Goldenhar Syndrome

Incidence: 1:5500 live births.

Second most common facial birth defect after cleft lip and palate.

Extracraniofacial anomalies can range from one anomaly (13%) to multiple affected organ systems (42%). No gender or side predominance was detected. Central nervous system, cardiac, and skeletal anomalies each occurred in more than 10% of cases. Surgical correction usually takes place in severe cases.

Cleft palate occurs in 25% of pts

Difficulties with airway management

Possible need for tracheostomy

High risk of anesthesia overdose in premature and low-weight pts

Stabilizing the heart rate.

Difficulty with airway management.

Ensuring good mask fit. There are many sizes to choose from, and the mask must fit prior to induction. Mask may have to be changed after induction for better fit. The degree of inflation of the facemask cuff may be adjusted in order to ensure an appropriate seal.

IV access.

Associated anomalies, such as cardiac and or cervical spine malformation that may influence decisions.

Cancelation of procedure owing to inability to intubate.

Increased severity of microsomia. Pruzansky classification type III is associated with increased intubation difficulties.

Craniofacial microsomia, also known as HFM or oculoauriculovertebral spectrum.

Bilateral microsomia can occur.

CNS, cardiac, and skeletal anomalies (expanded HFM spectrum) may occur

Pulmonary, gastrointestinal, and renal deformities are less common.

The majority of associated heart defects involve the outflow tract or septum. The increased frequency of cardiac anomalies with this condition suggests that abnormal development of the neural crest may result in both HFM and conotruncal heart defects.

Children with HFM may have fused or hemivertebrae, resulting in limitation of neck flexion and extension and increasing the difficulty of intubation.

There are positive correlations between the number of involved abnormal components and the degree of difficulty in visualizing the larynx in pts with both bilateral and unilateral microtia.

Bilateral mandibular and auricular malformations increase the risk of difficult intubation.