Management of cleft lip and palate

by Dr. Zainab Mohammed Al-Tawili 1

Introduction
• Epidemiology:
◦ Clefts of the lip and cleft palate are fusion disorders that affect the mid-
facial skeleton.
• They are one of the most common congenital anomalies with a
worldwide incidence of between 0.8 and 2.7 cases per 1000 live births.
• The incidence of clefting varies by region, gender, ethnicity and
maternal characteristics.
• Racial differences are apparent with Native Americans having the
highest incidence (followed by Maoris, Chinese, Anglo Saxons) and
African-Americans the lowest.

Aetiology
• Despite being common, the aetiology of many clefts of the lip/palate remains
obscure.
• In addition to chromosomal abnormalities and teratogenic effects, there are
over 500 recognized Mendelian syndromes associated with a cleft of the
lip/palate.
• However, over 70% of clefts of the lip and palate and up to 50% of clefts of
the palate only are apparently isolated anomalies.
• The aetiology of these non-syndromic forms of cleft is multifactorial and the
relative genetic and environmental contributions remain unclear.

Embryology
• Orofacial clefts result from a failure of complete fusion between any of the independently
derived facial primordia that form the orofacial complex.
• Development of the human face begins around day 22 with the migration of cranial neural
crest cells (ectomesenchyme) from the dorsal region of the anterior neural tube into the
facial region.
• On either side of the foregut, ectodermal swellings form five pairs of branchial (pharyngeal)
arches establish during the 4th week of gestation, consisting of five distinct prominences:
• A pair of mandibular prominences develop into the lower jaw.
• An unpaired frontonasal prominence that gives rise to a pair of lateral and medial nasal
processes during the 5th week.
• During the following 2 weeks, the medial nasal processes enlarge and merge with each
other and the flanking maxillary prominences, completing the formation of the upper
lip.
• A pair of bilateral maxillary prominences that result in the upper jaw.

Embryology
• The shelves grow vertically (downwards) initially from the bilateral maxillary processes during
the 6th week.
• Growth of the embryo causes rotation and extension of the head, dropping the tongue that was
lying in the nasal cavity into the mouth (stomodeum). At the same time, the palatal shelves elevate
to the horizontal position above the tongue during the 7th week of gestation.
• Following elevation, the palatal shelves continue to grow towards each other during the 8th week.
• Events that disrupt the fusion may result in clefting:
• Clefts of the primary palate (i.e. lip, alveolus and palate anterior to the incisive foramen) are
caused by disruption of fusion of the medial nasal processes and maxillary prominences
around Week 6.
• Clefts of the secondary palate (i.e. hard and/or soft palate) are caused by disruption of the
elevation and/or fusion of the palatal shelves around week 9.

Anatomy
• Clefts of the lip and palate may be unilateral or bilateral, complete or incomplete.
• The presentation of clefting conditions can be categorized into the following groups:
• Clefts of the lip and alveolus (primary palate):
• The primary palate forms anterior to the incisive foramen.
• A cleft of the primary palate may vary from an incomplete cleft (formefruste) to a
minimal defect involving just the vermilion border, to a complete defect extending
from the vermilion border to the floor of the nose with clefting of the alveolus.
• Even in the absence of a frank cleft of the bony alveolus there may be evidence of
clefting in the form of a dental anomaly such as a missing or microdont lateral incisor.
• Clefts of the primary palate may be unilateral or bilateral.

Anatomy
• Cleft lip and palate (CL/P):
• Varying degrees of clefting of the lip and palate can also exist; complete to incomplete, in a wide range of combinations; uni-
and bilateral, symmetrical and asymmetrical.
• In complete clefts, a direct communication exists between the oral and nasal cavities on the cleft side.
• There can be substantial variation in the degree of palatal shelf separation and while the maxillary arch form generally
appears normal at birth, medial collapse of the maxillary segments occurs soon.
• In the bilateral complete cleft lip and palate, both nasal chambers are in direct communication with the oral cavity; the
palatal processes are divided into two equal parts and the turbinates are clearly visible within both nasal cavities.
• The nasal septum forms a midline structure that is firmly attached to the base of the skull but is fairly mobile anteriorly,
where it supports the premaxilla and columella. In these clefts, the premaxilla protrudes considerably forward of the facial
profile and is attached to a stalk-like vomer and the nasal septum.
• The ‘lip’ component of the medial segment contains only collagenous connective tissue. It is, therefore, grossly deficient in
bulk and lacks the features normally produced by muscle.
• Cleft palate:
• A cleft of the palate may involve only the soft palate, both soft and hard palates, but almost never the hard palate alone.
• Deficiency of mucosa and bone are the main features of clefts of the hard palate.
• The cleft may extend forward from the uvula to varying degrees, from a bifid uvula (Figure 15.3) to a ‘V’-shaped cleft
extending through the hard palate to the incisive foramen.

