Anesthesia Considerations 

Goldenhar Syndrome

 

Background 

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• Also called oculo‐auriculo‐vertebral syndrome or hemifacial microsomia

• Developmental disorder of the first & second branchial arches

• Most frequently unilateral

• Characterized by: ​

  • Malformations/hypoplasia of the external & middle ear often with sensorineural hearing loss

  • Mandibular hypoplasia

  • Congenital heart disease in about a third of patients, most commonly septal & conotruncal defects (e.g. Tetralogy of Fallot) 

  • Eye abnormalities (e.g. microphthalmus & epibulbar dermoids)

  • Vertebral anomalies including cervical spine malformations & scoliosis

  • Developmental delay & autism spectrum disorder in some patients

 

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Considerations

 

• Pediatric patient considerations 

• Potentially very difficult airway​

  • Airway is difficult due to mandibular hypoplasia & inadequate space for direct laryngoscopy

  • Airway plan essential with multiple adjuncts, have ENT/surgical option on stand-by, plan on spontaneously breathing sleep technique (titrated TIVA vs IH).  Fiberoptic or video‐assisted laryngoscopy are almost always required. 

• Congenital heart disease in about a third of patients​

  • Most commonly septal & conotruncal defects (e.g. Tetralogy of Fallot)

• OSA more common in this patient population