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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.

an oral airway or short tube along the top of the tongue can be placed so that the airway stays open. . coughs or cries the mouth will open again. endoscopic or open. 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). In either case. but most often an endoscopic approach is used (using a small telescope and instruments through the nose). These spaces connect with one another. a suitable airway is attainable in the vast majority of patients. This area is called the nasopharynx. a small flexible endoscope may also be passed into the nose at the patient’s bedside to examine the inside of the nose and confirm the anatomy. the area behind the oral cavity. It is possible that the opening may begin to close. 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 patient’s symptoms. The procedure is done through the nostrils. With an endoscopic approach there are no incisions on the face or the palate (the roof of the mouth). Once an airway is established and the patient is stable. with or without a stent.SIGNS AND SYMPTOMS OF BILATERAL CHOANAL ATRESIA If the atresia is present on both sides. and aspirate milk. INITIAL TREATMENT OF BILATERAL CHOANAL ATRESIA Bilateral choanal atresia can be a life-threatening situation. the palate is then closed. Only in a small portion of cases is the narrowing composed of only soft tissue and no bone. More than one surgery is often necessary to try to keep the nasal airway open. 70 to 90% of choanal atresia involves bone and the soft tissue covering the bone. 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. There are multiple techniques that can be used during surgery. SURGERY TO REPAIR OR OPEN THE CHOANA The choanal atresia can be repaired or opened with a surgical procedure. (It is above the oropharynx. and symptoms will often temporarily resolve. a temporary stent may or may not be placed to keep the newly created choana open.) 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. Feeding slowly can allow the baby to breathe between swallows. it is much more likely that the newborn infant will have symptoms with significant difficulty breathing and feeding. After the atresia is opened. allowing air to move through to the lungs. However. Patients may have noisy breathing. turn blue while sleeping or feeding. When the patient awakens. However. Similarly. In an emergency. a feeding tube is often placed from the oral cavity into the stomach to make sure that the patient safely receives adequate feeds. (See Figures 2 and 3) A medication called mitomycin may be used during surgery to reduce postoperative scarring. 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).

Once the atresia is repaired and well-healed.Figure 2: This is a similar view as in Figure 1. The timing of removal of the tracheotomy will vary by patient. as there is above. This endoscopic view shows a stenosis or narrowing of the choana. This is especially true for those with bilateral (both . Figure 3: The choana is shown immediately after opening during endoscopic surgery. Saline spray is used at home to help rinse the nose and keep it clean. there are syndromes associated with choanal atresia for which every patient should be evaluated. 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. The stenosis in this patient is present about one or two years after a single attempt at repair in infancy. The nose heals quickly. 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. There may be mild to moderate pain for a few days. there would be no hole. In a complete obstruction. However.

dpuf Choanal Stenosis (Atresia) Choanal stenosis/atresia is a congenital problem presenting in the neonatal period.qbneVMve. Crying bypasses the obstruction because crying infants breathe though their mouths. It may be bilateral or unilateral and is relatively rare. Inability to easily pass a small catheter through the nostrils should raise the suspicion of choanal atresia. An oral airway may be useful in the short term. retardation of growth or development. neither of the patient’s parents would have the mutation or be affected. Patients with this may have other findings such as coloboma (a slit in the eyelid or iris). and ear problems (malformation and/or hearing loss). genital or urinary problems. One of the patient’s parents is usually affected.See more at: http://care. inherited in a dominant pattern. as other congenital anomalies are more common in those affected bilaterally. . Bilateral choanal atresia classically presents in neonates as cyclic cyanosis because neonates are preferential nose breathers. Microdeletion of 5q11. retardation. but the definitive treatment is surgery. ear anomalies).2 has also been associated with choanal atresia. 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. The diagnosis is confirmed by CT scan and by inspecting the area directly with a flexible nasopharyngoscope. especially when feeding. so obstruction of nasal passages can cause significant respiratory distress. Genetic mutations can also be sporadic (not inherited. meaning that the symptoms or features may be more or less noticeable. genitourinary defects. 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. choanal atresia. In this case. This particular syndrome is genetic. About 60% of patients with CHARGE syndrome have bilateral choanal atresia.american-rhinologic. Mutations in the CHD7 gene should be tested and are identified in 75% of patients with CHARGE. although the parent can be more or less affected. Neonates are generally obligate nose breathers. congenital heart disease. Revised 01/20/2015 ©American Rhinologic Society . Choanal atresia is the most common congenital anomaly of the nose and consists of a bony or membranous septum between the nose and pharynx. but spontaneous) and they can be mosaic (affecting some of the patient’s cells and not others).sides) choanal atresia. heart problems.org/choanal_atresia#sthash. either unilateral or bilateral. The remaining 25% of patients may have a mutation in another gene or no genetic mutation. The most common syndrome associated with choanal atresia is CHARGE syndrome. Unilateral choanal atresia may go undiagnosed until later in life and presents with symptoms of unilateral nasal obstruction and discharge.