Cleft and Craniofacial Center

Cleft Lip and Palate

Cleft lip occurs in one in every 700 Caucasian births, one in every 500 Asian and one in every 2500 African-American births. The causes are not well understood, but are generally thought to be multi-factorial (i.e. genetics as well as the environment are thought to play a role). As a result of these varied origins, there is a wide variation in severity of clefts, from very subtle to very wide. One or both sides of the face may be affected.

Repair of the cleft lip as well as the associated nasal deformity is usually carried out in a single operation at approximately three months of age, after allowing for growth and optimal nutrition. Most infants spend a single night in the hospital after surgery. The stitches are dissolving and therefore do not have to be removed. Feedings resume as tolerated following anesthesia.

Cleft palate (roof of the mouth) may occur with cleft lip or as an isolated finding. Repair of the palate is very important for future speech development. Ongoing evaluation and treatment by a Speech Language Pathologist specially trained to work with cleft affected individuals is a critical component of care. Despite adequate repair, approximately 15% of children may require additional surgery to help improve speech. Like cleft lip, there is a wide variation in the appearance of clefts of the palate. Repair is usually carried out in a single operation, when the baby is about ten months old. The child will usually spend two nights in the hospital after surgery. Again the sutures are dissolving. Feedings are resumed as tolerated following anesthesia.

Children with cleft palates have a higher incidence of hearing problems. The eustachian tube connects the middle ear space to the back of the throat. It normally opens and shuts to relieve pressure that builds up behind the eardrum. If the eustachian tube does not open, then the pressure increases until mucous or fluid accumulates behind the eardrum. Muscles responsible for opening the eustachian tube usually do not function as well in children with cleft palates, and other craniofacial disorders, resulting in more frequents problems with fluid, otitis media and ear infections, which can be very painful. Because of this problem, it is important to have the infant's hearing tested during the first few months of life. If hearing is impaired by fluid buildup or unequal pressure, it may be necessary for the otolaryngologist to place pressure equalizing tubes (PE tubes). Tubes are often placed at the time of the lip or palate surgery. It is crucial that children with cleft palates have regular hearing tests to monitor middle ear problems that could alter the development of normal hearing, as well as speech. As the child grows, the frequency of ear infections and fluid in the ears seem to decrease. The cleft in the gums (alveolus) is usually treated at age 8-10 years. This time corresponds with the appearance of adult or secondary teeth. Bone is taken from the hip and placed in the gap in the gums. This stabilizes the two halves of the palate and provides a strong foundation into which the teeth may move. Most cleft palate children will require braces to straighten their teeth. Individuals with clefts may have missing teeth. Dental prosthetics, crowns and implants are available. In addition, Pediatric Dentists are available for complex pediatric dental problems. For reasons that are only beginning to be understood, a small percentage of children with cleft lip and palate will have a diminished potential for growth of the upper jaw. This will result in a severe under-bite. Surgery to correct this is usually carried out at age 16-18 years. A definitive rhinoplasty to correct the nose may also be addressed at this age.

FAQ's:

Q: What is a cleft palate?
A: The cleft palate, a congenital split in the roof of the mouth, occurs in approximately one in every 750 births. It is also the cause of many speech problems, impairing speech if not surgically repaired. Readily detected because of a thin layer of tissue covering the defect, surgery is usually performed before the child learns to speak, generally between the ages of 12 and 18 months. Many children born with cleft palates achieve normal speech following surgery, although therapy is often required to correct inappropriate nasal/air flow and articulation problems.

Q: What is a cleft lip?
A: About one baby in 750 is born with a cleft lip, a congenital split in the upper lip. If left uncorrected, a cleft lip can cause physical and emotional problems and affect a child's speech, so surgery is performed around the age of 10 weeks. Today, through medical advancements, it's encouraging to know that babies born with cleft lip, palate or both have a better chance than ever before to lead a completely normal life.


Craniofacial

The UAB Cleft and Craniofacial Center Mission: We are an interdisciplinary team of specialists whose mission is to deliver the highest quality treatment for patients with facial differences. We strive to do so in a thoughtful and compassionate manner, which provides support and assurance to the families of our patients.

Our center provides families and patients with comprehensive information about facial birth defects, facial deformities due to trauma and facial differences due to tumors. This information will allow them to better understand and participate in, the treatment plan chosen. We then provide the professional expertise and dedication necessary to accomplish that plan with a minimum number of surgical procedures. For more information, please contact us at 205-638-9369.

Our team includes: Audiologists, Craniofacial Surgeon, Geneticist, Neurosurgeons, Nurses, Occupational Therapists, Oral and Maxillofacial Surgeons, Orthodontists, Otorhinolaryngologists (ENT), Pediatrician, Pediatric Anesthesiologists, Pediatric Dentists and Prosthodontists, Pediatric Radiologists, Physical Therapists, Plastic Surgeons, Speech Language Pathologists and Surgeon's Assistant and Clinic Coordinator.

