CLEFT LIP AND CLEFT PALATE

                                CLEFT LIP AND CLEFT PALATE

Cleft lip and cleft palate are birth defects that occur when a baby’s lip or mouth do not form properly. Together, these birth defects commonly are called “orofacial clefts”. These birth defects happen early during pregnancy. A baby can have a cleft lip, a cleft palate, or both.

Children with a cleft lip with or without a cleft palate or a cleft palate alone often have problems with feeding and talking. They also might have ear infections, hearing loss, and problems with their teeth.

The Centers for Disease Control and Prevention (CDC) recently estimated that each year 2,651 babies in the United States are born with a cleft palate and 4,437 babies are born with a cleft lip with or without a cleft palate.¹ Cleft lip is more common than cleft palate. Isolated orofacial clefts, or clefts that occur with no other birth defects, are one of the most common birth defects in the United States. About 70% of all orofacial clefts are isolated clefts.

Cleft Lip

The lip forms between the fourth and seventh weeks of pregnancy. A cleft lip happens if the tissue that makes up the lip does not join completely before birth. This results in an opening in the upper lip. The opening in the lip can be a small slit or it can be a large opening that goes through the lip into the nose. A cleft lip can be on one or both sides of the lip or in the middle of the lip, which occurs very rarely. Children with a cleft lip also can have a cleft palate.

Cleft Palate

The roof of the mouth is called the "palate." It is formed between the sixth and ninth weeks of pregnancy. A cleft palate happens if the tissue that makes up the roof of the mouth does not join correctly. Among some babies, both the front and back parts of the palate are open. Among other babies, only part of the palate is open.

Causes and Risk Factors

Just like the many families affected by birth defects, CDC wants to find out what causes them. Understanding the risk factors that can increase the chance of having a baby with a birth defect will help us learn more about the causes. CDC currently is working on one of the largest studies in the United States―the National Birth Defects Prevention Study―to understand the causes of and risk factors for birth defects. This study is looking at many possible risk factors for birth defects, such as orofacial clefts. The causes of orofacial clefts among most infants are unknown. Some children have a cleft lip or cleft palate because of changes in their genes. Cleft lip and cleft palate are thought to be caused by a combination of genes and other factors, such as exposures in the environment, maternal diet, and medication use

Did You Know?

Women can take steps before and during pregnancy to reduce the risk of having a baby born with birth defects. Such steps include taking a daily multivitamin with folic acid (400 micrograms), not smoking, and not drinking alcohol during pregnancy.

Recently, CDC reported on important findings about some factors that increase the risk of orofacial clefts:

• Smoking―Women who smoke during pregnancy are more likely to have a baby with an orofacial cleft than women who do not smoke.
• Diabetes―Women with diabetes diagnosed before pregnancy have been shown to be an increased risk of having a child with a cleft lip with or without cleft palate.⁴

CDC continues to study birth defects, such as orofacial clefts and how to prevent them. If you smoke or have diabetes, and you are pregnant or thinking about becoming pregnant, talk with your doctor about ways to increase your chances of having a healthy baby.

Diagnosis

Orofacial clefts sometimes can be diagnosed during pregnancy, usually by a routine ultrasound. Most often, orofacial clefts are diagnosed after the baby is born. However, sometimes minor clefts (e.g., submucous cleft palate and bifid uvula) might not be diagnosed until later in life.

 Treatments

Services and treatment for children with orofacial clefts can vary depending on the severity of the cleft; the presence of associated syndromes or other birth defects, or both; and the child’s age and needs. Surgery to repair a cleft lip usually occurs in the first few months of life and is recommended within the first 12 months of life. Surgery to repair a cleft palate is recommended within the first 18 months of life.⁵ Many children will need additional surgeries as they get older. Although surgical repair can improve the look and appearance of a child’s face, it also may improve breathing, hearing, speech, and language. Children born with orofacial clefts also might need different types of treatments and services, such as special dental or orthodontic care or speech therapy.

