Paediatrics: Orofacial clefts

Orofacial clefts

Orofacial clefts are due to failure of fusion of maxillary and pre-maxillary processes. They may be unilateral or bilateral and result in cleft lip and/or cleft palate. The incidence is 71/1000 live births.

Causes

Multifactorial and includes genetic and environmental factors. 66% of clefts are isolated. The majority have no obvious cause.

• Environmental factors: maternal folic acid deficiency; maternal exposure to alcohol, tobacco, steroids, anticonvulsants, and retinoic acid.
• 730% are syndromic, e.g. Pierre–Robin syndrome (large midline posterior cleft palate, mandible hypoplasia, prone to upper airway obstruction due to a posteriorly displaced tongue).

Treatment

• Refer to specialized ‘cleft lip and palate’ multidisciplinary team.
• Possible upper airway obstruction is a recognized complication of a large cleft palate, e.g. Pierre–Robin syndrome. If it occurs or is likely:

nurse prone;

the nasopharyngeal airway may be helpful;

monitor SpO₂—a low or worsening SpO₂ is an ominous sign and should be taken very seriously;

intubation may be difficult and require specialist (ENT) support.

• Feeding problems are common. Specialist nursing input, special feed devices and prosthetic plate (obturator) may all be required if the cleft palate is too large to allow adequate suck.
• Be aware of the increased risk of infections (aspiration pneumonia and, later, secretory otitis media with conductive hearing loss). Treat as appropriate.
• Surgical repair of the lip is usually at 3mths; palate at 6–12mths.
• Later speech defects and dental problems can occur requiring speech therapy and dental input respectively. 

Prognosis

Repair of unilateral complete or incomplete lesions usually produces a good result. As well as those complications described above later problems may include:

• Hindered parental bonding.

Psychological morbidity.