The treatment of choanal atresia

Choanal atresia occurs in approximately 1 out of every 8000 live births. In about 60% of these cases it is unilateral and is often associated with other major craniofacial anomalies or visceral malformations such as the so-called CHARGE syndrome (i.e. Choloboma, Heart defects, choanal Atresia, Retarded growth and development, Genital anomaly, and Ear defect with deafness) first described by Pagon in 1981. In newborns the clinical manifestations of choanal atresia consist of respiratory distress strictly related to the entity of nasal obstruction. Total bilateral congenial choanal atresia must, therefore, be considered a surgical emergency since nasal obstruction impedes the suction mechanism and hence normal feeding in the newborn. For this reason, prompt surgery is mandatory. The surgical approach employed in choanal atresia consists of both the trans-nasal and trans-palatal approaches. Between 1985 and 1997 31 patients with choanal atresia were treated in our department (16 males, 15 females; age range 2 days-5.5 years; mean 11.4 months). In 25 cases (80.6%) the malformation was bilateral. Associated anomalies were seen in 8 patients (25.8% of cases). All of these patients were treated using the trans-nasal approach, under general anesthesia, and endoscopic control. In 27 cases a trocar was employed to perforate the bony atresia and a stent was then positioned. Three patients underwent contact-laser resection of the stenosis without requiring any further stent and without any complications. None of these patients developed total recurrences, although after varying amounts of time, in 8 of the 27 patients operated using the trocar (29.6% of cases) a partial reduction of the airway occurred, the substenosis requiring dilatation with progressive Hegar dilators. In the remaining cases contact-laser therapy was associated with dilation. Three of the patients were treated by laser-therapy alone and none developed a recurrence. One patient dropped out of the follow-up for other serious malformations.