Case Report Delhi Journal of Ophthalmology

Iridotomy in Pigmentary Glaucoma - ASOCT perspective

Prakash Agarwal1 MD, VK Saini1 MS, Saroj Gupta1 MS, Anjali Sharma1 MS, Reena Sharma2 MD, Tanuj Dada2 MD

Abstract A pigmentary glaucoma is a form of secondary open angle glaucoma caused by pigment liberated from the posterior iris surface in pa ents with pigment dispersion syndrome. The pigment cells slough off from the back of the iris due to its concave confi gura on causing it to rub against the zonules and lens. These pigment cells accumulate in the anterior chamber in such a way that it begins to clog the trabecular meshwork causing eleva on of intraocular pressure. Anterior segment op cal coherence tomography (ASOCT) is a non contact, easy to use, reproducible method for examina on of the anterior segment. It allows detailed evalua on of the cornea, the angle of eye and the iris. It has extensively been used to evaluate angle closure glaucoma. It can also be used in cases of pigmentary glaucoma. We present a male, myopic pa ent with advanced stage of pigmentary glaucoma at a rela vely young age. We used ASOCT to demonstrate the concave iris confi gura on in our pa ent and its disappearance following laser iridotomy. We thus highlight the importance of use of ASOCT in pa ents of pigmentary glaucoma Del J Ophthalmol 2012;23(3):203-206. Key Words: pigmentry glaucoma, ASOCT, laser iridotomy DOI: h p://dx.doi.org/10.7869/djo.2012.70

The relationship of pigment and glaucoma was fi rst There is only one study evaluating the role of ASOCT in given by von Hippel in the 20th century.1 The modern assessing the anterior chamber parameters in pigmentary concept of pigmentary glaucoma was conceived by Sugar in glaucoma.6 However, there is no study, using ASOCT, 1940 when he described pigment dispersion and glaucoma documenting the iris changes after iridotomy in these in a 29 year old man.2 The term “Pigment glaucoma” was patients. described in a series published by Sugar and Barbour in 1949.3 Campbell suggested that the pigmentation resulted Case report from friction of the zonules rubbing on the neuroepithelium A 25 year old male patient presented to department 4 of the iris. In 1993, Karickhoff gave the mechanism of of ophthalmology at our hospital with decrease in vision reverse pupillary block which caused iris to rub against in right eye for 6 months. On Snellen visual acuity chart, 5 the zonules. The concave midperipheral iris drapes over vision of right eye was hand movement close to face with the lens, working as a fl ap valve, which does not permit inaccurate projection of rays in two quadrants and in left movement of aqueous, trapped in the anterior chamber, eye was 6/36 with inaccurate projection of rays in nasal to the posterior chamber causing pigment release and rise quadrant. of intraocular pressure(IOP). Anterior segment optical coherence tomography (ASOCT) can be used for imaging The anterior chamber was deep. Pupillary examination the iris and its confi guration in patients of pigmentary revealed a relative afferent pupillary defect in the right eye. glaucoma. This is a case report of a patient of pigmentary Goldmann applanation tonometry revealed IOP of 27 mm glaucoma presenting with advanced disease and low IOP Hg OD and 22 mm Hg OS. Fundus examination revealed at an early age. ASOCT was used to document the loss of total glaucomatous disc cupping in right eye and near total concave iris confi guration following Yag laser iridotomy. cupping in the left eye (Figure 1a, b). Gonioscopy using Goldmann single mirror gonioscope revealed homogenous brown pigment dispersion in both the eyes (more in 1 Peoples College of Medical Sciences and research centre, Bhopal. right eye) and open angles in both the eyes (Figure 2 a,b). 2 Dr Rajendra Prasad centre for ophthalmic sciences, All India Detailed examination revealed pigment dispersion over the Ins tute of Medical Sciences, New Delhi endothelium. Confi guration of the iris was concave with atrophy of the peripheral iris as compared with central Correspondence to : Dr. Reena Sharma portion of iris with heterochromia iridium (Figure 3). E-mail : [email protected] Confrontation perimetry revealed grossly constricted visual fi elds in both eyes. Right eye visual fi eld assessment using

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Figure 1(a & b) Fundus photograph showing total glaucomatous cupping OD; Near total disc cupping OS

Figure 2 (a & b) Gonioscopy of the eyes revealing pigmentation of angles OD,OS

Humphrey perimetry could not be done due to poor vision. was documented on ASOCT. Based on the deposition of Left eye visual fi eld assessment was not reliable due to high pigment at the angles and typical concave iris confi guration, loss of fi xation. On retrospective questioning there was a diagnosis of reverse pupillary block and pigmentary no history of trauma or intraocular surgery. There was no glaucoma was made (Figure 1,4). The differential diagnosis history of chronic medication or signifi cant medical illness. which could be possible in such a scenario was juvenile open There was no family history of glaucoma. angle glaucoma (JOAG). However, points against JOAG ASOCT of the angles revealed concave iris confi guration were concave iris confi guration well shown by ASOCT, with extensive irido-lenticular touch and reverse pupillary atrophy of peripheral iris and pigment dispersion at the block in both the eyes (Figure 4). Central corneal pachymetry angles. Secondary glaucoma was ruled out in view of no using the OCT revealed 533 microns OD and 534 microns relevant history. There were no ophthalmic signs of uveitis, OS. The patient had concave iris confi guration which hyphaema, trauma, melanosis or any other ocular disease

