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Chapter: Paediatrics: Neonatology

Paediatrics: Orofacial clefts

Orofacial clefts are due to failure of fusion of maxillary and pre-maxillary processes.

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.

 

 

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Paediatrics: Neonatology


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