Dr. Dirk Lazarus, Plastic Surgeon, Cape Town, South Africa

CLEFTS OF THE LIP AND/OR PALATE are commonest congenital abnormality of the face, occurring in about 1 in 665 newborn babies. Overall, they are the second most common congenital abnormality seen in children (after club foot).

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What causes clefts?

In the vast majority of cases, we do not know what causes a child to be born with a cleft of the lip and/or palate. It seems as if both genetic and environmental factors may be involved and that the aetiology is multifactorial.

A specific genetic abnormality is rarely identified as being the cause of a cleft. Chromosomal abnormalities, such as Down's syndrome or trisomy 13 may be associated with a cleft.

Drugs, medication and infections during very early pregnancy may result in the formation of clefts. Mothers who drink excessive alcohol during pregnancy may give birth to a child with foetal alcohol syndrome which may be associated with clefting. Some drugs or medications, such as anticonvulsants (epilepsy medication), anti cancer drugs and huge doses of steroids may also lead to the development of cleft lip and palate. Roaccutane, used for the treatment of acne, can also cause cleft lip and palate. Conflicting evidence regarding smoking exists, and despite the well known detrimental effects of smoking in other arenas, no clear evidence yet exists to link it to cleft formation. Importantly, folic acid and vitamin B6 (found in green leafy vegetables) seem to have a protective effect (similar to preventing neural tube defects, such as meningomyelocoele).

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What are the different types of clefts?

Clefts occur in a wide variety of different types and different severity's. Clefts may affect the nose, the lip, the gum, the hard palate, the soft palate and the uvula (that bit which hangs down in the back of your mouth). For simplicity sake, they are divided into clefts of the lip (which includes the nose, lip, gum and front of the palate) and clefts of the palate (most of the hard palate, the soft palate and uvula). Clefts may affect one side (so called, unilateral clefts) or both sides (bilateral clefts). In addition, they may be complete or incomplete. Some forms of cleft are so minor that they present as only a notch in the lip or gum, or a mild weakness of the palate muscles while some types may be complete, bilateral clefts of the lip and palate.

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What are the risks of the next baby having a cleft?

After the birth of a cleft child, the risk of subsequent children being born with a cleft is small. If normal parents produce a cleft child, the chances of subsequent siblings having a cleft are about 4-5%, more so if the cleft is bilateral and more so if the cleft involves the hard palate rather than the soft. If the parents produce two children with clefts, the risk of further children being affected increases to 10-20%. The chances of a cleft parent producing a child with a cleft are about 2-6%. If the parent already has a child with a cleft this chance increases to about 14%. Parents can therefore usually be reassured that the risk of subsequent child being born with a cleft is small.

The incidence of clefts increases with parental age, especially the father's. Lower socio-economic class appears to be associated with a higher incidence of clefts, possibly related to nutritional factors.

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What should I do if a cleft is diagnosed on ultrasound examination before the birth of the child?

An increasing number of children are having their cleft diagnosed prenatally on ultrasound examination. It is important to note that there is about a 10% false negative and false positive rate of prenatal diagnosis with ultrasound. Currently, intra-uterine surgery carries far too many risks (such as causing miscarriages or severe bleeding) to be considered and option in these patients.

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My child has a cleft, what are the potential problems?

All the potential problems can be treated and sorted out by a multi-disciplinary cleft team consisting of plastic surgeon, speech therapist, ENT surgeon, audiologist, orthodontist, dentist, maxillo-facial surgeon, geneticist, and often social worker and psychologist. Children with clefts should be seen by the cleft team as soon as possible after birth. This will allow complete diagnosis and appropriate planing of treatment. In addition the cleft team is better able to reassure the parents, often using photographs of similar children previously treated to show the results of surgery. These children will require follow up by the cleft team, usually annually, well into adulthood.

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Potential problems include the following:

1. Breathing - this is usually not a problem unless the child has an associated anomaly such as the Pierre Robin sequence (a very small, backwardly displaced jaw).

