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Choanal atresia

Last updated: January 31, 2019

Summary

Choanal atresia is a congenital condition characterized by a bony and/or membranous obstruction of the posterior nasal passage. This obstruction may occur either unilaterally or bilaterally. Unilateral choanal atresia often presents late with chronic inflammation (e.g., rhinorrhea, congestion) of the affected nasal passage. Bilateral choanal atresia manifests as obstructed nasal breathing with intermittent cyanosis immediately after birth; breathing improves when crying, as it allows the infant to breathe through his or her mouth. The diagnosis is confirmed with contrast rhinography or CT imaging. The definitive treatment is surgery, in which the obstructive membrane or bony portion is perforated.

Definition

  • Congenital condition; characterized by a bony (90% of cases) and/or membranous (10%) obstruction of the posterior nasal passage

References:[1]

Epidemiology

  • Sex: > (∼ 2:1)
  • Unilateral choanal atresia is twice as common as bilateral choanal atresia.
  • Frequently associated with other anomalies (“CHARGE syndrome” - coloboma, heart defects, atresia choanae (also known as choanal atresia), retardation of growth, genital abnormalities, and ear abnormalities)

References:[2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Choanal atresia has a positive association with periconceptional maternal exposure to:

References:[2]

Pathophysiology

  • It has been hypothesized that the condition is due to:
    • Failed resorption of the oronasal membrane during the embryonic period
    • Outgrowth of the palatine bone
    • Atypical mesodermal adhesions
    • Altered local growth factors

References:[2]

Clinical features

  • Bilateral choanal atresia
    • Early presentation
    • Infants are only able to breathe through the mouth (immediately postpartum)
    • Cyanosis that worsens when feeding and improves when crying
    • Upper airway obstruction (e.g., noisy breathing, dyspnea)
    • Food intake impossible: Complete airway obstruction is a life-threatening condition immediately following birth because of the worsening dyspnea associated with feeding.
  • Unilateral choanal atresia
    • Typically presents later in life
    • Chronic rhinitis in the affected nasal passage with purulent nasal discharge over several weeks

References:[2][3]

Diagnostics

References:[2][4][5]

Treatment

In suspected cases of bilateral choanal atresia, do not feed! A feeding tube is required due to the increased risk of aspiration!
References:[3][6]

References

  1. Tewfik TL. Choanal Atresia. Choanal Atresia. New York, NY: WebMD. http://emedicine.medscape.com/article/872409-overview. Updated: December 24, 2015. Accessed: February 16, 2017.
  2. Chaudhry S, Kochhar L, Galagali J, Kumar NA. Unilateral choanal atresia. Med J Armed Forces India. 1997; 53 (2): p.131-132. doi: 10.1016/s0377-1237(17)30683-4 . | Open in Read by QxMD
  3. Kwong KM. Current Updates on Choanal Atresia. Front Pediatr. 2015; 3 (52). doi: 10.3389/fped.2015.00052 . | Open in Read by QxMD
  4. Choanal atresia. https://radiopaedia.org/articles/choanal-atresia. Updated: February 16, 2017. Accessed: February 16, 2017.
  5. Choanal Atresia. http://care.american-rhinologic.org/choanal_atresia. Updated: January 20, 2015. Accessed: February 16, 2017.
  6. Prasad M, Ward RF, April MM, Bent JP, Froehlich P. Topical mitomycin as an adjunct to choanal atresia repair. Arch Otolaryngol Head Neck Surg. 2002; 128 (4): p.398-400.
  7. Choanal atresia. https://medlineplus.gov/ency/article/001642.htm. Updated: May 8, 2015. Accessed: February 16, 2017.