eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Pediatric Otolaryngology

Choanal Atresia

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
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

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 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

Workup

Imaging Studies

Rhinography is a procedure that involves the administration of radiopaque dye into the nasal cavity as illustrated in Image 1.

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

CT scanning is the radiographic procedure of choice in the evaluation of choanal atresia. For good results, careful suctioning is performed to clear excess mucus, and a topical decongestant is applied (see Image 2). The purpose of CT scanning is outlined as follows:
- Confirm the diagnosis of choanal atresia (unilateral or bilateral).
- Evaluate choanal atresia (vomer bone width and choanal airspace distance).
- Exclude other possible nasal sites of obstruction.
- Determine the degree of bony, membranous, or mixed atresia.
- Delineate abnormalities in the nasal cavity and nasopharynx.

Choanal atresia. CT scan showing membranous and bony choanal atresia. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

Diagnostic Procedures

Failure to pass an 8F catheter through the nasal cavity more than 5.5 cm from the alar rim
The lack of movement of a thin wisp of cotton under the nostrils while the mouth is closed
The absence of fog on a mirror when it is placed under the nostrils
Acoustic rhinometry
Listening for breath sounds with either a stethoscope or a Toynbee auscultation tube
Gently blowing air into each nasal cavity with a Politzer bag
Administering into the nose a colored solution that is visible in the pharynx

Treatment

Surgical Therapy

Treatment can be divided into emergent and elective definitive categories. Bilateral choanal atresia in a neonate is an emergency that is best initially treated by inserting an oral airway to break the seal formed by the tongue against the palate. This oral airway can be well tolerated for several weeks. The method of repair is controversial, with no technique having gained universal acceptance. Bilateral choanal atresia in the newborn requires prompt diagnosis and airway stabilization. An oral airway, McGovern nipple, and intubation are viable options. The ideal procedure for choanal atresia restores the normal nasal passage, prevents damage to growing structures important in facial development, is technically safe, requires short operative time, and provides short hospitalization and convalescence.

Procedures

Transnasal puncture, with or without a microscope, became unpopular because of the high rate of failure that then required revision. This was attributed to the difficulty in visualizing the choanal area that required special surgical attention, such as the vomerine septal bridge and bony narrowing of the lateral walls. The transnasal approach becomes more difficult in the presence of septal deviation, turbinate hypertrophy, nasal discharge, and elongation of the depth from the nasal vestibule to the posterior choanae as patients grow.

The transseptal technique consists of making a window in the septum anterior to the atretic plate.

Transpalatal repair is a technique that provides excellent exposure and has a high success rate but requires more operative time. The increased blood loss, possible occurrence of palatal fistula, palatal dysfunction, and maxillofacial growth disturbance are complications of this procedure (see Image 3).

Choanal atresia. Diagram illustrating the transpalatal correction of choanal atresia.

The endoscopic technique (nasal or retropalatal), with or without powered instrumentation, offers excellent visualization with great ease in removing the bony choanae.² Microdebriders will continue to advance the field of endoscopic surgery, providing clearer operative fields and causing less tissue trauma for experienced surgeons. However, the severity of complications, including the potential for rapidly aspirating orbital and cerebral contents when laminae are violated, must be appreciated and respected.

Carbon dioxide and potassium titanyl phosphate (KTP) lasers are easy and quick and create minimal discomfort to the patient. The time of hospitalization is short, and the operation can be repeated if a good result is not initially achieved. Most importantly, a stent is not usually needed. The use of mitomycin C topically as an adjunct to the surgical repair of choanal atresia may offer improved patency with a decreased need for stenting, dilatations, and revision surgery.³

Postoperative Details

Infants with documented gastroesophageal reflux disease (GERD) require prolonged stenting and dilatations for choanal restenosis and removal of granulation tissue. Stenting is usually performed using an endotracheal tube or Foley catheter. The advantages of Foley catheter stenting for choanal atresia are as follows:

Well tolerated by the patient
Simple to introduce, fix, and remove
Minimizes septal or columellar necrosis
Minimizes nasal cavity and paranasal sinus infections
Adjustable with inflation or deflation of the balloon that controls the pressure on the choanal walls
Easy to fix in cases of unilateral atresia

The use of stents in the treatment of patients with choanal atresia is a controversial subject. Some surgeons believe that stents are useful in stabilizing the nasal airway in the postoperative period to prevent the development of stenosis by maintaining a lumen. However, others believe that stents may act as a nidus for infection and may induce a foreign body reaction. This may contribute to choanal restenosis, much as an endotracheal tube may cause subglottic stenosis. Therefore, the use of stents following repair of choanal atresia requires the use of prophylactic antibiotic and antireflux medications. Cedin et al (2006) analyzed the long-term results of a new stentless surgical technique for choanal atresia.⁴ They reported that, using neither stents or nasal packing, this technique allowed fast recovery in a one-step surgery.

Follow-up

Following surgical repair of choanal atresia, patients may require operative debridement or periodic dilatations. Periodic dilations can sometimes be performed as an outpatient procedure with local decongestant and topical anesthesia using urethral sounds.

Multimedia

Media file 1: Choanal atresia. Rhinogram demonstrating blockage of radiopaque dye at the posterior choanae. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

Media file 2: Choanal atresia. CT scan showing membranous and bony choanal atresia. From T.L. Tewfik and V.M. Der Kaloustian, with permission.

Media file 3: Choanal atresia. Diagram illustrating the transpalatal correction of choanal atresia.

References

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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

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

Further Reading