Cleft Lip Repair

Cleft Lip Repair

Orofacial clefts are birth defects in which there is an opening in the lip and/or palate (roof of the mouth) that is caused by incomplete development during early fetal formation.

Cleft lip and cleft palate occur in about 1 or 2 of every 1,000 babies born in the United States each year, making it one of the most common major birth defects. Clefts occur more often in children of Asian, Latino, or Native American descent.

The good news is that both cleft lip and cleft palate are treatable. Most kids born with these can have surgery to repair these defects within the first 12-18 months of life.

About Oral Clefting

An orofacial cleft occurs when parts of the lip or palate do not completely fuse together during the first 3 months of pregnancy. A cleft lip may appear as a small notch in the edge of the lip only or extend into the nose. It may also extend into the gums.

A cleft palate may also vary in size, from a defect of the soft palate only to a complete cleft that extends through the hard palate. Because the lips and the palate develop separately, it is possible for a child to be born with a cleft lip only, cleft palate only, or both.

Most clefts can be categorized into three broad categories:

1. cleft lip without a cleft palate
2. cleft palate without a cleft lip
3. cleft lip and cleft palate together

A cleft can occur on one side of the mouth (unilateral clefting) or on both sides of the mouth (bilateral clefting).

Cleft lip with or without cleft palate is generally more common among boys; however, cleft palate occurring alone is more common in girls than boys.

For the most part, because a cleft lip is visible it is often easier to identify than a cleft palate alone. A cleft lip may be detected through prenatal ultrasound; however, diagnosing a cleft palate this way is more difficult and it might not be seen.

Even if a cleft condition is detected during pregnancy, the diagnosis and extent of cleft lip and palate is confirmed by physical examination after the birth of the child.

Causes

Sometimes a cleft occurs as part of a syndrome, meaning there are birth defects in other parts of the body, too. Other times, it's genetic and runs in families — the risk may be higher for children whose sibling(s) or parents have a cleft or who have a history of cleft in their families. In these situations, both mothers and fathers can pass on a gene or genes that can contribute to the development of cleft lip or cleft palate.

Sometimes a cleft may be associated with environmental factors such as a woman's use of certain medications, exposure to cigarette smoke, or lack of certain vitamins while pregnant.

Most of the time, though, the cause isn't known.

Associated Problems

Cleft lip and palate can be associated with other problems, including feeding difficulties, middle ear fluid buildup and hearing loss, dental abnormalities, and speech difficulties.

Feeding Problems

Infants with a cleft lip alone usually have fewer problems feeding than those with a cleft palate. Feeding can be a big problem for a newborn baby with a cleft palate. Normally, the palate prevents food and liquids from entering the nose. The baby with an unrepaired cleft palate has difficulty sucking on a regular nipple and will usually require a special nipple and bottle along with proper positioning in order to feed. With these techniques, the caregiver will learn how to feed the baby before taking the baby home from the hospital. The child's doctor will carefully monitor the child's weight.

Middle Ear Fluid Buildup and Hearing Loss

Many children with cleft palate are prone to the buildup of fluid in the middle ear and/or ear infections caused by malfunction of the Eustachian tube. This fluid buildup behind the eardrum can cause hearing loss. For this reason, kids with cleft palate usually need small pressure equalization (PE) tubes placed in their eardrums to help them to drain the fluid and improve hearing. Kids with cleft palate should have their ears and hearing checked once or twice a year; more often if there is ear drainage or a child seems to be having difficulty hearing.

Dental Abnormalities

Children with a cleft lip and palate frequently have dental problems. These include small teeth, missing teeth, extra teeth (called supernumerary), or malpositioned teeth. They may have a defect in the gums or alveolar ridge (the bone that supports the teeth). Defects of the alveolar ridge can displace, tip, or rotate permanent teeth, or prevent permanent teeth from coming in properly.

Speech Difficulties

Kids with cleft lip have fewer speech problems than those with cleft palate. Approximately 15%-20% with cleft palate may have speech problems after repair of the cleft palate (palatoplasty). The most common is excess nasality or hypernasality. This happens because the palate that normally separates the nose from the mouth for most sounds does not close adequately. This condition makes it sound as if the person is talking through the nose.

Children with clefts also can have other types of speech problems unrelated to the cleft condition; for example, age-related errors such as saying "wed" instead of "red." Sometimes, the dental problems associated with the cleft will cause some sounds to be distorted, particularly "s," "sh," "ch," and "j" sounds.

A speech-language pathologist will carefully assess a child's speech and language skills. If your child does have a speech problem, the pathologist will identify the cause and recommend treatment.

Treatment

The complex needs of a child with cleft lip and cleft palate are best met by an interdisciplinary team of professionals from various specialities who work together. This is a standard of care that begins soon after the child's birth and continues to adulthood.

The members of the cleft lip and palate treatment team include:

• geneticist
• pediatrician
• plastic surgeon
• ear, nose, and throat physician (otolaryngologist)
• oral surgeon
• orthodontist
• dentist
• speech-language pathologist
• audiologist
• nurse
• social worker
• psychologist
• team coordinator

The frequency of team visits will depend on the child's needs and can range from two to three times per year to once every 2 to 3 years. Which team members the child needs to see during a given visit will depend on his or her health needs, including psychosocial issues.

After each visit, a team report will be sent to the family and other professionals involved in the child's care. The team coordinator will help organize the visits with team members and other professionals.

Surgical Treatment for Cleft Lip and Cleft Palate

A cleft lip is usually repaired between the ages of 3 to 6 months. Some children who have very wide clefts of the lip may require a procedure such as lip adhesion or a device such as a molding plate to bring the parts closer together before the full lip repair. A child with a cleft lip that is repaired will have a scar on the lip under the nose. Surgery is performed in the hospital under general anesthesia.

A cleft palate is usually repaired between 9 and 12 months of age. By repairing the palate, the soft palate muscles from each side are connected to each other and the normal barrier between the mouth and nose created. Surgery for cleft palate is performed under general anesthesia and usually requires a 2-night stay in the hospital.

The goal of surgery is to create a palate that works well for speech. Some kids, however, will continue to sound nasal after cleft palate repair and some may become nasal due to natural growth changes or adenoid shrinkage.

In some cases, additional surgery may be needed to improve speech. This surgery is called a pharyngoplasty. It is often done when kids are in their early school years, but also can be done later.

As kids grow older, they might need additional surgeries, such as an alveolar bone graft, which is used to close the gap in the bone or gums near the front teeth. This provides stability for the permanent teeth and is usually done when kids are between 6 and 10 years old.

Other procedures might be options as kids get older. They may want to have their scars made less noticeable, improve the appearance of their nose and upper lip, or improve their bite with orthognathic surgery. These operations may improve speech and breathing, dental occlusion, and appearance.

Your child's surgeon will talk with you about the timing and nature of these surgeries.