Incidence

• Incidence is 1:700 births.

Clefts can range with varying degrees of complexity from incomplete cleft lip to complete bilateral cleft lip and palate

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.

Immediate Management

1. Paediatric assessment (SHO, NS-ANP, Registrar, or Consultant). In particular examination for other abnormalities, airway and breathing difficulties.
2. Babies are normally managed with their mothers on the postnatal wards.
3. Complete  written referral to the Cleft nurse coordinator (ph 021574434)  and fax to:276 0004
4. Referral to the Lactation Consultant, National Women’s Hospital.
5. Referral to the Speech Language Therapist, Starship Hospital
   • Phone 0212415614
   • Plus written referral by fax to SLT.
6. Additional Information can be found on the Middlemore Hospital website
7. Cleft Lip and Palate Support:  The information pack is the Blue Book which is given out by cleft nurse coordinator and is available on NICU and is given to and discussed with the parents (available from the Cleft Lip & Palate Support Group and available online (downloadable in Adobe Acrobat format)). A visit can be arranged if parents request via the email address in the Blue Book.

Feeding Guidelines

• Click here to open the Feeding Guidelines

Timing of Surgery

• Lip and primary palate is usually repaired at around 5 months of age.
• Soft palate repair is usually around nine 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:

1. To give the best possible appearance of the lip, nose and face
2. To repair the palate to enable production of normal speech and no difficulty eating/drinking.
3. The correct alignment of the teeth and the jaws.
4. To ensure adequate hearing.

Other Issues

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.
• Although clefts are usually an isolated condition, there are more than 300 syndromes known to be associated with orofacial clefting, 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.
  • These investigations may be included if there are concerns: Chromosomal testing

Consider Velocardiofacial Syndrome also known as Chromosome 22q11 syndrome as this is the most common syndrome associated with cleft palate

Follow Up

The Cleft nurse coordinator will visit the newborn infant within 24-48 hours of birth and will arrange appropriate followup. 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 National Women’s newborn service 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.