INTRODUCTION: Recurrent respiratory papillomatosis (RRP), benign tumors of the airway with potential to undergo malignant transformation to squamous cell carcinoma (in 3–4%), are caused by the human papilloma virus (HPV). These tumors usually involve the larynx, epiglottis, sub glottis, vocal cords and trachea. Pulmonary parenchymal and endobronchial involvement is seldom seen. We describe a case of endobronchial obstruction caused by RRP in a non-compliant patient.
CASE PRESENTATION: A 36-year male smoker with a past medical history significant of recurrent respiratory papillomatosis (RRP), s/p multiple laser excisions and microdebridement over the last ten years, presented with a two weeks history of hoarseness, productive cough and shortness of breath. Examination revealed an acutely ill man in moderate respiratory distress with respiratory rate of 30/minute, heart rate of 114/minute, blood pressure of 166/82 and oxygen saturation (SpO2) of 89%, increasing to 94% with supplemental oxygen. Chest examination revealed bilateral inspiratory and expiratory wheezing and stridorous breath sounds, prominent over the neck and the upper airway. The remaining physical examination was noncontributory. Complete blood count, metabolic panel and coagulation panel were normal, as was a portable single view chest radiograph. The patient became progressively hypoxic and developed respiratory failure, with arterial blood gas showing pH 7.214, pCO2 73mmHg and pO2 105mmHg. Non-invasive ventilation was not well tolerated. Direct laryngoscopic visualization of the upper airway during intubation showed multiple polyps in the supraglottic region and on the right vocal cord. He was treated with intravenous steroids and bronchodilators. Thoracic computed tomography demonstrated diffuse nodular opacities within the trachea and the bronchi, occluding the right main stem bronchus and bronchus intermedius, multiple scattered small nodules in the left lower lobe, left upper lobe and right upper lobe, including one with cavitation, and sub centimeter hilar lymphadenopathy. The next day, in the OR, fiberoptic bronchoscopy showed multiple papillomas in the supraglottic region, right vocal cord, trachea, right main bronchus, and right bronchus intermedius, the latter occluding the airway. The RUL and left-sided bronchi were patent. The patient underwent direct laryngoscopy and rigid bronchoscopy with excision and microdebridement of the upper airway, tracheal and endobronchial papillomas. The histopathology of the excised tissue showed laryngeal and tracheal papillomas consistent with the diagnosis of recurrent respiratory papillomatosis and revealed diffuse low as well as high grade dysplasia. Immuno-histochemistry was positive for HPV 6 and 11. After he was extubated and he reported considerable improvement of his symptoms.
DISCUSSIONS: Recurrent respiratory papillomatosis (RRP) are rare benign respiratory tumors with bimodal age distribution with peak age of diagnosis around 4 years in children and third to fourth decade in adults. RRP is caused by HPV subtypes 6 and 11. They are typically seen in the larynx, supraglottic area and trachea but rarely extend to the bronchial tree and lung parenchyma, presenting as nodules that may cavitate. Most common symptoms in adult patients include hoarseness of voice, sore throat, cough, and dyspnea. Bronchial RRP may be misdiagnosed as asthma, croup or bronchitis. The course of RRP varies from spontaneous remission to recurrence of papillomas, requiring multiple laser or surgical interventions. Our patient had respiratory failure due to endobronchial obstruction caused by RRP. He was not a candidate for laser resection at this time, due to the extensive endobronchial involvement. Excision of papillomas and microdebridement through rigid bronchoscopy in our patient was successful and he had significant improvement of his symptoms.
CONCLUSION: Recurrent respiratory papillomatosis (RRP), although uncommon, should be diagnosed early and treated aggressively with either laser resection or surgery. If left alone they have the potential to become malignant, and increased morbidity and mortality is seen in patients, with endobronchial obstruction.
DISCLOSURE: Jaswinderpal Sandhu, No Financial Disclosure Information; No Product/Research Disclosure Information