Centre of excellence in cleft care in India

What is cleft lip and cleft palate?

Cleft lip and palate are facial birth deformities. A cleft lip is a gap or discontinuity in the upper lip. A cleft palate is an opening in the roof of the mouth, which divides the mouth and nose. Clefts result from incomplete development of the lip and/or palate in the early weeks of pregnancy. It is during this time that the face is being formed. Separate plates of bone and tissue gradually move towards each other and join to form the lip and palate. When these parts do not join completely, it results in a cleft.

On an average, one in every 700 children worldwide is born with a cleft. It is the fourth most common birth defect, and the first most common facial birth defect.

Causes

Genetic factors: Sometimes a cleft can be genetic. 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. Consanguineous marriage (marriage between close relatives or cousins) can also be a cause.

Environmental factors: Not all birth deformities are hereditary. Even in the absence of any genetic factors, there are some external factors that may result in cleft formation.

1. Maternal exposure to smoking, use of tobacco, illicit drugs or alcohol consumption during pregnancy is extremely harmful for the developing baby.
2. Certain medications like steroids and others as those prescribed for Seizures, Cancer, Arthritis, Tuberculosis etc have been linked to the development of a cleft. Harmful medications and erratic dosages taken without consulting a doctor may have detrimental effects on the foetus. Women taking oral contraceptive pills unaware of their pregnancy may also contribute to clefting.
3. Studies have shown cleft is also related to advanced maternal age and also to some hormone deficiencies and dietary deficiencies namely that of folic acid, iron, iodine and zinc.
4. Cleft lip and cleft palate may also occur as a result of exposure to viruses or infectious diseases like measles while the fetus is developing in the womb.
5. Exposure to certain harmful chemicals, vapours, pesticides, gun powder fumes, nuclear radiation, X-rays, gamma rays or dangerous reagents like in industrial workplaces have dangerous effects on the genetic composition and growth of the developing baby.

The frequency of diagnosing cleft lip and palate before birth is increasing with improvements in technology. A cleft lip may be detected in a routine ultrasound. Using advanced molecular genetics occurrence of clefts may be tested. Counseling can help families better prepare for their baby’s needs. Current research continues to investigate how use of multivitamins and folic acid can reduce the chances of cleft. - See more at:

1. Cleft can occur either in the lip, palate or both. The cleft of both the lip and palate mostly involves the upper alveolar (teeth bearing portion of the jaw) bone.
2. A cleft lip can be either unilateral (one-side only) or bilateral (both sides).
3. Cleft lip can either be incomplete (mild notching of the lip) or complete (large gap from lip to nose).
4. Cleft palate can either be Cleft of the Hard Palate- cleft in bony portion of roof of the mouth, Cleft of the Soft Palate- in the soft, muscular part of the palate, behind the hard palate or Cleft of both the Hard and Soft palates including the Uvula (soft tissue projection from middle of soft palate).

Submucous Cleft Palate - This cleft occurs in the tissues beneath the mucous membrane that covers the palate. Hence it is often called the “invisible cleft”. This cleft is detected only when the child has a difficulty in speaking.

The common problems that are faced by cleft children and their parents are related to appearance, nutrition and speech. As the child grows up, multiple surgeries are required to be performed to restore good health and appearance and for the child to lead a normal life.

Primary lip repair

The main concern is creating a lip seal to enable the child to suck milk. But before the child is healthy enough to undergo the surgery, a feeding plate may be given to prevent aspiration of milk from the mouth to the nose, through the gap in the palate (roof of the mouth). Specially designed feeding bottles may also be used to help in feeding.

The primary repair of the cleft lip is done at the age of 3 months. It is important to recreate the natural shape and muscle of the upper lip.

Palate Repair

The cleft palate repair surgery is performed at about 9 months of age. Early repair is done to close the gap in the roof of the mouth creating a barrier between the mouth and the nose. Since the palate plays a key role in speech, voice and phonation the cleft hard palate should be closed as early as possible. This surgery is very essential for normal speech to develop.

Pharyngoplasty

Because of the nature of the problem, the child with a cleft is more at risk of having a speech and/or language problem, and close attention must be given to the child's speech development. Most problems can, however, be resolved with speech and language therapy.

Cleft children commonly have Velopharyngeal incompetency (VPI). In this, the soft palate and posterior wall of the throat fails to separate the mouth from the nose during speech. This leads to a nasal twang in their voice. Their speech may be difficult to understand due to escape of air through the nose during speech. To help treat this problem, a surgery called Pharyngoplasty may be needed. In this procedure, soft tissues in the throat are surgically rearranged to treat VPI.

Pharyngoplasty combined with speech therapy helps the child to speak well.

Premaxillary set back

Premaxillary protrusion (protruding anteriormost portion of the upper jaw) is a characteristic feature of infants with complete bilateral cleft lip and palate. The surgical procedure premaxillary setback is designed to facilitate lip repair and to fit the premaxilla into the maxillary arch to achieve an idealized arch form at an early age. This is usually done in the pre-school age to enhance appearance.

