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. Majority have no obvious cause.

• Enviromental 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;

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 (obdurator) may all be required if cleft palate is too large to allow adequate suck.

• Be aware of increased risk of infections (aspiration pneumonia and, later, secretory otitis media with conductive hearing loss). Treat as appropriate.

• Surgical repair of 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 prob-lems may include:

• Hindered parental bonding.

Psychological morbidity.