Cryptogenic Progressive Tracheal Obstructio

Abstract

INTRODUCTION: Cryptococcosis is a fungal infection than can result in pulmonary involvement after inhalation of Cryptococcus neoformans spores.Although cryptococcosis is a common pulmonary complication among patients with AIDS, airway involvement has only rarely been reported in an immunocompetent host (1).We report a case of limited endotracheal cryptococcosis in an otherwise healthy patient with a history of asthma. CASE PRESENTATION: A 66 year old woman with asthma, previously well controlled with Advair 250/50 and occasional albuterol, presented with increased cough and hoarseness.She had been in her usual state of health until 1 week prior when she noted nonproductive cough, hoarseness and worsening dyspnea.She denied fever or headache.The past medical history was notable only for hypertension and hyperlipidemia with no prior history of diabetes or alcohol use.Family history was unremarkable.She was a housewife with no recent travel, exposure to animals or soil excavations. On physical examination she was afebrile, the vital signs were normal, but she had oral thrush, mild bilateral expiratory wheezing and normal skin.The neurological exam was nonfocal.Initial blood work was unremarkable, the chest X-ray unrevealing and a CT scan of the chest showed no infiltrates or adenopathy. A CT scan of the neck obtained to evaluate the hoarseness revealed minimal circumferential prominence of the subglottic region with 2-3 mm thickening of the upper tracheal wall. ENT was consulted;a direct laryngoscopy showed oropharyngeal and laryngeal thrush but with normal supraglottic region and vocal cords.She was treated with nystatin in addition to oral steroids and discharged home with continued Advair. She was readmitted to the hospital twice more in the next two months for increased dyspnea, each time treated with systemic steroids and inhaled bronchodilators with marginal improvement. Three months into this illness the patient was referred to the Pulmonary Clinic. Although she did not appears distressed at rest, inspiratory stridor was now quite marked. Spirometry suggested a fixed large airways obstruction and the patient underwent immediate fiberoptic bronchoscopy with a pediatric scope.This again demonstrated the laryngeal thrush and normal vocal cord appearance and motion. However just below the vocal cords the airway was nearly completely occluded with a circumferential friable mass-like process with overlying shaggy white exudate which extended for several centimeters. A rapid exam of the remaining airway was unremarkable. ENT was notified and the patient was taken for emergent tracheostomy during which the airway could only be secured by the use of a rigid pediatric bronchoscope. Microscopic examination of the obstructing lesion demonstrated acute and subacute inflammation with numerous fungal organisms with features suggestive of Cryptococcus. PCR analysis confirmed the organism as Cryptococcus neoformans. A serum HIV and cryptococcal antigen were negative. CSF cryptococcal antigen was also negative with an otherwise unremarkable lumbar puncture. The patient was started on oral fluconazole 400 mg daily and discharged. At 2 weeks follow up her dyspnea had resolved; followup bronchoscopy with anticipated decannulation is pending. DISCUSSION: Although pulmonary cryptococcosis can occurs in normal individuals, infection limited only to the airway is quite rare with only two reported cases, a right and left upper lobe bronchial lesion respectively (2). To our knowledge, this patient represents the first reported case of cryptococcal infection strictly limited to the subglottis and trachea in an otherwise immunocompetent individual. CONCLUSIONS: This case highlights the fact that cryptococcal infection may at times clinically and bronchoscopically mimic upper airway neoplasm and should be considered in the differential diagnosis of a rapidly progressive endotracheal obstruction even in the immunocompetent patient.