluate reported anesthetic and airway management strategies in patients with Goldenhar syndrome. A review of literature on Goldenhar syndrome was completed on January 11, 2026, using “PubMed” and “Embase,” and included articles published in the past 10 years. Search terms included “Goldenhar syndrome”, “oculo-auriculo-vertebral syndrome”, “hemifacial macrosomia”, “anesthesia”, “airway”, and “intubation”. Articles were eligible for inclusion if they reported patients with confirmed Goldenhar syndrome undergoing any surgical or procedural intervention with detailed airway and anesthetic management. Exclusion criteria for this review included non-English reports without translation and papers that featured patients without perioperative or anesthetic discussion. The articles were screened to ensure the inclusion criteria were met, and any discrepancies were assessed by the researchers utilizing the Joanna Briggs Institute critical appraisal guidelines. A total of 28 cases met the inclusion criteria, with the majority of patients being infants and children. Cardiovascular and oromaxillofacial surgeries were the most commonly reported procedures. Sevoflurane was the most frequently used anesthetic agent. The most successful first-attempt intubation strategy was found to be Macintosh-style curved blade video laryngoscope followed by Miller-style straight blade video laryngoscope. Successful first-attempt intubation was achieved in only half the cases, while the remaining patients required multiple attempts or escalation to alternative airway strategies. Cases that used the curved Macintosh video laryngoscope and did not yield successful intubation on the first attempt ultimately necessitated invasive rescue interventions such as retrograde intubation and tracheostomy. While several cases that attempted straight Miller blade video laryngoscopy were unsuccessful on the first attempt at intubation, cases using this technique saw eventual success after repeated attempts. Patients requiring multiple attempts or rescue techniques more frequently experienced perioperative complications, including delayed extubation, hemodynamic instability, and one perioperative death. Although definitive airway control was ultimately achieved in most patients, these findings emphasize the potential severity of airway-related complications in patients with Goldenhar syndrome and reinstate the importance of minimizing repeated intubation attempts when possible. Clinicians should be prepared for difficult airway scenarios to minimize airway-related morbidity and mortality in affected children.

DOI

10.7759/cureus.105687

Publication Date

3-23-2026

Keywords

difficult airway management, goldenhar, goldenhar syndrome surgery, oav, oculo-auriculo-vertebral syndrome

ISSN

2168-8184

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