Multidetector Computed Tomographic Imaging of Erdheim-Chester Disease

Abstract

E rdheim-Chester disease (ECD) is a rarely reported form of multisystemic, non-Langerhans-cell histiocytosis. The clinical manifestations range from no symptoms to life-threatening conditions. Involvement of the bones, lungs, retro-orbital space, perirenal space, nervous system, cardiovascular system, and skin has particularly been observed. The cardiovascular involvement of ECD is probably more frequent than was originally thought; technological advances in multidetector computed tomography (MDCT) have enabled better views of patients' coronary arteries at low radiation doses. We report the case of a young woman in whom we used 256-slice, dual-source MDCT with high pitch value to evaluate the coronary arteries and reveal the effects of ECD. Case ReportIn April 2013, a previously healthy 29-year-old woman was referred to our hospital with a 3-month history of progressive angina. She had no history of other medical illnesses or familial diseases. Results of physical examination, laboratory tests, electrocardiography (ECG), and echocardiography were normal. To analyze her coronary arteries, we used a 256-slice Somatom ® Definition Flash MDCT system (Siemens Medical Solutions; Forchheim, Germany) with a high pitch value of 3.2. The patient's effective radiation dose was 1.1 mSv for the cardiac imaging. The MDCT angiogram revealed large mediastinal soft tissue with muscle attenuation surrounding the ascending aorta, extending into the proximal segment of the left main coronary artery, and causing stenosis by compressing the proximal segment of the left anterior descending coronary artery (Figs. 1 and 2). This periaortic effect resembles "coated aorta," associated with ECD. A specimen from a computed tomographic-guided biopsy of the mediastinal mass displayed fibrosis and many foamy histiocytes (CD68 + and CD1a -). The imaging and histologic findings supported the diagnosis of ECD. Skeletal radiographs showed no bone involvement, and thoracoabdominal computed tomograms and magnetic resonance images of the brain and orbit revealed no effect on those organs. The patient was referred for hemato-oncologic treatment. She was subsequently lost to follow-up. DiscussionTo our knowledge, this is the first report of the use of low-dose, dual-source, 256-slice multidetector computed tomography to characterize Erdheim-Chester disease

Cardiovascular Involvement in Erdheim–Chester Disease

“… 12 , 13 Second, myocardial localized or diffuse infiltration is often observed with frequent pseudotumoral infiltration of right atrium and involvement of the auricoloventricular sulcus. 14 , 15 Myocardial infiltration, in association with pericardial one, were both present in our patient. Moreover, coronary disease with myocardial infarction, valvular dysfunction, and heart failure have been described in the literature.…”

A Case of Incidentally-diagnosed Erdheim-Chester Disease

“…Cardiovascular manifestations of ECD include pericardial effusion, tamponade, congestive heart failure, valvular disorders, renovascular hypertension, periaortic fibrosis, and myocardial infarction, as was the case in our patient’s history [ 5 ]. In a review of 72 cases of ECD, Haroche, et al demonstrated that 56% of patients had circumferential aortic encasement, the so-called “coated aorta” [ 6 ].…”

A case report of Erdheim-Chester disease—clinically characterized by recurrent fever, multiple bone destruction, and antinuclear antibodies

“…Due to its invasion of bilateral pararenal and perirenal space, “hairy kidney” is manifested. The typical CT of cardiovascular ECD shows soft tissue infiltration around the aorta, forming the “coated aorta” sign [ 15 , 16 ].…”