Choanal Atresia

CHOANAL ATRESIALaura 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. These spaces connect with one another. and symptoms will often temporarily resolve. a suitable airway is attainable in the vast majority of patients. It is possible that the opening may begin to close. allowing air to move through to the lungs. with or without a stent. endoscopic or open. 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. a temporary stent may or may not be placed to keep the newly created choana open. but most often an endoscopic approach is used (using a small telescope and instruments through the nose).SIGNS AND SYMPTOMS OF BILATERAL CHOANAL ATRESIA If the atresia is present on both sides. With an endoscopic approach there are no incisions on the face or the palate (the roof of the mouth). it is much more likely that the newborn infant will have symptoms with significant difficulty breathing and feeding. After the atresia is opened. the area behind the oral cavity. The procedure is done through the nostrils. In an emergency. SURGERY TO REPAIR OR OPEN THE CHOANA The choanal atresia can be repaired or opened with a surgical procedure. Once an airway is established and the patient is stable. .) 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. However. However. (It is above the oropharynx. turn blue while sleeping or feeding. INITIAL TREATMENT OF BILATERAL CHOANAL ATRESIA Bilateral choanal atresia can be a life-threatening situation. There are multiple techniques that can be used during surgery. Patients may have noisy breathing. Only in a small portion of cases is the narrowing composed of only soft tissue and no 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. Feeding slowly can allow the baby to breathe between swallows. Similarly. In either case. 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. an oral airway or short tube along the top of the tongue can be placed so that the airway stays open. 70 to 90% of choanal atresia involves bone and the soft tissue covering the bone. the palate is then closed. More than one surgery is often necessary to try to keep the nasal airway 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). When the patient awakens. 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). and aspirate milk. coughs or cries the mouth will open again. 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. The stenosis in this patient is present about one or two years after a single attempt at repair in infancy. In a complete obstruction. The timing of removal of the tracheotomy will vary by patient. 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. 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.Figure 2: This is a similar view as in Figure 1. there would be no hole. However. This is especially true for those with bilateral (both . There may be mild to moderate pain for a few days. as there is above. Saline spray is used at home to help rinse the nose and keep it clean. Once the atresia is repaired and well-healed. Figure 3: The choana is shown immediately after opening during endoscopic surgery. The nose heals quickly. there are syndromes associated with choanal atresia for which every patient should be evaluated. This endoscopic view shows a stenosis or narrowing of the choana. Some newborns show respiratory difficulty while feeding only. especially when feeding. meaning that the symptoms or features may be more or less noticeable. so obstruction of nasal passages can cause significant respiratory distress. neither of the patient’s parents would have the mutation or be affected.qbneVMve.org/choanal_atresia#sthash. Crying bypasses the obstruction because crying infants breathe though their mouths.2 has also been associated with choanal atresia. Patients with this may have other findings such as coloboma (a slit in the eyelid or iris). Inability to easily pass a small catheter through the nostrils should raise the suspicion of choanal atresia. choanal atresia. The diagnosis is confirmed by CT scan and by inspecting the area directly with a flexible nasopharyngoscope. Genetic mutations can also be sporadic (not inherited. Microdeletion of 5q11. The remaining 25% of patients may have a mutation in another gene or no genetic mutation. Unilateral choanal atresia may go undiagnosed until later in life and presents with symptoms of unilateral nasal obstruction and discharge. retardation.dpuf Choanal Stenosis (Atresia) Choanal stenosis/atresia is a congenital problem presenting in the neonatal period. but the definitive treatment is surgery. 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. . Nearly half of infants with choanal atresia have other congenital anomalies as a part of the CHARGE association (coloboma. It may be bilateral or unilateral and is relatively rare. Bilateral choanal atresia classically presents in neonates as cyclic cyanosis because neonates are preferential nose breathers. Choanal atresia is the most common congenital anomaly of the nose and consists of a bony or membranous septum between the nose and pharynx. ear anomalies). although the parent can be more or less affected. as other congenital anomalies are more common in those affected bilaterally. Mutations in the CHD7 gene should be tested and are identified in 75% of patients with CHARGE.See more at: http://care. Revised 01/20/2015 ©American Rhinologic Society . either unilateral or bilateral. In this case. An oral airway may be useful in the short term.sides) choanal atresia. About 60% of patients with CHARGE syndrome have bilateral choanal atresia. This particular syndrome is genetic. and ear problems (malformation and/or hearing loss).american-rhinologic. retardation of growth or development. One of the patient’s parents is usually affected. congenital heart disease. inherited in a dominant pattern. but spontaneous) and they can be mosaic (affecting some of the patient’s cells and not others). genital or urinary problems. heart problems. The most common syndrome associated with choanal atresia is CHARGE syndrome. genitourinary defects. Neonates are generally obligate nose breathers.