We treat a wide variety of facial differences including, Amniotic Band, Apert Syndrome, Cleft Lip and or Palate, Craniosynostosis, Crouzon Syndrome, Encephalocele, Fibrous Dysplasia, Frontonasal Dysplasia, Goldenhar's Syndrome, Hemifacial Microsomia, Hypertelorism, Microtia, Obstructive Sleep Apnea, Orthognathic Problems, Post Traumatic Facial Differences, Saethre-Chotzen Syndrome and tumors of the face.

Timing
In general, we prefer to see children soon after birth, in order to assess feeding ability and provide early information to concerned family members. Thereafter, visits are individualized to the patient's diagnosis and specific medical needs. Full follow-up is provided through early adulthood. At the first visit, many or all of the above specialists will see the child. The team will then provide a summary opinion of our recommendations, in writing, to the patient's primary care physician as well as to the family. Input from the parents is encouraged. Once the child is old enough, they too are included in the decision making process. An effort is made to maximize outcome with a minimum number of operations in all cases. However scar healing is an individual trait, and some children need small revisions at various times.

Facial Trauma
Complex facial trauma is ideally managed acutely, within days after the injury. Complex craniofacial injuries are managed in a team fashion analogous to the approach used for congenital facial differences. This team approach leads to a more efficient and comprehensive treatment plan. Facial deformities arising as a consequence of trauma can also be managed in a delayed fashion. Some individuals may experience facial growth disturbances after trauma. Others have persistent post-traumatic deformity after severe facial skeletal and or soft tissue injuries. In many cases, secondary correction of these disturbances (even years after the original injury) can be accomplished to improve function and self esteem. Again, a team concept is utilized in an effort to optimize outcomes and to minimize surgical procedures.

Tumors
Removal of certain types of tumors may also benefit from a team approach. Surgeons with different, but complementary training and skills can combine these talents to allow safe removal of the tumor and immediate reconstruction of any bony or soft tissue defects. This team approach has been demonstrated to allow for more complete tumor removal, shorter hospitalization and a lower complication rate.

Craniofacial Surgery
Craniosynostosis is the premature fusion of the growth centers of the skull. It may involve single or multiple sutures (growth joints between the bones of the skull). Fusion of a particular suture will result in a characteristic head shape. It is generally believed that fusion of a single suture does not cause a significant increase in pressure on the brain. Correction is carried out for reasons of appearance and to aid psychosocial development. In some cases a fused suture can cause a twisting of the face, which could result in dental abnormalities if not corrected. Multiple fused sutures are thought to increase pressure on the brain, and may risk injury to brain function and vision if left uncorrected. Careful evaluation by the neurosurgeons as well as a craniofacial surgeon, pediatrician and a pediatric ophthalmologist will help to determine timing of surgical intervention. In general, surgery for craniosynostosis is carried out at age three to ten months. It involves a three to five day stay in the hospital.

The need for craniofacial surgery is highly individualized. Consequently the timing of craniofacial surgical procedures is customized (whenever possible) by the team to best meet the needs of the patient. Every effort is made to carry these procedures out in such a manner that the number of surgical procedure is minimized. Midface hypoplasia is seen in a number of syndromes such as Apert and Crouzon Syndrome. Surgery can be performed to move the facial bones forward. This is done to improve eye protection, to improve the airway (or breathing passages) and to help with alignment of the teeth. The timing of such surgery may vary depending on the individual patient's needs. The earliest age for midface advancement would be four years of age (for example to allow removal of a tracheostomy). However midface advancement at this early age may increase the likelihood of a repeat procedure later in life. In general, surgery for conditions such as facial clefting, fronto-nasal dysplasia, Treacher-Collins Syndrome and hemifacial microsomia are dictated by the severity of the condition as well as the specific anatomic region to be addressed. For example skull growth is very rapid until age three years, then slows dramatically. The orbits (eye sockets) are mature by approximately age ten years. The tooth bearing facial skeleton will continue to mature until age 14-16 years in girls, and 16 to 18 years in boys. Discussions with the patient, family and craniofacial team, help to prioritize issues of greatest concern.

Orthognathic Surgery
"Ortho" means to straighten or align, and "gnathic" means jaw so orthognathic surgery means, surgery to straighten the jaws and improve function. Frequently children born with facial differences have disproportionate growth of the jaws. Cleft palate may cause a small upper jaw (maxilla) and a relatively larger lower jaw (mandible). Sometimes the bones of the jaws can be small, fused, or even missing as in hemi-facial microsomia. Facial syndromes, such as Crouzon, Apert, Pfieffer etc. may affect the size of the jaws, and require surgery, after the child's growth is complete. Fractures of the jaws may sometimes affect their ability to grow normally. In some injuries, the jaws may have healed in a way that affects alignment. The lower jaw is the one that moves, so abnormal growth or position may affect, eating, chewing, talking and/or breathing. In such cases surgical repositioning of the jaws can frequently be beneficial. In teenage patients, orthodontic braces are needed to straighten the teeth, before jaw surgery. Our team orthodontists and oral surgeons will work together to develop a treatment plan which minimizes the time spent in braces.