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SVC Syndrome Secondary to Fibrosing Mediastinitis
Disorders of the Mediastinum SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM
SVC Syndrome Secondary to Fibrosing Mediastinitis Badar Siddiqui MD* Ahmed AlSheyyab MD Desiree Lehmann MD; and Girendra Hoskere MD Department of Internal Medicine, East Tennessee State University, Johnson City, TN
DISORDERS OF THE MEDIASTINUM
INTRODUCTION: Fibrosing mediastinitis (FM) is a rare chronic inflammatory disorder characterized by diffuse fibrosis of the soft tissues of the mediastinum leading to the obstruction of vital mediastinal structures. In this case report, we present a case of fibrosing mediastinitis leading to Superior Vena Cava (SVC) Syndrome. CASE PRESENTATION: A 41-year-old Caucasian male with remote diagnosis of Histoplasmosis, presented with a 6-week history of diffuse throbbing headache and facial pain with swelling. He reported worsening of his symptoms in the morning and night, further exacerbated by positional changes such as lying flat or leaning forward. Vitals were within normal limits. Physical exam was unremarkable except for tender anterior cervical lymphadenopathy, and prominent venous distension over the anterior upper chest. Computerized tomography (CT) scan of the chest showed marked narrowing of the superior segment of the SVC with prominent collateral circulation, calcification and mild fat stranding of the superior midline soft tissues in the pre-pectoral space. Biopsy of the cervical lymph nodes revealed, a benign lymphocytic reaction without granulomatous reaction or evidence of malignancy. Balloon angioplasty was attempted without results. Bare-metal stent was placed, the patient’s symptoms resolved and repeat Venogram demonstrated near complete improvement of contrast flow. DISCUSSION: In our case patient’s young age, gradual development of the symptoms with development of the collateral circulation, and absence of a mass constricting SVC all suggest SVC of benign etiology. Based on the collection of radiological findings and history of Histoplasmosis, our patient was diagnosed with FM. FM may constitute approximately half of the cases of SVC syndrome. Theorotically, FM secondary to Histoplasmosis is thought to occur due to leaking of antigen in mediastinum leading to progressive fibrosis. Diagnosis is usually made based on radiological findings, and also help in assessing extent of involvement and disease progression. Invasive diagnostic biopsies are not usually recommended given the risk of pulmonary hemorrhage unless diagnosis is not clear based on radiological findings. FM result in structural changes that are unresponsive to antifungal agents. In contrast, FM usually respond to interventions directed to relief obstruction, such as stent placement in our case. CONCLUSIONS: It can be difficult to establish histological diagnosis of SVC syndrome. Middle aged patients with SVC syndrome needs careful radiological evaluation for FM. The appearance on CT scan and the behavior on venocavogram can provide useful clues to the diagnosis. Reference #1: Rizvi, AZ, Kalra, M, Bjarnason, H, et al. (2008). Benign superior vena cava syndrome: Stenting is now the first line of treatment. J. of Vascular Surgery, 47(2), 372-380. Reference #2: Mathisen, DJ, & Grillo, HC (1992). Clinical manifestation of mediastinal fibrosis and histoplasmosis. The Annals of Thoracic Surgery, 54(6), 1053-1058. DISCLOSURE: The following authors have nothing to disclose: Badar Siddiqui, Ahmed AlSheyyab, Desiree Lehmann, Girendra Hoskere No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.644
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
556A
[
150#4S CHEST OCTOBER 2016
]
SVC Syndrome Secondary to Fibrosing Mediastinitis Badar Siddiqui MD* Ahmed AlSheyyab MD Desiree Lehmann MD; and Girendra Hoskere MD Department of Internal Medicine, East Tennessee State University, Johnson City, TN
DISORDERS OF THE MEDIASTINUM
INTRODUCTION: Fibrosing mediastinitis (FM) is a rare chronic inflammatory disorder characterized by diffuse fibrosis of the soft tissues of the mediastinum leading to the obstruction of vital mediastinal structures. In this case report, we present a case of fibrosing mediastinitis leading to Superior Vena Cava (SVC) Syndrome. CASE PRESENTATION: A 41-year-old Caucasian male with remote diagnosis of Histoplasmosis, presented with a 6-week history of diffuse throbbing headache and facial pain with swelling. He reported worsening of his symptoms in the morning and night, further exacerbated by positional changes such as lying flat or leaning forward. Vitals were within normal limits. Physical exam was unremarkable except for tender anterior cervical lymphadenopathy, and prominent venous distension over the anterior upper chest. Computerized tomography (CT) scan of the chest showed marked narrowing of the superior segment of the SVC with prominent collateral circulation, calcification and mild fat stranding of the superior midline soft tissues in the pre-pectoral space. Biopsy of the cervical lymph nodes revealed, a benign lymphocytic reaction without granulomatous reaction or evidence of malignancy. Balloon angioplasty was attempted without results. Bare-metal stent was placed, the patient’s symptoms resolved and repeat Venogram demonstrated near complete improvement of contrast flow. DISCUSSION: In our case patient’s young age, gradual development of the symptoms with development of the collateral circulation, and absence of a mass constricting SVC all suggest SVC of benign etiology. Based on the collection of radiological findings and history of Histoplasmosis, our patient was diagnosed with FM. FM may constitute approximately half of the cases of SVC syndrome. Theorotically, FM secondary to Histoplasmosis is thought to occur due to leaking of antigen in mediastinum leading to progressive fibrosis. Diagnosis is usually made based on radiological findings, and also help in assessing extent of involvement and disease progression. Invasive diagnostic biopsies are not usually recommended given the risk of pulmonary hemorrhage unless diagnosis is not clear based on radiological findings. FM result in structural changes that are unresponsive to antifungal agents. In contrast, FM usually respond to interventions directed to relief obstruction, such as stent placement in our case. CONCLUSIONS: It can be difficult to establish histological diagnosis of SVC syndrome. Middle aged patients with SVC syndrome needs careful radiological evaluation for FM. The appearance on CT scan and the behavior on venocavogram can provide useful clues to the diagnosis. Reference #1: Rizvi, AZ, Kalra, M, Bjarnason, H, et al. (2008). Benign superior vena cava syndrome: Stenting is now the first line of treatment. J. of Vascular Surgery, 47(2), 372-380. Reference #2: Mathisen, DJ, & Grillo, HC (1992). Clinical manifestation of mediastinal fibrosis and histoplasmosis. The Annals of Thoracic Surgery, 54(6), 1053-1058. DISCLOSURE: The following authors have nothing to disclose: Badar Siddiqui, Ahmed AlSheyyab, Desiree Lehmann, Girendra Hoskere No Product/Research Disclosure Information DOI:
http://dx.doi.org/10.1016/j.chest.2016.08.644
Copyright ª 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.
556A
[
150#4S CHEST OCTOBER 2016
]