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The Official Scientific Journal of Delhi Ophthalmological Society
Bilateral Posner Schlossman Syndrome with Underlying Open Angle Glaucoma
Madhu Bhoot, Suneeta Dubey, Saptarshi Mukherjee
Dr. Shroff’s Charity Eye Hosptial, New Delhi, India
Corresponding Author:
Madhu Bhoot 
DNB
Dr. Shroff’s Charity Eye Hospital
5027, Kedar Nath Road, Daryaganj
New Delhi - 110002, India
Email id: drmadhubhoot@gmail.com
Published Online: 31-JAN-2019
DOI: http://dx.doi.org/10.7869/djo.420
Abstract
Purpose: To describe a case of Posner Schlossman Syndrome (PSS) with bilateral presentation with underlying open angle glaucoma. 
Case Report: A young patient presented with blurring of vision in the right eye. On examination, he had mild anterior chamber reaction and open angles on gonioscopy. IOP was 30mm Hg in the right eye and 11mm Hg in the left eye. He had recurrent episodes of IOP spikes in the right eye despite being on ocular hypotensives. Trabeculectomy was done in the right eye. Few months later, he had similar episodes of elevation of IOP with inflammation in the left eye also for which he underwent trabeculectomy. Following trabeculectomy, his IOP was well controlled in both the eyes. 
Conclusion: PSS should be considered in any patient with increased IOP without any other signs because the inflammation may be so mild as to be overlooked or it may follow the acute rise in IOP by some days. Though PSS is unilateral, a bilateral presentation should also be kept in mind. Follow up of the patients should be at regular intervals and they need to have their visual fields and optic discs frequently monitored because of the recurrent nature and progression of the optic disc damage.
Keywords : Posner Schlossman, Bilateral, Open angle, Glaucoma, Inflammation
Introduction
Posner Schlossman Syndrome (PSS), also known as glaucomatocyclitic crisis, is characterised by recurrent episodes of mild non granulomatous anterior uveitis, which produces unilateral spikes in intraocular pressure (IOP). This is a rare clinical entity with uncertain aetiology. A significant number of patients develop glaucoma over time. This case report describes a patient with an unusual presentation of bilateral Posner Schlossman syndrome which progressed with time. 

Case Report
A 40 year old male presented to our hospital with the complaints of blurring of vision in the right eye for the last 4 months. He was previously diagnosed with glaucoma in the right eye with acute anterior uveitis in both the eyes elsewhere, one month prior to this visit. He had completed a course of topical steroids for a month. His current medications included Alphagan P (brimonidine tartarate 0.1%), Dortas (dorzolamide 2 %) in the right eye and Timolet (timolol maleate 0.5%) in both eyes. The patient had also completed a course of treatment for tuberculosis, 19 years back. He had no known allergies and no significant family history of ocular disease.
On examination, his best corrected visual acuity was 6/12, N6 in the right eye and 6/6, N6 in the left eye. Pupils were equal in diameter with brisk direct and consensual responses. There was no RAPD. Preliminary and gross external examinations were within normal limits.
Anterior segment evaluation of the right eye showed grade 1 cells in the anterior chamber with mild posterior sub-capsular cataract whereas the left eye showed a clear lens with a few pigments on the anterior lens capsule. IOP was 30 mm Hg in the right eye and 11 mm Hg in the left eye with Goldmann applanation tonometry. Gonioscopy revealed open angles in both eyes. Optic nerve head examination showed an average sized disc with cup disc ratio of 0.8:1 with bipolar notch in the right eye and cup disc ratio of 0.5:1 with healthy neuro-retinal rim in the left eye. 
Central corneal thickness was 469 µm and 472 µm in the right and left eye respectively. The patient underwent baseline Humphrey visual field threshold testing which showed severe constriction in the right eye and normal fields in the left eye. 
With a working diagnosis of secondary open angle glaucoma in the right eye, following an attack of uveitis, treatment was initiated. He was advised to stop his previous medication and was prescribed topical steroids in tapering doses and topical prostaglandin analogues along with topical and oral carbonic anhydrase inhibitors for his right eye. Two weeks later, his IOP was 27 mm Hg in right eye and 10 mm Hg in left eye. Fundus examination revealed increase in optic disc cupping to 0.9:1 in the right eye (Figure 1). So a combination of alpha-2 agonist and beta blockers was added to his treatment regimen in the right eye. Despite being on maximally tolerated medical therapy for one month, his IOP was uncontrolled, so he was advised surgical treatment with trabeculectomy in the right eye. Following trabeculectomy his IOP was under control, ranging between 8-10 mm Hg.
Six months later, he had an episode of IOP spike in the left eye. His IOP was 10 mm Hg in the right eye and 26 mm Hg in the left eye. Slit lamp examination showed grade 1 cells in the anterior chamber of the left eye. Optic disc examination showed subtle increase in cupping with thinning of the superior neuro-retinal rim and optic disc haemorrhage in the left eye.(Figure 2). Visual field examination showed similar results as recorded earlier. A prostaglandin analogue was prescribed for his left eye. In the following 14 months period, his IOP was under control ranging from 8-10 mm Hg in the right eye and 14-16 mm Hg in the left eye.



