International Journal of Rare Diseases & Orphan Drugs Cite this article: Nandavaram S, Alam B, Amzuta I (2017) Pulmonary Vascular Tangle: Unusual Cause of Hemoptysis. Int J Rare Dis Orphan Drugs 2(1): 1003 Abstract Pulmonary vascular malformation is an unusual cause of hemoptysis. These abnormal vascular communications between pulmonary circulations or between pulmonary and systemic circulations can result in signifcant dyspnea, hemoptysis and hypoxemia. Pulmonary vascular malformations can take various forms. Here we present a case of an abnormal collection of thrombosed arteries and veins, without the classic features of pulmonary arterio-venous malformation and presented as a focal opacity on imaging, that can be easily confused as air space process or lung consolidation. Identifcation and treatment of these abnormal vascular communications is vital as they might result in life threatening hemoptysis. Keywords • Hemoptysis • Dyspnea • Consolidation • Vascular Anomaly *Corresponding author Sravanthi Nandavaram, 750 E Adams Street, Syracuse, NY, USA Tel: +1-315-464-4534 Email: drsnandavaram@gmail.com Submitted: 11 November 2016 Accepted: 15 February 2017 Published: 6 March 2017 Copyright: © 2017 Nandavaram et al. OPEN ACCESS Case Report Pulmonary Vascular Tangle: Unusual Cause of Hemoptysis Sravanthi Nandavaram*, Bisma Alam, Ioana Amzuta Department of Medicine, SUNY Upstate Medical University, Syracuse, New York, USA Abbreviations: WBC: White Blood Cell count; AFB: Acid Fast Bacilli; CT: Computed Tomography; LUL: Left Upper Lobe; VATS: Video Assisted Thoracoscopic Sur- gery; MIP: Maximum Intensity Projection; AVM: Arterio Venous Malformation; PAVM: Pulmonary Arterio Venous Malformation; AV: Arterio Venous INTRODUCTION Pulmonary vascular malformation is an unusual cause of hemoptysis. Pulmonary vascular malformations are abnormal vascular communications between pulmonary circulations or between pulmonary and systemic circulations and can result in significant dyspnea, hemoptysis and hypoxemia [1-4]. Here we present an unusual pulmonary vascular anomaly with abnormal collection of arteries and veins, however, lacking the classic features of pulmonary arterio- venous malformation, presenting with chronic hemoptysis and as a focal opacity on radiographic imaging. CASE PRESENTATION A 47-year-old female with medical history significant for hypertension, type 2 diabetes mellitus presented to our emergency room with complaints of chronic intermittent hemoptysis for the last 23 years. She moved from Somalia to United States 1 month prior to the presentation. She did not report any fever, chills, and shortness of breath or chest pain or epistaxis. She has been a lifelong non-smoker. At the time of presentation, she was afebrile, blood pressure was 115/54, and pulse was 88, respiratory rate was 20, oxygen saturation 96% on room air. Physical exam was benign without any evidence of any muco-cutaneous lesions or telangiectasias. Diagnostic work up showed white blood cell count 7.3, hemoglobin 11.8, platelets 210, and erythrocyte sedimentation rate 30. AFB sputum was negative; legionella urine antigen was negative, blood cultures were negative, INR was 0.9. Non-contrast transthoracic echocardiogram was normal. There was no evidence of shunt on the two-dimensional transthoracic echocardiogram bubble study. Computed tomography of the chest revealed focal opacity in the inferior segment of the lingula [Figure 1 & 2]. Fiberoptic bronchoscopy revealed streaks of blood in the left main bronchus. Fresh blood and blood clot was found in the inferior lingular segment of the left upper lobe. Broncho-alveolar lavage was performed in the left upper lobe inferior lingular segment and the return was bloody. Broncho-alveolar lavage specimens were sent for microbiology and cytology and were negative for infection and malignancy. Given the findings of active persistent bleed, 3-D and MIP reconstructions were done from the prior CT Chest which did not show any bronchial artery branches or parasitized intercostal artery branches supplying the lingular consolidation. There were only normal sized pulmonary arteries and veins supply the inferior segmental lingular consolidation. It was deemed by Interventional Radiology that the vessels are not amenable for embolization, given the lack of classic features of pulmonary arterio-venous malformation. Thoracic Surgery was consulted for resection of the lingula. Patient underwent video assisted thoracoscopic surgery and was found to have marked discoloration of the lingula [Figure 3] and wedge resection of the lingula was performed. Pathology sections from the left lingula resection revealed prominent areas of hemosiderin deposition both within alveolar macrophages and within the interstitium [Figure 5], especially