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A six year-old child with cough and facial swelling

Reviewed By Pediatrics Assembly

Submitted by

Ferdinand L Coste DO

Department of Pediatrics

Washington University School of Medicine


Thomas Ferkol MD

Department of Pediatrics

Washington University School of Medicine


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A 6-year-old African-American girl presented for outpatient evaluation with a four-month history of nonproductive cough.  Her mother initially characterized the cough as ‘whooping,’ which became increasingly harsh and brassy over time.  Treated with azithromycin, her respiratory symptoms persisted.  Her cough became more episodic, and precipitated by exertion or exposure to tobacco smoke.  Review of systems revealed intermittent rhinorrhea and periorbital edema, which the patient’s mother attributed to seasonal allergies.  She has not had fevers, weight loss, or night sweats.  She has not had recurrent or chronic infections involving the lungs, skin, middle ear, or paranasal sinuses.  No history of foreign body ingestion or aspiration was elicited. 

Two months before presentation, the patient was evaluated in a local emergency department for her coughing and diagnosed with viral pneumonitis.  A chest radiographs performed at that visit revealed mediastinal widening.  Computerized x-ray tomography (CT) of the chest was performed five days later to further evaluate this finding, which revealed calcified and non-calcified right paratracheal and hilar adenopathy as well as a calcified granuloma located in the right upper lobe.  Mild compression of the right main pulmonary artery was noted.  The patient had been treated with an albuterol metered-dose inhaler and cetirizine without relief.  Her mother states that she had received a course of systemic corticosteroid with modest symptom improvement.  Over time, her cough improved, such that her family had considered cancelling the appointment. 

Review of social history was significant for recent relocation of her family to the greater St. Louis area after living in Mississippi.  Their previous home had repeated water damage and mold contamination.  Her father lives in Indianapolis, Indiana, and he is being treated with isoniazid for latent tuberculosis.  She has not had other infectious or environmental exposures.  Allergy skin testing was negative for thirty inhalational allergens.  Testing for hereditary angioedema was negative.  Urine Histoplasma capsulatum antigen was not detectable.  Intradermal tuberculin (PPD) test was negative and decision was made to defer further testing in the absence of any constitutional symptoms.  Our working diagnosis was resolving histoplasmosis.  The patient was referred to a cardiologist for evaluation of the significance of the right main pulmonary artery compression in the setting of exertional dyspnea.  Follow up was scheduled for two months, with plans to repeat chest imaging studies in four months.

The patient returned for evaluation three months later and reported continued coughing with mild worsening, now with intermittent low-grade fever.  She was having continued periorbital and facial and lip swelling, which worsened.  She was seen in the emergency department twice for the coughing and discharged with diagnoses of pneumonia and asthma exacerbation, treated with third-generation cephalosporin and nebulized beta agonist, without any contact with pulmonary team.  She had not yet had a cardiac evaluation, and the patient was admitted from clinic for further evaluation and testing.

Further testing for histoplasmosis was pursued, with negative Histoplasma serum antibody using immunodiffusion and complement fixation assays.  Blastomyces antibody (immunodiffusion) immunoassay was also negative.  Blood lactic dehydrogenase concentration was mildly elevated and uric acid level were normal.  Her serum immunoglobulin E level was normal.  Serological assays for human immunodeficiency virus 1 and 2 antibodies were negative.  H. capsulatum yeast phase antibody was positive (1:64) and repeat chest CT revealed increased size of right paratracheal mass with mass effect on trachea and occlusion of right upper lobe bronchus with stenosis of bronchus intermedius, right main pulmonary artery, and superior vena cava.

Cardiothoracic surgeon reviewed the case and deferred biopsy or debulking via mediastinoscopy.  The patient began treatment with itraconazole and required placement of stents between the innominate vein and cavoatrial junction.  She has had gradual resolution of coughing and facial edema, with improved exercise tolerance and spirometric measures.

Question 1

    What is this patient’s diagnosis?


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