The patient was a 13-year-old Caucasian female that presented to the hospital with complaints of fever, nasal congestion, and worsening cough over four weeks. This was her second admission within a one-month period. On her previous hospitalization, she had presented to the emergency department with ketoacidosis, was diagnosed with type 1 diabetes mellitus, and started on insulin therapy. An outpatient chest CT had demonstrated pneumonia. Laboratory workup via serology studies immediately prior to admission included Aspergillus, Blastomyces, Coccidiodes, Histoplasma, Mycoplasma, Chlamydia, and tuberculosis, which were all negative. She was consequently placed on four separate courses of antimicrobials including cefdinir, azithromycin, clindamycin, and levofloxacin. However, she developed a brown, purulent sputum and her respiratory function progressively declined.
On admission, the patient was febrile at 38.3 C, heart rate was 148 beats per minute, respiratory rate was 36 breaths per minute, blood pressure was 138/99 mmHg, and O2 saturation was 94% on room air. Physical examination revealed decreased breath sounds in right lower lobe and scattered rhonchi. Initial laboratory investigations demonstrated total WBC count 15.2 x  cells per liter (81.3% neutrophil), hemoglobin 10.8 g/dL, ESR 76 mm, and CRP 312 mg/L Chest radiography indicated right lower lobe homogenous opacity suggesting consolidation, and chest CT demonstrated right middle and lower lobe pneumonia. Blood cultures were drawn, and she was empirically started on ceftriaxone and vancomycin.
Overnight, the patient had increased oxygen requirement and was transferred to the pediatric intensive care unit. Infectious disease recommended sputum culture, screenings for pneumococcus and legionellosis, and change antimicrobial regimen to linezolid and meropenem. Further laboratory investigations for potential source of infection including Legionella, Pneumococcus, chlamydia, mycoplasma, and atypical mycobacteria were negative. The patient was initially improving. However, by hospital day 7, she began to experience low-grade fever.
Pediatric pulmonology was consulted due to persistent tachypnea and accessory muscle use. The patient was recommended budesonide and racemic epinephrine. For the next two days, the patient and her family reported improvement. However, after four days, the patient's respiratory function declined again, bronchoscopy and bronchoalveolar lavage were performed.
During the bronchoscopy, only the larynx was visualized before the patient desaturated, suffered a cardiopulmonary arrest and required immediate resuscitative efforts including CPR, epinephrine, endotracheal intubation, central line placement, and vasopressor support. The patient was subsequently placed on high-flow oscillating ventilator, and the respiratory arrest was believed to be caused by increased secretions and mucus plugging within the airways. Infectious disease changed from meropenem to cefepime once she was stabilized, and subsequently added voriconazole due to prolonged broad-spectrum coverage. While the patient stabilized over the next four days before re-attempting another bronchoscopy, all previous cultures of blood, sputum, and urine continued to remain negative for any suspected pathogens.
A second bronchoscopy was performed successfully. During the procedure, a large foreign mass was removed from the patient's trachea (Fig. 1). Direct visualization revealed severe tracheitis, fibrosis of the left mainstem bronchus, and lavage was performed in the right lower lobe. Initial pathology report from the foreign body suggested possible mucormycosis, and she was placed on liposomal amphotericin B. CT of the chest demonstrated bilateral infiltrates involving right upper, middle, and lower lobes, as well as the left lower lobe with a left-side pleural effusion.
On hospital day 20, the throat culture confirmed the diagnosis of mucormycosis, and both cefepime and linezolid were discontinued. Repeat chest CT revealed dilatation of the trachea and right mainstem bronchus, and an occluded left mainstem bronchus was noted. The final tracheal biopsy showed the presence of broad, non-septate hyphae in both fibroconnective tissues and cartilage, indicating invasive disease. In spite of clinical improvement of respiratory symptoms, complemented by serial chest radiography, the patient was transferred on hospital day 40 to a tertiary medical center for surgical debridement.