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INTRODUCTION
When the back of the nose is not open or not communicating with the rest of the airway this is
called choanal atresia. It is a congenital condition (meaning a person is born with it) that occurs
in about 1 in 6000 to 8000 live births. The exact reason for the anomaly is unknown and probably
multifactorial (from many causes), or a mix of genetic and environmental causes. It has been
reported in association with maternal use of methimazole. When the nasal airway develops, it
starts at the level of the skin of the face, where two indentations or nasal sacs are formed. At the
inferior aspect is the primitive palate. An oronasal membrane develops behind the palate
between the nose and the oral cavity. As the nasal pits become deeper and deeper, they
eventually connect with the oral airway (behind the palate). If this canalization (tunneling) is
incomplete, the result is choanal atresia. Figure 1 shows the endoscopic view (using a small
telescope to look through the nose) of the choana, with the floor of the nose (or palate) below
and the adenoid bed (a region of lymphoid tonsil-like tissue at the very back of the nose) behind.
Figure 2 shows a similar vantage point in a patient with choanal stenosis (or a small choana). It
occurs on only one side about twice as often as on both sides. About 1/3 are bilateral (two-sided)
and 2/3 are unilateral (one-sided).
Figure 1: Endoscopic view of the back of the normal nose. The opening is called the choana. The space behind the nose is the
nasopharynx. Adenoids (A), lumpy pink tissue similar to tonils, can normally be seen in the nasopharynx. In choanal atresia, this area is
completely closed. All that can be seen is tissue similar in appearance to the floor of the nose (F).
Figure 2: This is a similar view as in Figure 1. This endoscopic view shows a stenosis or narrowing of the choana. In a complete
obstruction, there would be no hole, as there is above. The stenosis in this patient is present about one or two years after a single
attempt at repair in infancy.
Figure 3: The choana is shown immediately after opening during endoscopic surgery. The nose heals quickly. There may be mild to
moderate pain for a few days. Saline spray is used at home to help rinse the nose and keep it clean.
Once the atresia is repaired and well-healed, patients who required a tracheotomy can usually
have the tracheotomy removed as long as there are no other problems for which the
tracheotomy would be necessary. The timing of removal of the tracheotomy will vary by patient.
sides) choanal atresia, as other congenital anomalies are more common in those affected
bilaterally. The most common syndrome associated with choanal atresia is CHARGE syndrome.
Patients with this may have other findings such as coloboma (a slit in the eyelid or iris), heart
problems, retardation of growth or development, genital or urinary problems, and ear problems
(malformation and/or hearing loss). About 60% of patients with CHARGE syndrome have
bilateral choanal atresia. This particular syndrome is genetic, inherited in a dominant pattern.
One of the patients parents is usually affected, although the parent can be more or less affected,
meaning that the symptoms or features may be more or less noticeable. Genetic mutations can
also be sporadic (not inherited, but spontaneous) and they can be mosaic (affecting some of the
patients cells and not others). In this case, neither of the patients parents would have the
mutation or be affected. Mutations in the CHD7 gene should be tested and are identified in 75%
of patients with CHARGE. The remaining 25% of patients may have a mutation in another gene
or no genetic mutation. Microdeletion of 5q11.2 has also been associated with choanal atresia.
Revised 01/20/2015
American Rhinologic Society