Craniofacial Surgery

The Treatment Journey and Milestones

At a Glance

Managing Goldenhar syndrome (OAVS) requires a multidisciplinary approach over many years. Treatment is highly individualized and carefully timed, ranging from early breathing and feeding support in infancy to complex ear reconstructions and final jaw surgeries in young adulthood.

Managing OAVS is not a single event, but a journey that unfolds over many years. Because it is a spectrum, not every patient will need major surgeries. However, when surgeries are necessary, they must be carefully timed to match skeletal maturity—the point at which the bones have finished growing ^[1]^[2].

Care should be led by a multidisciplinary team, which typically includes craniofacial surgeons, geneticists, ophthalmologists, ENTs, dentists, and speech therapists ^[3]^[4]. This team ensures that every intervention is timed to support both physical health and social development.

A General Roadmap of Care

While every path is unique, interventions often follow this general timeline:

Infancy to Early Childhood (Ages 0–5)

Non-Surgical Support: This period heavily focuses on daily functioning. Hearing aids may be fitted early to support speech development ^[5]. Feeding support and early speech therapy are critical if swallowing issues exist ^[6].
Airway and Feeding: If a very small jaw (mandibular hypoplasia) makes it hard to breathe or eat, doctors may perform Mandibular Distraction Osteogenesis (MDO) ^[7]^[8]. This involves gradually lengthening the jaw bone to open the airway ^[9].
Eye Care: Benign growths on the eye called epibulbar dermoids are often removed early if they interfere with vision or eye movement ^[10]^[11].

School Age and Pre-Teens (Ages 6–12)

Ongoing Therapies: Continued speech therapy and audiological monitoring are common to ensure academic environments are properly accommodated ^[12].
Ear Reconstruction (Microtia): There are two main ways to reconstruct the ear:
- Autologous Rib Cartilage: This uses the patient’s own rib cartilage to “sculpt” a new ear. It is usually done around age 10 or older once the ribs are large enough ^[13]^[14].
- Synthetic Frames (Medpor): This uses a synthetic frame and can sometimes be done earlier (around age 3–5), but has a slightly higher risk of infection compared to autologous tissue ^[15]^[16].
Speech Surgeries: If Velopharyngeal Insufficiency (VPI) persists, surgeries to help the palate close properly may be timed to support clear speech ^[12]^[17].

Adolescence to Young Adulthood (Ages 13–18+)

Orthognathic Surgery: Once skeletal maturity is reached, surgeons can perform final “two-jaw” surgeries to correct facial asymmetry and ensure the teeth meet properly (malocclusion) ^[1]^[2].
Spinal Care: If severe scoliosis or vertebral issues are present, a posterior spinal fusion may be performed ^[18].
Transition of Care: A vital milestone in this phase is transitioning from pediatric care to adult specialists, empowering the young adult to manage their own craniofacial and systemic health.

Important Considerations and Risks

Interventions for OAVS require extra caution due to the complex anatomy of the spectrum:

Anesthesia and Airway: Because the jaw and neck may be shaped differently, patients can be more difficult to intubate. Specialized “video” tools are often used to ensure safety ^[19].
Respiratory Risk: Patients—especially those undergoing spinal surgery—have a higher risk of temporary breathing issues after surgery and should be monitored closely ^[20].
Skeletal Maturity: Performing some jaw surgeries too early (before growth is finished) may mean the asymmetry returns, requiring a second surgery later in life ^[21]^[22].

Common questions in this guide

When is jaw surgery usually performed for Goldenhar syndrome?

Jaw surgeries are typically performed after a patient reaches skeletal maturity in adolescence or young adulthood. However, if a very small jaw causes breathing issues in infancy, a procedure called mandibular distraction osteogenesis may be done much earlier to open the airway.

What are the options for ear reconstruction with microtia?

There are two main surgical methods for ear reconstruction. One uses the patient's own rib cartilage and is usually done around age 10, while the other uses a synthetic frame and can sometimes be performed earlier, around ages 3 to 5.

Do the benign eye growths in OAVS need to be removed?

Benign growths on the eye, known as epibulbar dermoids, are typically removed during early childhood if they begin to interfere with the child's vision or affect normal eye movement.

Are there specific anesthesia risks for patients with Goldenhar syndrome?

Yes, patients with this condition can have differently shaped jaws and necks, which makes intubation more challenging. Anesthesia teams often use specialized video tools to safely manage the airway and monitor breathing closely after surgery.

Curated prompts to bring to your next appointment.

1.Are the ocular dermoids currently affecting vision, or can we wait to remove them?
2.Is Mandibular Distraction Osteogenesis (MDO) indicated early, or should we wait for orthognathic surgery at skeletal maturity?
3.At what age do you recommend starting the process for ear reconstruction, and which method do you suggest?
4.What are the specific anesthesia risks, especially regarding the cervical spine?
5.How will you monitor breathing and sleep as the jaw grows?
6.How does the care team facilitate the transition from pediatric to adult specialists?

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References (22)

1
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PMID: 40305773
2
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8
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9
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10
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11
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12
Congenital abnormalities associated with microtia: A 10-YEARS retrospective study.
Paul A, Achard S, Simon F, et al.
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13
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14
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15
[The application of porous polyethylene biological scaffolds combined with temporoparietal fascial flaps in auricular reconstruction].
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16
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17
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20
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22
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This timeline provides a general overview of Goldenhar syndrome and OAVS treatments for educational purposes. Every patient's journey is unique; always consult your child's multidisciplinary craniofacial team for personalized medical advice.

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