Cleft Lip and Palate
Palatal clefts can range with varying degrees of complexity from incomplete cleft lip to complete bilateral cleft lip and palate. The incidence is 1:700 births.
Background
Palatal clefts can range with varying degrees of complexity from incomplete cleft lip to complete bilateral cleft lip and palate. The incidence is 1:700 births.
A type of cleft which is often missed is a submucous cleft palate. The signs to look for are - bifid uvula, transparent line down soft palate, presence of notch at junction of hard and soft palate, poor feeding with milk coming from nose during feeding.
Immediate management
Paediatric assessment (SHO, NS-ANP, Registrar or Consultant). Include examination for other abnormalities, airway and breathing difficulties.
Babies are normally managed with their mothers on the postnatal wards.
Complete a written referral to the cleft nurse specialist (CNS) (ph 021574434) and fax to:276 0004
Make a referral to the Lactation Consultant, National Women's Hospital - see breastfeeding and lactation support guideline for more information
Make a referral to the Speech-language Therapist (SLT), Starship Hospital by phone 0212415614 for a feeding assessment during normal working hours, plus written referral by fax to SLT. After hours contact the cleft nurse specialist.
All babies should be put to the breast as a start to establishing sucking feeds.
Additional information can be found on the Middlemore Hospital website
Cleft Lip and Palate Support: The information pack is called the Blue Book and it was developed by the Cleft Lip and Palate Support Group 'Face It' . This is given out by cleft nurse specialist and is also available from NICU and on-line. A visit can be arranged if parents request via the email address in the Blue Book.
Additional diagnoses
Although clefts are usually an isolated condition, there are more than 300 syndromes known to be associated with clefts, with cleft palate being more likely to be syndromic than cleft lip and palate.
Investigations should include:
A detailed family history
Thorough examination of head, neck and extremities.
Chromosomal testing if there are concerns - consider Velocardiofacial Syndrome (also known as Chromosome 22q11 syndrome) as this is the most common syndrome associated with cleft palate and cardiac anomalies.
Pierre Robin Sequence (PRS): Children with Pierre Robin Syndrome or severe micrognathia are at high risk of airway obstruction and may need to be nursed prone, or on their side, so that the tongue falls forward and they may need tube feeding and saturation monitoring and apnoea monitoring on discharge. In mild cases the relative mandibular under-development and glossoptosis usually disappears by infancy.
Feeding guidelines
All babies will be assessed by a cleft palate nurse specialist and an SLT. In the first instance, recommendations will include:
Encourage skin to skin. Put baby to breast to encourage attachment and milk production. Assist with breast milk expression at the breast if breast colostrum/milk not transferred. Mother should start expressing.
Or provide colostrum via syringe or cup if sucking and swallowing not present. Only insert NGT if colostrum is not available or baby requires further nutrition or there is a medical requirement.
By day 2-3 baby will be ready for introduction of a bottle if breast feeding is not meeting nutritional requirements.
Management of feeding according to cleft type
Feeding recommendations will vary depending on the type of cleft the baby has. They can often be categorised into the following:
| Cleft of the lip alone (no palate involvement): • Breastfeeding outcomes for a unilateral cleft lip should be positive as the breast partly obscures the cleft to complete the seal required to establish breastfeeding. • Breastfeed with the cleft uppermost, use underarm hold and switch to the cradle hold on the other breast • Use the breast tissue to help seal the cleft/s • Mother can place her finger over the cleft to occlude air entry and create a seal. |
| Cleft palate only (soft or soft and hard): • Breastfeeding - if baby can take the nipple and some of the breast tissue into the mouth the breast may form a seal stopping air from entering from the nose and breastfeeding proceed naturally. • Once breastfeeding is initiated observe for slipping off the breast, need for frequent re-latching, signs of transferring milk. Clicking sounds may persist during feeds or milk spilling from the nostrils. • Where breastfeeding is not or only partially achieved a Pigeon cleft palate bottle and teat will be required. This will be provided by the SLT. If out of work hours the CNS can be contacted. • The baby should be given colostrum via a syringe and have skin to skin initially. Bottles are usually introduced on day 2 or 3 by the SLT. • Maintain lactation by expressing and provide expressed breast milk if possible. Refer to community LC for follow up. |
| Wide unilateral or wide bilateral cleft of the hard palate, with or without lip and soft palate involvement: • Initiate breastfeeding and observe if the breast tissue is able to seal the cleft. • Where breastfeeding is not initially achieved use a syringe for colostrum and if larger volumes are required refer to the CNS and SLT. • The Pigeon cleft palate bottle/teat will be provided by the SLT (2 x bottles/teat sets to be given per baby). • Maintain lactation by expressing and provide expressed breast milk to the baby where possible. Refer to the community LC for follow up. • Babies with clefts involving the lip and palate receive a presurgical orthodontic plate within 2 weeks of life and this is arranged by the cleft nurse coordinator. |
Additional suggestions for the mother
Stimulate the let-down before bringing baby to the breast may help baby to latch
Hold baby in an upright position
Support the breast for the entire feed
Place mother's first finger under baby's jaw to stabilise it and minimise tiring (dancer hold)
Wind baby frequently
Understand that feedings may be "noisy"
Accept that feeds may take a while but should take no longer than 40mins - if having difficulty mothers should discuss with their SLT or cleft nurse coordinator
Pump the breasts after breastfeeds, if suckling was not strong or the feed did not proceed well.
