Cleft Lip and Cleft Palate
Cleft lip and cleft palate are the most common craniofacial differences. The instance is about 1 in 1000 births. The modern comprehensive cleft care multidisciplinary approach among different specialties includes a plastic surgeon, speech pathologist, orthodontist, dentist, oral surgery, speech, occupational therapy, social worker and dietician. The first priority in cleft care is making sure the baby can feed appropriately and gain weight. A special nipple, such as Haberman Feeder, is required for children with a cleft palate. Breast feeding is not an option for a child with a cleft palate, however, the breast milk can be given through the feeder. Children with cleft lip only may be able to breast feed, depending upon the size of their cleft. However, they may require the feeder as well. Many children who have wide clefts and bilateral cleft lip and palate may require a pre-surgical orthopedic, performed by a cleft orthodontist, to narrow the gap and make the subsequent surgeries easier.
Dr. Chaiyasate typically repairs the lip at the age of 3-4 months old. The cleft palate is usually repaired at the age of 9-12 months. It is recommended in children with clefts to follow up annually with a craniofacial team. At around the age of 3, an initial speech evaluation will be performed by a speech pathologist. This may be evaluated by several methods including speech therapy, videofluoroscopy and nasendoscopy. About 20% of children with cleft palates will require a speech surgery to correct hypernasality. It is not uncommon for children with cleft lips to require cleft lip revisions before starting kindergarten.
During mixed dentition age, the children with cleft lip and palate will require orthodontic treatment to align an asymmetrical gumline. Many of them will require taking a piece of bone from their hip and placing it into a gap in their gumline to restore the arch of the gum. About 20 % of children with cleft lip and palate will require definitive jaw surgery to achieve a normal bite. Many of these children will also require a definitive rhinoplasty (nose job) during their late teens.
Dr. Chaiyasate is a leader of Beaumont’s Craniofacial Clinic and works closely with the other specialists.
Treacher Collins Syndrome
Treacher Collins syndrome is a condition that affects the development of bones and other tissues of the face. The signs and symptoms of this disorder vary greatly, ranging from almost unnoticeable to severe. Most affected individuals have underdeveloped facial bones, particularly the cheek bones, and a very small jaw and chin (micrognathia). Some people with this condition are also born with an opening in the roof of the mouth called a cleft palate. In severe cases, underdevelopment of the facial bones may restrict an affected infant’s airway, causing potentially life-threatening respiratory problems.
People with Treacher Collins syndrome often have eyes that slant downward, sparse eyelashes, and a notch in the lower eyelids called an eyelid coloboma. Some affected individuals have additional eye abnormalities that can lead to vision loss. This condition is also characterized by absent, small, or unusually formed ears. Hearing loss occurs in about half of all affected individuals; hearing loss is caused by defects of the three small bones in the middle ear, which transmit sound, or by underdevelopment of the ear canal. People with Treacher Collins syndrome usually have normal intelligence. This condition affects an estimated 1 in 50,000 people.
The treatment of individuals affected by TCS needs a multidisciplinary approach and may involve the intervention of different professionals. The primary concerns in individuals with TCS are breathing and feeding problems, which are a consequence of the hypoplasia of the mandible and the obstruction of the hypopharynx by the tongue. Sometimes even a tracheostomy is necessary to maintain an adequate airway. Also a gastrostomy could be necessary to assure an adequate caloric intake while protecting the airway. Surgery to restore a normal structure of the face is normally performed at defined ages, depending on the development state.
An overview of the present guidelines:
- If there is a cleft palate, the repair normally takes place at the age of 9–12 months. Before surgery, it is necessary to perform a polysomnography with a palatal plate in place, this may predict the postoperative situation and gives insight on the chance of the presence of OSAS after the operation.
- The hearing loss is treated by bone conduction amplification, speech therapy and educational intervention to avoid language/speech problems. The bone-anchored hearing aid (Baha) is an alternative for individuals with ear anomalies.
- The zygomatic and orbital reconstruction is performed when the cranio-orbitozygomatic bone is completely developed. Usually at the age of 5–7 years. In children an autologous bone graft is mostly used. In combination with this transplantation, lipofilling can be used in the periorbital area to get an optimal result of the reconstruction.
- The reconstruction of the lower eyelid Coloboma includes the use of a myocutaneous flap, which is elevated and in this manner closes the eyelid defect.
- The external ear reconstruction is usually done when the individual is at least 8 years old. Sometimes also the external auditory canal or middle ear can be treated.
- The optimal age for the maxillomandibular reconstruction is still a major point of discussion, at present, this classification is generally used: Type I (mild) and Type IIa (moderate) 13–16 year, Type IIb (moderate to severe malformation) at skeletal maturity, and Type III (severe) 6–10 years
- Orthognathic treatments usually take place after the age of 16 years; at this point all teeth are cut and the jaw and dentures are mature. Whenever OSAS is detected, the level of obstruction is determined through endoscopy of the upper airways. Mandibular advancement can be an effective way to improve both breathing and aesthetics, while a chinplasty only restores the profile.
- If a nose reconstruction is necessary it is usually performed after the orthognathic surgery and after the age of 18 years.
- The contour of the facial soft tissues generally requires correction at a later age, because of the facial skeletal maturity. The use of microsurgical methods, like the free flap transfer, has improved the correction of facial soft tissue contours. Another technique to improve the facial soft tissue contours is lipofilling. For instance, lipofilling is used to reconstruct the eyelids.