P1091 - Rare Manifestation of Aspergillus in a Pediatric Patient with Cystic Fibrosis

Introduction: Endobronchial aspergilloma is a rare clinical presentation of aspergillus infection involving a fungal growth within the large airways. It is distinct from pulmonary aspergillomas which can form within damaged parenchyma such as cavitary lesions. Endobronchial aspergilloma most commonly presents in patients with underlying malignancy or an immunocompromised state. We present the first reported case of endobronchial aspergilloma in an immunocompetent pediatric patient with known cystic fibrosis (CF).

Case Report: Our patient is a 12 year old female with pancreatic insufficient CF who presented with a several month history of chronic non-productive cough, dyspnea, and difficulty expectorating. She denied any associated hemoptysis, but was found to have progressive decline of her pulmonary function testing. She also reported a globus sensation. During this period of time, she was treated with multiple courses of systemic antibiotics, to include Trimethoprim-sulfamethoxazole, Linezolid, and parenteral ceftazidime. Despite both outpatient treatment and an inpatient admission for presumed CF pulmonary exacerbation, her symptoms persisted. She was subsequently evaluated via bronchoscopy and found to have a large subglottic mass obstructing approximately 95% of the airway. In addition, she had increased bronchial secretions, but no evidence of foreign body or external compression. She was promptly referred to ENT for surgical removal of the airway mass. Histopathological evaluation demonstrated septated hyphae consistent with aspergillus without tissue or vascular invasion or evidence of malignancy. Tissue cultures were positive for Aspergillus fumigatus, confirming the presumed diagnosis of endotracheal aspergilloma. Prior imaging included CT of the chest demonstrating mild bronchiectasis consistent with CF. The mass was not visible on imaging nor was there evidence of invasive aspergillus. The patient was referred to pediatric infectious disease for medical management and treatment was initiated with voriconazole. Since surgery and initiation of antifungal therapy, she has demonstrated complete clinical resolution of her cough, dyspnea, and globus sensation. Repeat bronchoscopy did not demonstrate recurrence of the mass.

Discussion: Although aspergillus is a common pulmonary complication in patients with CF, our patient demonstrated a rare manifestation of an endobronchial aspergilloma. A careful literature search revealed that this entity is more commonly associated with underlying malignancy or immunocompromised states. This is the first reported case of endobronchial aspergilloma in an immunocompetent patient with CF. This case highlights that rare manifestations of common aspergillus infections should be considered in high-risk patients, such as those with CF, especially in the setting of worsening or unexpected clinical course.