(A) (B)
(A) Unilateral complete cleft lip and palate,
showing the extent of the malformation in the
palate.
(B) Bilateral complete cleft lip and palate. The
premaxillary segment is clearly visible as an
extension of the nasal septum. The central
incisors are contained within this process.

(C)
C. Anterior view of a child with a
bilateral complete cleft of the lip
and palate. The columella and
philtrum are extremely short and
there is a wide defect between the
segments.
A bifid uvula associated with a submucous cleft palate.
The fibres of tensor palati are not joined, although the
epithelium is intact. These children may present with
nasal air escape due to a shortened palate and
velopharyngeal incompetence. There is often a notch
felt at the posterior border of the hard palate and a
bluish median line extends to the uvula.

Anatomy
• Cleft palate:
• Some babies have a ‘U’-shaped cleft of the palate that is described in ‘Robin sequence’.
• Robin sequence is characterized by upper airway problems associated with a very small
mandible and glossoptosis and is thought to be secondary to mandibular hypoplasia in
the 1st trimester, which causes the tongue to sit high in the mouth and prevent fusion
of the palatal shelves.
• Submucous cleft palate:
• Clefting of the velum or soft palate can be ‘submucous’ where the mucous membrane
remains intact, despite clefting of the underlying musculature.
• Submucous clefts of the palate occur in around 1 in 1200 live births, with only half of
the cases having clinically significant symptoms.

(A) (B)
(A) Cleft of the palate with an intact lip and alveolus. This U-shaped cleft was associated with the
Robin sequence and resulted from a failure of embryonic head rotation.
This maintained the tongue in the oral cavity and the palatal shelves subsequently formed around
the tongue, giving rise to the characteristic cleft shape.
(B) Another manifestation of this condition is extreme micrognathia. The Robin sequence may also
be associated with transverse clefts of the face. These transverse clefts may be rather subtle as
demonstrated in (C).

(C) (D)
(C), or severe as in (D). This latter patient has a first and second branchial arch
deformity associated with Goldenhar’s syndrome. Note the preauricular skin tag.

Diagnosis
• Clefting disorders are now increasingly diagnosed prenatally as part of routine ultrasound
screening between 18 and 20 weeks in utero.
• Much higher accuracy is achieved using 3D ultrasound but again isolated clefts of the
palate often remain undetected.
• The reported diagnostic accuracy of routine 2D ultrasonography in a low risk population
varies between 9–100% for complete clefts of lip and palate but is much lower (0–22%) for
clefts of the palate only (Maarse et al 2010).
• Prenatal screening provides knowledge of the impending birth of a baby with a cleft, which
allows both the family and appropriate clinicians to prepare for this event.

Management of individuals with clefts of the
lip and/or palate
• Although surgery can correct many of the structural defects, affected individuals can still face a
lifetime of functional, social and aesthetic challenges.
• The care of these infants often starts antenatally and continues from birth through to adulthood and
involves a large multidisciplinary team of clinicians. The specialties involved in this team vary greatly
but usually include:
◦ Plastic surgery.
◦ ENT (otolaryngology) and/or maxillofacial surgery.
◦ Speech pathology and audiology.
◦ Orthodontics.
◦ Paediatric dentistry.
◦ Paediatrics.
◦ Psychology.
◦ Specialist nursing and social services.
• In addition, genetics, ophthalmology, neurosurgery, periodontics and implantology may be involved
at different times.

