Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR imaging findings.
OBJECTIVE
To retrospectively review the brain magnetic resonance (MR) imaging and computed tomographic (CT) findings in patients with Erdheim-Chester disease (ECD).


METHODS
The ethics committee required neither institutional review board approval nor informed patient consent for retrospective analyses of the patients' medical records and imaging data. The patients' medical files were retrospectively reviewed in accordance with human subject research protocols. Three neuroradiologists in consensus analyzed the signal intensity, location, size, number, and gadolinium uptake of lesions detected on brain MR images obtained in 33 patients with biopsy-proved ECD.


RESULTS
Thirty patients had intracranial, facial bone, and/or orbital involvement, and three had normal neurologic imaging findings. The hypothalamic-pituitary axis was involved in 16 (53%) of the 30 patients, with six (20%) cases of micronodular or nodular masses of the infundibular stalk. Meningeal lesions were observed in seven (23%) patients. Three (10%) patients had bilateral symmetric T2 high signal intensity in the dentate nucleus areas, and five (17%) had multiple intraaxial enhancing masses. Striking intracranial periarterial infiltration was observed in three (10%) patients. Another patient (3%) had a lesion in the lumen of the superior sagittal sinus. Nine (30%) patients had orbital involvement. Twenty-four (80%) patients had osteosclerosis of the facial and/or skull bones. At least two anatomic sites were involved in two-thirds (n = 20) of the patients. Osteosclerosis of the facial bones associated with orbital masses and either meningeal or infundibular stalk masses was seen in eight (27%) patients.


CONCLUSIONS
Lesions of the brain, meninges, facial bones, and orbits are frequently observed and should be systematically sought on the brain MR and CT images obtained in patients with ECD, even if these patients are asymptomatic. Careful attention should be directed to the periarterial environment.
Drier
Aurélie
A
Department of Neuroradiology, Pitié-Salpêtrière Hospital, 47-83 Bd de l'Hôpital, 75013 Paris, France. aureliedrier@gmail.com
Haroche
Julien
J
Savatovsky
Julien
J
Godenèche
Gaelle
G
Dormont
Didier
D
Chiras
Jacques
J
Amoura
Zahir
Z
Bonneville
Fabrice
F
D000293:Adolescent; D000328:Adult; D000368:Aged; D001706:Biopsy; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000473:pathology*; D003287:Contrast Media; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging / Q000473:pathology*; D005147:Facial Bones / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008536:Meglumine; D008875:Middle Aged; D009915:Orbit / Q000000981:diagnostic imaging / Q000473:pathology*; D009942:Organometallic Compounds; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods*
D003287:Contrast Media; D009942:Organometallic Compounds; D008536:Meglumine; C072417:gadoterate meglumine