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A5586 - Not All that Wheezes Is Asthma - and Not All Are Foreign Bodies Either!
Author Block: W. S. Stoudemire1, M. S. Muhlebach2; 1Pediatrics, University of North Carolina, Chapel Hill, NC, United States, 2Univ of N Carolina At Chapel Hill, Chapel Hill, NC, United States.
Introduction: While asthma is common in children, other disorders may mimic asthma, and delayed recognition of alternative diagnoses may lead to significant morbidity. Here we describe the case of a young girl initially diagnosed with asthma found to have a tracheal mass causing airway obstruction.
Case Description: BH is a 4 year old girl admitted for respiratory distress. She was diagnosed with asthma six months ago due to intermittent cough and difficulty breathing. Two months ago, she was admitted for progressive respiratory distress requiring mechanical ventilation. She tested positive for parainfluenza virus, and was treated with albuterol and steroids for a presumed asthma exacerbation. She improved over several days, and was then discharged. She had intermittent cough and wheeze over the past two months with minimal response to albuterol.
BH was most recently admitted following acute onset of difficulty breathing. Her symptoms started four days prior to admission with rhinorrhea, cough, wheezing, as well as intermittent stridor. She then developed progressive respiratory distress refractory to maximum asthma therapy, and ultimately required intubation.
Following intubation, she continued to have poor oxygenation. Given her rapid decompensation, recent intubation, and intermittent stridor, CT scans of the neck and chest were obtained, which revealed opacity of the right mainstem bronchus suspicious for a mucus plug. A flexible bronchoscopy was performed which revealed a large pedunculated mass located on the distal anterior trachea superior to the carina. The mass was subsequently resected by otolaryngology. The patient was extubated immediately after surgery, and was discharged three days later. Pathology of the mass revealed a juvenile xanthogranuloma. Following resection, she had no further respiratory symptoms. A repeat endoscopy six months later revealed no evidence of residual tumor or regrowth.
Significance: Our patient was found to a juvenile xanthogranuloma of the trachea, an extremely rare location of a lesion typically found on the skin of young children. Juvenile xanthogranulomas are benign tumors caused by proliferation of dermal dendrocyte histiocytic cells. While they are the most common form of non-Langerhans cell histiocytosis, they typically occur as solitary granulomas in the skin during the first year of life. Juvenile xanthogranulomas of the airway are extremely rare, with only a handful of reported cases. While uncommon, lesions causing upper airway obstruction may be misdiagnosed as asthma in children, as in this case. Understanding of alternative diagnoses mimicking asthma, including airway masses, is important for accurate diagnosis and management.