Cleft Lip and Palate: Craniofacial Malformation

Cleft Lip and Palate (CLP)

• Cleft lip and palate is the most common craniofacial malformation, accounting for 65% of all anomalies affecting the head and neck.
• The prevalence of CLP varies geographically and between different racial groups, occurring in approximately 1 in every 700 live births among Caucasians (0.42/1000 live births).

Developmental Process

• In normal development, fusion of the maxillary and medial nasal processes occurs around the sixth week of intra-uterine life.
• The palatal shelves 'flip-up' from a vertical to a horizontal position, followed by fusion to form the secondary palate, around the eighth week.
• The embryological processes must grow until they come into contact before fusion can take place.

Feeding and Nutrition

• Feeding difficulties are associated with fatigue, poor weight gain, excessive air intake, and nasal regurgitation.
• Feeding problems depend on the type and severity of the cleft and whether both lip and palate are involved.

Speech and Hearing

• Velopharyngeal insufficiency affects speech development, as the soft palate is unable to make adequate contact with the back of the pharynx to close off the nasal airway.
• A cleft involving the posterior part of the hard and soft palate can cause hearing difficulties, which can also retard speech development.
• Cleft lip only is usually associated with minimal problems.

Dental and Orthodontic Issues

• Unilateral cleft lip and palate is associated with segmentation of the dental arch into lesser and greater segments.
• Bilateral clefts involve both lateral segments, often collapsed behind a prominent premaxilla.
• Delayed eruption, hypodontia, microdontia, and enamel defects are common dental anomalies.
• Individuals with unoperated clefts do not experience a significant restriction of facial growth.

Management and Treatment

• Management is a long-term, complex, multidisciplinary process that starts from birth and extends to over 20 years of age.
• The clinical nurse provides initial support and advice, and educates the mother on feeding the cleft baby.
• Presurgical orthopaedic alignment of the cleft alveolar segments may be carried out in the neonate.
• Surgical repair of cleft lip is usually carried out between 3 and 6 months of age, and cleft palate repair between 6-9 months or 9-12 months of age.
• Speech assessment starts by the age of 2 years, and pharyngeal surgery may be needed to improve velo-pharyngeal insufficiency.
• Alveolar or secondary bone grafting is performed around 8-10 years of age, before the eruption of the permanent canine.
• A period of orthodontic treatment is required prior to graft placement, followed by a period of retention.
• Orthognathic surgery may be required in 25% of CLP patients, and prosthetic replacement may be needed to replace missing teeth.

Importance of Multidisciplinary Care

• The most important thing in management of CLP is being able to audit the treatment progress among the different disciplines involved in care provision.
• Concentration of expertise and experience at certain centres of excellence produces superior results to those obtained by discrete practitioners.
• Standardised treatment protocols for management of CLP have been set in many countries, and careful standardised records should be taken before and after any intervention.
• Patients' registration systems are still lacking in most centres, and initiatives for establishment of a standardised national protocol have been taken to achieve a more efficient care of cleft patients.