CLEFT LIP AND PALATE

A cleft is a gap or split in the upper lip, the roof of the mouth (palate), or sometimes both. It occurs when there is non-union of the two halves of the face during development.

The face and upper lip develop between the fifth and ninth weeks of pregnancy. Most clefts are picked up either at the time of the routine 20 week scan, or soon after birth.

The cleft can affect the lip area, the roof of the mouth (palate) or both. The cleft can involve part or all of these areas and may be one-sided (unilateral) or affect both sides (bilateral).

What causes cleft lip and palate?

The exact cause of clefts is not known. However, evidence suggests they are often caused by a combination of genetic and environmental factors.

A healthy lifestyle during pregnancy is likely to reduce the risk of a cleft occurring. For example, avoiding smoking and alcohol, having a healthy diet and avoiding excessive weight gain.

Cleft lip and palate can occur on its own (non-syndromic) or can sometimes be part of a wider series of birth defects (syndromic).

Symptoms of cleft lip and palate 

The symptoms of a cleft lip and palate can vary depending on which area is affected.

There are two types of cleft lip:

  • unilateral cleft, where the cleft occurs on one side of the lip
  • bilateral cleft, where the cleft occurs on both sides of the lip

The appearance of the cleft lip can vary from a small notch in the lip (incomplete cleft) to a wide gap that runs all the way up to the nostril (complete cleft lip).

The appearance of a cleft palate can range from an opening at the back of the soft palate to a complete cleft of the roof of the mouth (hard and soft palate).

A cleft lip is usually fairly obvious, while a cleft palate alone is hidden inside the mouth. Side cleft palates are not easily visible, they may be noticed soon after birth. The palate should be carefully examined especially when feeding difficulties, failure to suckle or, frequent back flow of milk down the nose are present.

Hidden cleft palate

A cleft lip and palate is visible at birth. However, a type of cleft palate called a submucous cleft palate can be hidden by the lining of the roof of the mouth. This type of cleft palate can be difficult to diagnose early on and in some cases may not be apparent for some months or years.

Consult with the pediatrician is recommended when the following symptoms occur:

  • milk frequently comes out of the nose when feeding
  • an inability to suck through a straw or blow out candles
  • nasal-sounding speech

These symptoms may indicate a submucous cleft palate.

Pierre-Robin cleft palate

Some children with a cleft palate may also have a small or ‘set-back’ (retruded) lower jaw. This may cause the tongue to interfere with the upper airway and breathing. This combination of features is called Pierre-Robin sequence.

A Pierre-Robin cleft palate may lead to difficult and noisy breathing. Breathing difficulties can be managed by careful positioning of the infant or sometimes by placing a special breathing tube into the nose. In very severe cases, a tracheostomy may be required.

In most cases, the lower jaw grows and catches up during the first few months. It is quite common for this type of cleft palate to be repaired a little later than usual, often around one-year of age.

Diagnosing cleft lip and palate 

Clefts affecting the lip are often, but not always, picked up during an ultrasound.  While clefts of the palate are not usually diagnosed during the ultrasound scan and are nearly always diagnosed soon after birth.

However, some clefts – such as a submucous cleft palate, where the cleft is hidden by the lining of the roof of the mouth – may not be detected for several months or even years, when speech problems may develop.

Treating cleft lip and palate 

Surgical correction is the definitive treatment for cleft lips and palates. Other treatments may be needed to treat associated symptoms, such as speech therapy or dental care.

In this section, the term cleft refers to a cleft lip, cleft palate, or a cleft lip and palate. Specific types of cleft are only referred to when necessary.

Cleft clinics and treatment teams

The cleft treatment team is made up of healthcare professionals from different specialist backgrounds who work closely together. The team includes:

  • a cleft surgeon, who will carry out the repair of the cleft
  • a paediatrician, a doctor who specialises in treating children
  • an audiologist and an ear, nose and throat (ENT) surgeon, who will both assess and treat hearing conditions
  • a speech and language therapist, who will assess and treat speech problems
  • a paediatric (children’s) dentist, who will help prevent dental decay
  • an orthodontist, who specialises in dental and jaw development
  • a psychologist
  • a nurse

Care plan timetable

Most children with clefts will receive the same type of care plan tailored to meet their individual needs. A typical care plan timetable for cleft lip and palate is described below:

  • following birth to six weeks: feeding assistance, support for parents, hearing test and paediatric assessment
  • three months: surgery to repair a cleft lip
  • six to 12 months: surgery to repair a cleft palate
  • 18 months: speech assessment
  • three years: speech assessment
  • five years: speech assessment
  • eight to 11 years: bone graft to the cleft in the gum area (alveolus)
  • 11-15 years: orthodontic treatment and monitoring jaw growth

Once the initial care plan has been completed, regular outpatient appointments at the cleft clinic are recommended for close monitoring and evaluation of other problems that needs to be managed.

Important records are taken at key stages of development at age five, 10, 15 and 20 years to monitor how treatment has progressed over time.

Feeding

If a cleft is present, feeding will need to be assessed so any problems can be resolved. Many babies with a cleft palate have problems breastfeeding due to the gap in the roof of their mouth.

In the presence of feeling problems alternative feeding methods may be taught. One method that works well for some women is expressing breast milk and using a specially made flexible bottle for feeding.

Lip repair surgery

Lip repair surgery is usually carried out at three months of age. The procedure is done under general anesthesia and the cleft lip carefully repaired, including underlying muscles. The nose is usually reshaped at the same time.

The operation usually takes between one and two hours, although it can take longer if the cleft is more extensive. The surgery leaves a slight scar, but the surgeon will attempt to line up the scar with the natural lines of the lip to make it less noticeable.

Palate repair surgery

Palate repair surgery is usually performed when the child is six to 12 months old. The muscles and the lining of the palate are rearranged and usually no extra tissue is needed to complete the operation.

The operation usually takes about two hours and is carried out under general anaesthesia.

Additional surgery

In some cases, additional surgery may be needed to improve the appearance and function of the lips and palate. Although the palate works well during speech for most children after surgery, in a small number of cases further surgery may be necessary. This may be necessary if the palate is not working sufficiently well for normal speech or if a hole has opened up in the palate during the early stage of healing.

Also, if there is a cleft in the gum (alveolus), the surgeon will perform a bone graft operation to repair the alveolar cleft, usually when the child is around nine to 12 years old.

In some young adults, surgery can be considered if the growth of the jaws are not equal.

Treating associated symptoms

Hearing

Children with a cleft palate are more likely to develop a condition called glue ear. This is because the muscles in the palate are connected to the middle ear. Because the muscles are not working properly due to the cleft, sticky secretions may build up within the middle ear which may reduce hearing. The condition may improve after cleft palate repair and if necessary, can be treated.

If hearing is significantly reduced, the ENT surgeon may recommend inserting a tiny plastic tube called a grommet into the eardrum. This lets out the sticky secretions and allows air in. Sometimes, a hearing aid may be recommended.

An audiologist will assess hearing at birth and a second hearing assessment will take place once your child has had reconstructive surgery. Hearing will be regularly assesed for the first few years.

Speech and language therapy

Repairing a cleft palate will significantly reduce the chance of future speech problems. However, approximately half of all children with a repaired cleft palate still need some form of speech therapy. Further corrective surgery may be required for a small number of children who have increased airflow through their nose when they’re speaking.

A speech and language therapist (SLT) will carry out an initial assessment after surgery, followed by a further assessment once the child is three years old. If the assessment reveals problems with your child’s pronunciation and use of language, the SLT will teach you speech exercises. They may also carry out a number of one-to-one exercises. Children born with cleft lip only do not normally need further therapy.

The SLT will work as long as assistance is needed. Therapy is not usually needed after the age of seven.

Dental care

When the cleft involves the gum area, it’s common for teeth on either side of the cleft to be tilted or out of position. Often a tooth may be missing, or there may be an extra tooth. The pediatric dentist will evaaluate and recommend additional treatment when necessary.

Orthodontics help improve the alignment and appearance of teeth. Treatment can include using braces or other dental appliances to help in the alignment of teeth. Orthodontic treatment will also monitor the development of the jaws and the bite during growth.

Children with a cleft are more vulnerable to tooth decay, so it’s important to encourage them to practice good oral hygiene.

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