eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Choanal Atresia

Author: Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Coauthor(s): Yaser ALI Alrajhi, MD, FRCSC, Fellow, Department of Otolaryngology, Division of Pediatric Otolaryngology, McGill University Faculty of Medicine; Abdulrahman A Hagr, MBBS, Staff Physician, Department of Otolaryngology, McGill University, Canada
Contributor Information and Disclosures

Updated: Oct 7, 2009

Introduction

Complete nasal obstruction in a newborn may cause death from asphyxia. During attempted inspiration, the tongue is pulled to the palate, and obstruction of the oral airway results. Vigorous respiratory efforts produce marked chest retraction. Increased cyanosis and death may occur if appropriate treatments are not available; however, if the infant cries and takes a breath through the mouth, the airway obstruction is momentarily relieved. Then the crying stops, the mouth closes, and the cycle of obstruction is repeated.


Choanal atresia. Rhinogram demonstrating blockage...

Choanal atresia. Rhinogram demonstrating blockage of radiopaque dye at the posterior choanae. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

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Choanal atresia. Rhinogram demonstrating blockage...

Choanal atresia. Rhinogram demonstrating blockage of radiopaque dye at the posterior choanae. From T.L. Tewfik and V.M. Der Kaloustian, with permission.


History of the Procedure

In 1755, Roederer first described congenital choanal atresia; therefore, this condition has been recognized for more than 200 years. In 1854, Emmert reported the first successful surgical procedure for congenital choanal atresia in a 7-year-old boy using a curved trocar transnasally. Over the years, the necessity of serial dilatations to maintain patency of the choanae has been clearly recognized.

Frequency

The average rate of choanal atresia is 0.82 cases per 10,000 individuals. Unilateral atresia occurs more frequently on the right side. The ratio of unilateral to bilateral cases is 2:1. A slightly increased risk exists in twins. Maternal age or parity does not increase the frequency of occurrence. Chromosomal anomalies are found in 6% of infants with choanal atresia. Five percent of patients have monogenic syndromes or conditions.

Race: Choanal atresia occurs with equal frequency in people of all races.

Sex: More studies report significantly more females than males affected.

Etiology

The nasal cavities extend posteriorly during development under the influence of the posteriorly directed fusion of the palatal processes. Thinning of the membrane occurs, which separates the nasal cavities from the oral cavity. By the 38th day of development, the 2-layer membrane consisting of nasal and oral epithelia ruptures and forms the choanae (posterior nares). Failure of this rupture results in choanal atresia. Although these choanae are not in the same location as the definitive choanae, which are eventually located more posteriorly, the unexpectedly anterior extent of choanal atresia is explained. In 2008, Barbero et al suggested that prenatal use of antithyroid (methimazole, carbimazole) medications was linked to choanal atresia.1

Pathophysiology

A number of theories have been proposed to explain the occurrence of choanal atresia, and they can be summarized as follows:

  • Persistence of the buccopharyngeal membrane
  • Failure of the bucconasal membrane of Hochstetter to rupture
  • Medial outgrowth of vertical and horizontal processes of the palatine bone
  • Abnormal mesodermal adhesions forming in the choanal area
  • Misdirection of mesodermal flow due to local factors

Presentation

The clinical evaluation includes a complete physical examination to assess for other congenital anomalies. A small feeding tube could be used to determine the patency of the choana, but a complete nasal and nasopharyngeal examination should be performed using a flexible fiberoptic endoscope to assess the deformity. A simple method using the automatic tympanometer to screen newborns for congenital choanal atresia was recently published. The sensitivity and specificity of the technique in diagnosing a patent nostril was reported as 100%. However, a high level of suspicion is required to diagnose bilateral choanal atresia.

Symptoms of severe airway obstruction and cyclical cyanosis are the classic signs of neonatal bilateral atresia. When crying alleviates respiratory distress in an obligate nasal breather, the neonatologist should be alerted to the probability of bilateral choanal atresia. Unilateral atresia may not be detected for years, and patients may present with unilateral rhinorrhea or congestion.

