Orthognathic surgery in patients

with craniofacial syndrome

Syndromes:

1.craniosynostosis syndromes:
I. Crouzon Syndrome.
II. Apert Syndrome.

2.Pierre Robin Sequence Syndrome.

 Crouzon and Apert Syndromes
• Belong to the family of Craniosynostosis.
• Apert Syndrome (Acrocephalosyndactyly).
• Crouzon Syndrome (Craniofacial Dysostosis).

 Craniosynostosis:
premature fusion of cranial sutures which
can impair proper brain and craniofacial
development, affecting the shape of the
skull.

 Could be syndromic or non-syndromic.

• Typical characteristics
1. Craniosynostosis :Coronal sutures fused at
birth.
2. Larger than average head circumference at
birth.
3. Midfacial malformation and hypoplasia.
4. Shallow orbits with exophthalmos.

• Apert Syndrome: symmetric syndactyly

of hands and feet Syndactyly: the condition of having
some or all of the fingers or toes
wholly or partly united, either
naturally (as in web-footed animals)
or as a malformation.
• Crouzon and Apert Syndromes facial features:

1. Shallow orbits with exophthalmos

2. Retruded midface with relative prognathism
3. Beaked nose
4. Hypertelorism
5. Downward slanting palpebral fissures

Class III Skeletal Pattern

 Pierre Robin Sequence
• Triad of :

1. Micrognathia: a condition in
which the jaw is undersized.

2. Glossoptosis: a medical
condition and abnormality
which involves the downward
displacement or retraction of
the tongue. It may cause non-
fusion of the hard palate,
causing cleft palate.

3. Cleft palate.
 Pierre Robin Sequence
• Syndromic - 80%
• Non-syndromic - 20%

• Bird face appearance:

I. Mandible is severely underdeveloped.
II. Cleft lip and palate.
III. Small Tongue.

• Severe Class II Skeletal Pattern.

• Early Surgical intervention because of the

effect on feeding and airways.
Edited By : Mays Khanfar