Double pumping with a hired electric pump is recommended for efficient ongoing expressing
Pre-term and long stay babies, e.g. Pierre Robin Sequence, are at risk for more complex feeding difficulties, and are likely to need more specialised assessment and recommendations from a feeding therapist. Please liaise with the Speech Language Therapists and Lactation Consultant.
Bottle feeding
If breast feeding is not an option for baby then a bottle with teat will be provided by the SLT or cleft palate co-ordinator. These are usually appropriate to introduce about day 2-3. The Pigeon cleft palate bottle and teat is the usual option, but other bottles/teats that are appropriate may be provided depending on the child.
Cleft palate bottle and teats are dedicated to babies with special feeding needs. A baby affected by a cleft palate often takes longer to feed due to weaker sucking strength. Because of the gap in their palate, the baby is unable to create a vacuum in the oral cavity.
Pigeon cleft palate bottle/teat
The teat is wider than usual - 16mm wide to cover the palate and avoid the risk of getting the tip of the teat stuck in the cleft.
Thicker (2mm) on the upper side - To act as a substitute palate and allow for better suction. Thinner (0.9mm) and softer on the lower side - To allow the baby to easily squeeze the liquid out by gently pressing the teat with their tongue.
Variable Flow Y Cut - To make sure the baby only gets the amount of liquid that he/she is able to swallow. Also, a Y cut is easier for the baby to get more milk by just pressing the tip with their tongue, allowing for the Y cut to open mechanically instead of due to suction strength. The cleft palate bottle comes with 2 sizes of teat - regular and small. The small teat is best for newborns. As baby grows, the regular teat maybe introduced.
Flow Regulator - This special regulator prevents milk flow into the nipple from going back to the bottle. It helps keep a constant flow of milk for a continuous milk flow, making it easier for babies to suck using their own efforts.
The Pigeon cleft palate bottle 240mL is made of soft Polypropylene material in an oval shape, allowing the care giver to hold it comfortably and squeeze it to regulate the flow of milk as needed to suit each baby's needs.
Instructions for use
Assemble the bottle and teat. Check you have the appropriate sized teat - small/regular. Ensure that the flow regulator/valve is inserted on the inside of the teat. Remember to position the notch facing upwards. The notch can get clogged and requires cleaning. Also check that the teat Y cut can open before first use.
Squeeze the teat with your finger and thumb and tilt the teat downwards so that milk runs into the teat before starting the feed. The bottle is now ready to be used
Ensure that the collar is not on too tight as air needs to be able to get into the bottle for the one way valve to work.
Cleaning
Wash bottle, teat, collar and valve in hot soapy water. Roll teat between fingers to clean opening
Use bottle brush to thoroughly clean bottle
Feeding equipment can then be sterilised with boiling water, with sterilising solution, (place in solution for 30 minutes then remove), or with a steam-sterilising unit.
Please note that a dishwasher does not sterilise bottles or feeding equipment.
After three months of age it is sufficient to wash bottles and feeding equipment with hot soapy water and then rinse.
Surgical management
Lip and primary palate is usually repaired at around 5 months of age.
Soft palate repair is usually around 10 - 12 months of age.
As well as being fundamental to 'normal' facial appearance, the structures involved in cleft lip and/or palate repair are vitally important to the development of normal speech, feeding and dentition.
A cleft lip and/or palate has tremendous aesthetic and functional implications for the patients in their social interactions therefore the multidisciplinary team need to take into account not only the anatomical impairment of the cleft, but the potential impact of the cleft on the patient's ability to communicate effectively, and their facial appearance.
The overall aims in treating cleft lip and palate are:
To give the best possible appearance of the lip, nose and face
To repair the palate to enable production of normal speech and no difficulty eating/drinking.
The correct alignment of the teeth and the jaws.
To ensure adequate hearing as children with clefts are prone to frequent ear infections and consequently potential for hearing loss.
Discharge/follow up
The Speech Language Therapist will assess the baby's feeding and apply for specialist feeding equipment as appropriate. If special feeding equipment is required, two bottle and teat sets which the mother has used are to be supplied by the SLT to the parents before discharge.
Follow up will be arranged on discharge from Newborn Services by the Speech Language Therapist to the Child Development Service for ongoing feeding and speech support. They will continue to supply the family with the bottle and teats as required.
The cleft nurse specialist will visit the newborn infant within 24-48 hours of birth and will arrange appropriate follow up with the Cleft Palate team. The team will then assess the child in clinic within 6 weeks. The surgeon will outline and individualise management for that child.
The team includes:
Plastic surgeon
Orthodontist
Speech-Language Therapist
Cleft Nurse Coordinator
ENT Surgeon
If other anomalies are present then the Specialist will need to follow up as appropriate, e.g. Paediatric Cardiology at Starship Hospital. A Newborn Services appointment may be offered for three months to ensure that all is progressing normally in terms of follow up arrangements, feeding, growth and early development.
Parents are not always aware that the management of cleft lip and palate is long term and follow up may be into the child's twenties before orthodontics is completed. Rhinoplasty, jaw and further lip surgery may also need to be scheduled at this late stage. We should endeavour to inform the parents with the help of literature that the immediate repair is only the beginning of a long term relationship with the Plastic Surgical Team.