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2. Feeding - children with clefts have an inability to suck properly although swallowing is normal. If the feed is delivered to the back of the mouth, swallowing can proceed normally. Parents must understand that children with clefts take longer to feed, tend to swallow more air, the milk regurgitates through the nose, they may seem to choke and splutter and weight gain may be poor. Nevertheless the child will feed and will try to suck - if enough time is allowed, the child will not go hungry. The child must therefore be allowed more time than normal to feed - an ideal opportunity for a strong bond to be established between parent and child. The mother must be encouraged to play an active role in feeding. Under no circumstances should the child be fed with a naso-gastric tube as this will tend to inhibit any sucking reflex. Things that will help include:

1. Holding the child more upright at about 45^o.
2. The use of an orthodontic feeding plate to facilitate sucking.
3. Delivery of the milk to the back of the child's mouth either:
a) directly expressed from the breast
b) using a bottle with a modified teat (longer with a larger hole)
c) using a cup or a spoon
4. Weight gain must be monitored.

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3. Hearing - normal hearing is not only critical for normal speech development, but also for proper functioning at school and later. Children with clefts are prone to getting ear infections which can lead to hearing impairment, even deafness. Early repair of cleft palate (before about 4-6 months) is associated with a lower incidence of hearing problems. These children should have regular hearing tests and check ups. Grommets must be inserted if necessary to drain the middle ear.

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4. Speech - children with cleft palates have short palates with abnormal muscles. Because of this, they cannot close the mouth off from the nose (the normal function of the palate). Typical cleft speech is associated with air escaping through the nose and a nasal sound to speech. In an attempt to speak better, the child may learn little tricks of tongue placement and will therefore not pronounce words properly. Speech development is a complex process. The child starts vocalising at birth and within a few months is babbling. The first words are usually said at about 1 year of age. It has been shown that for optimal results as far as speech is concerned, cleft palate repair is ideally performed at about 6 months of age and certainly before 7-9 months. Speech therapy is an important component of post-operative care.

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5. Teeth - children with clefts may have a variety of dental abnormalities including misplaced or absent teeth and abnormalities of alignment of the upper and lower jaws. Soon after birth of the cleft child, the orthodontist may make a palatal plate to be worn inside the mouth (an artificial palate). It is very important that dental hygiene is meticulous - brushing twice a day - to prevent caries and ultimate extractions which can result in abnormal facial growth. Whenever possible teeth should be maintained and filled rather than extracted. The orthodontist is involved in aligning the teeth to normal. A bone transplant, from the hip to the gum is usually needed at around the age of 9 years. If facial growth is abnormal, or the dental relations be out of kilter, a maxillo-facial surgeon will need to correct this. The operation is a Le Fort I advancement osteotomy which is only performed after the completion of facial growth in the late teens. Good primary surgery and proper dental care can often prevent the need for this major operation.

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6. Aesthetics - prior to surgery, children with clefts look grossly abnormal and often the parents and even the medical staff are shocked by the appearance of these children. Corrective surgery in the past aimed merely to close the cleft whereas the modern approach to cleft care is to restore normal landmarks and aesthetics. Plastic surgeons therefore recognise the subtleties of form of the nose and mouth. The aim of surgery is to leave a normal looking child. All normal landmarks should be present and the lip proportion restored after surgery. Greater attention is being placed on the nose and early corrective nasal surgery to straighten and restore the nose is the current practice. Cleft lip repair is usually undertaken between 3 and 6 months of age.

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What treatment is given?

Usually the child will be seen shortly after birth. Counselling and reassurance is given as well as advice about feeding. A palatal plate may be made by the orthodontist. A plan is formulated and the future treatments required will be discussed.

Repair of the lip and/or palate is performed at 3-6 months of age. Surgery before this time is not widely advocated as the baby is small, the anaesthetic more risky and the surgery harder. Results are not better if surgery is done before this time. General anaesthesia is used so the child sleeps through the operation and strong pain killers are used after the operation. The child should therefore not experience much pain. A cleft lip repair takes about 1-1.5 hours, a cleft palate repair about the same time. After a cleft palate repair, children are usually looked after in a high care unit during the first night. Usually the children only need to spend a night or two in hospital before they are allowed home.

Thereafter, regular check ups are performed. Dental care, orthodontics, audiology, speech assessments, plastic surgeon assessment, etc. should all be done. For children with clefts of the gum, a bone transplant operation (from the hip to the gum) is usually done at about 9 years of age. Often, other minor touch ups can be done at this same operation.

Finally, when the child enters the late teens, any corrective jaw or nasal surgery might be done.

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What will the outcome be - will my child be normal?

With current surgical techniques and multi-disciplinary team care, the cosmetic and functional outcome is highly satisfactory and likely to produce a normal adult.

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