Chief Complaint: Cough, hemoptysis, chest pain

1. Chief Complaint: Cough, hemoptysis, chest pain Kelly Kawaoka, M.D. Loma Linda University Medical Center

2. Case Presentation • 17 yo Hispanic female with Type I Diabetes Mellitus, multiple previous admissions for diabetic ketoacidosis • Presented initially with 10 days of chest pain, cough, and later developed hemoptysis • Diagnosed with diabetic ketoacidosis (DKA) and pericarditis secondary to pneumonia by chest CT at an outside facility • Bronchoscopy revealed necrotic tissue on the left mainstem bronchus • DKA resolved with appropriate treatment, but only minor clinical improvement of respiratory status with antibiotics • On presentation, afebrile, saturating well on 2 liters/min oxygen • Bilateral rhonchi, diminished on left with crackles, high-pitched expiratory wheezes

3. Labs/Imaging • White blood cell count: 19,700 per µL • Serum glucose 268 mg/dL • HgbA1C 10.1% • Westergren sedimentation rate (ESR) >140 mm/hr [normal 0-20 mm/hr] • C-reactive protein (CRP) 8.5 mg/dL [0-0.8 mg/dL] • Chest CT • Progressive consolidation in the lower left lobe with persistent bilateral pleural effusions • Thickening of the left lower lobe mainstem bronchus • Enlarged subcarinal and left hilar lymph nodes

4. Chest CT • Mediasinal mass with infiltration into the left atrium

5. Hospital Course • Bronchoscopy: no viral cytopathic changes, atypia or malignant cells on washings • Chest tube drainage (3) • Video-Assisted Thoracoscopic Surgery (VATS) Pathology • Abscess, granulation tissue, chronic inflammation • Lymph node  benign • Inconclusive for infection or malignancy • Endoscopic ultrasound (EUS)

6. Endoscopic Ultrasound Irregularly shaped hypoechoic mass in the left posterior mediastinum measuring approximately 2.5 x 1cm

7. Pathology Non-septated hyphae in an inflammatory background

8. Treatment • Ambisome started, changed to posaconazole and rifampin  slight clinical improvement • Repeat bronchoscopy confirmed Mucor • Pneumonectomy when the mass and symptoms did not resolve with antibiotics

9. Case Discussion • Pulmonary zygomycosis • Rapidly progressive • Affects the immunocompromised • Present with fever and hemoptysis • Spread locally to the mediastinum and heart or hematogeneously to other organs • Most common etiology: hematologic malignancy • May see with diabetes, more frequent with rhino-orbital-cerebral infection

10. Conclusion • When available, transesophageal biopsy with EUS is preferred over thoracoscopy • high diagnostic yield • less invasive technique • fewer complications • No other cases using EUS to diagnose Mucor in the current literature

11. Learning Objectives • Know that diabetic patients are at higher risk for developing infections • Know that fungal infections can be devastating in the immunocompromised host • Know that the diagnosis of pneumonia in an immunocompromised host may require aggressive procedures, including bronchoscopy • Review the differential diagnosis of a mediastinal mass in children and adults • Review presentation of mediastinal masses

12. Mediastinal Masses: Ddx A = anterior, M = middle, P = posterior *Due to infectious, malignant/metastatic, idiopathic causes

13. Mediastinal Masses: Presentation • Airway compression  Recurrent pulmonary infection or hemoptysis • Esophageal compression  dysphagia • Spinal column involvement paralysis • Phrenic nerve damage  elevated hemidiaphragm • Recurrent laryngeal nerve damage  hoarseness • Sympathetic ganglion compression  Horner's • Superior vena cava involvement  SVC syndrome

14. References • Krasnik M; Vilmann P; Larsen SS; Jacobsen GK (2003). “Preliminary experience with a new method of endoscopic transbronchial real time ultrasound guided biopsy for diagnosis of mediastinal and hilar lesions” Thorax. 58(12):1083-6. • Tedder, M, Spratt, JA, Anstadt, MP, et al. “Pulmonary mucormycosis: Results of medical and surgical therapy.” Ann Thorac Surg 1994; 57:1044. • Brown, RB, Johnson, JH, Kessinger, JM, Sealy, WC. “Bronchovascular mucormycosis in the diabetic: An urgent surgical problem.” Ann Thorac Surg 1992; 53:854. • UpToDate. “Evaluation of Mediastinal Masses” http://www.utdol.com

15. Question • A 3-year-old female is transported by ambulance to the emergency department. She had been treated with amoxicillin for the past eight days for suspected pneumonia and now presents with worsening of symptoms: cough, fever, and most recently coughing up blood. Physical examination includes a respiratory rate of 40 breaths/min, heart rate of 85 beats/min, oxygen saturation of 92% on room air, blood pressure of 100/70 mm Hg, and temperature of 102.3°F (39°C). She is awake and alert but has difficulty speaking in full sentences. On auscultation, you note diffuse crackles throughout her lung fields. Chest x-ray shows a mediastinal mass, which is confirmed to be anterior on CT.

16. Question • After initial stabilization, the BEST next step in the management of this patient is to • Administer methylprednisolone • Start a different oral antibiotic • Measure the pH of the bloody secretions • Transfuse packed red blood cells

17. Answer - C • Hemoptysis, is uncommon in pediatrics, but acute lower respiratory tract infection is the leading cause today, accounting for 40% or more of cases. Other causes include cystic fibrosis and congenital heart disease, both can present as recurrent bleeding. In children younger than 4 years of age, foreign body aspiration should be considered. Unlike in adults, neoplasm is an uncommon cause of hemoptysis in children. • The first step in the evaluation of a child who has hemoptysis is to determine the source of the bleeding. Blood from hemoptysis is typically bright red and frothy with an acidic pH rather than the dark or "coffee ground" alkaline material produced in hematemesis. Epistaxis generally can be established after careful examination of the oropharynx and nasopharynx. • The source of the bleeding for the child in the vignette likely is either pulmonary infection or foreign body obstruction. Methylprednisolone may be of benefit for a foreign body aspiration prior to bronchoscopy. The presence of the mediastinal mass makes this scenario less likely.

18. Answer - C • Initial therapy with antibiotics is appropriate only after collection of blood and sputum samples if pneumonia is suspected. IV antibiotics would be a more appropriate choice given that she has failed oral therapy. Most hemoptysis in children resolves spontaneously without the need for invasive measures. • This child had one episode of hemoptysis without massive bleeding so would most likely not need a blood transfusion. • Patients whose hemoptysis does not resolve spontaneously or who experience marked blood loss may require bronchoscopy to determine the source of the bleeding.