Many patients have an associated narrowed nasopharynx, widened vomer, medialized lateral nasal wall, and/or arched hard palate. Associated malformations occur in 47% of infants without chromosome anomalies. Nonrandom association of malformations can be demonstrated using the CHARGE association, which appears to be overused in clinical practice. The components of the CHARGE association are as follows:

  • Coloboma of the iris, choroid, and/or microphthalmia
  • Heart defect such as atrial septal defect (ASD) and/or conotruncal lesion
  • Atresia of choanae
  • Retarded growth and development
  • Genitourinary abnormalities such as cryptorchidism, microphallus, and/or hydronephrosis
  • Ear defects with associated deafness (The external, middle, and/or inner ear may be involved. Only a small proportion of infants with choanal atresia and related components probably represent this entity.)

The percentages of the different anomalies in CHARGE association are as follows:

  • Coloboma - 80%
  • Heart defect - 58%
  • Atresia of choanae - 100%
  • Mental retardation - 94%
  • Growth deficiency - 87%
  • Genital hypoplasia in males - 75%
  • Ear anomalies - 88%

Differential diagnosis

Deviated nasal septum

Dislocated nasal septum

Septal hematoma

Mucosal swelling

Turbinate hypertrophy

Encephalocele

Nasal dermoid

Hamartoma

Chordoma

Teratoma

More on Choanal Atresia

Overview: Choanal Atresia
Workup: Choanal Atresia
Treatment: Choanal Atresia
Multimedia: Choanal Atresia
References
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References

  1. Barbero P, Valdez R, Rodríguez H, et al. Choanal atresia associated with maternal hyperthyroidism treated with methimazole: a case-control study. Am J Med Genet A. Sep 15 2008;146A(18):2390-5. [Medline].

  2. Assanasen P, Metheetrairut C. Choanal atresia. J Med Assoc Thai. May 2009;92(5):699-706. [Medline].

  3. Prasad M, Ward RF, April MM, Bent JP, Froehlich P. Topical mitomycin as an adjunct to choanal atresia repair. Arch Otolaryngol Head Neck Surg. Apr 2002;128(4):398-400. [Medline].

  4. Cedin AC, Fujita R, Cruz OL. Endoscopic transeptal surgery for choanal atresia with a stentless folded-over-flap technique. Otolaryngol Head Neck Surg. Nov 2006;135(5):693-8. [Medline].

  5. Al-Ammar AY. Presentation of choanal atresia in Saudi children. Saudi Med J. Nov 2006;27(11):1680-2. [Medline].

  6. Brown OE, Burns DK, Smith TH, Rutledge JC. Bilateral posterior choanal atresia: a morphologic and histologic study, and computed tomographic correlation. Int J Pediatr Otorhinolaryngol. Aug 1987;13(2):125-42. [Medline].

  7. Brown OE, Pownell P, Manning SC. Choanal atresia: a new anatomic classification and clinical management applications. Laryngoscope. Jan 1996;106(1 Pt 1):97-101. [Medline].

  8. Cozzi F, Steiner M, Rosati D, Madonna L, Colarossi G. Clinical manifestations of choanal atresia in infancy. J Pediatr Surg. Mar 1988;23(3):203-6. [Medline].

  9. Crockett DM, Scamman FL, McCabe BF, Lusk RP, Gray SD. Venturi jet ventilation for microlaryngoscopy: technique, complications, pitfalls. Laryngoscope. Nov 1987;97(11):1326-30. [Medline].

  10. Deutsch E, Kaufman M, Eilon A. Transnasal endoscopic management of choanal atresia. Int J Pediatr Otorhinolaryngol. May 4 1997;40(1):19-26. [Medline].

  11. Effat KG. Use of the automatic tympanometer as a screening tool for congenital choanal atresia. .J Laryngol Otol. 2005;119:125-8.

  12. Friedman NR, Mitchell RB, Bailey CM, Albert DM, Leighton SE. Management and outcome of choanal atresia correction. Int J Pediatr Otorhinolaryngol. Jan 30 2000;52(1):45-51. [Medline].

