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CASE REPORT
Year :   |  Volume :   |  Issue :   |  Page :  

Histopathology of Pigment dispersion syndrome and glaucoma


1 Department of Ocular Pathology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India
2 Department of Ophthalmology, Sri Sankaradeva Nethralaya, Guwahati, Assam, India

Date of Submission14-May-2021
Date of Decision06-Jul-2021
Date of Acceptance17-Jul-2021
Date of Web Publication18-Jul-2022

Correspondence Address:
Dipankar Das,
Department of Ocular Pathology, Uveitis and Neuro-Ophthalmology Services, Sri Sankaradeva Nethralaya, 96 Basistha Road, Beltola, Guwahati, Assam
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ojo.ojo_141_21

   Abstract 


Pigment dispersion syndrome (PDS) can have varied manifestations. Anterior and posterior segment involvement by dispersed pigments was documented in gross pathology and by staining under microscopy. Pigmentary changes in the sclera, cornea, anterior chamber, iris, trabecular meshwork, lens, and retinal pigment epithelium and optic nerve findings were consistent with PDS. External scleral and vitreous pigmentation has never been reported before in the scientific literature. Retinal pigment degeneration and granule dispersion were seen throughout the retina which could be a contributing factor in the etiology of PDS.

Keywords: Enucleation, optic nerve, pigment dispersion



How to cite this URL:
Das D, Kuri GC, Tayab S, Bhattacharjee H, Deshmukh S, Deka A. Histopathology of Pigment dispersion syndrome and glaucoma. Oman J Ophthalmol [Epub ahead of print] [cited 2022 Dec 8]. Available from: https://www.ojoonline.org/preprintarticle.asp?id=351367




   Introduction Top


Pigment dispersion syndrome (PDS) and glaucoma can present with diverse appearance.[1],[2],[3],[4],[5],[6] There are multiple theories about the dispersion of pigments in PDS.[1],[2],[3],[5],[7] Pathological findings in PDS were documented in the literature, but very few observations were related to retinal pigment epithelial change.[6],[7] Pigmentation on the external sclera and vitreous has never been reported in PDS in the scientific literature before. We report an appealing case of PDS with pathological observations of different pigment dispersions in gross and under microscopy.


   Case Report Top


A 49-year old-female reported to a tertiary institute of Northeast India with chief complaint of eye pain in the left eye (OS) for 3 months' duration, diminution of vision in the same eye since birth, and blurring of vision in the right eye (OD) for 1 week. The patient was seen elsewhere and reported to have an intraocular pressure (IOP) (Schiotz tonometry) of 17.3 mmHg in OD and 56 mmHg in OS. There was no past history of trauma or surgery in either eye. The patient did not give a history of myopia or glaucoma in the family. She was using local and systemic antiglaucoma medications. Her visual acuity in OD was counting finger 3 m and perception of light and projection of rays inaccurate in OS. On slit lamp examination, Von Herrick's grading of anterior chamber revealed grade 4 in OD and slit angle in OS. IOP (applanation tonometry) recorded in the OS and showed 60 mmHg in spite of all possible medical treatments. In gonioscopy, iridocorneal touch was seen in OS and anterior chamber was flat. Fundus examination OD revealed large peripapillary atrophic area with peripapillary pigment clumping. OS had no fundus view. B-scan ultrasound of OS revealed optic nerve head cupping and suspected posterior staphylomatous change. Lens echo was normal with increase in axial length. Diagnosis of painful blind eye with secondary angle change was made and enucleation of OS was advised. Enucleation with ball implant was done in the OS with proper consent. A conformer was put in the empty socket after the enucleation. Eyeball was subjected to histopathology.

Grossly, external hyperpigmentation of the sclera was noted posteriorly near the optic nerve. Eyeball was relatively enlarged and cut-section showed tessellated fundus after sectioning vertically. Posterior surface of the cornea showed pigment depositions around the empty looking endothelial cells [Figure 1]a. Pigments were more prominent and large on the pupillary area. Separate iris tissue showed transillumination defects which were in the mid portion of the iris. Lens was dislocated. Portion of the lens showed pigment deposition, more on the periphery and in a linear distribution. Zonules of crystalline lens could not be studied. Vitreous showed pigment clumps in spindle-shaped arrangement. Retina was attached. Retinal pigment epithelium (RPE) showed hypertrophic and atrophic areas stained under fluorescein dye in raw tissue [Figure 1]b. Optic nerve from the eyeball seen separately revealed deep glaucomatous cup and peripapillary blood vessels arrangement indicated such deep cup.
Figure 1: (a) Gross enface view of posterior surface of cornea showing extensive pigmentation of the endothelial surface. (b) Gross photograph of retinal pigment epithelium degeneration

