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CHOANAL ATRESIA

Laura H. Swibel Rosenthal, MD

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).

SIGNS AND SYMPTOMS OF UNILATERAL CHOANAL ATRESIA


When unilateral, the right side is affected more often than the left. If the atresia (narrowing or
complete blockage) is only on one side, sometimes it is identified at birth but it can go unnoticed
for a few years. Most commonly, children will have chronic thick drainage from one side of the
nose.

SIGNS AND SYMPTOMS OF BILATERAL CHOANAL ATRESIA


If the atresia is present on both sides, it is much more likely that the newborn infant will have
symptoms with significant difficulty breathing and feeding. Patients may have noisy breathing,
turn blue while sleeping or feeding, and aspirate milk. When the patient awakens, coughs or cries
the mouth will open again, allowing air to move through to the lungs, and symptoms will often
temporarily resolve. Feeding slowly can allow the baby to breathe between swallows. However, a
feeding tube is often placed from the oral cavity into the stomach to make sure that the patient
safely receives adequate feeds.

INITIAL TREATMENT OF BILATERAL CHOANAL ATRESIA


Bilateral choanal atresia can be a life-threatening situation. In an emergency, an oral airway or
short tube along the top of the tongue can be placed so that the airway stays open. Patients will
often require an intubation (a breathing tube from the mouth to the airway below) or
tracheostomy (a tube bringing air directly from an opening made in the neck down to the airway
below). One of the first signs in the delivery room or neonatal intensive care unit (NICU) that a
patient may have a bilateral choanal atresia is the inability to pass a small catheter (tube) down
either side of the nose into the stomach. Similarly, a small flexible endoscope may also be
passed into the nose at the patients bedside to examine the inside of the nose and confirm the
anatomy. Once an airway is established and the patient is stable, a CT scan (computed
tomography) and possibly an MRI (magnetic resonance imaging) may be obtained to confirm the
choanal atresia or identify other reasons for the patients symptoms.

SURGERY TO REPAIR OR OPEN THE CHOANA


The choanal atresia can be repaired or opened with a surgical procedure. 70 to 90% of choanal
atresia involves bone and the soft tissue covering the bone. Only in a small portion of cases is
the narrowing composed of only soft tissue and no bone. There are multiple techniques that can
be used during surgery, but most often an endoscopic approach is used (using a small telescope
and instruments through the nose). With an endoscopic approach there are no incisions on the
face or the palate (the roof of the mouth). The procedure is done through the nostrils. The bone
and soft tissue that is blocking the opening is taken down until it is open to the area where it
connects with the pharynx (the throat behind). This area is called the nasopharynx. (It is above
the oropharynx, the area behind the oral cavity. These spaces connect with one another.) An
open approach can be used in which an incision is made into the palate from the oral cavity to
access the narrowing in the nose. After the atresia is opened, the palate is then closed. In either
case, endoscopic or open, a temporary stent may or may not be placed to keep the newly
created choana open. It is possible that the opening may begin to close, with or without a stent.
More than one surgery is often necessary to try to keep the nasal airway open. (See Figures 2
and 3) A medication called mitomycin may be used during surgery to reduce postoperative
scarring. However, a suitable airway is attainable in the vast majority of patients.

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.

CHOANAL ATRESIA ASSOCIATED WITH OTHER CONGENITAL ANOMALIES


Choanal atresia usually occurs in the absence of any other anomalies (problems with other parts
of the body) or syndromes. However, there are syndromes associated with choanal atresia for
which every patient should be evaluated. This is especially true for those with bilateral (both

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

- See more at: http://care.american-rhinologic.org/choanal_atresia#sthash.qbneVMve.dpuf

Choanal Stenosis (Atresia)


Choanal stenosis/atresia is a congenital problem presenting in
the neonatal period. It may be bilateral or unilateral and is relatively
rare. Neonates are generally obligate nose breathers, so
obstruction of nasal passages can cause significant respiratory
distress, especially when feeding. Crying bypasses the obstruction
because crying infants breathe though their mouths. Inability
to easily pass a small catheter through the nostrils should
raise the suspicion of choanal atresia. The diagnosis is confirmed
by CT scan and by inspecting the area directly with a
flexible nasopharyngoscope. An oral airway may be useful in
the short term, but the definitive treatment is surgery.

Choanal atresia is the most common congenital anomaly


of the nose and consists of a bony or membranous septum
between the nose and pharynx, either unilateral or bilateral.
Bilateral choanal atresia classically presents in neonates as
cyclic cyanosis because neonates are preferential nose breathers.
Airway obstruction and cyanosis are relieved when the
mouth is opened to cry and recurs when the calming infant
reattempts to breathe through the nose. Some newborns show
respiratory difficulty while feeding only. Nearly half of infants
with choanal atresia have other congenital anomalies as a part
of the CHARGE association (coloboma, congenital heart disease,
choanal atresia, retardation, genitourinary defects, ear
anomalies). Unilateral choanal atresia may go undiagnosed
until later in life and presents with symptoms of unilateral
nasal obstruction and discharge.