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• 영문명: A Case of Erdheim-Chester Disease with Diplopia
• 발행기관: 대한안과학회
• 저자명: 김세은 김숙영 김근해,Se Eun Kim, MD, Sook Young Kim, MD, PhD, Keun Hae Kim, MD, PhD
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume55,number2, 283~288쪽, 전체 6쪽
• 주제분류: 인문학 > 역사학
• 파일형태: PDF
• 발행일자: 2014.02.15

영문 초록

Purpose: We present a case of Erdheim-Chester disease (ECD) with diplopia. Case summary: A 56-year-old woman came to the hospital with a 6-week history of diplopia on left lateral gaze. The right eye showed mildly limited adduction. Humphrey automated perimetry demonstrated inferior bitemporal quadrantanopia. Orbital and brain magnetic resonance imaging revealed well-defined orbital masses in both intraconal orbits with homogenous enhancement, as well as multiple masses of homogenous signal intensity in the brain. Systemic evaluation showed involvement of the long bones, and retroperitoneum, but no involvement of the heart, or lungs. Incisional biopsy of the right orbital mass was performed. Histopathological examination showed numerous lipid-laden histiocytes and few multinucleated Touton giant cells. Immunohistochemical staining showed positivity for CD68, but negativity for CD1a, and ECD was therefore diagnosed. The patient received treatment with radiation therapy and interferon-α, but died due to sepsis secondary to urinary tract infection after 2 months. Conclusions: Except exophthalmos, diplopia may be the only initial symptom of an orbital mass. Although rare, the possibility of ECD should be considered in the differential diagnosis of both retrobulbar and orbital masses with diplopia. J Korean Ophthalmol Soc 2014;55(2):283-288

키워드

Diplopia, Erdheim-Chester disease, Orbital tumor, Retrobulbar mass