Management of individuals with clefts of the
lip and/or palate
• In addition, genetics, ophthalmology, neurosurgery, periodontics and implantology
may be involved at different times.
• The appropriate specialists (usually a specialist nurse and/or primary surgeon) within
a cleft team aim to provide a rapid initial evaluation, often within hours of birth.
• Subsequent regular contact through either home visits or attendance at specialist cleft
review clinics ensures that families are supported appropriately and social and
psychological issues identified and resolved early.
• Treatment plans can then be formulated and implemented collaboratively by the
multidisciplinary team in partnership with the families.
• The goals of the treatment of a child with a cleft of the lip and/or palate are to restore
both form (appearance) and function (especially speech and mastication), while
optimizing an individual’s general health and well-being.

Management in the neonatal period
• Presurgical orthopaedics:
• Presurgical orthopaedics (PSO) takes advantage of the plasticity of the nasal
cartilage in the neonate by using orthodontic-like plates, with or without tapes and
nasal stents to reposition the maxillary segments and the nasal structures. PSO
needs to be started as soon after birth as possible to maximize the chances of an
infant tolerating the appliances.
• It is technically complex and demanding, not least because taking a maxillary
impression in a neonate is particularly challenging with the associated risks to the
airway.
• PSO should only be done by appropriately trained and experienced clinicians.
• Its use remains controversial, not least because of the associated financial burden
coupled with the lack of strong evidence of effectiveness.

• Counselling and parent support:
• Careful counselling by an appropriately informed cleft specialist is often needed by
parents.
• In many cleft teams, this is provided by the specialist cleft nurses who may also
carry out home visits to provide additional support.
• Parents not only have many questions but are also often confused and anxious and
may seek advice about the intermediate and long-term implications of the cleft
defect and the necessary surgery.
• They also often appreciate, and benefit from, the assistance available from
members of a parent support group such as CleftPALs (Australia), CLAPA (UK) and
American Cleft Palate-Craniofacial Association.

(A) (B)
(A) Presurgical orthopaedic appliance. The plate is held by micropore tape to the cheeks.
(B) Strapping aids in the positioning of the labial segments, especially in cases of bilateral
complete clefts. Depending on the institution, these appliances are used in cases of bilateral
cleft lip and those with very wide unilateral clefts as shown in (A).

• Primary surgery:
◦ There is still controversy regarding the timing of surgery and which is the most reliable technique consistent with
ensuring optimal growth of the face and development of speech.
• Lip repair:
◦ Is less controversial than is palate repair and is generally undertaken around 10–12 weeks and almost certainly
by 6 months of age, provided the infant is otherwise developing well.
• The aim of the lip repair is to restore the continuity of the orbicularis oris muscle of the lip, and with it, the
appearance of the upper lip.
• Palate repair:
• Aims to reconstruct the abnormally inserted musculature of the soft palate to normalize movements of the
soft palate and permit the development of normal speech.
◦ The extent and timing of palatal surgery is one of the major and continuing controversies in cleft management;
this relates to the perceived balance between the benefits of good speech development (which is promoted by
early closure) versus the deleterious effects on midfacial growth through surgical trauma and associated scarring
(which can be minimized by delaying the surgery).
◦ To date, there is no strong evidence regarding the best approach to primary surgery.
◦ Attempts to address this gap in the evidence are being made through large multicentre international clinical trials.

Surgical repair of a bilateral complete cleft lip with columella
lengthening.
The alar base is symmetrical, although there is an accentuation of
the cupid’s bow and eversion of the vermillion border.

Management during childhood
• Speech and language and ENT problems:
• Children with clefts and other craniofacial malformations are at an increased
risk of speech and language difficulties.
• Regular assessments are required to monitor the speech and language
acquisition process, to assist in making decisions about the need for either
further surgery or speech therapy or both.
• During childhood, care needs to be exercised to ensure hearing is optimal for
speech and language development; Otitis media is common in infants and
children with cleft palate and some may also have sensorineural loss.
• Suboptimal hearing can affect speech and language development so regular
audiology assessments for all infants with clefts of the lip and palate is
important.

