choanal atresia alyssa brzenski. case 33 year old mother has delivered a term baby boy by c-section....
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Choanal Atresia
Alyssa Brzenski
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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.
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Choanal Atresia
Congenital narrowing at the posterior choanae
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http://www.edoctoronline.com
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The Basics
• 1:5000-1:7000 live births
• 2:1 female predominance
• More commonly unilateral
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CHARGE Syndrome
• Coloboma
• Heart disease (TOF, PDA, DORV, VSD, ASD, Right Aortic Arch)
• Atresia choanae
• Retarded growth (CNS anomalies)
• Genital anomalies (hypogonadism)
• Ear anomalies
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CHARGE criteria
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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
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Associations
• Retinoic Acid
• Thionamides (methimazole or carbimizole)
• Genetic Syndromes-• CHARGE• Crouzon• Pfeiffer• Treacher Collins
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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
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Work up
• Attempt to pass a catheter through the nare
• ENT exam
• CT of sinuses and skull base after decongestion and suctioning
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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
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Surgical Repair
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Surgical Repair- Transpalatal
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Surgical Repair- Endoscopic
• More commonly used
• Size of child is a limitation
• Small restenosis rate (12%)
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Stents
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Lasers
• CO2 laser ineffective for treating choanal atresia
• KTP laser can be used for membranous lesions
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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
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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.