Case of the week 0814: ErdheimChester Disease

1
Case of the week 08-14 Erdheim-Chester Disease
History History 41 year old black female
admitted for chest pain, malaise, weakness and
increasing peripheral edema. Tibial biopsy had
demonstrated typical findings of Erdheim-Chester
disease (ECD) 8 years earlier. She has previously
documented involvement of the long bones,
pancreas, pericardium and thoracic aorta with her
ECD. Cardiac biomarkers were negative for
myocardial necrosis. Physical examination No
murmur, gallop or rub. 3 pedal edema, scattered
rales at the right lung base. CMR Findings Cines
show extensive infiltration and thickening of the
pericardium, and a large loculated pericardial
fluid collection. There is a mild septal
bounce. Axial T1 images with and without Fat
Sat show infiltration of the intra-atrial-septum,
mild changes in the lung parenchyma and
wrapping of the aorta (). The pericardium
enhances after gadolinium (not from fat). CMR
Points Erdheim-Chester disease is a rare (lt200
reported cases), distinctive lipoidosis
characterized by deposition of cholesterol-laden
foam cells in the bone marrow associated with a
granulomatous reaction affecting the lungs,
pericardium, heart, orbit and retroperitoneum.
CMR is useful in identifying the extent of
infiltration of the pericardium and the presence
of cardiac constriction. In addition, CMR can
demonstrate the involvement of the great vessels
and wrapping of the aorta.
Dion E, Graef C, et al, Imaging of
Thoracoabdominal Involvement in Erdheim-Chester
DiseaseAm. J. Roentgenol. 2004 183 1253-1260
(?Click to view PDF)
Patricia Mergo, Mohammed Tabesh, Carsten
Schmalfuss, Gary Cooper Cardiac MRI Unit,
University of Florida