Cleft Lip and Palate

 

Condition Description

Cleft lip and palate is a facial deformity due to failure of fetal tissues to properly come together to form the lips and roof of the mouth (palate). These facial structures are formed between the 6 th and 10 th weeks of fetal life. Unknown factors (genetic and environmental) prevent this fusion process from fully closing the midline facial structures. The cleft may be minimal, a small notch barely visible, or it may be complete with the opening extending fully through the lip, palate and bottom of the nostril. The cleft in the lip can be on the right side (30% of unilateral clefts), left side (70% of unilateral clefts) or both sides. Similarly, the palate cleft can extend partially or fully through the soft and hard palates. There is a genetic pattern for cleft lip and palate in many cases, with a different inheritance pattern for each type of cleft. Maternal smoking, alcohol use and folic acid deficiency can be associated with the development of cleft lip and palate in the fetus. Medications that treat a mother's seizure disorder may also be linked to development of this congenital defect.

Prevalence

Cleft lip and palate is the commonest birth defect. It occurs together in 1 of every 1,000 babies, somewhat more in males than females. About 20% have the cleft lip only, 30% have cleft palate only, and 50% of children with a cleft lip will also have a cleft palate. It is more common in Asians (1.7 per 1,000 births) and certain groups of American Indians (3.6 per 1,000). It is less frequent in African Americans (1 in 2,500 births). The risk of a second child having cleft lip and palate is 4-8%.

Common Associated Conditions

About 400 syndromes have cleft lip and palate as part of the affected systems. About 13% of babies with cleft lip and palate have other birth defects. Feeding problems, ear infections and speech difficulties as well as dental problems are associated with cleft lip and palate.

Short-term Treatment and Outcomes

Each child with a cleft lip and palate will have a unique plan of care and will need to be treated by a specialized interdisciplinary team, typically composed of pediatricians and expert nurses, plastic surgeons, dental specialists, otolaryngologists, speech-language pathologists, dieticians, audiologists and geneticists. Surgery to correct the cleft lip and palate will occur in stages, beginning with the lip repair at about 3 months of age. Sometimes flaps of skin and muscle will need to be moved to the lip in order to bridge the gap. The palate will be repaired later once the child's facial structures have grown bigger in order to be able to successfully pull the tissues together.

Children will need maximum calories to grow well and must be fed in an upright position to protect the airway and prevent aspiration of milk into the lungs. Patience, trial and error will be essential to determine the type of feeding aids that are most helpful (special bottles and nipples). Breastfeeding can be attempted, and if not possible, the baby will benefit from expressed breast milk because it helps ensure good growth and offers protection from infections.

Babies are prone to upper respiratory infections, including ear and sinus infections. Hearing should be tested because hearing may be muffled by fluid in the middle ear, further making the development of normal speech more difficult.

Long-term Treatment and Outcomes

Multistage surgeries will occur for children with significant clefts. If the gums are involved the teeth will need special bracing and perhaps dental surgery. Some children are missing teeth or have abnormally positioned or unusually shaped teeth. These problems can be corrected with satisfactory cosmetic and functional results. Some children need tympanostomy (also called pressure equalizing or PE tubes) to allow ventilation of the middle ear for healing to occur. Hearing testing periodically is often indicated. In adolescence, more surgery might be offered to get better correction of the area under the nose and to obtain a better appearance of the border of the upper lip.

Families will be offered the services of a genetic counselor to discuss the chances of recurrence in future pregnancies.

Common Complications

Children with clefts are especially susceptible to upper respiratory infections and ear infections because the Eustachian tube connecting the middle ear drains into the back of the throat and may have an unusual shape as well. Middle ear fluid can cause hearing loss that is typically not permanent.

Implications for Children's Development

Babies with cleft lip generally can feed well, although a special bottle with a phalange to close the cleft may be needed. A cleft palate often makes it difficult for the baby to suck and breathe at the same time. Special feeding bottles may be helpful, and in some cases a small shield (obturator) will be inserted in the roof the baby's mouth to help the baby feed successfully. Breastfeeding is possible for some babies with extra patience and careful positioning. If not successful, the mother can express milk and give it to the baby in a special feeder. Careful attention to feeding is very important to be sure the baby grows well and avoids pneumonia from aspirating milk.

Speech therapy is very helpful to children, who may learn to speak a bit later than other children because of the surgical procedures and discomfort. They may have a nasal quality to their speech as well as some difficulty with certain sounds. However they will catch up with speech therapy, and in most cases their speech will be normal.

Having multiple surgeries can be difficult for children and families, and ongoing visits to special professional teams will usually be necessary to anticipate difficulties and do further corrections for cosmetic or functional reasons as the child grows.

Children and families may benefit from counseling to support the child's self-esteem and help with developing self-confidence and independence. This very visible defect can make a child feel different and affect self-confidence and body image. Focusing on the child's strengths and abilities is very important throughout childhood.

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