This is a life threatening condition seen in children with a recurring potential like malignancy presenting with abnormal cry, change in voice and noisy breathing. It is caused by Human Papilloma Virus (HPV) a DNA virus of Papova group. There are 70 serotypes types of which HPV-6&11 have been implicated in this clinical condition.This information is important as drugs acting on one virus sero type may not work for the other. Example is the homeopathic drugs with prooven use in skin warts papilloma but have no effect on the respiratory papillomatosis.

HPV’s are tissue-specific, targeting stratified Squamous epithelium of the orpharynx, larynx (Fig. 1), and anogenital region but not targeting epidermis It is commonly seen in the true vocal cord with Supra-and subglottic extension. Other areas of involvement, in order of decreasing frequency, are the trachea and bronchi, palate and nasopharynx, and pulmonary parenchyma. Involvement of the lung parenchyma leads to pulmonary nodules, atelectasis, pneumonia, bronchiectasis, cavitations, neoplasia, and even death. Other sites of HPV viral warts being vulva, Cervix, Penis & Perianal area. Studies have shown evidence of disease transmission at the time of delivery. If the child has disease before the age 5 there is 60% probability that mother had genital warts at the time of delivery. Hence the mother has to be screened for the same and if found to have lesions then advised to have subsequent children by caesarian section.

Pathogenesis: During infection virus remains dormant in the basal layer of the epithelium. The disruption of the normal flow of mucous blanket leads to invasion of larynx. The lesions are noted at the site of air way constriction where there is lot of drying, crusting and irritation, like anterior 1/3 of glottis, anterior commissure and tracheostoma. Recurrence is due to viral episome in normal cells even after complete removal. Children often require multiple surgical procedures, and their disease often becomes quiescent in adolescence. Radiotherapy for controlling disease may lead to malignant transformation to Squamous cell carcinoma or verrucous carcinoma.

Remission is unrelated to thoroughness of the removal of disease. Age and remission are inversely related. To achieve cure the latent infection has to be eliminated or the reactivation has to be prevented.

Management is usually by surgical laser vaporization; use of the surgical microdebrider is the best method to remove these lesions. Various modalities of treatment had been tried to prevent recurrences. Acyclovir, ribavirin, Interferon, Isoretinoin, Vaccines and antineoplastic drugs etc but the results have been variable. Air way block is the result if, papillomas are left untreated. Repeated surgery leads to scarring and either laryngeal stenosis or severe voice box scarring with resultant severely hoarse voice or no voice.The other alternative of doing a tracheostomy and leaving the child to reach adolescence and then remove the laryngeal papilloma is a practical alternative but the absence of voice and precence of tracheostomy makes this impractical. MANAGEMENT OF COMPLICATIONS OF TREATMENT IS A CHALLENGING TASK.

CASE 1 : We describe here two ends of spectrum following repeated laser surgery. The first example is a 17 year old girl with a near silent voice and a pendant of tracheostomy tube in the neck. She underwent MLS with laser excision 27 times at Mumbai, for recurrent respiratory Papillomatosis from the age of 5 years. Laryngeal examination showed mobile false cords with total glottic stenosis (Fig. 3). There was no papilloma seen in the larynx. CT SCAN neck confirmed the same and also showed mild subglottic stenosis. FLS through the tracheostoma showed no evidence of papilloma in the bronchus.She underwent Laryngoplasty via a laryngofissure approach under GA with release of the thick fibrosed scar tissue followed by mucosal reconstruction of one vocal cord using vein graft. Silastic sheet was placed between glottal surfaces to prevent adhesions.

 

1 Month later the Silastic sheet was removed. Inter arytenoids region appeared to be free; however there was growth of granulations in the scarred region with restenosis. The glottic chink was re opened by debridement of granulations and Fibrotic tissue, followed by placement of a stent (Fig. 3 ). She developed aspiration hence the stent was repositioned with blockage of the lower end.

2 months later the stent was removed the residual stenotic segment was debrided and dilated using dilators. Inj.Kenocort was injected into surrounding fibrotic area. MITOMYCIN-c was applied to the scar tissue. Adequate glottic chink was created (Fig. 4). Patient is still on treatment and has recently undergone subglottle widening with open method.

CASE 2 : The Second example is a patient with loss of bulk of vocal cord due to laser surgery. A 22yr old female patient presented with a manly voice. She had undergone MLS excision for Juvenile respiratory papillomatosis elsewhere. Stroboscopy showed loss of entire one vocal cord with a wide phonatory gap and dysphonia plica ventricularis (Fig .6).

She underwent modified type 1 Thyroplasty using goretex graft and the paraglottis was medialised with significant improvement in voice. We did not favour silicone as it is not the ideal material for paraglottic shift. 2 months later her voice became breathy for which fat augmentation was done using Brunnings Syringe (Fig. 6). Post op BW scopy shows adequate medialisation as seen in figure 7, voice improved and MPD [maximum phonatory duration] was within normal range.

CASE 3 : The 3rd example is a 36 year old lady with change in voice and difficulty in breathing. Laryngoscopy showed multiple papillomas occupying the whole right vocal cord .she had similar problem earlier at the age of 2 years and a tracheostomy was done. After 10 years subsequently underwent MLS twice at a another centre . At our centre she underwent MLS with excision of the superficial papilloma using CO2 laser and subsequently the remaining tissue was debrided. We use the tissue sparing skimmer microdebrider to avoid damage to underlying vocal ligament. After a follow up of 2 years she is asymptomatic and no recurrence (Fig. 8), with closure of the tracheostoma.

Laser does have a role to play in the management of Recurrent Respiratory Papillomatosis, however the controlled use of laryngeal microdebrider helps in minimizing the complication and results in the successful outcome.Management may also include silicon soft stents , mitomycin C,depot steroids intralesionally and dilators .