2. General information
• It is often inherited as autosomal dominant
trait
• it is derived from first and second branchial
arch
• it results from failure or incomplete migration
of neural crest cells to the facial region
3. Clinical features
• Underdevelopment of zygomatic bone,
resulting in midfacial deformities
• Downward inclination of palpebral fissure
• deficiency of eyelashes
• there is varying degree of visual impairment
• Underdeveloped and /or malformed years
• Secondary mental deficiency
4. Oral manifestations
• Underdevelopment of mandible steep
mandibular angle
• fish or bird appearance
• micrognathia
• macrostomia that is unusually large mouth
• high arch palate with cleft palate
• malocclusion is seen
5. Radiographic features
• Zygomatic bone — reduction in size of
zygomatic bone
• maxillary sinus is underdeveloped or
completely absent
• articular eminence is either shallow or absent
• hypoplasia of mandible and maxilla showing
accentuation of antegonial notch and steep
mandibular angle
• partial anodontia and malformation of teeth
6. Management
• Cosmetic surgery – improvement and surgical
interventions should be done to improve
osseous and ear defect