  13. Hackman TG, Ferguson BJ. Powered instrumentation and tissue effects in the nose and paranasal sinuses. Curr Opin Otolaryngol Head Neck Surg. Feb 2005;13(1):22-6. [Medline].

  14. Josephson GD, Vickery CL, Giles WC, Gross CW. Transnasal endoscopic repair of congenital choanal atresia: long-term results. Arch Otolaryngol Head Neck Surg. May 1998;124(5):537-40. [Medline].

  15. Lazar RH, Younis RT. Transnasal repair of choanal atresia using telescopes. Arch Otolaryngol Head Neck Surg. May 1995;121(5):517-20. [Medline].

  16. Panwar SS, Martin FW. Trans-nasal endoscopic holmium: YAG laser correction of choanal atresia. J Laryngol Otol. May 1996;110(5):429-31. [Medline].

  17. Pasquini E, Sciarretta V, Saggese D, Cantaroni C, Macrì G, Farneti G. Endoscopic treatment of congenital choanal atresia. Int J Pediatr Otorhinolaryngol. Mar 2003;67(3):271-6. [Medline].

  18. Schoem SR. Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg. Oct 2004;131(4):362-6. [Medline].

  19. Schoem SR. '. Otolaryngol Head Neck Surg. 2004.

  20. Schoem SR. Transnasal endoscopic repair of choanal atresia: why stent?. Otolaryngol Head Neck Surg. 2004.

  21. Vickery CL, Gross CW. Advanced drill technology in treatment of congenital choanal atresia. Otolaryngol Clin North Am. Jun 1997;30(3):457-65. [Medline].

  22. Wiatrak BJ. Unilateral choanal atresia: initial presentation and endoscopic repair. Int J Pediatr Otorhinolaryngol. Nov 15 1998;46(1-2):27-35. [Medline].

  23. Yaniv E, Hadar T, Shvero J, Stern Y, Raveh E. Endoscopic transnasal repair of choanal atresia. Int J Pediatr Otorhinolaryngol. Mar 2007;71(3):457-62. [Medline].

  24. Zeitouni AG, Shapiro RS. Congenital anomalies of the nose and anterior skull base. In: Tewfik TL, Der Kaloustian VM, eds. Congenital Anomalies of the Ear, Nose, and Throat. New York, NY: Oxford University Press; 1997:189-200.

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Further Reading

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Keywords

choanal atresia, complete nasal obstruction in a newborn, congenital choanal atresia, choanae, unilateral atresia, bilateral choanal atresia, complete nasal obstruction, definitive choanae, respiratory distress, atresia

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Contributor Information and Disclosures

Author

Ted L Tewfik, MD, FRCS(C), Professor, Department of Otolaryngology, Director of Continuing Medical Education of Otolaryngology, McGill University Medical School; Director, Director of Professional Affairs of Otolaryngology, Department of Otolaryngology, Montreal Children's Hospital; Senior Staff, Montreal General Hospital and Royal Victoria Hospital
Ted L Tewfik, MD, FRCS(C) is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Society of Pediatric Otolaryngology, Canadian Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Quebec Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Yaser ALI Alrajhi, MD, FRCSC, Fellow, Department of Otolaryngology, Division of Pediatric Otolaryngology, McGill University Faculty of Medicine
Disclosure: Nothing to disclose.

Abdulrahman A Hagr, MBBS, Staff Physician, Department of Otolaryngology, McGill University, Canada
Abdulrahman A Hagr, MBBS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery and Canadian Society of Otolaryngology-Head & Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

Russell A Faust, MD, PhD, Consulting Staff, Department of Otolaryngology, Columbus Children's Hospital
Russell A Faust, MD, PhD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Legal Medicine, American Laryngological Rhinological and Otological Society, American Rhinologic Society, American Society for Head and Neck Surgery, and American Society of Law Medicine and Ethics
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gregory C Allen, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Gregory C Allen, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Academy of Pediatrics, American Cleft Palate/Craniofacial Association, American College of Surgeons, American Laryngological Rhinological and Otological Society, American Medical Association, Christian Medical & Dental Society, and Colorado Medical Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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