Long-Standing Temporal Visual Field Defect with Peripapillary Pigmentary Changes-Two Case Reports

長期顳側視野缺損合併視乳突周圍色素變化－兩個病例報告

孫仁彬(Jen-Pin Sun) ； 葉伯廷(Po-Ting Yeh) ； 楊中美(Chung-May Yang)

《中華民國眼科醫學會雜誌》 50卷2期 (2011/06) Pp. 434-441

https://doi.org/10.30048/ACTASOS.201106.0035

摘要

目的：視乳突周圍色素變化與長期顳側視野缺損之兩個病例報告。方法：臨床表現與治療。結果：這兩位年輕男性病人均表現長期顳側視野缺損並伴隨慢性閃光出現在視野缺損之區域。病例一之患眼為右眼而病例二為左眼。螢光眼底血管攝影顯示出視神經周圍視網膜色素上皮缺損。病例一患眼之網膜電圖不正常而兩位病人患眼之眼電圖結果均不正常。在第一位病人可以發現到第一級玻璃體發炎與輕微視乳突周圍色素變化。腦部核磁共振檢查與全身性發炎或自體免疫測試結果都為陰性。Recoverin抗體呈現陽性但是並沒有發現惡性腫瘤。在給予全身性類固醇後玻璃體發炎與視力均獲得改善。病例二的中心視力沒有受到影響且也沒有眼內發炎的現象。眼底檢查發現視神經盤周圍呈現區域性上皮色素細胞萎縮並延伸到上、下鼻側。Recoverin抗體為陰性。這位病人並沒有接受任何治療。我們懷疑這兩位病人為非典型多發輝閃性白點症候群（MEWDS）或急性區塊隱蔽性視網膜外層病變（AZOOR）。結論：單邊顳側視野缺損合併視乳突周圍色素變化可以單獨出現，而不伴隨著潛在疾病或其他視網膜病灶。鑑別診斷至少應包含MEWDS與AZOOR。不一定需要中樞神經系統影像學檢查。給予全身性類固醇對於減少玻璃體發炎是有幫助的。

並列摘要

Purpose: To report two cases with peripapillary pigmentary changes and long-standing temporal visual field defect.Methods: Clinical findings and treatments are presented.Results: These two cases both included young-aged males with presentation of long-standing temporal visual field defect with chronic photopsia in the visual field defect area of the right eye (case 1) or the left eye (case 2). Fluorescein angiography (FA) showed retinal pigmentary epithelium (RPE) defect around the disc. Subnormal scotopic and photopic electroretinogram (ERG) was noted in case 1. Decreased Arden ratio was demonstrated on electroculography (EOG) in these two cases. In case 1, grade I vitreous cell and subtle peripapillary pigment disturbance were observed. Brain MRI, systemic inflammatory and autoimmune profiles all showed negative results. The anti-recoverin antibody was positive, but no underlying malignancy was found. Vitritis and visual acuity improved after systemic steroid therapy. For case 2, central vision was spared and there was no intraocular inflammation throughout the course. The fundus appearance of case 2 revealed an ill-defined zone of RPE atrophy around the disc extending into the superior and inferior nasal quadrants. The result of anti-recoverin antibody was negative. No specific treatment was given to this patient. Atypical multiple evanescent white dots syndrome (MEWDS) or acute zonal occult outer retinopathy (AZOOR) was suspected in these two cases.Conclusion: Unilateral temporal field defect with peripapillary pigmentary changes may occur without specific underlying diseases or other focal retinal lesions. Differential diagnosis at least includes MEWDS and AZOOR. Extensive central nerve system imaging studies may not be necessary. Systemic steroid might be helpful to reduce vitreous inflammation.

並列關鍵字

acute zonal occult outer retinopathy (AZOOR) ； enlarged blind spot ； multiple evanescent white dots syndrome (MEWDS) ； peripapillary pigmentary change