Shah Imtiaz Ali, Shah Shujaat Ali, Nagdev Partab Rai, Abbasi Safdar Ali, Abbasi Naeem Ali, Katpar Safdar Akhtar
Department of Ophthalmology, Chandka Medical College, Larkana, Sindh, Pakistan.
J Ayub Med Coll Abbottabad. 2017 Jul-Sep;29(3):412-414.
Pigment Dispersion Syndrome (PDS) is an autosomal dominant disorder of white males between 20 to 40 years of age characterized by deposition of pigment on the lens, zonules of lens, trabecular meshwork and corneal endothelium (Krukenberg's spindle) in addition to radial, spoke like transillumination defects in the mid peripheral iris. This study was conducted to determine the frequency of occurrence of Pigmentary Glaucoma in patients with Pigment Dispersion Syndrome (PDS).
This longitudinal follow up study included patients presenting with Krukenberg's spindle on the endothelial side of cornea and pigmentation of angle of anterior chamber seen on slit lamp examination and gonioscopy.
Seventy-two cases of PDS were included in the study, amongst them 63 (87.50%) were males. Mean age was 35.00±6.54 years (range 24-46 years). Forty-seven (65.28%) patients had an IOP in the range of 10-14 mmHg, 22 (30.56%) patients had an IOP in the range of 15-18 mmHg and 3 (4.17%) patients developed an IOP of greater than 19 mmHg. Fundoscopy showed myopic degeneration in 49 (68.06%) patients and optic disc cupping in 3 (4.17%) patients. Four (5.56%) patients had refractive error between +1D to +3D, 9 (12.50%) patients had refractive error between -1D to -4D, 21 (29.17%) patients had refractive error between -5 D to -8 D and 38 (52.78%) patients had refractive error between -9 D to -12 D. Our study showed that one patient having PDS developed glaucoma at 5 years of follow up and three patients developed glaucoma at 14 years of follow up.
On the basis of this study we conclude that early onset primary open angle glaucoma associated with PDS or Juvenile glaucoma associated with PDS might have been mistaken as Pigmentary Glaucoma in Pakistani patients and a distinct entity in the form of Pigmentary Glaucoma may be non-existent.
色素播散综合征（PDS）是一种常染色体显性疾病，好发于20至40岁的白人男性，其特征为色素在晶状体、晶状体悬韧带、小梁网和角膜内皮（ Krukenberg梭）沉积，此外，中周部虹膜还存在放射状、轮辐状透照缺损。本研究旨在确定色素播散综合征（PDS）患者中色素性青光眼的发生率。
本纵向随访研究纳入了在裂隙灯检查和前房角镜检查中发现角膜内皮侧有Krukenberg梭和前房角色素沉着的患者。
本研究共纳入72例PDS患者，其中63例（87.50%）为男性。平均年龄为35.00±6.54岁（范围24 - 46岁）。47例（65.28%）患者眼压在10 - 14 mmHg之间，22例（30.56%）患者眼压在15 - 18 mmHg之间，3例（4.17%）患者眼压高于19 mmHg。眼底检查显示49例（68.06%）患者有近视性退变，3例（4.17%）患者有视盘杯状凹陷。4例（5.56%）患者屈光不正度数在+1D至+3D之间，9例（12.50%）患者屈光不正度数在-1D至-4D之间，21例（29.17%）患者屈光不正度数在-5D至-8D之间，38例（52.78%）患者屈光不正度数在-9D至-12D之间。我们的研究表明，1例PDS患者在随访5年时发生青光眼，3例患者在随访14年时发生青光眼。
基于本研究，我们得出结论，在巴基斯坦患者中，与PDS相关的早发性原发性开角型青光眼或与PDS相关的青少年青光眼可能被误诊为色素性青光眼，而色素性青光眼这种独特的类型可能并不存在。
