Chief Complaint

							Chief Complaint: Cough, hemoptysis, chest pain




Kelly Kawaoka, M.D.
Loma Linda University Medical Center
                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
                    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
                      Chest CT




Mediasinal mass with infiltration into the left atrium
              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)
           Endoscopic Ultrasound




Irregularly shaped hypoechoic mass in the left posterior
   mediastinum measuring approximately 2.5 x 1cm
                    Pathology




Non-septated hyphae in an inflammatory background
               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
               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
                  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
            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
             Mediastinal Masses: Ddx
             Children                                  Adults
 Neurogenic tumors (P)                 Neurogenic tumors (P)
 Enterogeneous cysts (A)               Thymomas (A)
                                       Thymic cysts (A)
                                       Lymphadenopathy* (M)
                                       Hodgkins/Non-Hodgkins
                                       lymphoma (A)
 More often symptomatic,       More often asymptomatic,
 respiratory distress or       Vague complaints such as aching
 recurrent pulmonary infection pain or cough
A = anterior, M = middle, P = posterior
*Due to infectious, malignant/metastatic, idiopathic causes
 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
                     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
                        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.
                    Question
• After initial stabilization, the BEST next step
  in the management of this patient is to
  A.   Administer methylprednisolone
  B.   Start a different oral antibiotic
  C.   Measure the pH of the bloody secretions
  D.   Transfuse packed red blood cells
                           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.
                    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.