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

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; Corrales C, Koltai P. Choanal Atresia: Current Concepts and Controversies. Current Opinions in Otolaryngology. 17: 2009; Ramsden J, Campisis P. Choanal Atresia and Stenosis. Otolaryngology Clin N Am. 42: 2009;