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"Coated aorta": a new sign of Erdheim-Chester disease
J Serratrice1, B Granel, C De Roux
1Service de Médecine Interne, Hôpital de la Timone, Marseille, France.
Insights
Erdheim-Chester disease, a rare histiocytosis, can present with unusual aortic involvement. Bone scintigraphy aids in diagnosing this "coated aorta" finding.
Area of Science:
- Oncology
- Radiology
- Pathology
Background:
- Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis.
- It typically involves bone sclerosis, diabetes insipidus, proptosis, and retroperitoneal fibrosis.
Observation:
- This study describes 3 unusual cases of Erdheim-Chester disease.
- These cases featured periaortic fibrosis encasing the entire aorta, creating a "coated aorta" appearance on CT scans.
Findings:
- Periaortic fibrosis involving the entire aorta is an uncommon manifestation of Erdheim-Chester disease.
- Computed tomography (CT) scans revealed a characteristic "coated aorta" morphology.
- Bone scintigraphy proved valuable in identifying Erdheim-Chester disease in these cases.
Implications:
- The findings expand the understanding of Erdheim-Chester disease's vascular manifestations.
- Recognizing the "coated aorta" sign is crucial for diagnosis.
- Bone scintigraphy should be considered in the diagnostic workup for suspected Erdheim-Chester disease with this imaging finding.
Abstract:
Erdheim-Chester disease is a rare, non-Langerhans cell form of histiocytosis characterized by osteosclerosis of the metaphyseal regions of long bones, diabetes insipidus, proptosis, and retroperitoneal fibrosis. The latter usually involves the perirenal area and leads to hydronephrosis. Periaortic fibrosis is less frequent. We describe 3 unusual cases of Erdheim-Chester disease with periaortic fibrosis involving the whole aorta and leading to a "coated aorta" appearance on computed tomography scans. Faced with such a singular "coated aorta," bone scintigraphy can be very helpful when searching for Erdheim-Chester disease.