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正视年轻男性的色素播散综合征和色素性青光眼。

Pigment Dispersion Syndrome and Pigmentary Glaucoma in an Emmetropic Young Male.

机构信息

Department of Ophthalmology, Sant Parmanand Hospital, New Delhi, India.

出版信息

Nepal J Ophthalmol. 2020 Jan;12(23):139-145. doi: 10.3126/nepjoph.v12i1.23972.

Abstract

INTRODUCTION

Pigment dispersion Syndrome (PDS) is a disorder with an onset in mid-twenties. There occurs a disruption of the iris pigment epithelium and deposition of pigment granules throughout the anterior segment. The incidence of PDS is 4-8/ 100,000. This condition is more commonly seen In Caucasians and is considered to be rare in Indians.

CASE

A 33-year-old male presented with the complaint of headache for three months. He had normal vision in both eyes with visual acuity of 6/6.

OBSERVATION

Krukenberg's spindle, a classic sign of pigment dispersion syndrome was evident on slit-lamp examination over the posterior corneal surface. Gonioscopy revealed a heavy and uniformly pigmented trabecular meshwork. OCT (Optical Coherence Tomography) demonstrated a characteristic iris configuration in the form of a mid-peripheral posterior bowing of the iris .Retinal nerve fibre layer analysis done on OCT revealed glaucomatous thinning in the right eye and a more advanced defect in the left eye. A visual field examination revealed the field to be outside normal limits in both the eyes pointing towards a diagnosis of pigment dispersion glaucoma.

CONCLUSION

The purpose of presenting this case is to caution the clinicians to carefully examine young emmetropes who present with Krukenberg's spindle as it could be associated with PDS. Patients with Krukenberg's spindle and without elevated lOP are often treated as normal. These patients must be cautioned regarding possible future consequences of the disease and counseled regarding the hereditary nature of the syndrome.

摘要

简介

色素播散综合征(PDS)是一种在二十多岁时发病的疾病。虹膜色素上皮发生紊乱,色素颗粒沉积在前节。PDS 的发病率为 4-8/10 万。这种情况在白种人中更为常见,在印度人中则被认为很少见。

病例

一名 33 岁男性因头痛三个月就诊。他双眼视力正常,视力为 6/6。

观察

在裂隙灯检查中,后角膜表面可见典型的色素播散综合征的 Krukenberg 纺锤体。房角镜检查显示小梁网色素沉着严重且均匀。OCT(光学相干断层扫描)显示虹膜呈中周后弓状的特征性虹膜形态。OCT 上的视网膜神经纤维层分析显示右眼青光眼性变薄,左眼更严重的缺损。视野检查显示双眼视野均超出正常范围,提示诊断为色素播散性青光眼。

结论

提出这个病例的目的是提醒临床医生仔细检查出现 Krukenberg 纺锤体的年轻正视眼患者,因为这可能与 PDS 有关。出现 Krukenberg 纺锤体但眼压不高的患者通常被视为正常。必须警告这些患者疾病可能带来的未来后果,并告知他们该综合征具有遗传性。

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