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Cleft lip and/or palate: clinical practice in the UK and the role of the clinical nurse specialist.

One in 700 babies born in the UK has either a cleft lip and/or palate. (1) Babies may have both cleft lip and palate, or either on its own. Prevalence varies by ethnic group, geographic origin and sex--cleft lip and palate is more common in boys, and cleft palate alone in girls. (2) The causes are still largely unknown, but several genetic and environmental factors are believed to interact. (3) Sodium valporate taken during pregnancy is known to cause clefting. (4) There is sometimes a clear genetic cause, but for most families it cannot be anticipated. (1)

Associated anomalies and conditions

Cleft is usually an isolated defect, but there are associated anomalies. The research gives a wide range of occurrence from 21% to 37%, and babies with cleft palate alone are most at risk. Clefting is associated with over 400 syndromes, including trisomy 13 (Patau syndrome), trisomy 18 (Edward's syndrome), velocardiofacial syndrome (22q11 deletion) and Stickler syndrome. (5,6) Pierre Robin sequence is the most common anomaly associated with an isolated cleft palate. They present with a small, set-back chin (micrognathia) and posteriorly-placed tongue, resulting in airway problems.

Organisation of services in the UK

UK cleft services have undergone a major overhaul following the 1998 Clinical Standards Advisory Group (CSAG) report, (7) which compared care and outcomes with other European countries. The number of hospitals treating cleft were reduced from around 57 to 10 regional cleft teams to improve skills and outcomes. Specialist surgeons carry out all operations in hospitals with appropriate support services, such as paediatric intensive care. Other services are offered locally, and cleft team members visit families and provide outreach clinics. The teams involve key professionals, including specialist speech and language therapists (SALTs), orthodontists, paediatric dentists, surgeons, psychologists and clinical nurse specialists (CNSs).

The Cleft Lip and Palate Association (8) (CLAPA) offers excellent support, with activities for young people, leaflets and a mail order service for specialist feeding equipment. Local branches provide support to families, such as trained parent contacts who can be contacted following diagnosis or at any stage of treatment.



Surgery usually starts with lip repair at around three months, but may continue over 15 to 20 years for some--cleft should not seen as a 'quick fix' condition. Neonatal surgery for cleft lip does not provide better results or any psychological benefit for parents. (9) Allowing three months before the first surgery gives the mother time to recover after childbirth, get to know her baby and adjust to the appearance of the cleft. (10) Children who cope well later are those whose parents are psychologically well adjusted to the cleft. (11)

Palate repair is done at around six to nine months, allowing for sufficient growth but before the child develops speech. Surgery involves the palate muscles being moved from their abnormal lateral position to form a sling across the midline, enabling the palate to move.

An alveolar bone graft will be needed for a child whose cleft affects the gum, and is carried out at eight to 11 years of age. It fills the gap in the bone prior to the eruption of permanent canine teeth.

Cleft may continue to cause facial asymmetry, with further surgery required well into teenage years. Psychologists have a key role with older children, who may need support in making decisions about lengthy, complex surgery and orthodontics, such as distraction osteotomy to allow forward movement of the upper jaw to improve the facial profile and bite, which may change their appearance significantly.


Specialist SALTs meet the family when they first come to clinic to provide advice about encouraging normal speech development, supported with written information following palate repair. From 18 months, they will see the child regularly to monitor development of speech and the function of the palate. Over 50% develop normal speech after palate repair with no more intervention. The SALT will determine if therapy or further surgery--necessary in about 25% of children (12)--is required.


Children with a cleft lip and/or palate may have missing or extra teeth. They have an increased incidence of tooth decay, so preventive dental care and advice about diet and drinks is needed. Orthodontics after the second teeth have erupted are required for many children, provided by a specialist consultant orthodontist in the hospital.


Children with a cleft palate have a 97% chance of being affected by glue ear at some stage of childhood, due to the altered muscle position of the palate reducing drainage of the eustachian tube. They need regular follow up during the first five years in case of significant hearing loss. (13) Grommets may be necessary for some, but are not done routinely with palate repair as in the past. (14)

The role of the CNS

The CNS will typically have 25 to 30 babies born a year who become part of their caseload. We work independently, but a lead nurse offers clinical and managerial support. Joint visiting with the lead nurse is often done in more complex cases, and strong clinical supervision is available.

