Choanal atresia seen during exam.
Choanal atresia is a congenital narrowing of the back of the nasal cavity that causes difficulty breathing. It is rare, occurring in approximately 1 in 7,000 live births, and is seen more often in females than in males. Choanal atresia can be bilateral, meaning it blocks both nasal passages, or unilateral, meaning only one nasal passage is blocked. The blockage can be either completely bony (30 percent of cases) or be composed of both bone and membranes (70 percent of cases).
Choanal atresia is often seen in association with other developmental anomalies such as CHARGE, Treacher Collins syndrome, Tessier syndrome and others.
Choanal atresia is a developmental abnormality. The anomaly is present at birth and can be associated with other developmental abnormalities. There is no known specific cause of choanal atresia. Most believe that choanal atresia occurs when the tissue that separates the nose and mouth area during fetal development remains after birth.
The symptoms of choanal atresia include:
CT scan demonstrating bilateral choanal atresia.
Further evaluation is done with endoscopy and CT scans.
Exam shows choanal atresia. Patients with bilateral choanal atresia often have respiratory distress episodes immediately after birth. These are relieved only when the patient begins to cry. The child can not breathe during normal circumstances and can only breath when they open their airway to cry. Because of this, infants with bilateral choanal atresia often require intubation immediately after delivery. Once the airway is secured, the patient can be evaluated for other developmental abnormalities.
Patients with unilateral choanal atresia often show symptoms later during infancy and childhood with signs of unilateral nasal obstruction (discharge, mouth breathing, inability to move air on one side). Since one side is open at birth, it is rare that these children have difficulty breathing when they are first born.
Patient after endoscopic repair of choanal atresia. Treatment depends on the severity of the anomaly (unilateral or bilateral), how much it affects the child's breathing and eating, and what other medical conditions the patient has.
There are cases of unilateral choanal atresia where the child is not having respiratory distress or significant feeding issues. These patients may initially be managed with close observation and occasionally supplemental oxygen. Nasal saline can help to keep the nasal linings healthy and free of discharge. Patients with unilateral choanal atresia can be allowed to grow, delaying repair until the child is older.
Severe cases include all patients with bilateral choanal atresia. Surgery should be performed as soon as the patient is stable and has been evaluated for other anomalies. The major goal of the surgical repair is to open the nasal airway sufficiently, allowing the infant to be extubated. There are several approaches available to accomplish this goal, all of which are performed in the operating room under general anesthesia.
The transnasal endoscopic approach is currently CHOP's procedure of choice for managing choanal atresia. Modern endoscopic equipment allows for the safe performance of this procedure in children as young as a few days old. The posterior nasal airway is opened using a combination of dilators, sinus instruments, ear curettes and drills. In some cases, a piece of endotracheal tube is placed inside the nasal passage as a stent to maintain the opening while the area heals. The stent will be removed a few weeks after the surgery at the bedside or during an office visit. Sometimes stenting is not required.
In rare circumstances where the skull base is abnormally developed (other craniofacial anomalies are present), the surgeon may need to use a transpalatal approach to access the area of choanal atresia through the roof of the mouth.
Infants with choanal atresia need to be evaluated for both airway and feeding issues. Management of the airway often requires a combination of supportive, medical and surgical care. Feeding and swallowing issues are very common in children with these conditions and often need to be addressed by speech pathologists and GI specialists. Children born with syndromes often need other subspecialty evaluations (cardiology, ophthalmology, etc.) and benefit greatly from the coordinated care provided in the multidisciplinary setting of the Center for Pediatric Airway Disorders.