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.