• Velopharyngeal inadequacy (VPI):
• Children with clefts are at increased risk of VPI.
• Normal speech requires that the muscles that make up the velopharyngeal sphincter
(predominantly the muscles of the soft palate and nasopharynx) work in a coordinated
fashion.
• Defects in any aspect of the nasopharyngeal anatomy and/or physiology may lead to
VPI; it is uncommon for otherwise healthy individuals to have VPI.
• For individuals with anomalies of their velopharyngeal sphincter such as a cleft of the
palate (hard and/or soft, repaired or unrepaired) VPI can, if severe, compromise speech
production rendering a child incomprehensible to others.
• The speech pathologist’s assessment of speech production is critical in the identification
and management of VPI and they work closely with the primary cleft surgeons in
deciding what, if any, intervention is required to optimize speech.

• Velopharyngeal inadequacy (VPI):
• If significant VPI is identified, then surgery is usually needed.
• There are a number of different approaches to the surgical management of VPI,
including: narrowing the velopharyngeal opening (pharyngoplasty), lengthening the
soft palate (intravelar veloplasty) and bulking up the posterior pharyngeal wall (fat
augmentation).
• Although surgery can be expected to improve structural obstacles which hinder the
development of normal speech, postoperative speech therapy may still be needed
(before and after surgery) to correct poor speech habits that have developed because of
the structural malformation.
• Occasionally, surgical correction of VPI is not possible or does not bring resolution. In
this case, a palatal obturator or a speech bulb may reduce the velopharyngeal space
sufficiently for normal speech to develop.

• Orthodontics:
• Children with clefts of the lip and palate and those with cleft of the hard-palate are at risk of
compromised growth of the maxilla as a result of post-surgical scarring.
• If severe, the growth restriction of the maxilla which affects all three dimensions can lead to the
development of a skeletal class III relationship and an associated buccal crossbite.
• Individuals with complete clefts of the lip and palate may require extensive orthodontic treatment.
• The first comprehensive orthodontic evaluation is generally made around the age of 8 years of age
although occasionally, there may be a need for earlier review.
• Orthodontic management at this age can be subdivided into three main components:
• Mixed dentition treatment:
• Interceptive treatment such as minor tooth alignment to facilitate anterior alignment or correction of
an anterior incisal cross-bite.
• Palatal expansion prior to alveolar bone grafting comprises a relatively short period of fixed appliance
therapy aimed at achieving an optimal arch shape to improve access for surgery.
• Bone grafting is usually undertaken when the cleft-side permanent canine (cuspid) tooth shows
between half and two-thirds of root development.

(A) (B)
(A) One of the effects of early surgery and scarring is collapse of the palatal segments due
to an inhibition of growth. Rotation of the central incisor adjacent the cleft can also
be seen.
(B) Palatal expansion with a quad helix appliance to correct the posterior cross-bite and
open space for the bone graft.

• Orthodontics:
• Orthodontic management at this age can be subdivided into three main components:
• Secondary alveolar bone grafting:
• Alveolar bone grafting should be timed to precede the eruption of the permanent canine tooth on the
cleft side, which usually occurs at approximately 11 years of age.
• The aim of bone grafting is to:
• Restore the bony contour of the alveolus.
• Stabilize the maxillary expansion.
• Provide a bony matrix through which teeth (especially the canine) may erupt.
• Allow the teeth to have a healthy supporting periodontium.
• Surgery:
• Presurgical palatal expansion (if required) with fixed appliances such as a quad helix.
• Preparation of recipient area may be prepared by extraction of retained primary teeth several
weeks prior to surgery.
• Bone harvest for the graft is usually autologous iliac crest cortico-cancellous bone, but may be
obtained from the mandibular symphysis, tibia, rib, or calvarium.

(A) Periapical radiograph of a cleft prior to bone grafting. The lateral incisor is present on
the palatal aspect of the canine tooth. A small supernumerary tooth (arrowed) is also
present, and was removed at the time of surgery.
(B) The cleft has been filled with cancellous bone, harvested from the iliac crest. After 3
months, there is already movement of the lateral and canine and both these teeth
erupted through the graft to the mouth, where they were aligned orthodontically.
(A) (B)

