Upper respiratory tract congenital malformations Notes

NOTES NOTES UPPER RESPIRATORY TRACT CONGENITAL MALFORMATIONS CHOANAL ATRESIA osms.it/choanal-atresia PATHOLOGY & CAUSES ▪ Congenital narrowing or blockage of the nasal passage (choana) by abnormal bony or soft tissue ▪ Most common nasal abnormality in newborns; more than 50% have other congenital conditions ▪ ⅔ present unilaterally, ⅓ bilaterally ▪ Cause unknown: can be associated with conditions that cause depression of the nasal bridge or midface retraction (craniosynostosis syndromes) MNEMONIC In context of CHARGE association Coloboma Heart defects Atresia of choanae Retardation (physical, mental) Genitourinary abnormalities Ear defects RISK FACTORS ▪ Possible association with: low thyroid hormone levels; smoking; coffee consumption; high maternal zinc and B12 intake; exposure to agricultural chemicals; anti-infective urinary tract medications COMPLICATIONS ▪ Aspiration while feeding ▪ Respiratory arrest 956 OSMOSIS.ORG ▪ Re-narrowing of the area after surgery SIGNS & SYMPTOMS ▪ Variance of presentation depends on unilateral or bilateral defect ▪ Newborns are obligate nasal breathers → difficulty breathing unless crying ▪ Unilateral choanal atresia may not be detected for years → newborn uses healthy nostril to breathe; distress may be intermittent ▪ Bilateral choanal atresia can be lifethreatening; causes acute breathing problems and cyanosis ▪ Marked chest retraction ▪ Inability to nurse and breathe at the same time ▪ Persistent one-sided mucous discharge ▪ Cyanosis DIAGNOSIS DIAGNOSTIC IMAGING CT scan Endoscopy of the nose Sinus radiography OTHER DIAGNOSTICS ▪ Inability to pass a catheter through nasal passage

Chapter 134 Upper Respiratory Tract Congenital Malformations Figure 134.1 A CT scan of the head in the axial plane demonstrating membranous atresia of the right choana. Figure 134.2 A CT scan of the head in the axial plane demonstrating bilateral osseous choanal atresia. TREATMENT ▪ Temporarily: oral airway placement; place infant prone SURGERY ▪ Definitive: surgical correction of the atresia LARYNGOMALACIA osms.it/laryngomalacia laryngeal muscle tone PATHOLOGY & CAUSES ▪ Congenital malformation of the larynx where the aryepiglottic folds are shorter than normal ▪ Short aryepiglottic folds cause folding of epiglottis in a characteristic omega shape that prolapses during inspiration ▪ Arytenoid cartilages are enlarged and softer than normal, so they flop into the airway ▪ Most common cause of congenital stridor and most common congenital lesion of the larynx ▪ Cause is unknown; associated with weak COMPLICATIONS ▪ Impaired growth and development caused by hypoventilation (hypoxemia) ▪ Associated with gastroesophageal reflux ▪ Swallowing dysfunction and choking SIGNS & SYMPTOMS ▪ ▪ ▪ ▪ High-pitched stridor Noisy respirations Breathing difficulties Gastroesophageal reflux OSMOSIS.ORG 957

DIAGNOSIS DIAGNOSTIC IMAGING Laryngoscopy or bronchoscopy ▪ Confirms diagnosis OTHER DIAGNOSTICS ▪ History and physical exam TREATMENT ▪ Can resolve spontaneously as throat muscles strengthen by age two MEDICATIONS ▪ If hypoxemic → supplemental oxygen Figure 134.3 A laryngoscopic view of the larynx in an individual with laryngomalacia in which there is an omega-shaped epiglottis. SURGERY ▪ If laryngomalacia persists, surgical treatment is necessary (tracheotomy or supraglottoplasty) Figure 134.4 Illustration of unique shape of larynx seen in laryngomalacia. 958 OSMOSIS.ORG