JUVENILE LARYNGEAL PAPILLOMATOSIS

JUVENILE LARYNGEAL PAPILLOMATOSIS (MULTIPLE LARYNGEAL PAPILLOMAS)

History: MacKenzie first described this condition 100 years ago. These papillomas affect not only the larynx, but other areas of the respiratory tract, hence they are also called recurrent respiratory papillomatosis.

Aetiology: 

  1. Age: 3-5 years. Majority of children present before 4 years of age.
  2. More than 1500 new cases every year.
  3. Found in all socioeconomic segments of society.
  4. Viral infection of the epithelial cells by the human papilloma DNA virus type 6 and 11.
  5. Hormonal imbalance.

Pathology:

  1. Papillomas have a tendency to grow on the anterior commissure of the vocal cords. They come in clusters and are sessile, spreading over a wide area of mucosa.
  2. Recurrence is due to the activation of dormant virus from the normal mucosa adjacent to the lesion.
  3. They mostly affect supraglottic and glottic regions of larynx but can also involve subglottis, trachea and bronchi.

Common sites:

  1. Anterior commissure of larynx.
  2. Vocal cords.
  3. Rarely – gingiva, lips, soft palate, tonsillar pillar and pulmonary parenchyma. The lesion has a predilection for points of airway constriction causing drying, crusting and irritation.

Microscopy:

Immunofluorescent techniques show evidence of human papilloma virus having incorporated into cellular DNA.

Symptoms:

  1. Initially, hoarseness of voice or abnormal cry.
  2. Gradually increasing stridor and respiratory distress as the disease progresses.
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Management:

Prevention:

Medical treatment:

    1. Interferon injection.
    2. Antivirals like Cidofovir, Ribavarin and Acylovir
    3. Photodynamic therapy.
    4. Tetracycline.
    5. Systemic hormones.
    6. The monoclonal antibody against Vascular Endothelial Growth Factor (VEGF), Bevacizumab has shown promising result as an adjuvant therapy in the management of recurrent respiratory papillomatosis.
    7. HPV vaccines can be used therapeutically after the infection has occurred. For most patients, the HPV vaccine significantly increases the length of time needed between surgeries.

Surgical treatment:

  1. Surgical removal by microlaryngoscopy and CO2 laser is used for removal of papillomas with minimal damage to the musculature.
  2. Surgical removal by microlaryngoscopy and coblation is a newer technique which is effective in complete disease removal.
  3. Excision by sharp cup forceps during microlaryngoscopy. However, in this technique the chances of residual disease with surrounding tissue damage is present.
  4. Application of chemical cauterizing agents like silver nitrate (rarely done).
  5. Tracheostomy should be done if the patient has severe respiratory distress.

Progress:

Most papilloma tend to disappear by puberty. The chances of recurrence are considerably reduced after use of laser or coblation surgery. Chances of malignant changes are also very minimal in this pathology.

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