In the second article in this series, the Cleft Team from Birmingham Children's Hospital outline the diagnosis of cleft lip and palate and the care likely to be needed during the first five years of the child's life
The second in a series on cleft lip and palate, this article outlines the diagnosis of these congenital anomalies, the problems that a child with a cleft lip and/or palate and their family may encounter in the first five years of life, and the multidisciplinary care needed. The input of individual members of the cleft team is described, including nurse specialists, speech and language therapists, paediatric dentists, ear, nose and throat surgeons, and audiologists. Journal of Family Health Care 2009; 19(4): 122-125
_ Most clefts are isolated anomalies with little risk of recurrence in siblings or offspring, but in a significant minority there is an associated syndrome or specific genetic cause, and the input of the geneticist is needed
_ Diagnosis of a cleft lip (but not a cleft palate) may be made antenatally, usually at the 20- week anomaly scan. The family should then be referred to the Regional Cleft Centre for specialised information and support, often from the specialist nurse
_ A cleft may be associated with other anomalies and health professionals should look for these antenatally and postnatally
_ At the routine postnatal check, the examiner should check for a possible isolated cleft palate using a torch and tongue depressor. This diagnosis may be missed, which can lead to serious feeding difficulties for the baby
_ Feeding can cause problems which vary according to the type of cleft. A nurse specialist will carry out a feeding assessment and offer advice and practical help
_ Some children with cleft lip and/or palate develop normal speech with few or no problems but many need help. Speech problems are rare in children who only have clefts of the lip, but a cleft palate may affect speech to varying degrees. Early assessment and input from a speech and language therapist is needed to ensure children learn to make the sounds for speech correctly at an early stage
_ Children with a cleft palate are more likely to have glue ear than other children, and hearing should be monitored. If reduced hearing is affecting speech, liaison between the speech and language therapists and the ENT team is important. Some speech problems may require specialist investigations and further surgery
_ Children born with cleft lip and/or palate may have dental abnormalities that require treatment from a consultant paediatric dentist. Their condition makes them more at risk of dental caries and they need regular dental examinations and dental health education
_ Like other families who have a child with a congenital abnormality, parents of a child with a cleft need support and information from health professionals. This is offered by the Regional Cleft Team, backed up by the local health teams and the voluntary organisation CLAPA
The first article in this series on cleft lip and palate1 described the different types of cleft that occur and gave an overview of the multidisciplinary long-term care required from birth to adulthood.
This second article describes in more detail the problems that a child with a cleft lip and/or palate and their family may encounter in the first five years and the care needed. Surgery will be discussed in a future article in this series. Care during this period includes input from the following:
_ nurse specialists
_ paediatric dentists
_ ENT surgeons
_ clinical psychologists
Although the majority of clefts occur as an isolated abnormality with a relatively small risk of recurrence in siblings or offspring, there will be an associated syndrome or underlying specific genetic cause in a significant minority. The role of the geneticist is to help with identification of syndromes and to advise families about the chances of clefting recurring within the family in future.
The diagnosis of a cleft lip (though not of an isolated cleft palate) may be made antenatally. Across the UK this happens for approximately 68%2 of babies born with a cleft lip and most usually occurs at the 20-week anomaly scan. A cleft lip may be visible but it may not be possible to see the full extent of the cleft, particularly whether the palate is involved. If a cleft lip is picked up on the ultrsasound scan the sonographer should look for other anomalies. If further anomalies are found, the fetal medicine specialists will discuss these with the family and further tests may be required.
Following a diagnosis of a cleft lip on an antenatal scan, the family should be referred to the Regional Cleft Centre. The family will then be contacted by the Cleft Team who will offer information and support. This contact is usually made by nurse specialists in the team, who may offer a home visit and will discuss the diagnosis and potential future care. Contact will then be maintained by the Cleft Team throughout the duration of the pregnancy and a subsequent home visit may be offered nearer to the time of delivery in preparation for the birth.
If not made antenatally, the diagnosis of a cleft lip is usually made immediately after birth as the gap in the lip is usually easily seen (Figure 1). Most diagnoses of clefts of the palate will also be made at or soon after birth. Following referral from a maternity unit to the appropriate Regional Cleft
Centre, a nurse specialist aims to visit the family within 24 hours. The nurse specialist examines the baby to confirm the cleft diagnosis and, together with the paediatric team, looks for associated problems. The nurse specialist can then discuss the plans for future care with the family and the paediatric team, and provide written information.
Diagnosing an isolated cleft palate
Diagnosis of an isolated cleft palate is sometimes missed at the newborn examination. The external appearance of the face looks normal (Figure 2a).
