Choanal Atresia

Alyssa Brzenski

Case• 33 year old mother has delivered a term

baby boy by C-Section. The baby was intubated in the delivery room for respiratory distress. The nurse attempted to place a NGT however it was unable to pass. ENT was consulted and have a presumptive diagnosis of choanal atresia. You are scheduled to do the anesthesia for the repair. When you arrive at the NICU the mother has a tracheostomy in place as does 2 older brothers.

Choanal Atresia

Congenital narrowing at the posterior choanae

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The Basics• 1:5000-1:7000 live births• 2:1 female predominance• More commonly unilateral

CHARGE Syndrome• Coloboma• Heart disease (TOF, PDA, DORV,

VSD, ASD, Right Aortic Arch)• Atresia choanae• Retarded growth (CNS anomalies)• Genital anomalies (hypogonadism)• Ear anomalies

CHARGE criteria

Embryology• Persistence of buccopharyngeal

membrane from the foregut• Abnormal persistence of mesoderm in

the nasochoanal region• Abnormal persistence of nasobuccal

membrane of Hochstetter• Misdirection of neural crest cell migration

with subsequent mesodermal migration

Associations• Retinoic Acid• Thionamides (methimazole or

carbimizole)• Genetic Syndromes-

• CHARGE• Crouzon• Pfeiffer• Treacher Collins

Presentation• Bilateral atresia-

• Cyanosis at birth that resolves with crying• Oral airway, McGovern Nipple or intubation

• Unilateral atresia• Presents later (5-24 months) with unilateral

nasal discharge

• Associated syndromes-• Other facial and airway issues• Thick pterygoid bone• May delay repair

Work up• Attempt to pass a catheter through the

nare• ENT exam• CT of sinuses and skull base after

decongestion and suctioning

Timing of Surgical Repair

• Most bilateral can be done within the first few days of life to aid in growth and development

• CHARGE syndrome may require a delay• Tracheostomy typically needed

• Unilateral atresia • Before school age

Surgical Repair

Surgical Repair- Transpalatal

Surgical Repair- Endoscopic

• More commonly used• Size of child is a limitation• Small restenosis rate (12%)

Stents

Lasers• CO2 laser ineffective for treating

choanal atresia• KTP laser can be used for membranous

lesions

Anesthesia Concerns• May be called to intubate at birth• Oral airway or a nipple can prevent

obstruction in the newborn• Oral airway helpful on induction to maintain

airway• If associated with a syndrome may have a

difficult airway• Extubation depending on clinical situation

Sources• Hengerer et al. Choanal Atresia: Embryologic

Analysis and Evolution of Treatment. Laryngoscope. 118: May 2008; 862-6.

• Corrales C, Koltai P. Choanal Atresia: Current Concepts and Controversies. Current Opinions in Otolaryngology. 17: 2009; 466-70.

• Ramsden J, Campisis P. Choanal Atresia and Stenosis. Otolaryngology Clin N Am. 42: 2009; 339-52.