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