By Dr Sandeep Kaur Jawanda, General Practitioner, MBChB MRCGP
A cleft lip and/or palate is the most common craniofacial abnormality in the UK. It affects 1 in 700 children and approximately 1,200 babies in the UK are born with a cleft each year1. It can be in the form of a cleft lip, cleft palate or a combination of the two.
A cleft palate occurs when the roof of the mouth has not joined completely and there is a gap. It can affect the soft palate, hard palate or both.
Diagnosis of cleft palate as part of the newborn examination is essential in order to best support the family with the various feeding issues surrounding the initial diagnosis.
Sometimes a baby with a cleft palate may have a small mandible and a few babies with this combination may have difficulties with breathing. This condition may be identified as Pierre Robin Sequence. This has a prevalence of up to 1 in 30,000 and about 50 are born in the UK each year with this condition2. Pierre Robin sequence is a set of abnormalities affecting the head and face, consisting of a small lower jaw (micrognathia), a tongue that is placed further back than normal (glossoptosis), and blockage (obstruction) of the airways. This can complicate feeding issues as much as the cleft alone.
After the initial diagnosis of cleft lip and or palate, there are a timeline of events, including preparation for surgery which may include early introduction of solids. Then post operatively, speech and language development, along with hearing and dental/orthodontic issues that commonly co-exist. These can continue into early adulthood.
Delay in detection of a cleft palate may adversely affect growth, nutrition, development and subsequent the timely medical and surgical management3. Overall, 15% of all clefts are syndromic clefts so GPs need to be vigilant during the 6-week checks for other features that may be linked1.
Guidelines were published by the Royal College of Paediatrics and Child Health on Identification of Cleft Palate in the Newborn in 20143, yet despite being a GP since 2010, it wasn’t until I gave birth to my own cleft baby, that even I became aware of this guidance which, among other things, highlights the impact a delay in diagnosis can have on families. Additionally, litigation brought for delayed detection of cleft palate costing the NHS £250,000 in a 10 year period3, 4. In the UK it was thought that this was due to inadequate examination. A prevalence of 30% delayed detection beyond the first 24 hours has not changed significantly over a 10 year period (12% >1 week, 7% < 3 months, 3% 1 year)5.
So how do we improve the pick up rate?
Firstly, to simply be aware of cleft palate as a cause of difficulty feeding in the early weeks and considering it as a differential diagnosis. We are accustomed to checking the red reflex and femoral pulses, but looking inside the child’s mouth before for example, signing a script for gaviscon and putting things down to reflux is important. Reflux is indeed common amongst cleft babies, particularly those with associated syndromes such as Pierre Robin.
How to examine the palate adequately?
Below are the recommendations for best current practice3:
- Healthcare professionals should examine a baby’s hard and soft palate as part of the full newborn physical examination and record this in the child health record.
- Examination of the baby’s palate should be carried out by visual inspection.
- A pen torch and method of depressing the tongue should be used to visualise the whole palate.
- Parents should be informed if the whole palate (including the full length of the soft palate) has not been visualised during the newborn examination.
- If the whole palate is not able to be visually inspected at first attempt then a further attempt at visual examination should be made within 24 hours.
- Trusts should provide training on the correct method of visual inspection of the palate to all healthcare professionals required to carry out the newborn examination.
Clefts come in all shapes and sizes and no longer is sweeping the roof of the mouth with the small finger adequate enough to ensure you don’t miss the small/posterior clefts.
Feeding infants with cleft lip and palate
Once the mother receives a diagnosis they will usually be assigned to a specialist cleft team. The assigned cleft nurse will be able to advise and assist in best methods of feeding. This can be advice during the antenatal period or postnatally.
Usually if a cleft lip is detected, this is known before birth and mums will be given the option of hand expressing colostrum and storing from 32 weeks onward. They can be educated about the use of squeezable and other specialist bottles, and the possibility of NG feeding.
If the diagnosis is after birth, then often the mother is advised to supplement and start expressing colostrum straight away should she wish. Isolated cleft lips are generally still compatible with breastfeeding, so long as the anatomy allows for forming suction. When there is any hole in the palate, hard or soft, direct breastfeeding becomes challenging, and often impossible due to the lack of suction. This is where squeezable bottles or bottles with a valve can be helpful. Again, with associated syndromes or any other birth problems, NG or PEG feeding may be required.
It is important to support mums with options available and expressing of breast milk should still be encouraged. Cleft babies that receive breast milk are less likely to suffer ear infections and with the anatomical variation leading to a higher prevalence of glue ear in this cohort, it can be protective against some of the complications of clefts6.
Finally, and arguably most importantly, is the support to the mother with her feeding decisions. The impact on perinatal mental health is paramount and well documented where this is concerned. There is a real grief and mourning associated with the loss of a breastfeeding journey where mothers have wanted to breastfeed their newborn but through ill health, for example NICU stays, they cannot. Couple this with the coming to terms with the diagnosis of cleft lip and/or palate itself, the emotional impact can be vast.
Exclusive expressing or expressing and combination feeding allow the provision and benefits of breast milk, even for those requiring NG and PEG feeds. However, these options require significant amounts of family and professional breastfeeding support.
Families will need support throughout the first year, particularly as cleft lips are often repaired around 3-6 months and palate repairs taking place between 6-12 months. The prospect of any surgery for a baby in the first year of life is daunting, but again is a risk factor for maternal mental health and should be monitored closely. Cleft teams in the UK are comprised of nurses, plastic surgeons, dentists, ENT surgeons, speech and language therapists and even psychologists for the child through the school years. A leading example of a multi-disciplinary model for excellent practice.
If we could improve the stats of 16% of babies being undiagnosed at first examination- then we’d really be on to a winner7.
Cleft Lip and Palate Association (CLAPA) A charity providing information and support for those affected by cleft lip and palate. Their website includes a ‘Feeding’ section with details on breastfeeding, expressing, bottles and teats.
- Cleft Lip and Palate Association (CLAPA)
- Cole A, Lynch P, Slator R. A New Grading of Pierre Robin Sequence. Cleft Palate–Craniofacial Journal. 2008;45(6), 603–606
- Royal College of Paediatrics and Child Health. Palate examination: Identification of cleft palate in the newborn – best practice guide.
- Freedom of Information communication NHS Litigation Authority 2013.
- CRANE Database Annual Report. 2012. London, Clinical Effectiveness Unit, The Royal College of Surgeons of England.
- Mills R. The management of childhood otitis media with effusion. J R Soc Med. 1996;89(3):132–134.
- Habel A, Elhadi N, Sommerlad B, Powell J. Delayed detection of cleft palate: an audit of newborn examination. Arch Dis Child 2006;91:238-240.