Paediatrics: Congenital abnormalities: upper airway

Congenital abnormalities: upper airway

Choanal atresia (CA)

Congenital obstruction of the posterior choana of the nose may be unilateral or bilateral. Babies are obligate nose breathers and bilateral obstruction presents with asphyxia during feeding and sleep. Unilateral obstruction may pass unnoticed. CA may be a presenting feature of the CHARGE association, which is:

•   Coloboma;

•   Heart defects;

•   Atresia of the choanae;

•   Retardation of growth and development;

•   Genitourinary abnormalities;

•   Ear abnormalities and hearing loss.

Diagnosis

•   NGT: the diagnosis is excluded by passage of a tube down each nostril.

•   CT scan will determine whether the obstruction is membranous or bony.

Treatment

•   Emergency treatment comprises an oropharyngeal airway and an orogastric tube for feeding.

•   Surgery (which is performed through a transnasal approach) restores the patency of the choanae.

Laryngeal atresia

A rare condition that is invariably fatal at birth. The condition is relatively easy to detect using antenatal US because the foetal lungs appear bright and large. The large airways can also be visualized because they are dis-tended with foetal lung fluid. The condition is not amenable to correction (due to lung hypoplasia) and termination of pregnancy should be offered.

Cleft lip and palate

Approximately 1 baby per 1000 is born with a cleft lip and palate. This may occur sporadically or there may be a family history. A cleft lip is immediately apparent. An isolated cleft palate may not be noticed immediately, but will present with feeding difficulties, particularly nasal regurgitation of milk. A cleft palate will interfere with breastfeeding as it precludes generation of suction. Bottle-feeding may also be difficult unless a squeezable bottle, rather than a rigid bottle, is used.

Management

•   Lip repair: at around 3mths of age.

•   Palate repair: at around 6mths of age.

Follow-up: long-term because of problems with speech, dentistry, and hearing.

Pierre–Robin sequence

The Pierre–Robin sequence (Fig. 23.1) is characterized by three features:

• micrognathia;

• glossoptosis;

• cleft palate.

Management

• The large tongue has a tendency to obstruct the airway causing apnoea, particularly during sleep.

• Prone positioning may help, allowing the tongue to fall forward, but occasionally tracheostomy is necessary.

• Endotracheal intubation is often difficult.

• Tube feeding may be necessary.

• The palate is generally repaired between 9 and 18mths of age.

The airway problems invariably improve with growth.