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首页> 外文期刊>Arquivos Brasileiros de Oftalmologia >Bilateral orbital involvement in Erdheim-Chester disease: case report
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Bilateral orbital involvement in Erdheim-Chester disease: case report

机译：双边轨道累及Erdheim-Chester病：病例报告

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Purpose: To report a case of Erdheim-Chester disease with bilateral orbital involvement and to inform ophthalmologists about this uncommon condition. Methods: Case report. A 41-year-old man presented with proptosis, lid xanthelasmas, disc edema and extraocular muscle restrictions. He was suspected of having a systemic lymphoma and had already been submitted to a retroperitoneal biopsy, however, the diagnosis was still uncertain. Results: The patient was evaluated by computed tomography and magnetic resonance imaging of the orbit. Orbital pseudotumor was suspected and a biopsy of the orbital lesion initially failed to provide the correct diagnosis which was only made after detailed analysis of the clinical and imaging findings, and review of the histopathologic studies. The systemic diagnosis was made after the orbital diagnosis although the patient had been extensively investigated for more than one year. Conclusions: Erdheim-Chester disease is a rare idiopathic systemic condition characterized by a xanthogranulomatous process involving retroperitoneum, heart, lungs, bone and other tissues. The condition is often fatal due to renal or cardiovascular complications. Ocular findings are rare but may be very helpful for the diagnosis. Therefore, ophthalmologists should be aware of the clinical manifestations and imaging findings of this rare disease in order to establish an early diagnosis.
机译：目的：报告双侧眼眶受累的Erdheim-Chester病，并将这种不常见的情况告知眼科医生。方法：病例报告。一名41岁的男性出现了眼球突出，眼睑黄瘤，椎间盘水肿和眼外肌受限。他被怀疑患有全身淋巴瘤，已经接受了腹膜后活检，但是，诊断仍不确定。结果：通过计算机断层扫描和磁共振成像对患者进行评估。怀疑有眼眶假瘤，眼眶病变的活检最初未能提供正确的诊断，只有在详细分析临床和影像学发现并回顾组织病理学研究后才能做出诊断。尽管对该患者进行了广泛的研究超过一年，但在眼眶诊断后进行了全身诊断。结论：Erdheim-Chester病是一种罕见的特发性全身性疾病，其特征是涉及腹膜后，心脏，肺，骨骼和其他组织的黄原体肉芽肿。由于肾脏或心血管并发症，这种疾病通常是致命的。眼部检查很少见，但可能对诊断非常有帮助。因此，眼科医生应了解这种罕见疾病的临床表现和影像学发现，以便建立早期诊断。

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