LARYNGOCELE

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    LARYNGOCELE

     

    DEFINITION:

    A congenital disorder of the larynx characterized by varying degrees of upper airway obstruction and a neck mass.

    EPIDEMIOLOGY:

    • incidence: rare (about 200 cases reported)
    • age of onset:
      • 40% detected at birth and 95% by 6 months of age
    • risk factors:
      • sporadic
      • M = F

    PATHOGENESIS:

    1. Background

    • in persons with congenitally large ventricular appendices, increased intraluminal laryngeal pressure (with straining, crying, singing, etc.) may cause the laryngeal appendix to dilate and form a saccular cyst which can remain either internal to the larynx or expand externally

    2. Classification

    1. Location

    • internal, external, or both
    • the internal cystic mass may expand through a perforation site in the thyrohyoid membrane at the neurovascular bundle to form an external cystic mass

    2. Content

    • aerocele, pyocele, mucocele
    • the cysts are lined with ciliated respiratory epithelium and contain mucous-secreting glands

    CLINICAL FEATURES:

    1. Internal Laryngocele

    • 90% - stridor
    • 55% - dyspnea
    • 50% - feeding problems
    • 20% - chronic cough

    2. External Laryngocele

    • presents as a cyst-like neck mass between the hyoid bone and the thyroid cartilage over the thyrohyoid membrane - tends to enlarge with straining and decrease in size at rest
    • may collapse with gentle pressure
    • compression of the mass may produce gurgling or hissing in the throat (Bryce sign)

    3. Complications

    • hoarseness, dysphagia
    • respiratory manifestations
      • aspiration +/- pneumonia
      • upper airway obstruction
      • laryngeal carcinoma
        • may develop in up to 50% of patients with laryngocele

    INVESTIGATIONS:

    1. Direct Laryngoscopy

    • diagnostic

    MANAGEMENT:

    1. Supportive

    • respiratory support with severe or life-threatening upper airway obstruction, i.e., intubation, tracheostomy

    2. Surgery

    • cyst decompression by needle aspiration or surgical unroofing
    • excision - surgical, laser

    3. Prognosis

    • normal life span and intelligence

     

     

    Pediatric Database - LARYNGOCELE

    Pediatric Organization - Pedbase [at] Gmail.com