Cleft management in adolescence and early
adulthood• Orthodontics:
• Full permanent dentition correction:
◦ Once the full permanent dentition is established (excluding the third molars), final correction of the
malocclusion and tooth alignment is undertaken.
• The reasons for postponing the definitive orthodontic management to mid to late adolescence include:
• In the event of a significant skeletal discrepancy orthognathic surgery may be required in conjunction
with orthodontic treatment the planning for which cannot occur until growth is complete.
• At 12–16 years dental suitability and motivation to cooperate with orthodontic treatment is assessed
prior to the commencement of full fixed appliance treatment.
• Rapid palatal expansion and/or fixed appliance orthodontic treatment is undertaken at the optimum
time – occasionally elective extraction of teeth for orthodontic reasons is indicated.
• Orthognathic surgery:
◦ If necessary, further aesthetic surgery such as a rhinoplasty can be undertaken as a final surgical
procedure.
• In some instances, functional lip and nose revisions are combined with alveolar bone grafting at an
earlier stage to reduce the impact of the aesthetic deformity on the growing child but in general, such
procedures are postponed until growth has ceased and orthodontic therapy completed.

Importance of dental care in overall
management• The paediatric dentist plays an essential role in coordinating the oral healthcare for these individuals, in
order to ensure they reach adulthood with a healthy dentition and positive attitude to dental care.
• The paediatric dentist will generally coordinate all dental care aspects with those of other disciplines
and as such, it is important for them, or their representative, to regularly review the child with a cleft
lip and palate.
• The first appointment:
◦ Many specialist paediatric dental academies support the notion that all infants should be seen by a
dentist within 6 months of eruption of the first tooth (i.e. before the age of 1 year). This is even more
important in infants born with a cleft.
• The purpose of this initial consultation is to explain:
• The dental aspects of the clefting process.
• The likely course of dental management. The involvement of different specialties including restorative,
radiological, orthodontic and possible later oral surgical care.
• The probability of the absence of the normal tooth in the region of the cleft, and conversely, the
possibility of one or more supernumerary teeth in the cleft region should be mentioned.

A tooth erupting in the cleft. Such
teeth often become carious, but
should be repaired and retained until
bone grafting is performed. Where
permanent teeth are congenitally
absent, especially the lateral incisor,
the supernumerary may be used if of
adequate size.

management• The first appointment:
• The purpose of this initial consultation is to explain:
• The likelihood of the presence of crown and/or root morphological abnormalities and enamel hypoplasia of the incisor
and canine teeth adjacent to the cleft should be indicated, with the positive reassurance that these can be treated relatively
simply soon after they appear.
• The absolute importance of sound preventive care and regular dental visits should be emphasized.
• Preventive dental care:
◦ Children with clefts of the lip and/or palate may be at increased risk of developing dental caries for a number of reasons,
however the evidence is fairly weak. Reasons include:
• PSO (if carried out) may predispose infant oral cavity to colonization by mutans streptococci and hence, to the early
development of dental caries.
• Early infant feeding problems may lead to prolonged and more cariogenic feeding habits.
• Presence of developmental defects of enamel particularly around the cleft site.
• Presence of dental crowding, malocclusion and orthodontic appliances.
• Existence of other comorbidities, which may increase caries risk, e.g. reduced salivary flow associated with
velocardiofacial syndrome.
• Risk of being ‘spoilt’ with sugary treats to compensate for hospitalizations and other healthcare exposures.

management
• Dental anomalies:
◦ Dental anomalies are extremely common in children with orofacial clefting. The most
commonly affected tooth is the maxillary lateral incisor on the cleft side. This is due, in
part, to disruption of the dental lamina.
• Anomalies may include:
◦ Agenesis of teeth.
◦ Supernumerary teeth.
◦ Concurrent agenesis and supernumerary teeth within or adjacent to the cleft.
◦ Disorders of morphogenesis (size and shape).

management
• Cosmetic restoration of malformed anterior teeth and alveolar cleft:
◦ The appearance of the teeth can be improved at any time, depending on the child’s
perceptions and wishes.
• Composite resin restorations may be placed for hypoplastic or morphologically
abnormal permanent incisor teeth adjacent to the cleft(s) soon after eruption,
however, enamel-bonded crowns or veneers should be reserved until after passive
eruption and establishment of the gingival margin at the cementoenamel junction.
• As a temporary measure, and in suitable cases, an adhesive retained bridge may be
placed to replace missing incisor teeth. This form of prosthesis is a superior
alternative to a removable partial denture, however an osseointegrated implant
remains the ultimate treatment solution when space cannot be closed orthodontically.

Reference:

Management of cleft lip and palate

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