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Figure 3 Heterochromia iridum with atrophy of the Figure 5 Anterior segment OCT images of the two eyes peripheral iris compared to the central portion of iris following Nd Yag laser iridotomy with fl attening of the irides

patient was started on topical bimatoprost (Lumigan 0.01%, Allergan, USA) and topical combination of timolol (0.5%) and Brimonidine (0.15%) (Combigan, Allergan, USA). The patient responded well and tolerated the medications without signifi cant side effects. The IOP was lowered to 11 in right eye and 12 in left eye at 3 month follow up. Discussion Pigmentary glaucoma is recognized as one of the most common forms of secondary open-angle glaucoma. It affects a younger patient population more than most other forms of open-angle glaucoma, and has a predilection for Caucasian males with myopia. The pigment is released due to irido- zonular contact owing to reverse pupillary block and blocks the fi ltering trabecular meshwork leading to decreased outfl ow and rise in IOP.7-11 The concave iris confi guration on gonioscopy is a strong clue to the diagnosis of pigmentary glaucoma. ASOCT is a simple, non-contact technique for evaluation of anterior chamber parameters and serves as an adjunt to gonioscopy.12 It can be used to assess the iris and angle changes before and after laser iridotomy.13

Figure 4 Anterior segment OCT images showing concave Our case was a 25 year old patient with advanced stage of iris confi gurtion in both eyes pigmentary glaucoma and low IOP. Our patient is relatively young for presentation with advanced disease; however giving rise to secondary elevation of IOP. The patient pigmentary dispersion has been described in as young as underwent a neodymium-yttrium aluminum garnet laser 14 year old patients.14 The patient presented with relatively (YAG) laser iridotomy in both eyes and post iridotomy, low IOP which is uncommon. The IOP in pigmentary the fl attening of iris was also documented with ASOCT glaucoma is typically higher, 35-40 mm Hg and diffi cult (Figure 5). In view of advanced glaucomatous cupping of to treat medically.15 The absence of iris transillumination both eyes; the target IOP of 10-12 was set for the patient. The defects and corneal endothelial deposits is not unusual as

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they are less common in black population.16 Thus, advanced parameters in pigmentary glaucoma using slit-lamp optic nerve damage with relatively lower values of IOP (22 optical coherence tomography. Eur J Ophthalmol 2010; and 27 mm Hg) at a relatively younger age as seen in our 20(4):702-7. patient, is uncommon in pigmentary glaucoma. The use of 7. Scheie HG, and HW Fleischhauer, Idiopathic atrophy ASOCT aided in our diagnosis by showing the concave iris of the epithelial layers of the iris and ciliary body. Arch. confi guration and the iridolenticular touch responsible for Ophth 1958; 59:216. the reverse pupillary block. The changes in iris confi guration 8. Peterson HP, Pigmentary glaucoma, Acta ophth 1961; after iridotomy could also be documented. 39:688. 9. Cavka V. Pigmentary glaucoma, Am. J. Ophth 1961; The ASOCT is a good tool for documenting the concave 52:880. iris confi guration required for the diagnosis and its changes 10. Evans, WH, RE Odom, and EJ Wenaas Krukenberg’s following laser iridotomy in pigmentary glaucoma. spindle: a study of 202 collected cases. Arch. Ophth 1941; 26:1023. 11. Speakman JS. Pigmentary dispersion. Br J Ophthalmol 1981; 65:249-51. 12. Dawczynski J, Koenigsdoerffer E, Augsten R, Strobel J. References Anterior optical coherence tomography: a non-contact technique for anterior chamber evaluation. Graefes Arch 1. Von Hippel E. Zur pathologischen Anatomie des Clin Exp Ophthalmol 2007; 245:423-5. Glaucoma. Arch Ophthalmol 1901; 52:498. 13. Radhakrishnan S, Huang D, Smith SD. Optical 2. Sugar HS. Concerning the chamber angle. I: Gonioscopy. coherence tomography imaging of the anterior chamber Am J Ophthalmol 1940; 23:853. angle. Ophthalmol Clin North Am 2005; 18(3):375-81. 3. Sugar HS and Barbour FA. Pigmentary glaucoma; a 14. Scheie HG, Cameron JD. Pigment dispersion syndrome: rare clinical entity. Am J Ophthalmol. 1949; 32(1):90-2. a clinical study. Br J Ophthalmol 1981; 65:264-9. 4. Campbell DG. Pigmentary dispersion and glaucoma: a 15. Thomas M. Richardson and Dianna H. Ausprunk. new theory. Arch Ophthalmol 1979; 97:1667–72. Pigmentary Dispersion Syndrome and Glaucoma. 5. Karickhoff JR. Reverse pupillary block in pigmentary Principles and Practice of Ophthalmology. 2nd glaucoma: follow-up and new developments. Edition:Chap 206;2731-40. Ophthalmic Surg. 1993;24:562–3.Mapstone R. Pigment 16. Roberts DK, Chaglasian MA, Meetz RE. Clinical signs release. Br J Ophthalmol 1981; 65:258-63. of the pigment dispersion syndrome in blacks. Optom 6. Dinc UA, Kulacoglu DN, Oncei B, Yalvac IS. Vis Sci 1997; 74:993-1006. Quantitative assessment of anterior chamber

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