High Resolution Images Obtained With Ultrasound and Magnetic Resonance Imaging of Pericarotid Fibrosis in Erdheim-Chester Disease
A 41-year-old man with Erdheim-Chester disease presented with several episodes of transient monocular blindness of the left eye lasting up to 3 minutes. Medical history included diabetes insipidus since 1986 related to a retrochiasmatic mass and kidney failure with retroperitoneal fibrosis for 1 year.
Ultrasound examination of the neck revealed a left carotid artery stenosis with a major thickening of the arterial wall and multiple hyperechoic stratifications (Figure 1), as well as a minor thickening of the right carotid arterial wall.
High resolution magnetic resonance images with phased array surface coil showed similar images with pericarotid infiltration around both internal carotid arteries and endoluminal concentric stratifications predominating on the left side. Transverse magnetic resonance images on T1-weighted images of the left internal carotid artery demonstrated a homogeneous and hypointense thick fibrous cap. In proton density-weighted images, different coats are seen as isointense and hyperintense caps (Figure 2A and 2B). No sign of hemorrhage on transverse 3-dimensional time of flight images was detected.
This case illustrates a rare case of pericarotid fibrosis in Erdheim-Chester disease. In the literature, severe extensive periarterial infiltrations in the retroperitoneal space and the mediastinum have only been described along aortic and renal arteries. Histological findings demonstrated a perivascular fat infiltration of the aorta and its branches secondary to foamy histiocytes with some lymphocytes and increased fibrosis.
This case study was supported by a research grant (AOR 02028).