Alveolar bone cleft closure

Closure of the cleft in the upper alveolar bone (tooth bearing region of jaw) is required to provide a solid surface for the permanent teeth to erupt into.

Conventionally, to close the gap in the upper alveolus a small amount of bone is taken from the child’s hip bone and placed in the region of the cleft in the upper jaw to close the gap. This incurs an additional surgery at the hip bone, increases blood loss and pain. More importantly, it results in a scar in the hip region. Nevertheless, there are no major complications.

Alveolar bone cleft closure using Bone Graft

This synthetic bone material is placed in the site which stimulates the body’s own cells to rapidly produce new bone thus promoting bone growth. Following this, normal teeth eruption and teeth alignment can be achieved.

With this, an additional surgery to harvest bone and subsequent scarring is prevented. Post-surgery recovery is faster and hospital stay is minimum. rhBMP-2 now ensures superior results to the conventional alveolar bone grafting technique by allowing cleft closure at a more younger age of 3-4 years than waiting till 7-8 years.

Cleft Orthodontics

A cleft can produce a variety of dental problems. The cleft displaces the teeth; teeth erupt in abnormal positions, disturbing their alignment.

Since children with clefts may have special problems related to missing, malformed, or malpositioned teeth, they require early evaluation by an orthodontist who is familiar with the needs of the child with a cleft.

Alveolar bone cleft closure using Bone Graft

Irregular teeth are also difficult to maintain. Teeth alignment is essential to maintain good oral hygiene. Patients with cleft lip and palate can suffer from deformed upper jaw and skeletal growth retardation. Jaw and teeth irregularities also affect speech. Continuous assessment and correction of jaw growth and dental development is required.

Nose Correction

As bone growth is retarded due to cleft, the nasal bones are also underdeveloped giving the nose a tilted appearance. The bridge of the nose and nose tip may be deviated to one side giving an asymmetrical appearance. Bony abnormalities may occur along the floor of the nose. This distortion in shape also affects breathing and nasal airflow.

Rhinoplasty or nose correction surgery aims at restoring normal form and function. These surgeries are approached from inside the nose and from deep below the upper lip so as to avoid any unsightly scars.

Distraction Osteogenesis

Cleft decreases the growth potential of the jaws. As a result, the upper jaw remains small while the whole face grows. Depending upon the degree of cleft , the severity of the jaw defect can range from slight depression to a dish face appearance.

In Distraction Osteogenesis, the jaw bone is cut and the distractor device is fitted between the cut ends. The screw connected to the device is gradually rotated. The rotation of the screw brings about a slow separation between the cut bone segments. New bone is formed to fill in the gap and ultimately the bone is lengthened.

Orthognathic Surgery

Corrective jaw surgery or orthognathic surgery may be required to correct disparity between upper and lower jaw, bring the jaws into proper alignment and to enhance the facial profile.

Secondary corrections

It may take more than one surgery to achieve the best possible appearance and function of the child’s lip and palate. This is especially true because as the child grows, the scars from previous surgeries may not grow and stretch in the same way as skin without any scars. As the child’s face develops, there may be a time when it would be appropriate and beneficial to have a “touch up” surgery to improve the appearance of the lip.

Abbe-flap surgery: After initial surgery, the scar on the upper lip thickens radically resulting in a thin upper lip. Abbe flap surgery may be done to correct this. A portion of the normal lower lip is taken, rotated across the mouth and placed into the defect in the upper lip maintaining blood supply. After blood supply is established and the blood vessel can be cut, the flap is divided.

Q. What are the causes of cleft lip or palate?

Genetics and environmental factors are both considered instrumental in causing clefts. Cleft parents are known to be predisposed to having cleft children. But the main cause is attributed to the deficiency of Folic Acid during pregnancy. The other causes are radiation, pollution, nutritional deficiency, psychologically disturbed pregnant women, trauma, alcohol consumption, smoking, tobacco chewing during pregnancy. The development of cleft is not influenced by simple x- rays, medications taken under medical supervision, trivial illness, injuries etc.

Is there a possibility of giving birth to another cleft child?

Yes. There is a high chance, if there is a genetic predisposition related to the familial history. Had the etiology been an environmental cause, there is a fair chance that the next child may be a normal one.

Is a cleft lip always associated with cleft palate?

Cleft lip is not always associated with cleft palate.

What can be done for my child born with a cleft?

Surgical repair of the cleft can be successfully done restoring appearance and function to normal. Most of the babies born with cleft have nasal deformity, so along with cleft surgery, nose correction is also done. We have seen excellent results in our patients. The treatment should begin as early as possible.

Why do cleft lip and palate require many surgeries? Is it not enough to do once?

Depending upon the deformity, surgeries are performed. The surgeon plans the surgeries with the ultimate aim of achieving normal form and function. The functional treatment always precedes the esthetic correction and has to be constantly revised as the child grows.

