Prevalence: 1/8,000 to 1/12,000 births, equal in all races, with a 2:1 female predominance. Bilateral forms (50% of cases) are often associated with other malformations, particularly the syndromic CHARGE association (see this term). Environmental and toxic factors (antithyroid drugs) could play a role and genetic abnormalities are found in 6% of cases. Choanal atresia is the result of hypoplasia of the palatine and nasal regions due to a lack of regression of the bucconasal membrane zt the 38th day of embryonic development.

The diagnosis is most frequently made at birth when impossibility to introduce a nasopharyngeal probe into both nostrils is noted. It is confirmed by nasal endoscopy and opacification and/or lateral CT scan of the face.

The clinical manifestations are variable, ranging from lack of symptoms or unilateral purulent rhinorrhea (unilateral atresia) to acute respiratory distress with cyanosis during neonatal period (bilateral atresia) disappearing when the baby is crying. This respiratory distress is relieved with an  oropharyngeal cannula and a positioning of the child allowing mouth breathing (which is not spontaneous in the newborn). In some cases of severe associated malformations, it may be necessary to intubate the child or to perform a tracheostomy. If the general condition of the child permits it, the surgical cure of atresia must be quickly performed in bilateral forms.

Corrective surgery is most often performed via the endonasal route. It consists in drilling the often osseous membrane, with a small endoscope and a microspacer. A rigid tube (stent) is then inserted to keep the nasal cavity open, to avoid the formation of synechiae and to ease nasal breathing. Some teams drill the membrane using a KTP laser. In some centers, no rigid stent is put in place but part of an endotracheal tube cut to fit with the length of the nasal passages is used instead. Some teams even prefer not to use stenting transchoanal tubes.

Anesthetic implications: 

risk of airway obstruction during induction of anesthesia. This risk can be reduced by the use of an oropharyngeal cannula after prior topical anesthesia of the tongue, if necessary. Oral intubation. Some surgeons use gauze soaked with adrenaline (lignocaine solution 0.5 or 1 % mixed with adrenaline 1/200,000) or with cocaine (Bonain solution [equal amounts of cocaine hydrochloride, menthol and phenol) to minimize local bleeding.

Postoperative management depends on the quality of hemostasis, the permeability of the nostrils and the lack of apnea. In the majority of cases, children are extubed and transferred to NICU with prophylactic corticosteroid therapy (dexamethasone 0.2 mg/kg) to minimize the local inflammatory reaction and edema as much as possible.

References : 

-        Harris J, Robert E, Kallen B.
Epidemiology of choanal atresia with special reference to the CHARGE association.
Pediatrics 1997; 99:363-7.

-        Eladl HM.
Transnasal endoscopic repair of bilateral congenital choanal atresia: controversies.
J Laryngol Otol 2010; 124:387-92.

-        Pototschnig C, Appenroth, Völklein C, Thumfart WF.
Transnasal treatment of congenital choanal atresia with the KTP laser.
Ann Otol Rhinol Laryngol 2001; 110:335-9.

-        Ibrahim AA, Magdy EA, Hassab MH.
Endoscopic choanoplasty without stenting for congenital choanal atresia repair.
Int J Pediatr Otorhinolaryngol 2010; 74:144-50.

-        Brihaye P, Delpierre I, De Villé A, Johansson A-B, Biarent D, Mansbach A-L.
Comprehensive management of congenital choanal atresia.
Int J Pediatr Otorhinolaryngol 2017; 2017: 9-18.

Updated: August 2017