1 Chief Complaint: Cough, hemoptysis, chest pain Kelly Kawaoka, M.D.Loma Linda University Medical Center
2 Case Presentation17 yo Hispanic female with Type I Diabetes Mellitus, multiple previous admissions for diabetic ketoacidosisPresented initially with 10 days of chest pain, cough, and later developed hemoptysisDiagnosed with diabetic ketoacidosis (DKA) and pericarditis secondary to pneumonia by chest CT at an outside facilityBronchoscopy revealed necrotic tissue on the left mainstem bronchusDKA resolved with appropriate treatment, but only minor clinical improvement of respiratory status with antibioticsOn presentation, afebrile, saturating well on 2 liters/min oxygenBilateral 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/dLHgbA1C 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 CTProgressive consolidation in the lower left lobe with persistent bilateral pleural effusionsThickening of the left lower lobe mainstem bronchusEnlarged subcarinal and left hilar lymph nodes
4 Chest CTMediasinal mass with infiltration into the left atrium
5 Hospital CourseBronchoscopy: no viral cytopathic changes, atypia or malignant cells on washingsChest tube drainage (3)Video-Assisted Thoracoscopic Surgery (VATS) PathologyAbscess, granulation tissue, chronic inflammationLymph node benignInconclusive for infection or malignancyEndoscopic ultrasound (EUS)
6 Endoscopic Ultrasound Irregularly shaped hypoechoic mass in the left posterior mediastinum measuring approximately 2.5 x 1cm
7 PathologyNon-septated hyphae in an inflammatory background
8 TreatmentAmbisome started, changed to posaconazole and rifampin slight clinical improvementRepeat bronchoscopy confirmed MucorPneumonectomy when the mass and symptoms did not resolve with antibiotics
9 Case Discussion Pulmonary zygomycosis Rapidly progressiveAffects the immunocompromisedPresent with fever and hemoptysisSpread locally to the mediastinum and heart or hematogeneously to other organsMost common etiology: hematologic malignancyMay see with diabetes, more frequent with rhino-orbital-cerebral infection
10 ConclusionWhen available, transesophageal biopsy with EUS is preferred over thoracoscopyhigh diagnostic yieldless invasive techniquefewer complicationsNo other cases using EUS to diagnose Mucor in the current literatureWe present a case in which transesophageal endoscopic ultrasound-guided fine needle aspiration was used to diagnose Mucor in a work up of a hilar mass.
11 Learning ObjectivesKnow that diabetic patients are at higher risk for developing infectionsKnow that fungal infections can be devastating in the immunocompromised hostKnow that the diagnosis of pneumonia in an immunocompromised host may require aggressive procedures, including bronchoscopyReview the differential diagnosis of a mediastinal mass in children and adultsReview presentation of mediastinal masses
12 Mediastinal Masses: Ddx ChildrenAdultsNeurogenic tumors (P)Enterogeneous cysts (A)Neurogenic tumors (P) Thymomas (A)Thymic cysts (A)Lymphadenopathy* (M)Hodgkins/Non-Hodgkins lymphoma (A)More often symptomatic, respiratory distress or recurrent pulmonary infectionMore often asymptomatic,Vague complaints such as aching pain or coughEnterogeneous cysts = Enteric duplications, ganglioneuromas, bronchogenic cysts, hemangiolymphangiomasNeurogenic tumors = neurofibromas, neurilemomas, origin includes nerve sheath, paraganglionic tissue, autonomic gangliaA = anterior, M = middle, P = posterior*Due to infectious, malignant/metastatic, idiopathic causes
14 ReferencesKrasnik 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):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”
15 QuestionA 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 QuestionAfter initial stabilization, the BEST next step in the management of this patient is toAdminister methylprednisoloneStart a different oral antibioticMeasure the pH of the bloody secretionsTransfuse packed red blood cells
17 Answer - CHemoptysis, 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 - CInitial 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.