Reconstruction - Craniofacial

Hemifacial Microsomia (Goldenhar syndrome)

Hemifacial Microsomia or Craniofacial Microsomia occurs in 1 in 5600 patients.

This facial deformity is typically asymmetric (one side more affected) and ranges from mild to severe. In its full form patients may have:

• Absence of the mandibular (lower jaw) ramus and an occlusal cant (crooked bite)
• Microtia or absence of the external ear
• Weakness of facial muscles of expression and/or mastication
• Vertical orbital dystopia or eye asymmetry.
• Goldenhar syndrome is a variant of craniofacial microsomia and has cervical (neck) and rib anomalies and epibulbar dermoids (excess eye tissue).

The UCLA Craniofacial Clinic Protocol is aimed at maximizing results and minimizing the number of procedures. The timing and types of procedures may vary depending on the severity of the deformity and the individual patient. Typically, corrections include many of the following:

1. Preauricular skin tags: (age under 1 year): Excision;
2. Macrostomia or wide mouth: (age under 1 year): Commisuroplasty;
3. Mandibular hypoplasia: (5-8 years of age): Internal distraction osteogenesis is used to lengthen the lower jaw . (For severe cases with absence of mandibular condyle and ramus, a rib graft may be necessary).
4. External ear deformity or absence: (6-8 years of age): Staged ear reconstruction with a rib graft framework, elevation, lobule (ear lobe) and tragus (front of ear) reconstruction are performed.
5. Orbital dystopia (asymmetric eyes): (6-11 years of age): Although rarely required, repositioning of the orbit and/or advancement of the forehead and brow (fronto-orbital advancement) may be performed.
6. Jaw asymmetry: (15-18 years or age of skeletal maturity): Preoperative orthodontics followed by jaw (orthognathic) surgery with Le Fort I (upper jaw) and mandibular sagittal-split (lower jaw) osteotomies are often necessary.
7. Soft tissue asymmetry: (after jaw surgery): Final facial contouring with autogenous fat grafting, dermal fat grafts or even a fascial-fat free flap from the upper back are often necessary after other corrections.

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