A 3-month-old infant with CHARGE syndrome presents for repair of choanal atresia. He has a systolic murmur.
All patients with choanal atresia need a thorough workup to detect other abnormalities of the CHARGE syndrome:
C: coloboma of the eye
H: heart disease (tetralogy of Fallot)
A: atresia of choanae
R: retarded growth or development
G: genital abnormalities (hypogonadism)
E: ear abnormalities (deafness)
Micrognathia may make endotracheal intubation difficult, and these patients are at increased risk for having laryngomalacia.
Choanal atresia can be membranous or bony, unilateral or bilateral. Bilateral posterior choanal atresia is associated with high mortality, particularly in patients with congenital heart disease and tracheoesophageal fistulas.
A small number of children require a tracheostomy to manage chronic airway obstruction. Severe gastroesophageal reflux and aspiration pneumonias may warrant a Nissen fundoplication. Swallowing and feeding problems may require insertion of a gastrostomy or jejunostomy tube.
Mask ventilation may be difficult because of the choanal atresia. Patients who snore heavily are at higher risk for airway obstruction after the induction of anesthesia.
An oral intubation with an RAE endotracheal tube is usually preferred. The intubation may be difficult because some patients have micrognathia.
Give dexamethasone 0.5 mg/kg, maximum 10 mg to decrease swelling of the airway.
Patients should be extubated awake. Dexmedetomidine might be used for pain relief and sedation without compromising the patient’s airway after the extubation.
Patients who have received stents should have a patent nasal passage immediately postoperatively; close monitoring is required.
Patients with CHARGE syndrome are at increased risk for airway events after general anesthesia and should be watched closely. Neonates should be extubated in a controlled setting, most likely in the neonatal intensive care unit, with continuous positive airway pressure available if they have had significant airway obstruction, increased secretions, or aspiration.
DOs and DON’Ts
✓ Do have a formal cardiac evaluation for patients with choanal atresia.
✓ Do plan an anesthetic that results in a calm, spontaneously breathing with a patent airway after the procedure.
⊗ Do not be surprised by a difficult intubation and difficult mask ventilation.
Using stents for management of choanal atresia may not improve the outcome and can bring on stent-related complications.
Restenosis in choanal atresia is an unsolved problem. No randomized controlled trials exist that show which surgical approach is most effective.
CHARGE syndrome was once thought to be an association, with no known genetic cause. In 1987, the underlying genetic cause was discovered (CHD7 mutation), and CHARGE association was renamed CHARGE syndrome.