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- Apert syndrome
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- Craniofacial clefts
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- Muenke syndrome
- Nose deformities, nasal breathing problems
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Craniofacial clefts
What is a craniofacial cleft?
A craniofacial cleft (cray-nee-oh-fay-shul KLEFT) is an abnormal opening or gap on the face or head that a baby has at birth. It can involve both bones and skin and other soft tissues. Craniofacial clefts are very rare, occurring in about one to five out of 100,000 births. They can range in severity from a small gap in a single area to a large gap that affects more than one part of the face.
Unlike cleft palates, which always affect the mouth, craniofacial clefts can be anywhere on the face and no two are alike. What they all have in common is:
- An abnormal gap in one or more facial features
- Facial features that don’t match
- Possible problems with breathing, eating, speaking or seeing
Because craniofacial clefts are so widely different, medical experts use a numeric system to categorize them. This system, developed in 1976 by a French surgeon named Paul Tessier, gives doctors guidance in determining the best treatment plan.
In the Tessier system, craniofacial clefts are defined by their position in relation to the eye. Each category has a number from zero to 14. There are four main groups:
- Numbers 0-3: oral-nasal, involving the mouth and/or nose
- Numbers 4-6: oral-ocular, involving the mouth and/or eye
- Numbers 7-9: lateral face, extending from the mouth outward across the cheekbone and possibly into the eye
- Numbers 10-14: cranial, extending from the top of eye into the forehead
The numbers correspond to the bones of the face. If there is an abnormal gap in the bone, there will also be gaps in the soft tissues in that area.
Craniofacial clefts can affect one or both sides of the face and a baby may have more than one type of cleft. Sometimes a cleft below the eyes (categories 0-9) combines with one above the eyes (categories 10-14).
What causes a craniofacial cleft?
A craniofacial cleft occurs while a baby is developing in the womb. A baby’s facial features form during weeks 3 to 8 of pregnancy. During that time, five distinct areas rotate together inward and downward, connecting in the middle, to form the forehead, eyes, cheeks, nose, mouth, chin and jaw. If any part of those five areas fails to meet and connect correctly, a cleft occurs.
Currently, no one fully knows why clefts happen. Some clefts have a genetic cause and are part of a larger syndrome, such as Treacher Collins syndrome. Other studies point to something that has affected the mother’s health. Most experts think more than one thing causes clefts.
Diagnosis of craniofacial clefts
Although a cleft may sometimes be seen before birth in routine prenatal ultrasounds, usually it is diagnosed at birth by physical examination. With the diagnosis, doctors will do more detailed imaging tests to see how severe the cleft is and to begin planning treatment. CT scans show the bones of the face. MRI tests show what the soft tissues look like. Because craniofacial clefts, particularly categories 7-9, can be part of a genetic syndrome, genetic counseling for the baby’s parents is also provided after diagnosis.
How are craniofacial clefts treated?
Craniofacial clefts are complicated conditions that can affect both appearance and functions such as eating, breathing, speaking and seeing. Treatment requires help from many different pediatric experts to deal with the various areas of the face and head that are involved.
Pediatric craniofacial surgeons work to repair the bones and soft tissues of the face to create normal openings of the mouth, nose, eyes and other areas. This may require several surgeries done in stages over the years as the baby grows.
If a cleft threatens the baby’s ability to breathe, surgery will be done soon after birth to correct problems in the baby’s airway. Another reason for urgent surgery would be a cleft that prevents the baby from closing an eye.
Because craniofacial clefts are all different, there is no single surgical technique that is always used. Instead, the surgeon combines information from imaging showing the baby’s specific cleft, expert knowledge of facial development patterns, and understanding of surgical principles and techniques to perform the best possible operations for the baby. The goal of surgery is to improve both the baby’s appearance and abilities.
In addition to surgeons, other pediatric experts who may be involved in the baby’s care include:
- Orthodontists
- Speech therapists
- Geneticists and genetic counselors
- Pediatricians
- Otolaryngologists (ear, nose and throat doctors)
- Ophthalmologists (eye doctors)
- Child psychologists
- Social workers
This team approach, with all the necessary pediatric specialists working together, gives babies with craniofacial clefts their best outcomes. It also provides families with ongoing support.
Get help at Children’s of Mississippi
The craniofacial team at Children’s of Mississippi has all the pediatric specialists needed to manage the unique needs of babies with craniofacial clefts. You can request a consultation with our pediatric craniofacial surgeons. They will be happy to answer your questions and help you understand what may be involved in your child’s treatment and ongoing care. Schedule an appointment online.
Last reviewed: July 16, 2026
UMMC