Supplementary Material for: Unilateral Congenital Lenticular Pigmentation

Introduction Release of pigments in the anterior chamber is frequently observed in Pigment Dispersion Syndrome (PDS), an autosomal dominant disorder marked by bilateral pigment deposition on the anterior and possibly posterior lens capsule, zonules of the lens, trabecular meshwork, and corneal endothelium, in addition to radial, spoke-like trans-illumination defects in the mid peripheral iris [1,4]. Pigmentation of the anterior lens surface has also been associated with intraocular inflammation, pseudoexfoliation syndrome, siderosis, antipsychotic medication usage, and remnants of the tunica vasculosa lentis [2]. Case presentation A 23 years old female presented to our Eye clinic with chief complaint of mild blurring of vision in the right eye and enquiring about refractive surgery. The patient denied any previous history of ocular inflammation, trauma, surgery, or use of topical or systemic medications. Slit lamp examination of the right eye anterior segment was within normal limit except for the crystalline lens anterior capsular which showed confluent pigment deposits stellate in shape over the pupillary axis whereas left eye examination was completely within normal limit. Ophthalmic examination of the posterior segment was normal in both eyes. Based on her previous ophthalmic history and slit-lamp examination of the right eye a diagnosis of unilateral congenital lenticular pigmentation was made. Conclusion Congenital lenticular pigmentation is a rare benign entity carrying no surgical indications with a relatively good visual response to optical correction. Recognition of this rare benign condition would add to the Ophthalmologist's differential of ocular pigmentation and avoid unnecessary concern and follow up in more potentially progressive disorders such as pigmentary glaucoma.

引言 前房色素释放常见于色素播散综合征（Pigment Dispersion Syndrome, PDS），这是一种常染色体显性遗传病，特征为双侧晶状体前（及后）囊膜、晶状体悬韧带、小梁网与角膜内皮出现色素沉积，同时伴随中周部虹膜呈放射状、辐辏样的透照缺损[1,4]。晶状体前表面色素沉着还与眼内炎症、假性剥脱综合征、铁质沉着症、抗精神病药物使用以及晶状体血管膜残留等情况相关[2]。
病例报告 一名23岁女性因右眼轻度视物模糊就诊于我院眼科门诊，并咨询屈光手术相关事宜。患者否认既往眼部炎症、外伤、手术史，亦无局部或全身用药史。右眼眼前节裂隙灯检查结果基本正常，仅瞳孔轴区的晶状体前囊膜可见融合性星状色素沉着；左眼检查完全未见异常。双眼后节眼科检查均正常。结合既往眼科病史与右眼裂隙灯检查结果，诊断为单侧先天性晶状体色素沉着症。
结论 先天性晶状体色素沉着症是一种罕见的良性病变，无手术指征，经光学矫正后可获得较好的视力预后。认识这一罕见良性病变，有助于眼科医师对眼部色素沉着相关疾病进行鉴别诊断，避免对色素性青光眼等潜在进展性疾病实施不必要的随访与过度担忧。