Gonçalves Allan Christian Pieroni, Moritz Rodrigo Bernal da Costa, Monteiro Mário Luiz Ribeiro
Department of Ophthalmology, Universidade de São Paulo, Brazil.
Arq Bras Oftalmol. 2011 Sep-Oct;74(5):374-6. doi: 10.1590/s0004-27492011000500015.
Primary localized amyloidosis is rare in the orbit. We report the case of a 63-year-old woman that presented with bilateral proptosis and ophthalmoplegia. A computed tomography scan revealed an infiltrative amorphous and markedly calcified mass in both orbits while a magnetic resonance scan showed a heterogeneous hypointense signal on T2-weighted images. A biopsy was performed through an anterior orbitotomy. Microscopy revealed extracellular amorphous and eosinophilic hyaline material which stained pink with Congo red and displayed green birefringence on polarized microscopy, leading to a diagnosis of amyloidosis. The results of the systemic workup were completely normal. A two-year follow-up period without any treatment disclosed no worsening of the condition. While calcification of nonvascular orbital lesions has often been regarded as suggestive of malignant disease, our case is a reminder that it can also be a characteristic presenting sign of orbital amyloidosis.
原发性局限性淀粉样变性在眼眶中较为罕见。我们报告了一例63岁女性患者，其表现为双侧眼球突出和眼肌麻痹。计算机断层扫描显示双侧眼眶有浸润性无定形且明显钙化的肿块，而磁共振扫描在T2加权图像上显示为不均匀低信号。通过前路眼眶切开术进行了活检。显微镜检查发现细胞外无定形嗜酸性透明物质，刚果红染色呈粉红色，在偏振显微镜下显示绿色双折射，从而诊断为淀粉样变性。全身检查结果完全正常。未经任何治疗的两年随访期内病情未恶化。虽然非血管性眼眶病变的钙化常被认为提示恶性疾病，但我们的病例提醒人们，它也可能是眼眶淀粉样变性的特征性表现体征。