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Microscopic examination revealed thinned out corneal epithelium with focal stromal scarring and edema. Pigment depositions were noted on the endothelial side of the cornea, and some of the pigments were in spindle-shaped pattern such as Krukenberg's spindles [Figure 2]a. Anterior chamber was deep. Pigment macrophages were seen in the anterior chamber. Iris showed posterior bowing. Pigment clumps were seen engulfing the iris vessels on the anterior surface of the iris. Peripheral iris was posteriorly inserted in the blunted ciliary body. Dilator muscle in the iris showed focal hyperplastic change. Angle of the anterior chamber was open at the one end and closed at the other end. Trabecular meshwork showed hyperpigmentation which was homogenous in nature [Figure 2]b. Pigmentation extended over the posterior trabeculum also. There were focal collapses of trabecular sheets with pigment granules. Pigmentation was also noticed on intratrabecular spaces. Retina showed variable size of RPE cells which were atrophic as well as hypertrophic. RPE degenerations were noted throughout the retina with dispersion of pigment granules. Photoreceptor degeneration was noted. Inner and outer nuclear cells of retinal layers were normal. Internal limiting membrane showed triangular projections toward the vitreous cavity. No retinal detachment or other degenerations in myopia were noted. Pigment cells were seen outside the sclera and in vitreous cavity [Figure 3]. Final impression on enucleated eyeball was PDS with glaucomatous and myopic changes.
Figure 2: (a) Pigment depositions being noted on the endothelial side of the cornea and some of the pigments being in spindle-shaped pattern like Krukenberg's spindles (H and E, ×40). (b) Homogeneous pigmentation of the trabecular mesh-work. Pigmentation extended to posterior trabeculum with pigmented granules seen in intratrabecular space (H and E, ×10)

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Figure 3: (a) Pigmented cell seen outside the sclera (marked with an arrow, H and E, ×40). (b) Pigmented cells (marked with arrows) in the anterior vitreous cavity (H and E, ×20)

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   Discussion Top


PDS has both anterior- and posterior-segment presentations.[1],[2],[3],[4],[5],[6] Histopathology of postmortem eyes were available in the literature, but few cases showed retinal pigment epithelial degenerations along with other constellation of findings.[1],[2],[3],[4],[7] Our case had corneal change in the endothelium with histopathological documentation of Krukenberg's spindles like previous studies.[6],[7] Iris bowing, pigment macrophages on the anterior chamber and homogenous trabecular meshwork pigmentation and lens changes were consistent with earlier observations.[1],[2],[3],[4],[5],[6],[7] External sclera and vitreous showed pigmentary changes which were not documented in the previous scientific literature. Grossly, optic nerve was documented having optic atrophy and the optic nerve blood vessels arrangement showing the evidence of deep glaucomatous cup. Eye had myopic change which can be seen in PDS.[1],[2],[3] RPE was studied grossly in the other half of the eyeball with fluorescein stain revealed significant RPE degenerations which were confirmed by hematoxylin and eosin stained slide RPE was studied grossly in one half of the eyeball in raw specimen with fluorescein stain which revealed significant RPE degenerations as seen in the [Figure 1] which were confirmed by hematoxylin and eosin stain. RPE changes in PDS were noted in two earlier case studies.[7] The present case highlighted few additional pigmentary changes in PDS which might be helpful in understanding this condition of glaucoma.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has/have given her consent for her images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity.

Acknowledgments

We would like to acknowledge Kanchi Sankara Health and Educational Foundation, Guwahati, Assam, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Sugar HS. Pigmentary glaucoma. A 25-year review. Am J Ophthalmol 1966;62:499-507.  Back to cited text no. 1
    
2.
Donaldson DD. Transillumination of the iris. Trans Am Ophthalmol Soc 1974;72:89-106.  Back to cited text no. 2
    
3.
Delaney WV Jr. Equatorial lens pigmentation, myopia, and retinal detachment. Am J Ophthalmol 1975;79:194-6.  Back to cited text no. 3
    
4.
Richter CU, Richardson TM, Grant WM. Pigmentary dispersion syndrome and pigmentary glaucoma. A prospective study of the natural history. Arch Ophthalmol 1986;104:211-5.  Back to cited text no. 4
    
5.
Niyadurupola N, Broadway DC. Pigment dispersion syndrome and pigmentary glaucoma--a major review. Clin Exp Ophthalmol 2008;36:868-82.  Back to cited text no. 5
    
6.
Kupfer C, Kuwabara T, Kaiser-Kupfer M. The histopathology of pigmentary dispersion syndrome with glaucoma. Am J Ophthalmol 1975;80:857-62.  Back to cited text no. 6
    
7.
Piccolino FC, Calabria G, Polizzi A, Fioretto M. Pigmentary retinal dystrophy associated with pigmentary glaucoma. Graefes Arch Clin Exp Ophthalmol 1989;227:335-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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