The CNS will contact families soon after diagnosis--CSAG recommends within 24 hours (7)--and this may be before birth, since a cleft lip might be diagnosed at the 20-week anomaly scan. We contact the family at least twice and may visit a few weeks before the birth to discuss feeding in more detail. This might include writing a feeding plan for the first 24 hours to go into the mother's notes. Cleft palate can only be diagnosed after birth. It is important that the palate is visualised during the newborn examination, as a cleft of the soft palate will only be diagnosed in this way--a national audit found that 23% of cleft palates were missed at first examination in 2005. (15)

The first member of the team to meet the family, the CNS is able to provide information about future care and treatment. We see them in hospital and in the community, in addition to the community midwife and health visitor. We also see families attending multidisciplinary cleft clinics, which are often held in outreach clinics.

The CNS is able to build a strong and supportive relationship with families through continuity of care. The role has a strong counselling element, since parents go through an adjustment process following diagnosis not unlike that of bereavement for the loss of an anticipated 'perfect' child. (16,17) Antenatal diagnosis can be very shocking for parents, and mothers often blame themselves for somehow causing it. (18) It is important that parents have the opportunity to meet with a cleft team member soon after diagnosis for accurate information. (7) A diagnosis of either cleft lip or palate following birth is equally traumatic, with similar feelings of bereavement and loss while the family copes with all the usual issues of having a new baby. (19)

Support with feeding is a large part of the CNS role (see Box 1), since it may be the biggest concern for 95% of parents. (20) We conduct a feeding assessment when first seeing the baby, offering on-going support and specialist feeding equipment. It is vital for both the parents and child that feeding should be a happy, relaxed experience. The CNS will liase with other primary healthcare team members, often with joint visits. CLAPA gives new families a free starter pack of bottles and teats, and local branches often supply further feeding equipment for free.

The CNS role does not stop with the babies and varies by team, but we get involved increasingly with older children, either through pre-admission clinics, support prior to alveolar bone graft or at events such as 'school change days', held to support children moving to senior school.
Box 1: Feeding issues for babies with cleft lip and/or palate

A national audit has found that the type of cleft has a significant
impact on whether the baby can feed directly at the breast or is better
able to have expressed breastmilk. (17)

Babies with only cleft lip are able to breastfeed or
bottle-feed--breastfeeding works well because breast tissue may mould
to fill the gap in the lip better than a more rigid teat. With
an antenatal diagnosis, the mother can express from 36 weeks to store

Babies with complete cleft lip and palate may not be able to
uccessfully breastfeed, but skin-to-skin contact should still be
encouraged. We lend mothers electric breast pumps with double pump kits
free of charge for as long as they wish to use them.

Cleft palate-only babies may manage some feeding at the breast, but
will normally need to be topped up with expressed breastmilk or
formula. They are unable to create negative intraoral
pressure to allow milk to be drawn from the breast or bottle. They get
frustrated quickly when they are unable to drain the breast, and will
often get insufficient milk and fail to thrive. Some mothers may use
breast compression to give the baby more milk at the breast, but many
fully express and give milk via a soft bottle using gentle squeezing
('assisted' feeding).

Cleft babies use more energy in feeding than non-cleft babies, so may
be a little slower to gain weight initially. We monitor their weight
and liase with health visitors and community children's nurses as
appropriate. Some babies require high-energy formula.

Weaning usually works well with cleft babies and takes less physical
effort than bottlefeeding. They can be weaned at the normal time,
though if surgery is planned for close to six months, it may be
suggested that they start prior to this (babies often do better
post-surgery with purees rather than milk). High-energy weaning is
often used with slow weight gain.

Families who have had contact with a CNS from birth will call when they have a query or a concern, and we continue to see them when they come to the multidisciplinary clinics throughout childhood.

