Facial deformity Flashcards
What are the types of cleft?
- LAHSAL - lip, alveolus, hard palate, soft palate, alveolus, lip. Upper case denotes complete (complete cleft going right up to the nose and involving the hard and soft palate
- lahsal - lower case denotes incomplete
What is the epidemiology of a cleft palate in isolation? (not associated with cleft lip)
It affects 1 in 2000 births. It can affect the hard palate, soft palate or both. It is associated with syndromes.
What is the epidemiology of a cleft lip and palate?
It affects 1 in 600/700 babies which is 1000 births per year in the UK. There are 2 distinct presentations which are cleft lip +/- cleft palate or isolated cleft palate.
What is the aetiology of cleft lip and palate?
- Genetic - family history in 40% of cases
- Environmental - possible causes include anti-convulsant drugs, nutritional deficiency, anaemia, alcohol, low socio-economic status
- Probably a genetic predisposition triggered by environmental factors
What is a sub-mucous cleft palate?
It affects 1 in 12000 births. It is not obvious and may not be suspected until the child is older. Usually there is a previous history of problems with feeding/failure to thrive. The diagnostic features are:
- Notch on palatal shelf
- Bifid uvula
- ‘Blue translucent zone’
- Or at surgery
When can cleft lip and palate be diagnosed?
In an antenatal scan around 20/40 weeks.
What is the prevalence of each type of cleft?
- 20% cleft lip (uni or bilateral)
- 50% cleft lip and palate (uni or bilateral)
- 30% cleft palate
15-40% of cleft children have associated anomalies.
What syndromes are associated with a cleft palate?
Van de Woude: - Autosomal dominant - Lip pits - CP - Linked with cardiac anomalies - Hypodontia Pierre Robin sequence: - Cleft palate, glossoptosis - Mandibular retrognathia - Around 1/3 of cases are linked to stickler syndrome which is a connective tissue disorder Foetal alcohol syndrome: - A wide range of symptoms: small head size, low body weight, learning difficulties, coordination issues which can require life long care
What is the organisation of cleft care in the UK?
- CSAG 1998 recommended reorganisation of cleft care so fewer centres caring for larger volumes of cleft patients
- 10 regional cleft units
- Trent regional cleft network set up in 2000
- Hub in Nottingham
- Spokes in Sheffield, Doncaster, Chesterfield, Lincoln, Boston, Derby and Leicester
What does cleft lip/palate affect in neonatal period?
- Breathing
- Feeding - babies will not be able to breastfeed as they cannot produce negative pressure in their mouth. Special types of bottle have been developed.
- Cormorbidity in syndromic babies
Why is there a risk with breathing in Pierre Robin sequence?
In Pierre robin sequence there can be problems with breathing as the tongue is too far back and occludes the airway.
How can breathing problems in cleft lip/palate be managed?
You can put the child in their side. Nasopharyngeal tube can be used alongside nasogastric tube. There are secondary interventions if the other things don’t work but these are not common. They include tracheostomy or making the mandible bigger but this will cause damage to tooth buds and nerves.
When can there be cleft lip and palate repair?
When the baby is thriving. Lip from 3 months and posterior palate from 6 months. Babies are obligate nose breathers so at 6 months babies have learnt to breathe through their mouths so the posterior palate can be operated on.
What are the important components when repairing a cleft?
Skin, muscle and bone.
The role of the palate is feeding and speech.
How is surgery done?
There is soft tissue mobilisation. Inside the mouth a vomerine flap is made and septal centralisation.
. In isolated cleft palate repair, incisions are made and there is flap elevation. Von Langernbeck flaps are raised off hard palate and muscles in soft palate. The greater palatine artery provides blood flow so healing is better. There is nasal mucosa and muscle repair. There is muscle retropositioning. We sometimes use a bioguide collagen membranes. Then oral layer closure.
