PULMONARY FUNCTION IN LARYNGOTRACHEOBRONCHIAL PAPILLOMATOSIS

Abstract

A 13 year old female(S) presented with asthma-like wheeze followed 2 months later by easy fatigability, orthopnea with paroxysmal nocturnal dyspnea, weight loss and hoarseness. Upper airway obstruction was suspected when the forced expiratory volume (FEV) tracings showed a “hump” and no flow recorded despite active expiratory efforts. Good attempts at inspiration resulted in an inspiratory capacity of 28% of predicted normal. Chest roentgenograms showed overaeration followed 3 mos. later by the appearance of multiple nodular densities throughout both lower lobes, middle lobe and lingula, and cavitation. The lateral view of the neck showed nodular densities obstructing about 50% of the airway. Endoscopy revealed large papillomas on the anterior half of the left vocal cord, trachea and main bronchi. Spirometry disclosed severe restrictive and obstructive pattern, intrapulmonary gas maldistribution and transit time of 3.25 to 6 seconds. Maximum expiratory flow volumes were all reduced. Diffusing capacity was decreased. Mechanics, ventilation and lung volumes were significantly changed after removal of the papillomas and the FEV curve became smooth. In the past 6 years, S experienced more respiratory obstruction every 2 to 8 months coinciding with enlargement of the papillomas. Repeated removal of the papillomas and chemotherapy have caused subjective relief but no significant change in serial lung function. Supported in part by NIH grant #RR-75

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