Will my child look normal after surgery?

Not immediately after surgery because of the swelling in the lip region and the scar appears red. The scar takes about 6 - 12 months to disappear to give a pleasing look.

How many days should my child be in the hospital after surgery?

The child is hospitalized for maximum of 2 - 3 days.

Can parents stay along with the child in the hospital?

Parents can stay and take care of the child in the hospital. The details about the stay can be cleared from the hospital staffs.

How should I feed my child before surgery?

If the child’s lip is incompletely split and the palate is intact, breast feeding or nursing bottle can be used. Regarding feeding please ask the doctor in charge as the child is always placed under 6 hour fasting prior to surgery.

How will my child feel about having cleft?

Insecurity in infants is due to parents’ depression, child’s lethargy and irritability. Social support, self-confidence and motivation might prove advantageous. So parents should have frank discussion and counseling about cleft management and should give their children proper guidance and encouragement.

Will my child speak like a normal child?

A child can speak well after the surgical repair of the cleft. Children with difficulty should undergo speech therapy and improvement in the speech depends on the co-operation and encouragement shown to the child.

How the palate helps in speech?

Before the palate is repaired, there is no separation between the nasal cavity (nose) and the mouth. This means that the child cannot build up air pressure in the mouth because air escapes out of the nose. The roof of the mouth has very less tissue for the tongue to touch. These problems can make it difficult for the child to talk.

Once the palate has been repaired, your child may be able to learn more words and speech can be improved with training and encouragement.

Will the nasal twang and fluid that escapes out of the nose stop after the treatment?

It cannot be estimated immediately because palatal repair takes time for healing. When the muscles become flexible the air can escape through the nose and mouth equally enabling the child to talk. Hence it is always mandatory to perform the surgery as advised by the Surgeon. Palate surgery should be done before the child learns to speak.

Q. What are the causes of cleft lip or palate?

Genetics and environmental factors are both considered instrumental in causing clefts. Cleft parents are known to be predisposed to having cleft children. But the main cause is attributed to the deficiency of Folic Acid during pregnancy. The other causes are radiation, pollution, nutritional deficiency, psychologically disturbed pregnant women, trauma, alcohol consumption, smoking, tobacco chewing during pregnancy. The development of cleft is not influenced by simple x- rays, medications taken under medical supervision, trivial illness, injuries etc.

Is there a possibility of giving birth to another cleft child?

Yes. There is a high chance, if there is a genetic predisposition related to the familial history. Had the etiology been an environmental cause, there is a fair chance that the next child may be a normal one.

Is a cleft lip always associated with cleft palate?

Cleft lip is not always associated with cleft palate.

What can be done for my child born with a cleft?

Surgical repair of the cleft can be successfully done restoring appearance and function to normal. Most of the babies born with cleft have nasal deformity, so along with cleft surgery, nose correction is also done. We have seen excellent results in our patients. The treatment should begin as early as possible.

Why do cleft lip and palate require many surgeries? Is it not enough to do once?

Depending upon the deformity, surgeries are performed. The surgeon plans the surgeries with the ultimate aim of achieving normal form and function. The functional treatment always precedes the esthetic correction and has to be constantly revised as the child grows.

Will my child look normal after surgery?

Not immediately after surgery because of the swelling in the lip region and the scar appears red. The scar takes about 6 - 12 months to disappear to give a pleasing look.

How many days should my child be in the hospital after surgery?

The child is hospitalized for maximum of 2 - 3 days.

Can parents stay along with the child in the hospital?

Parents can stay and take care of the child in the hospital. The details about the stay can be cleared from the hospital staffs.

How should I feed my child before surgery?

If the child’s lip is incompletely split and the palate is intact, breast feeding or nursing bottle can be used. Regarding feeding please ask the doctor in charge as the child is always placed under 6 hour fasting prior to surgery.

How will my child feel about having cleft?

Insecurity in infants is due to parents’ depression, child’s lethargy and irritability. Social support, self-confidence and motivation might prove advantageous. So parents should have frank discussion and counseling about cleft management and should give their children proper guidance and encouragement.

Will my child speak like a normal child?

A child can speak well after the surgical repair of the cleft. Children with difficulty should undergo speech therapy and improvement in the speech depends on the co-operation and encouragement shown to the child.

How the palate helps in speech?

Before the palate is repaired, there is no separation between the nasal cavity (nose) and the mouth. This means that the child cannot build up air pressure in the mouth because air escapes out of the nose. The roof of the mouth has very less tissue for the tongue to touch. These problems can make it difficult for the child to talk.

Once the palate has been repaired, your child may be able to learn more words and speech can be improved with training and encouragement.

Will the nasal twang and fluid that escapes out of the nose stop after the treatment?

It cannot be estimated immediately because palatal repair takes time for healing. When the muscles become flexible the air can escape through the nose and mouth equally enabling the child to talk. Hence it is always mandatory to perform the surgery as advised by the Surgeon. Palate surgery should be done before the child learns to speak.