Because children and individuals with orofacial clefts often require a variety of services that need to be provided in a coordinated manner, services and treatment by cleft teams is recommended. Cleft teams provide a coordinated, interdisciplinary team approach to care for children with orofacial clefts. These teams usually consist of experienced and qualified physicians and health care providers from different specialties. Cleft teams and centers are located throughout the United States and other countries. Resources are available to help in choosing a cleft team. With treatment, most children with orofacial clefts do well and lead a healthy life.

 Cleft lip and cleft palate are two conditions that occur during the facial development of a foetus. It occurs once in approximately every 600-800 births and can occur separately or combined.If the condition is left untreated, babies can have difficulties breastfeeding and therefore will be at risk of lacking the vital nutrients they need for their development. This can lead to malnutrition and even death.Clefting has been known to occur in other parts of the body during development, including the eyes, nose, cheeks and the forehead.In addition, an untreated cleft lip and palate can mean that a child and family experience social stigma that forces the parents to hide their children away.

Surgical intervention mends the damage, but finding children who need our help is essential. To find these children, often hidden away because of their appearance, CBM uses a network of Community Health Workers. Surgery can successfully correct a cleft lip or palate, although it is best to operate when the child is young.The surgery is conducted in partnership with local projects, and if required on-going rehabilitation services such as speech therapy can be made available.People with cleft lip or palate may also experience the following:

• Malnutrition*Deafness or Hearing Impairment *Problems with speech development *Psychosocial Problems

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.

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.

Approximately one in seven hundred children throughout Europe are born with cleft lip and/or cleft lip and palate, yet it remains little understood.  The condition is completely treatable, and with the right medical care and social support, children born with clefts can go on to achieve the same as any other child. Yet access to good treatment varies enormously throughout Europe, meaning that many children born with clefts are never given the opportunity to realise their full potential.

The first three months of a pregnancy are a critical time for a growing fetus: In addition to many other developments taking place throughout the body, the structure of the mouth and face also begins to take shape.Normally, at around the fifth or sixth week, the two sides of a baby’s upper lip begin to fuse together. Sometimes, though, the fusing doesn’t happen the right way and the upper lip is split, or cleft.A child with a cleft lip has a visible separation in the skin of her top lip. This space can be a small hole, or it can be a significant opening that extends from the base of the baby’s nose all the way down to her top jaw and gums. Between the eighth and twelfth weeks of pregnancy, the roof of a baby’s mouth begins to form. The roof of the mouth is made up of two parts:*the hard palate, the firm and bony plate hugging the horseshoe-shaped curve of the top teeth *the soft palate, the flexible, fleshier tissue that spans the back of the mouth *When the development of one or both of these parts is incomplete, the baby has what is known as a cleft palate. A child can be born with a cleft lip, a cleft palate or both. Cleft lip/cleft palate:

• when combined, affect one in every 700 babies born in the U.S., making them the fourth most common birth defect nationwide
• can be unilateral—involving only one side of the mouth and face—or bilateral, involving both
• are more common in boys than girls 
• affect more children of Asian, Latino and Native American descent—and fewer of African-American descent—than children of other ethnicities

The good news is that cleft lip/cleft palate are very treatable. Although children with more advanced cases may require assistance in several areas, and may need multiple procedures over time, there are several minimally invasive treatment options available to help them regain a normal appearance and range of functions.

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.

More than 2.5 lakh children have been operated upon in the country over a decade. But Smile Train says that an estimated 10 lakh more cases have to be covered.

The finding, so far, is that the cleft is a disorder impossible to prevent, though various reasons (see graphics) have been cited for its occurrence. The option is to identify cases continuously through Smile Train's network. The focus is on making more people aware of a movement that takes upon itself the financial burden of surgery for poor children.

Ashish Sabharwal, in charge of Smile Train's project in Gujarat, Madhya Pradesh, Rajasthan and a part of Maharashtra, says cleft does not differentiate between the rich and the poor. But, poverty stands in the way of a 45-minute surgery that costs only Rs. 12,000. That is why social workers of Smile Train go deep into villages to tell the poor that surgeons trained in bridging the cleft are doing these free of cost.

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.

                                                                      Prof. John Kurakar