Choanal atresia

CHOANAL ATRESIA
Occurence:
  • Most cases of choanal atresia are sporadic, although familial cases suggest autosomal dominant or autosomal recessive modes of a single gene defect. 
  • Persistence of bucconasal membrane
Clinical features
Most cases of choanal atresia are sporadic, although familial cases suggest autosomal dominant or autosomal recessive modes of a single gene defect. Approximately one-half to two-thirds of patients with choanal atresia have associated malformations, which occur more frequently with bilateral atresia. The most common of these is the CHARGE syndrome:
  • Coloboma of the eye
  • Heart malformations
  • Choanal Atresia
  • Retarded growth, development, or both
  • Genital hypoplasia
  • Ear malformations, deafness, or both
Types
  • Bony – 90%; membranous - 10%
  • Unilateral atresia is more common
  • Bilateral atresia presents with respiratory obstruction (new born – a natural nose breather, does not breath from mouth)
In some cases, this may present as cyanosis while the baby is feeding because the oral air passages are blocked by the tongue.
The cyanosis may improve when the baby cries, as the oral airway is used at this time.
These babies may require airway resuscitation soon after birth.
  • Diagnostic features
  1. Mucoid discharge in the nose
  2. Absence of air bubbles in the nasal discharge
  3. Failure to pass a catheter from nose to pharynx(posterior rhinoscopy)
  4. Investigated using CT scan
Treatment :
  • McGovern's technique – feeding nipple with a large hole
  • Definitive treatment – surgical correction at one and half years
Exam Question
Clinical symptoms:
Persistence of bucconasal membrane associated with CHARGE syndrome:
  • Coloboma of the eye
  • Heart malformations
  • Choanal Atresia
  • Retarded growth, development, or both
  • Genital hypoplasia
  • Ear malformations, deafness, or both
Bilateral atresia type presents with respiratory obstruction
Diagnosed by:
  • Absence of air bubbles in the nasal discharge
  • Failure to pass a catheter from nose to pharynx(posterior rhinoscopy)
Investigated using CT scan


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