A 32-year-old man with no previously known medical history presented to our institution, from a primary care office, for worsening dyspnea, cough and elevated glucose. Very limited information was obtained from the patient as he spoke a unique language from Guatemala called Tz'utujil, for which language interpretation was not available. In the emergency department he was diaphoretic, in the tripod position, and in moderate respiratory distress.
His condition was guarded initially on 10L oxymizer. Blood glucose was noted to be 415mg/dL. Chest x-ray was immediately performed and demonstrated subcutaneous air and pneumomediastinum. Chest CT was subsequently performed, revealing extensive right sided infiltration with probable erosion of the right mainstem bronchus, pulmonary emphysema in the right-sided airways, as well as right upper lobe (RUL) and right middle lobe (RML) infiltrates and centrilobular ground-glass nodules were also noted (Fig. 1). He was subsequently intubated due to increasing distress with a 35 french left sided, double lumen endobronchial tube (ET), performed under endoscopic guidance with a bronchoscope. Bronchoscopy revealed erythema at the carina along with copious thick, white secretions that extended into the left and right mainstem bronchus. Saline was used to wash and suction the secretions. Empiric antibiotic and anti-fungal treatment were started with Vancomycin, Piperacillin-Tazobactam, Doxycycline and Voriconazole. In addition, he was maintained on airborne precautions due to concern for tuberculosis. HIV testing, respiratory multiplex PCR, aspergillus antibodies, and blood cultures were obtained at this time. He was then transferred via helicopter to our tertiary institution for evaluation for interventional pulmonology.
Cardiothoracic surgery evaluated the patient for bronchomediastinal fistula and recommended proceeding with further bronchoscopic intervention. Fiberoptic bronchoscopy was performed in the intensive care unit. Bronchoscopy revealed extensive submucosal sloughing and necrosis of the carina, right mainstem, right bronchus intermedius and RML lateral segment. In addition, there was an endobronchial flap with purulent material in the right mainstem bronchus. Broncheoalveolar lavage was obtained of the right lung as well as endobronchial biopsies of the right mainstem (Fig. 2). Continued broncoscopic management was recommended by cardiothoracic surgery at this time, with repeat debridement. The patient later became hypotensive requiring vasopressors, and broad-spectrum antibiotics and anti-fungal agents were continued.
On hospital day 3, acid fast bacilli smear resulted as negative, bronchiolar alveolar lavage cultures grew rhizopus, candida (albicans or candida dubliniensis), and endobronchial biopsies revealed necrotic tissue with fungal hyphae suggestive of mucor species, as well as actinomyces (Fig. 3). His anti-fungal coverage was switched to liposomal Amphotericin B and micafungin. It was agreed that the etiology of patient's disease was invasive mucormycosis causing bronchial erosion and an MRI of the brain was ordered to look for fungal disease. Right sided mastoiditis was found with no other findings identified.
The following day, the ventilator alarmed for high peak pressures. The ET appeared to be obstructed. An approximately 10cm long blood clot was retrieved from the ET, followed by large amounts of bright red blood. The patient was unable to be adequately ventilated or oxygenated and went into PEA arrest. Advanced cardiac life support (ACLS) protocol was immediately started. ET exchange was performed under glidescope visualization. Massive transfusion protocol was initiated simultaneously. Subsequently, crepitus was noted from mid-thigh to forehead, and bilateral needle decompression was performed with air and bright red blood return. ACLS protocol was continued for a total of 35 minutes, at which time the patient was pronounced dead.
Top left: Initial chest x-ray on presentation showing right sided opacities and sub-cutaneous air in the neck. Top right: Axial cut of chest CT showing mediastinal air. Bottom right: Axial cut of CT demonstrating bronchomediastinal fistulas with necrotizing pneumonia. Bottom right: Coronal cut of the chest CT further demonstrating necrotizing areas in the right main bronchus and necrotizing pneumonia.