|Year : 2017 | Volume
| Issue : 1 | Page : 52-53
Pigment dispersion syndrome associated with spontaneous subluxation of crystalline lens
Vikas Veerwal, Jawahar Lal Goyal, Parul Jain, Ritu Arora
Department of Ophthalmology, Guru Nanak Eye Centre, New Delhi, India
|Date of Web Publication||21-Feb-2017|
A-109, Sector 61, Noida - 201. 301, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
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.
Keywords: Concave iris configuration, Krukenberg's spindle, lens subluxation, pigment dispersion syndrome, Zentmayer's line
|How to cite this article:|
Veerwal V, Goyal JL, Jain P, Arora R. Pigment dispersion syndrome associated with spontaneous subluxation of crystalline lens. Oman J Ophthalmol 2017;10:52-3
|How to cite this URL:|
Veerwal V, Goyal JL, Jain P, Arora R. Pigment dispersion syndrome associated with spontaneous subluxation of crystalline lens. Oman J Ophthalmol [serial online] 2017 [cited 2017 Aug 19];10:52-3. Available from: http://www.ojoonline.org/text.asp?2017/10/1/52/200683
| Introduction|| |
Pigment dispersion syndrome (PDS) is a condition that results from idiopathic atrophy of the pigment layers of the iris with the liberation of pigment, which is carried forward and deposited along the routes of aqueous flow. PDS typically affects young, myopic adults with a slight male predominance., The pigment in PDS is deposited in a characteristic manner on the corneal endothelium as Krukenberg's spindle, on the anterior surface of the iris, in the trabecular meshwork, on the lens and zonules and occasionally on the anterior hyaloid face.,,, The zonular fibers may have a bronze like appearance. Even with diffuse pigment deposition on zonular fibers, no zonular weakness or laxity has been reported in cases of PDS.,,, PDS has been known to be associated with Marfan's syndrome where reduced fibrillin expression in the iris and lens zonules may lead to increased irido-zonular contact promoting increased iris concavity and zonular weakness and thus, may lead to Spontaneous crystalline lens subluxations.
However, to the best of our knowledge, spontaneous subluxation of crystalline lens in isolated PDS without any systemic manifestations has not been reported.
| Case Report|| |
We report a unique case of a 43-year-old male with isolated PDS with spontaneous subluxation of crystalline lens. The patient presented with blurring of vision of both eyes for 6 months with BCVA of 6/36 in his right eye and 6/24 in the left eye. There was no history of trauma. Systemic and family history was insignificant. On systemic examination, the patient had a normal habitus with no abnormal facies, and no limb or joint deformities. No abnormality was detected on cardiovascular evaluation, ruling out Marfan's syndrome, Ehler's Danlos syndrome, and Weill Marchesani syndrome as the cause of lens subluxation. Homocysteine levels were 8.3 mmol/l (normal value-6.3–11.2 mmol/l). Ocular examination on slit lamp evauation revealed the presence of Krukenberg's spindle in both eyes [Figure 1]b and [Figure 2]b. Anterior chamber was deep with the absence of cells and flare. Pigment deposition was also seen on anterior lens capsule, zonules, and at the insertion of zonules on posterior lens capsule suggestive of Zentmayer's line [Figure 1]a and [Figure 2]b. The patient had 180° of zonular loss with temporal subluxation of clear lens in both eyes [Figure 1] and [Figure 2]. On gonioscopy, heavily homogenously pigmented trabecular meshwork with concave iris configuration was seen [Figure 3]. Gonioscopic and slit lamp findings confirmed the diagnosis of PDS. Intraocular pressure was normal. No posterior segment pathology was noted, and patient had a cup: Disc ratio of 0.3:1. Patient underwent an uneventful right eye followed by left eye intracapsular cataract extraction with scleral fixation of posterior chamber intraocular lens by ab-interno technique. On follow-up, the patient had a BCVA of 6/9 in both eyes after 6 weeks.
|Figure 1: (a) 180° of zonular loss with temporal subluxation of lens in right eye. Diffuse pigments on zonules are visible. (b) Presence of Krukenberg's spindle and a faint Zentmayer's line|
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|Figure 2: (a) 180° of zonular loss with temporal subluxation of lens in left eye. (b) Presence of Krukenberg&339;s spindle|
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|Figure 3: Gonioscopic findings of the patient. Significantly concave iris configuration with a heavily pigmented trabecular meshwork is seen|
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| Conclusion|| |
Patients with PDS show disruption of pigment epithelial cell membranes with dispersion of their pigment granule contents. Pigment may also be deposited on the posterior lens surface and zonules (Zentmayer's line or Scheie's stripe)., However, no zonular weakness is reported in these cases and spontaneous subluxation of crystalline lens is not known to occur.,, To the best of our knowledge, no case of isolated PDS with zonular weakness and spontaneously subluxated lens has been reported. By means of this report we would like to highlight this case of diagnosed PDS with bilateral spontaneous crystalline lens subluxation. We recommend thorough assessment of zonular status in all cases of PDS especially before undertaking them for any surgical procedure.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Scheie HG, Fleischauer HW. Idiopathic atrophy of the epithelial layers of the iris and ciliary body: A clinical study. Arch Ophthalmol 1958;59:216-28.
Migliazzo CV, Shaffer RN, Nykin R, Magee S. Long-term analysis of pigmentary dispersion syndrome and pigmentary glaucoma. Ophthalmology 1986;93:1528-36.
Sugar HS. Pigmentary glaucoma. A 25-year review. Am J Ophthalmol 1966;62:499-507.
Zentmayer W. Association of an annular band of pigment on the posterior capsule of lens with Krukenberg spindle. Arch Ophthalmol 1938;20:52-7.
Scheie HG, Cameron JD. Pigment dispersion syndrome: A clinical study. Br J Ophthalmol 1981;65:264-9.
Niyadurupola N, Broadway DC. Pigment dispersion syndrome and pigmentary glaucoma – A major review. Clin Experiment Ophthalmol 2008;36:868-82.
Campbell DG. Pigmentary dispersion and glaucoma. A new theory. Arch Ophthalmol 1979;97:1667-72.
[Figure 1], [Figure 2], [Figure 3]