Cleft Palate without Cleft Lip

 

Condition Description

Cleft is a congenital defect where there is a separation in the middle of the roof of the mouth (the palate). The palate has 2 parts: the “hard palate”, which is bone, and the “soft palate”, which is the mucous membrane at the back of the roof of the mouth. Cleft palate can involve only the soft palate, or it can also extend towards the teeth and involve the hard palate as well. The cleft can be on the left, right or in the middle of the palate.

The causes of isolated cleft palate are unknown, but the problem occurs early in fetal life. In the 10th week the palate cells should come together and fuse but for some reason this process is disrupted. Maternal smoking, alcohol use and nutritional deficiency of folic acid may contribute to causing cleft palate, and it also runs in certain families so it has a genetic cause as well.

Prevalence

Cleft palate alone (without a cleft in the lip) occurs in about 1 of 2000 babies and is less common than the combined cleft lip/palate defect. It occurs in all racial groups and affects females more than males. Many genetic syndromes will have cleft palate as one of the baby's problems. The recurrence of a cleft in subsequent pregnancies is 2-8%.

Common Associated Conditions

There are at least 400 syndromes in which babies have a palate cleft as well as other defects. At least 50% of babies with cleft palate will have other birth defects.

Short-term Treatment and Outcomes

Ideally children with cleft palates will be treated by an interdisciplinary team that will plan the best possible comprehensive plan of treatment. The best team will include a pediatrician, a plastic surgeon, a dental specialist, an otolaryngologist (ear, nose and throat specialist), a speech-language pathologist, an audiologist (hearing specialist), a geneticist, a psychologist and social worker. A pediatrician or pediatric nurse practitioner is also an important team member. The palate will be surgically repaired between 9 and 18 months of age because by that time the baby's palate will have grown sufficiently to permit closure.

The family will usually need assistance and special equipment to feed a baby with a cleft palate. 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. Holding the baby in an upright position for feedings will be important to protect the airway and prevent milk from being aspirated into the lungs. It is very important that babies receive good nutrition so that they grow well and brain growth is not affected. Speech therapy will be an important part of the treatment plan, as well as dental care. Wound care to prevent scarring after surgery is very important.

Long-term Treatment and Outcomes

Children with cleft palate defects often need further surgery as they grow. Dental work will need to be carefully planned as teeth placement may be affected. Thus dental care is very important from an early age. An orthodontist and oral surgery will need to work together to improve the placement of teeth and braces are common.

Sometimes sinus and middle ear infections are chronic concerns affecting overall health of the child and decreasing hearing due to middle ear fluid. Many children will have small drainage tubes (tympanostomy or PE tubes, also called pressure equalizing tubes) placed in the eardrum to allow air to enter the middle ear to help the infection to heal.

Common Complications

Complications include feeding problems, ear infections, dental problems and speech impediments. The cleft in the palate makes it hard for babies to create a suction at the nipple or breast, and the opening between the mouth and the nose can cause choking and gagging as the baby tries to prevent milk from going into the lungs. Ear infections are common because the Eustachian tube opens into the area at the back of the throat. Hearing loss can occur from the ear infections, but it is usually from fluid in the middle ear and is not permanent if the ear infections are properly treated. Sometimes the cleft in the palate will extend into the gum line and involve the teeth. Teeth may have unusual placement or shapes or some teeth may not be present.

Implications for Children's Development

Feeding is a major issue for babies with cleft palate defects. Some babies with less severe clefts can successfully breast-feed with careful upright positioning. Mothers can also express their milk and feed the baby by bottle. Special bottles can be used, and in some cases a small plastic plate can be put in the roof of the baby's mouth during feedings to make sucking more successful. Good nutrition is so important for the baby in the months before surgical repair to ensure maximum growth and good health.

Speech is another area where the child may need some special therapy in order to have intelligible speech. Children with cleft palate may need speech therapy to pronounce consonants and to reduce the nasal sound quality of their speech.

For more information, including resources for parents and general information about congenital cleft conditions, visit the following websites: