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 separate areas of the face do not join together properly when a baby is developing during pregnancy.
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).
Read more about diagnosing cleft lip and palate.
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 enviromental 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).
Read more about the causes of cleft lip and palate.
Who is affected?
A cleft lip and palate is the most common facial birth defect in the UK. One in every 700 babies is born with a cleft. The type of cleft and its severity vary widely between children.
Nearly half of all affected children have a cleft palate. Around a quarter of affected children have a cleft lip and a quarter have both a cleft lip and palate.
Unilateral cleft lip and palate is more common than bilateral cleft lip and palate which accounts for about 10% of all cases. For unknown reasons, left sided cleft lip and palate is twice as common as right sided cleft lip and palate.
A cleft lip or combined cleft lip and palate are more common in boys, but a cleft palate on its own is more common in girls.
Treating cleft lip and palate
In England and Wales, there are nine specialist cleft lip and palate centres, with separate specialist networks in Scotland and Northern Ireland.
Surgery is the usual treatment for cleft lip and palate, with good results. Other forms of treatment may also be required depending on the type of cleft, such as speech and language therapy and orthodontics. After treatment most children have a normal appearance with minimal scarring and normal speech.
All types of treatment for cleft lip and palate are available on the NHS. A long-term, structured care plan from a team of cleft specialists is usually needed to help children born with clefts.
Excellent results are possible with well-coordinated treatment provided by specialists working together within a dedicated cleft lip and palate centre.
Learn more about treatment for cleft lip and palate.
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).
Whilst a cleft of the lip is usually fairly obvious, a cleft palate is hidden inside the mouth and may not be easily visible. However, most cleft palates are picked up soon after birth. If there are feeding difficulties soon after birth, such as a failure to suckle or frequent escape of milk down the nose during feeding, then the palate should be carefully examined by a health professional.
Hidden cleft palate
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.
You should take your child to see your GP if they develop the following symptoms:
- 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.
Learn more about diagnosing cleft lip and palate.
Causes of cleft lip and palate
A cleft lip or palate happens when the structures that form the upper lip or palate fail to fuse together properly when a baby is developing in the womb.
In most cases the exact cause is unknown, but it’s thought to be a combination of genetic (internal) and environmental (external) factors.
Research indicates that the genes a child inherits from their parents occasionally makes them more vulnerable to developing a cleft lip or palate. A number of genes have been identified that may be responsible.
In some cases there is a family history of clefts, although most children of parents with clefts will not develop them.
Environmental risk factors
A number of things have been identified that may increase a child’s chance of being born with a cleft lip or palate. These are outlined below.
Lack of folic acid during pregnancy
All pregnant women are advised to take a daily supplement of folic acid during the first four months of pregnancy. Folic acid helps reduce the possibility of birth defects.
While it is known that folic acid deficiency during pregnancy increases the risk of a cleft developing, there is no firm evidence yet to suggest an intake of higher doses of folic acid prevents clefts. However, mothers from families with a history of clefting are often advised to take higher doses of folic acid during pregnancy. This should be discussed with your doctor.
A mother who smokes during pregnancy increases her baby’s chance of being born with a cleft. The risks from passive smoking are not fully known, but it is a good idea to avoid breathing in high levels of secondhand smoke.
Read more about treatments to help you stop smoking.
Some studies have shown a link between a mother’s alcohol consumption during pregnancy and the development of a cleft lip or palate in her baby.
Read more about alcohol in pregnancy.
Obesity and nutrition
Mothers who are obese have a higher chance of their child being born with a cleft. Poor nutrition during pregnancy can also increase the risk.
Medications during pregnancy
It has been suggested that some medications taken during pregnancy may increase the risk of cleft lip and palate. These include:
- methotrexate, a medicine used to treat cancer, arthritis and psoriasis
- isotretinoin, a medicine used to treat acne
- anti-seizure medicine
Speak to your GP or midwife if you are concerned about any medication you are taking during your pregnancy.
Pierre Robin syndrome
Pierre Robin syndrome is a rare condition where a baby is born with a small lower jaw and a tongue positioned further back in the mouth than usual. This can result in breathing difficulties which requires careful positioning of the baby and, sometimes, a breathing tube placed through the nose.
Most infants with Pierre Robin syndrome will also have a cleft palate.
The cleft can usually be repaired with surgery, although treatment may be delayed if there are continuing issues with airway obstruction. The lower jaw usually has some ‘catch-up’ growth later and orthodontic treatment can further help the bite.
Diagnosing cleft lip and palate
Clefts affecting the lip are often, but not always, picked up during an ultrasound scan carried out in mid-pregnancy. The cleft nurse specialist will then normally be in contact with you and arrange to meet you.
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 (see symptoms of cleft lip and palate).
Specialist cleft lip and palate team
As soon as a cleft has been diagnosed, you and your child will be referred to a specialist cleft team. An arrangement can also be made for a specialist cleft nurse to visit you in hospital or at home to help with feeding and answer any questions you may have.
The specialist nurse will liaise with other healthcare professionals involved with your child’s care and arrange for you to attend a cleft lip and palate clinic appointment at the hospital.
Learn more about your child’s care team in treatment of cleft lip and palate.
Coping with a diagnosis
Being told that you or your child has a condition such as cleft lip – either at birth or as a result of screening – can be a confusing and frightening experience.
You’ll be given the chance to talk to specialists and the specialist cleft lip and palate team about what having a baby with a cleft lip might mean to you and your family, including the available treatments, and how it can affect your life.
Below is a list of charities and other organisations you may find useful:
- The Cleft Lip and Palate Association organises parent-to-parent support networks across the UK as well as providing a specialist feeding advice service.
- The British Association of Plastic, Reconstructive and Aesthetic Surgeons has detailed information on the reconstructive surgery options for cleft lip and palate.
- The Craniofacial Society is a good source of information on the latest research into cleft lip and palate.
Treating cleft lip and palate
Cleft lip and palate is usually treated with surgery. 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
In England, all children born with a cleft are referred to a specialist multi-disciplinary cleft team within a specialist centre.
The cleft treatment team is made up of healthcare professionals from different specialist backgrounds who work closely together. The team includes:
- a specialist cleft nurse, who will provide initial advice about feeding and act as your first point of contact
- 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
The core members of the team will be available at the outpatient cleft clinic appointments to give you advice and answer any questions you may have.
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, your child will attend regular outpatient appointments at the cleft clinic so their condition can be monitored closely and any problems can be dealt with.
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.
If your baby has a cleft, their 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.
If your baby has difficulty feeding, your specialist cleft nurse can advise on alternative feeding methods. Some babies respond better to alternative feeding methods than others.
One method that works well for some women is expressing breast milk and using a specially made flexible bottle for feeding.
Lip repair surgery
All types of surgery required for cleft lip and palate are available on the NHS. There are nine centres in England and Wales and further specialist centres in Scotland and Northern Ireland.
Lip repair surgery is usually carried out when your child is three months old. Your child will be given a general anaesthetic (where they are asleep) 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 carried out when your 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 anaesthetic.
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
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 (hearing specialist) will assess your child’s hearing at birth and a second hearing assessment will take place once your child has had reconstructive surgery. Your child’s hearing will also be regularly assessed during 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 your 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 to help your child’s development. They may also carry out a number of one-to-one exercises with your child. Children born with cleft lip only do not normally need further therapy.
The SLT will work with your child for as long as they need assistance. Therapy is not usually needed after the age of seven.
Watch a video on speech and language therapy.
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 paediatric dentist will monitor the health of your child’s teeth and recommend treatment when necessary. It’s also important that you register your child with a family dentist.
Orthodontic treatment, which helps improve the alignment and appearance of teeth, may also be required. Treatment can include using braces or other dental appliances to help straighten the teeth. Orthodontic treatment will also monitor the development of the jaws and the bite during growth.
Read more about complications of cleft lip and palate.
Problems related to a cleft lip and palate
Giving birth to a baby with a cleft can have a profound psychological impact both on the parents and the child.
It’s common for parents to feel guilty, angry, shocked, helpless or disappointed.
However, it’s important to realise that in most cases a cleft cannot be prevented and you are not to blame. Some parents may worry that they will be unable to bond with a child who has a cleft, but there’s no evidence this is the case.
Your cleft care team will be used to supporting parents during the difficult time that can follow the birth of a baby with a cleft. Take this opportunity to express any concerns and worries you have and ask as many questions as you want.
Some parents find that learning as much as they can about the condition helps them cope better. Talking to other parents in similar circumstances can also help reduce feelings of anxiety and isolation.
Psychological impact in children
A cleft lip and palate can also have a psychological impact on a child, particularly as they get older and begin to mix with other children.
Experts advise explaining the condition to your child as soon as they are old enough to understand. Treat it as a normal topic of conversation. The clinical psychologist in the team will be able to give further support with this.
You can also help strengthen your child’s independence by involving them in the decision-making process about their treatment and their life in general.
If you are worried about your child’s social or psychological development, speak to your specialist cleft nurse. They can arrange an assessment and any necessary counselling and support.
Babies with a cleft may have problems feeding, particularly if the cleft includes the palate, as the baby may not be able to suck adequately.
However, feeding can usually be established quickly with assistance and sometimes the use of a special feeding bottle. Tube feeding is very unlikely unless there are also other feeding or swallowing difficulties.
Children with a cleft palate often develop a condition called glue ear, where sticky fluid collects in the middle ear behind the eardrum. This may reduce their level of hearing and can sometimes cause ear infections.
It is therefore important that all children with a cleft palate have their ears and hearing checked regularly by an audiologist.
See glue ear for more information.
Surgery to repair the cleft lip and palate is generally successful and complications are uncommon.
Cleft lip repair is often carried out when a child is between three and four months old and cleft palate repair when they are between six and 12 months old. Sometimes the timing of surgery may be later if there are good medical reasons for delaying the surgery. The cleft team will discuss with you the appropriate timing of the surgery.
Healing after surgery is good in most cases however healing complications do occur occasionally such as a wound infection or opening of the palate repair line. If this occurs then further surgery may be required.
As the child grows, the palate usually continues to function well for speech. However if it does not, then the cleft team may recommend investigating the palate function further.
Speech and language problems
The roof of the mouth (palate) plays an important role in helping your child form sounds for speech.
If a cleft palate is not repaired with surgery, it will lead to speech problems when the child is older. The cleft palate is therefore carefully repaired before speech development starts and in most cases the child goes on to develop normal speech.
About half of all children with a repaired cleft palate will need speech therapy, and a small number may need further surgery. Over time, a specialist speech and language therapist will monitor the child’s speech development.
A cleft lip and palate can cause changes to the structure of the mouth and lead to problems with the development of teeth, making children more vulnerable to tooth decay. It is therefore important for the child to have their teeth checked regularly and practice good oral hygiene.
Children with clefts are more likely to develop tooth decay. This is partly due to crowding of the teeth, which makes it more difficult to keep them clean. The advice below may help reduce your child’s risk of developing tooth decay.
- Your child should have a regular dental check-up.
- Encourage your child to brush their teeth after every main meal. When they are at school, you could put a small tube of toothpaste and a toothbrush in their lunchbox.
- Using a fluoride mouthwash once a day will help protect your child’s teeth against decay.
- Limit the amount of sugary foods and drinks your child consumes, especially between meals, as these foods increase tooth decay.
- Limit the amount of starchy foods such as crisps, white bread and biscuits that your child eats, especially between meals, to lower the risk of tooth decay.