Health professionals have traditionally been taught to introduce a finger into the baby's mouth to feel the palate for a cleft. If the cleft affects only, or mainly, the soft palate the cleft may not be detected by such an examination. A recent national review showed that 23%3 of isolated cleft palates were diagnosed more than 24 hours after birth. If the palate is examined visually using a tongue depressor and a torch, a cleft palate is unlikely to be missed4 (Figure 2b). A late or missed diagnosis of cleft palate usually results in feeding difficulties for the baby, which in turn causes anxiety for the family. Such feeding difficulties can be severe enough to lead to faltering growth and readmission to hospital.
Babies with clefts of the lip and/or palate have the same nutritional need as any other baby. Where there is a cleft of the lip only, breast-feeding often works better than bottle-feeding. This is because the breast is likely to fill the gap in the lip, enabling the baby to have an effective suck. A teat may not fill the gap in the lip so well, resulting in the baby sucking in air as well as or instead of milk. When there is a cleft palate, the baby is likely to have a non-nutritive suck because the gap in the palate will allow air to be sucked in through the nose, preventing milk from being sucked from bottle or breast. Babies with a cleft palate can, however, usually be fed by using a normal latex teat and a soft squeezy bottle. The soft bottle is squeezed in rhythm with the baby's normal sucking pattern, thus squeezing the milk into the mouth to be swallowed normally. Breast-feeding a baby with a cleft palate is likely to be difficult but it is possible for a mother to express her milk and give it to her baby with a soft bottle. The nurse specialists establish the mother's preferred method of feeding, whether breast or bottle, and support her with that, making modifications for cleft type where necessary. Feeding should be a pleasurable experience for both parent and baby and a baby with a cleft should not take any longer to feed than any other baby (Figure 3).
A feeding assessment is part of the examination carried out by the nurse specialist. A minority of babies with a cleft lip and/or palate will have problems with feeding not directly related to the cleft. If the nurse specialist has concerns about the baby's ability to feed normally then the baby will initially be fed by nasogastric tube. Further assessment and liaison with other health professionals such as a speech and language therapist who is a dysphagia specialist may be required. More detailed information on feeding babies with clefts of the lip or palate will be given in the next article in this series.
Support for parents
Giving birth to a baby who is different from others will have an emotional impact on the parents and the wider family. This can be an isolating experience as family and friends may be less willing to help care for the baby and participate in activities such as feeding.
The early months can be particularly difficult and challenging for parents who not only have a new baby but also have to assimilate and understand all the various aspects of their child's treatment and care, involving a large number of health care professionals. The nurse specialist provides support and advice at this time. This may include visits to the family at home to offer support with feeding or other wider issues. They are also available to families of children of all ages for advice when needed. The clinical psychologist on the Cleft Team is also available to provide advice and further support.
The Cleft Lip and Palate Association (CLAPA) (see Resources) is a valuable source of advice and support to all those affected by cleft lip and/or cleft palate.
Cleft palate and speech
The soft palate is important in speech because it moves upwards and backwards when we talk. This movement, combined with the movement of the walls of the throat, separates the nose from the mouth and enables air to be pushed out of the mouth. This is necessary in order to make most of the sounds of speech, e.g. "p, b, t, d, k, g, s, sh, ch". The soft palate does not have to lift up for the nasal sounds "m, n, ng". If the soft palate does not move very well, this can affect speech in two ways. Speech may sound nasal if the palate does not separate the nose from the mouth properly and allows air to escape into the nose when it should not. This can give speech a nasal tone (the opposite to how people sound when they have a bad cold and a blocked nose). Also, if the palate is allowing air to escape into the nose when it should not, some speech sounds may be difficult to make. They might, therefore, seem weak or be replaced by incorrect or unusual sounds. A problem affecting the palate may therefore affect speech development and production. Some children with cleft lip and/or palate develop normal speech with few or no problems but many need help. They may have difficulty learning how to use their tongue, lips and palate correctly for speech. Also, air may escape down their nose when they are speaking because the palate is not working well enough.
Problems are rare in children with clefts of the lip only, but like any child they may have speech and language problems that are unrelated to cleft palate4. Speech and language therapists are therefore involved with the care of children with cleft palate from the earliest stages. Following palate repair, they provide information and advice to parents on how to encourage speech sound development. In addition, they monitor the communication development of all children who have had clefts of the palate in order to identify those who have problems and to ensure that they get the right help when they need it. There is some variation over the timing and type of contact, but children with cleft palate in the UK will normally have been seen by a specialist speech and language therapist from the Cleft Palate Team by the time they are two years old. Their progress continues to be monitored at specific ages, e.g. at three years, five years, 10 years and 15 years.
