Veerwal Vikas, Goyal Jawahar Lal, Jain Parul, Arora Ritu
Department of Ophthalmology, Guru Nanak Eye Centre, New Delhi, India.
Oman J Ophthalmol. 2017 Jan-Apr;10(1):52-53. doi: 10.4103/0974-620X.200683.
Pigment dispersion syndrome (PDS) is an ocular condition characterized by a dispersion of iris pigment throughout the eye. This pigment is deposited in a characteristic manner on the corneal endothelium as Krukenberg's spindle, anterior surface of the iris, in the trabecular meshwork, on the lens and zonule and occasionally on the anterior hyaloid face. Even with deposition of pigment on zonular fibers, no zonular weakness, or zonular dehiscence has been reported in these cases. We report a unique case of PDS with bilateral spontaneous subluxation of crystalline lens. With characteristic findings of pigment distribution in both his eyes, the patient had concave iris configuration with heavily pigmented trabecular meshwork confirming the diagnosis of PDS. The patient had bilateral 180° temporal subluxation of crystalline lens in both his eyes. The usual cause of lens subluxation such as Marfan's Syndrome and Ehler's Danlos Syndrome was ruled out. The patient underwent right eye followed by left eye intracapsular cataract extraction with ab-interno technique with postoperative best-corrected visual acuity (BCVA) of 6/9 in both eyes. Spontaneous subluxation of crystalline lens in isolated PDS is not known to occur and has been reported by means of this case. We recommend a thorough assessment of zonular status in all cases of PDS.
色素播散综合征（PDS）是一种眼部疾病，其特征是虹膜色素在整个眼内播散。这种色素以特征性方式沉积在角膜内皮上形成Krukenberg梭，虹膜前表面、小梁网、晶状体和悬韧带，偶尔也沉积在前玻璃体表面。即使色素沉积在悬韧带纤维上，这些病例中也未报告有悬韧带薄弱或悬韧带裂开。我们报告一例独特的色素播散综合征病例，晶状体双侧自发性半脱位。患者双眼有色素分布的特征性表现，虹膜呈凹形，小梁网色素沉着严重，确诊为色素播散综合征。患者双眼晶状体均有180°颞侧半脱位。排除了晶状体半脱位的常见原因，如马方综合征和埃勒斯-当洛综合征。患者右眼先行囊内白内障摘除术，随后左眼采用经内路技术进行囊内白内障摘除术，术后双眼最佳矫正视力（BCVA）均为6/9。孤立性色素播散综合征中晶状体自发性半脱位尚未见报道，本文通过该病例进行了报告。我们建议对所有色素播散综合征病例进行悬韧带状态的全面评估。