Health visitors: identifying cleft

Health visitors may pick up late-diagnosed palate babies or those with submucous cleft palates. These babies may present as failing to thrive, or be slow feeders who have been unable to breastfeed. They might also regurgitate milk nasally or make a clicking noise when feeding at breast or bottle. Mothers will often have raised concerns already with many professionals. It is important to look inside the mouth using a torch and preferably a tongue depressor or when the baby is crying to identify the cleft. A baby with a submucous cleft palate appears to have an intact palate, but the muscles are not joined properly. They may appear to have a notched or split (bifid) uvula and the palate may appear transparent, and may have some or all symptoms of a baby with cleft palate.

Immediate telephone referral should be made to the cleft team, whose details will be available at the local maternity hospital.


(1) Watson ACH. Embryology, aetiology and incidence. In: Watson ACH, Sell DA, Grunwell P (Eds.). Management of cleft lip and palate. London: Whurr, 2001.

(2) Bender PL. Genetics of cleft lip and palate. Paediatric Nursing, 2000; 15: 242-49.

(3) Amaratunga NA. A study of etiologic factors for cleft lip and palate in Sri Lanka. Journal of Oral Maxillofacial Surgery, 1989; 47: 7-10.

(4) Clayton-Smith J, Donnai D. Fetal valproate syndrome. Journal of Medical Genetics, 1995; 32: 724-7.

(5) Millerad J, Larson O, Hagberg C, Ideberg M. Associated malformations in infants with cleft lip and palate: a prespective population based study. Pediatrics, 1997; 100: 180-6.

(6) Stoll C, Alembik Y, Dott B, Roth MP. Associated malformations in cases with oral clefts. Cleft Palate Craniofacial Journal, 2000; 37: 7-10.

(7) Clinical Standards Advisory Group. Cleft lip and/or palate. London: Stationery Office, 1998.

(8) Cleft Lip and Palate Association. CLAPA. Available at: (accessed 11 June 2009).

(9) Slade P, Emerson DJM, Freedlander E. A longitudinal comparism of the psychological impact on mothers of neonatal and three-month repair of cleft lip. British Journal of Plastic Surgery, 1999; 52: 1-5.

(10) Coy K, Spelz M, Jones K. Facial appearance and attachment in infants with orofacial clefts: a replication. Cleft Palate-Craniofacial Journal, 2002; 39: 66-72.

(11) Bradbury E, Bannister P. Prenatal, perinatal and postnatal counselling. In: Watson ACH, Sell DA, Grunwell P (Eds.). Management of cleft lip and palate. London: Whurr, 2001.

(12) Spriestersbach DC, Dickson DR, Fraser FC, Horowitz SL, McWilliams BJ, Paradise JL, Randall P. Clinical research in cleft lip and palate: the state of the art. Cleft Palate Journal,1973; 10: 113-65.

(13) Paradise JL, Bluestone CD, Felder H. The universality of otitis media in 50 infants with cleft palate. Pediatrics, 1969: 44; 35-42.

(14) Sheahan P, Blayney AW Sheahan JN, Earley MJ. Sequelae of otitis media with effusion among children with cleft lip and/or cleft palate. Clinical Otolaryngology, 2002; 27: 494-500.

(15) Butcher S. Cleft palate: the value of early diagnosis. RCM Midwives., 2005; 10(8): 382-3.

(16) Martin V, Bannister P. Cleft care: a practical guide for health professionals on cleft lip and/or palate. Salisbury: APS, 2004.

(17) Martin V. Helping parents cope. Nursing Times, 1995; 91: 38-44.

(18) Hodgkinson P, Brown S Duncan D, Grant C, McNaughton A, Thomas P, Rye Mattick C. Management of children with cleft lip and palate. Fetal and Maternal Medicine Review, 2005; 16: 1-27.

(19) Young J. What information do parents of newborns with cleft lip and palatewant to know? Pediatrics, 2001; 38: 55-8.

(20) Nurses' Special Interest Group, Cleft Lip and Palate. Breastfeeding audit (unpublished). London: Nurses' Special Interest Group, Cleft Lip and Palate.

Ann Young Clinical nurse specialist, South Thames Cleft Service, Royal Surrey Hospital Guildford
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Title Annotation:CLINICAL UPDATE
Author:Young, Ann
Publication:Community Practitioner
Geographic Code:4EUUK
Date:Jul 1, 2009
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