Assessing palate function
One of the important tasks for the speech and language therapists on the Cleft Team is to assess how well the soft palate is working during speech. The specialist speech and language therapist will be able to confirm whether a child's speech is developing correctly or not. For a very young child, information is obtained by talking to the parents, playing with the child and listening to the child's speech. For older children, more formal tests in which they are asked to say specific sounds and sentences are used. Assessments are often audio and video recorded (Figure 4) with the parent's permission so that they can be shared with other members of the Cleft Team and progress can be monitored. If the results of the speech assessment suggest that the child is learning to produce speech sounds incorrectly, speech therapy may be needed. It is extremely important that children learn how to make the sounds for speech correctly early in life and have speech and language therapy input where necessary at an early stage. It becomes more and more difficult to change incorrectly learned speech sounds as the children become older. If the speech assessment suggests that the child's palate is not working properly, more detailed tests are needed.
These include palate investigations such as video X-rays of the palate moving during speech, and nasendoscopy (examination of the palate from above by a small, very bendy tube [endoscope]). These investigations are carried out in conjunction with the cleft surgeon. Surgery may then be needed to help to reduce the amount of air escaping into the nose during speech and to make it easier for the child to produce sounds correctly, which can then facilitate progress in speech and language therapy.
Children with a cleft palate are more likely to have glue ear than other children. This is because the muscles controlling Eustachian tube function are in the soft palate and are disrupted by a cleft of the palate. As with other children, children with a cleft palate and glue ear will usually grow out of the problem in time. However, it is important that hearing is monitored and glue ear treated when appropriate. This may be if the hearing is reduced to an unacceptable level affecting day-to-day activities. Intervention may also be needed if it is felt that reduced hearing is affecting speech development. Close liaison is therefore required between the speech and language therapists and the Ear, Nose and Throat (ENT) team Treatment may involve hearing aids or grommets. Regular monitoring of hearing is part of the care provided by the Cleft Team. Routine treatment is also provided more locally.
Dental development and dental health
Children born with cleft lip/palate will usually have normal timings for eruption of their first and second teeth, apart from the area of the cleft site.
There can be a delay in eruption of the primary teeth, most commonly the lateral incisor in this area. These teeth can also erupt in an abnormal position, quite often in the palate. They can also be smaller and more pointed in size. In some children both the primary and secondary teeth in this area can fail to develop and will therefore be missing. Children with cleft lip and palate are more at risk of dental decay and this can be for a variety of reasons. For example, teeth may be crooked and more difficult to clean or lip scar tissue can also make tooth brushing difficult. The teeth in the cleft site can be poorly formed and more susceptible to decay. It is important to prevent decay, so a healthy diet, keeping the intake of sugar to a low frequency, and tooth brushing from the start of tooth eruption are encouraged, as well as attending the dentist regularly. The Cleft Team will provide advice about oral hygiene and dental care and will monitor the child's oral health as they grow.
Rona Slator DPhil FRCS FRCS(Plast) Consultant Plastic Surgeon and ClinicalDirector - Cleft Service(West Midlands Regional Cleft Centre)
Jane Russell PhD FRCSLT Lead Specialist Speech and LanguageTherapist
Annie Cole SRN RSCN Lead Cleft Nurse Specialist
Jayne Tomlinson RGN RSCN Cleft Nurse Specialist
Melody Bridges BA(Hons) MSc MIHM Cleft Service Manager
Victoria Clark BChD(Hons) MDentSci FDSRCS(Paed Dent)Ed Consultant Paediatric Dentist
Jenny Morton MBChB FRCP Consultant Clinical GeneticistJohn ReadingCleft Information Manager West Midlands Regional Cleft Centre Birmingham Children's Hospital NHS Foundation Trust Birmingham
1. Slator R, Russell J, Bridges M, Tomlinson J, Cole A, Morton J. Understanding cleft lip and palate. 1: An overview. Journal of Family Health Care 2009; 19(3): 101-103
2. Clinical Nurse Specialists' Special Interest Group. National audit undertaken by the Cleft Nurses Special Interest Group January 2006-December 2007 and presented at the annual Craniofacial Society Conference 2008. "The Incidence of Facial Clefts Not Diagnosed In the Antenatal Period. A Postcode Lottery?". Clinical Nurse Specialists' Special Interest Group www.nurses4cleft.org.uk
3. Butcher S. Cleft palate - the value of early diagnosis. RCM Midwives 2007; 10(8): 382-383
4. Vitkovitch J. Speech and language skills: their importance in development. Journal of Family Health Care 2008;18(3): 93-95
Birmingham Children's Hospital NHS Foundation Trust Diagnosis of a Cleft Palate Video http://www.bch.org.uk/departments/cleftlipandpalate.htm CLAPA (Cleft Lip and Palate Association) http://www.clapa.com CLAPA is a registered charity set up to give information and support to all those affected by cleft lip and/or cleft palate. CLAPA have local support groups throughout the UK and organise activities for children of all ages and their families. They also supply specialised feeding equipment, leaflets and publications. CLAPA leaflet "Speech: The Impact of a Cleft Palate" is available from CLAPA at http://www.clapa.com