巩膜脉络膜钙化的临床表现和全身合并症

Santosh G. Honavar, MD; Carol L. Shields, MD; Hakan Demirci, MD; Jerry A. Shields, MD


研究背景：巩膜脉络膜钙化是一种少见的眼病，大多数病因不清。

目的：描述巩膜脉络膜钙化的临床表现，并观察与全身病之间可能的关系。

方法：根据临床特点和诊断试验结果诊断的巩膜脉络膜钙化的系列病例无对照研究。作者观察分析了27例患者的人口学，全身及眼部特点。对19例患者进行了钙磷代谢相关的全身检查，13例患者进行了肾小管低钾性代谢性碱中毒综合症的全身检查。

结果：所有的患者均是老年（平均70岁）白人，无症状，是在常规检查时偶然发现脉络膜病变。38只眼中有诊断意义提示包括：10眼（26%）中有脉络膜转移，8眼（21%）有脉络膜黑色素瘤，4眼（11%）有脉络膜色素痣。16名（59%）患者有单侧眼的临床表现，双侧表现的有11例（41%）。37眼（97%）snellen视力不低于20/50。10眼（26%）可以在水平直肌止点之前的巩膜上发现Cogan斑块。在77个病灶中，平均每只眼有2个巩膜脉络膜钙化病灶，41个是黄色的，32个是黄白色，2个白色，2个桔黄色，平均直径2.6毫米，1.1毫米厚。最常见的位置是位于赤道部后（45眼，58%）30眼（39%）沿颞侧血管分布，43眼（56%）位于颞上象限。A、B超检查均发现类似钙的强回声和其后阴影。所有的病变在随诊38周后大小和形态还是保持稳定不变。1例患者在巩膜脉络膜钙化上发生了脉络膜新生血管。19例患者的钙磷代谢检查发现1例患者原发性甲状旁腺亢进症，13例患者的临床和生化检查发现6例高镁血症，4例患者符合Gitelman综合症的诊断标准。

结论：巩膜脉络膜钙化通常表现为老龄白人眼底中周部多灶性离散的无症状黄色鱼鳞状病变。虽然大多数病例病因不明，一些患者合并了钙磷代谢异常或者肾小管低钾性代谢性碱中毒综合症。所有巩膜脉络膜钙化的患者都应该进行这些可治疗的疾病的检查。

Arch Ophthalmol. 2001;119:833-840

Sclerochoroidal Calcification

Clinical Manifestations and Systemic Associations

Santosh G. Honavar, MD; Carol L. Shields, MD; Hakan Demirci, MD; Jerry A. Shields, MD


Background Sclerochoroidal calcification is an unusual ocular condition that is believed to be idiopathic in most cases.

Objectives To describe the clinical manifestations of sclerochoroidal calcification and to investigate its possible systemic associations.

Methods This noncomparative consecutive case series included patients diagnosed as having sclerochoroidal calcification based on clinical characteristics and diagnostic test findings. We analyzed the demographic, systemic, and ocular features of 27 such patients. Systemic evaluation included tests for calcium-phosphorus metabolism in 19 patients and renal tubular hypokalemic metabolic alkalosis syndromes (Bartter or Gitelman syndrome) in 13.

Results All the patients were asymptomatic older (mean age, 70 years) white individuals, incidentally noted as having a choroidal lesion on routine examination. Among 38 eyes, the main referral diagnoses were choroidal metastasis in 10 eyes (26%), choroidal melanoma in 8 (21%), and choroidal nevus in 4 (11%). Sixteen patients (59%) had unilateral clinical findings, while 11 (41%) had bilateral. The Snellen visual acuity was 20/50 or better in 37 eyes (97%). Cogan scleral plaque was visible anterior to the insertion of horizontal rectus muscles in 10 eyes (26%). Among 77 foci, there were a mean of 2 foci of sclerochoroidal calcification in each eye, 41 yellow (53%), 32 yellow-white (42%), 2 white (3%), and 2 orange (3%), measuring a mean 2.6 mm in diameter and 1.1 mm in thickness. The most common locations were postequatorial in 45 (58%), along the temporal vascular arcades in 30 (39%), and in the superotemporal quadrant in 43 (56%). A-scan and B-scan ultrasonography revealed dense echoes compatible with calcium, with orbital shadowing. All the lesions remained stable in size and configuration during a mean follow-up of 38 months. One patient developed a choroidal neovascular membrane over the area of sclerochoroidal calcification. Investigations for abnormal calcium-phosphorus metabolism in 19 patients revealed primary hyperparathyroidism in 1 patient (5%). Clinical and biochemical evaluation of 13 patients demonstrated hypomagnesemia in 6 (46%). Four patients (31%) met the criteria for the diagnosis of Gitelman syndrome.

Conclusions Sclerochoroidal calcification usually manifests as multiple discrete yellow placoid lesions in the midperipheral fundus of asymptomatic older white individuals. Although most cases may be idiopathic in nature, some patients may have underlying systemic disorders involving abnormal calcium-phosphorus metabolism or renal tubular hypokalemic metabolic alkalosis syndromes. All patients with sclerochoroidal calcification should be tested for these treatable systemic associations.

Arch Ophthalmol. 2001;119:833-840