Laryngocele and Saccular Cyst

• LaryngoceleLaryngoceleo Abnormal dilation of the saccule Abnormal dilation of the saccule

that communicates with the that communicates with the lumen of the larynxlumen of the larynx

o Filled with air =/- mucousFilled with air =/- mucous• Saccular CystSaccular Cyst

o Cystic dilation of the saccule that does not communicate with the laryngeal lumen

o Thought to develop due to nonpatent saccular orifice

o Cyst is distinctly submucosal and are covered with normal mucosa

Saccular Cyst

Subglottic Hemangioma

• Classic scenarioClassic scenarioo ““Croup” symptoms generally begin 6-8 weeks of ageCroup” symptoms generally begin 6-8 weeks of age

• No fever, good cryNo fever, good cryo ““Croup” recurs several weeks laterCroup” recurs several weeks latero Mean age of diagnosis is 4 monthsMean age of diagnosis is 4 monthso Delay due to misdiagnosis of symptomsDelay due to misdiagnosis of symptoms

• Proliferative phase (Birth to 1 year) then involutional phase Proliferative phase (Birth to 1 year) then involutional phase (usually by 3-5 years)(usually by 3-5 years)

• 50% of children with Subglottic hemangioma also have 50% of children with Subglottic hemangioma also have cutaneous hemangioma (esp. facial)cutaneous hemangioma (esp. facial)

• Female:Male= 2:1Female:Male= 2:1

Subglottic Hemangioma

Subglottic Hemangioma- Treatment

• Medical treatment:o Systemic corticosteroidso Intralesional steroidso Interferon alpha-2Ao Propranolol

• Surgical treatment:o Endoscopic excisiono Laryngofissure with complete resection

Subglottic Hemangioma- Propranolol

• Discovered serendipitously by Discovered serendipitously by Le´atue´-Labre`ze in 2008 when propranolol was used to treat steroid-induced hypertrophic cardiomyopathy in a patient with a large facial hemangioma

• Mechanism- Unknowno VasoconstrictionVasoconstrictiono Inhibition of VEGF or other growth factorsInhibition of VEGF or other growth factors

• Side Effects: Side Effects: Bradycardia, Hypotension, Hypoglycemia, and Bronchospasm

• Dose: 2-3 mg/kg divided tidDose: 2-3 mg/kg divided tid

Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al. Propranolol for severe hemangiomas of infancy. N Engl J Med 2008;358:2649e51.

Congenital Disorders of the Trachea

• Tracheal Anomalies:Tracheal Anomalies:o TracheomalaciaTracheomalacia 45%45%• PrimaryPrimary• SecondarySecondaryo Innominate arteryInnominate artery 34%34%o Vascular SlingVascular Sling 9%9%o Tracheal StenosisTracheal Stenosis 6%6%o Tracheal DiverticulaTracheal Diverticula 3%3%o Double aortic archDouble aortic arch 3%3%

Holinger, LD. Etiology of Stridor in the Newborn, Infant, and Child. Annals of ORL, 1980; 89: 397-400.

Tracheomalacia

• Flaciddity of the tracheal/ bronchial cartilage leading to collapse of the airway

• Cartilaginous to membranous ratio may be significantly decreasedo Normal trachea Cartilaginous:

Membranous trachea is 4.5:1o Primary tracheomalacia

Cartilaginous: Membranous tracheal is 3:1 or 2:1

• Incidence of primary tracheomalacia is about 1 in 2100

Tracheomalacia

• Symptoms:o Tracheal wheeze/ Expiratory wheeze or stridoro Harsh, barking cough/ “Brassy” cougho Failure to thriveo Increasing respiratory distress with growtho Transmitted vibration through the backo Difficulty clearing secretionso Symptoms exacerbated with viral infectionso Reflex Apnea- “Dying Spells”

Tracheomalacia- Treatment

• Supportive careo Disease resolves between ages 2-5 years

• Medical treatmento Only to help manage concomittant issues

• CPAP/ BiPAP• Tracheotomy• Airway stenting• Surgical options

o Aortopexyo Tracheostomy

Secondary Tracheomalacia

I. Aberrant innominate arteryII. Vascular Rings

A. Double aortic archB. Right aortic arch with left ligamentum arteriosum

III. Pulmonary artery slingIV. Aberrant right subclavian arteryV. Congenital cardiac defects

Aberrant Innominate Artery

• Leftward origin of the innominate artery (brachiocephalic trunk) is common variant of normal anatomyo Most common vascular anomalyo Present in 0.5% of humans

McLaughlin R, Wetmore R, Tavill M, Gaynor J, Spray T. Vascular Anomalies Causing Symptomatic Tracheobronchial Compression. Laryngoscope. 109:312-319, February 1999.

Double Aortic Arch

• Bifurcation of the ascending aorta that surrounds the trachea and esophagus, then rejoins to form the descending aorta

McLaughlin R, Wetmore R, Tavill M, Gaynor J, Spray T. Vascular Anomalies Causing Symptomatic Tracheobronchial Compression. Laryngoscope. 109:312-319, February 1999.

Subglottic Stenosis

• Subglottic Stenosiso Full term infant:Full term infant:

• Subglottic diameter Subglottic diameter << 4.0 mm 4.0 mmo Premature Infant:Premature Infant:

• Subglottic diameter Subglottic diameter << 3.5 mm 3.5 mm

• Congenital Subglottic Stenosis: (5%)o Defined as SGS in absence of internal or external

laryngeal traumao Third most common congenital laryngeal anomalyo No Croup in Infants Younger Than 1 Year!

• Acquired Subglottic Stenosis: (95%)o Secondary to laryngeal trauma

• External Trauma: Blunt/ Clothesline injury• Internal Injury: Endotracheal tube; Nasogastric tube

o Other factors contribute to progression

Congenital Subglottic Stenosis

• MembranousMembranous• CartilaginousCartilaginous

o Cricoid Cartilage Cricoid Cartilage DeformityDeformity

• Normal shape, Small sizeNormal shape, Small size• Abnormal Shape:Abnormal Shape:

– EllipticalElliptical– FlattenedFlattened– Laryngeal CleftLaryngeal Cleft– OssificationOssification

o Trapped First Tracheal Trapped First Tracheal RingRing