2. Cleft lip and palate (CLP) is the most common congenital craniofacial deformity.
Its incidence varies according to the ethnic group studied: American Indians (1/300 live births)
> Japanese (1/400) > Chinese (1/500) > Caucasians (1/600) > black people (1/2500).
Cleft lip (CL) ± cleft palate (CL(P)), forms a separate entity from isolated clefts of the secondary
palate (CP), with a difference in incidence, gender bias and genetic contribution.
CL(P) is approximately twice as common in males as females, whereas, isolated CP is twice as
common in females.
Cleft lip and palate
3. CL arises from failure of fusion (at 7 weeks in utero) between the medial nasal, lateral nasal
and maxillary swellings.
CP arises from failure of fusion (8-9 weeks in utero) of the lateral palatal swellings.
CLP may be isolated or as part of a syndrome.
The aetiology of isolated CLP is multifactorial with both genetic and environmental influences.
There is a family history of CL(P) in approximately 40% of individuals whereas the
corresponding figure in CP is 20%.
Environmental risk factors include maternal alcohol intake, smoking and phenytoin intake.
Folic acid may have a protective effect.
Many conditions can be associated with CP including Pierre-Robin sequence, hemifacial
macrosomia, Treacher Collins syndrome and Stickler syndrome.
Aetiology
4. The severity of a cleft can vary from a mild deformity (submucous cleft, forme fruste of the lip)
to a complete bilateral CLP.
Many classifications exist, a popular one is that of Kernahan and Stark.
In this classification, primary palate refers to the lip, alveolus and palate anterior to the
incisive foramen.
A complete cleft of the primary palate will involve the full thickness of these structures.
Classification of CLP
5. The following figures show clinical examples of various types of cleft.
Classification of CLP
(A) Examples of cleft lip and palate:
(i-iii) unilateral complete cleft of the lip and palate
(primary and secondary)
(iv) Unilateral complete cleft of the lip and primary
palate,
(v) Bilateral complete cleft of the lip and primary palate,
and
(vi) A bifid uvula which may suggest a submucous cleft.
6. (B) (i) Care pathway for the management of cleft lip and palate,
(ii) pre-surgical orthopaedics with lip strapping,
(iii) A tri-helix appliance used to achieve expansion before alveolar
bone grafting (ABG), and
(iv) A Class III relationship that is often evident and requires joint
orthodontic-orthognathic treatment.
7. Clinical problems depend on the severity and location of the cleft. These include:
Feeding difficulty due to communication between the oral and nasal cavities.
Hearing problems secondary to poor middle ear drainage due to Eustachian tube dysfunction.
Speech defects due to velopharyngeal incompetence and secondarily to poor hearing.
Dental anomalies include: (a) hypodontia (50% have a missing lateral incisor on the cleft side),
(b) supernumerary or supplemental lateral incisors, (c) maxillary canine impaction (x10 risk),
(d) delayed dental development, (d) hypoplastic teeth, (e) microdontia and (f) impaction of
first permanent molars.
Malocclusion including anterior and posterior crossbites.
Deficient maxillary growth related to scarring of the maxilla following palate repair.
Low self-esteem.
Clinical Problems in CLP
8. The treatment of CLP involves a multidisciplinary approach by a dedicated cleft lip and palate
team.
An example of a care pathway is shown in the following Figure.
Treatment
9. At the time of birth the parents should receive counselling and the contact details of a
support group, e.g. Cleft Lip and Palate Association (CLAPA).
Special feeding bottles (e.g. Haberman feeder), which eject fluid without the infant having to
generate negative intra-oral pressure, can be helpful if breast feeding is unsuccessful.
Treatment
10. Presurgical orthopaedics, with a maxillary removable appliance, can be used to encourage
lateral palatal shelf growth by stopping the tongue from sitting within the cleft site.
Such plates, used up to the time of palatal surgery, facilitate palate repair by approximating
the cleft segments.
Extra-oral lip strapping can be used in bilateral CLP to control growth of the premaxilla which
facilitates lip closure.
Treatment
11. The surgical protocol for CLP can vary between teams due to the lack of evidence to support
any one protocol.
Many undertake lip repair at 6 months by re-alignment of muscle fibres, to encourage normal
function, and skin closure.
Palatal repair is undertaken at 9-12 months to encourage development of normal speech.
Dissection should be minimal to limit scarring that may hinder future maxillary growth.
Treatment
12. As the deciduous teeth erupt, preventive dental advice (oral hygiene, dietary and use of
fluoride) is important to establish good dental health.
A speech assessment should be undertaken by 2 years to detect any speech abnormality.
An ENT opinion, to assess hearing, can also be useful as patients often have middle ear
drainage problems.
Treatment
13. At 6-8 years psychological support maybe required as children start to notice that they are
different and may be teased.
Preventive dental advice should continue into the mixed dentition where fissure sealing may
be helpful.
A full orthodontic assessment is also important at this stage.
Removable/fixed appliances can be used to correct anterior crossbites in concerned patients.
Care should be taken not to move teeth towards the cleft as the lack of bone may cause root
exposure.
Treatment
14. Alveolar bone grafting (ABG) is usually undertaken between 8.5-10.5 years when the root of
the maxillary canine is half to two-thirds formed.
The role of ABG is to provide bone, usually taken from the iliac crest, for canine eruption, offer
bony support to teeth on either side of the cleft, close residual palatal fistulae and provide
nasal support.
Expansion, with a quad/tri-helix, is often necessary before bone grafting to expand the
collapsed cleft segment and improve access to the site.
Fixed appliances should be used to stabilize the mobile premaxilla in bilateral complete CLP
prior to ABG.
Treatment
15. Once in the permanent dentition, definitive orthodontic treatment can be undertaken.
Patients often have a Class III malocclusion due to deficient maxillary growth.
The tight cleft lip and a lack of overbite may potentiate relapse following treatment.
Where there is a severe skeletal discrepancy, an orthognathic approach, almost certainly
involving maxillary advancement, may be undertaken near the completion of growth.
This should be planned with care because it may further compromise speech, by effecting
velopharyngeal function, and there is a high risk of relapse.
Distraction osteogenesis may help to reduce these complications as it produces skeletal
change by slow movement that produces gradual stretching of the soft tissues.
Treatment
16. The replacement of missing teeth with implants can also be undertaken at the end of growth
assuming there is adequate bone volume at the site of implant placement.
Patients may have a number of plastic surgery procedures to improve nasal aesthetics, lip
revision and close residual palatal fistulae during the CLP care pathway.
Treatment