However, 2 months later he had yet another episode of high IOP (30 mm Hg in left eye) associated with mild anterior chamber reaction. Optic disc examination showed a cup disc ratio of 0.9:1 in the right eye and a cup disc ratio of 0.7:1 with further thinning of the superior neuro-retinal rim (Figure 3). Visual field testing showed slow progression in the right eye and severe progression in the left eye (Figure 4 a,b) (Figure 5 a,b,c) (Figure 6 a,b,c).







Based on the above findings, a provisional diagnosis of advanced secondary open angle glaucoma with possible bilateral PSS was made. In view of his uncontrolled IOP & visual field changes in the left eye, he was advised surgical treatment with trabeculectomy in the left eye also. The IOP was stable at 8-10 mm Hg in both eyes on the follow up visits without any anti-glaucoma medication and the patient continues to be seen at 3 months intervals.

Discussion
PSS was first reported by Adolph Posner & Abraham Schlossman in 1948.1 It is characterized by episodes of acute unilateral spikes in IOP associated with mild anterior chamber reaction. PSS accounts for 2.5% of all cases of anterior uveitis. In 92% of patients, the disease is found to be unilateral,3 though rarely it may occur simultaneously or may manifest in both eyes at different times.2,4
Patients with PSS may have concomitant primary open angle glaucoma which may lead to optic nerve head damage and visual field changes. There are well documented reports on this.5,6,7 In one study it was seen that 45% of PSS patients had underlying POAG.5 PSS patients can also develop chronic secondary open angle glaucoma. Jap et al found that patients with more than 10 years of PSS had 2.8 times greater risk of developing glaucoma.8
The patient described here fits into the typical age group as it usually affects individuals between the second and fourth decade with male predominance. It is also clear from this report that there is a risk of subsequent glaucomatous development with repeated IOP spikes and that it can manifest in both eyes at separate times. 
The characteristic feature of PSS is that the IOP does not correlate to the amount of inflammation in the eye. Patients usually present with ocular discomfort and blurred vision or halos around lights. On examination, there is mild anterior chamber reaction with corneal epithelial oedema with little or no hyperaemia. Small, round, non pigmented keratic precipitates may be seen in the lower third of the corneal endothelium. Gonioscopy reveals normal, open angles without abnormal pigmentation. Diagnosis of PSS can thus be challenging due to the mild nature of the inflammation. Our patient had episodes of mild inflammation along with recurrent episodes of IOP spikes in both eyes, one followed by the other. 
The aetiology and pathophysiology of PSS remains unknown. The acute IOP elevation in PSS is thought to be due to trabeculitis and subsequent infiltration of the trabecular meshwork by mononuclear cells. Several theories have been postulated. However, role of cytomegalovirus and herpes simplex virus infections has frequently been reported.9,10
Treatment of patients with PSS includes  a combination of topical steroids to decrease the inflammation and ocular hypotensive agents to decrease the IOP. Preferred choice of ocular hypotensive agents are topical beta blockers, alpha-2 agonists and carbonic anhydrase inhibitors. PG analogues can be used, but with caution.4,11 Our patient was on maximally tolerated medical therapy which was insufficient in controlling the IOP, so surgical treatment with trabeculectomy was finally done in both eyes. Our patient responded well to trabeculectomy with antimetabolites. 
The differential diagnosis of PSS is Fuch’s heterochromic uveitis, phacolytic glaucoma, Herpes simplex uveitis, acute angle closure glaucoma and acute anterior uveitis.

Conclusion
PSS should be considered in any patient with increased IOP without any other signs because the inflammation may be so mild as to be overlooked or it may follow the acute rise in IOP by some days. Though PSS is unilateral, a bilateral presentation should also be kept in mind. Follow up of the patients should be at regular intervals and they need to have their visual fields and optic discs frequently monitored because of the recurrent nature and progression of the optic disc damage. 

References
  1. Posner A, Schlossman A. Syndrome of unilateral recurrent attacks of glaucoma with cyclitic symptoms. Arch Ophthal 1948; 39:517-535. 
  2. Levatin, P. Glaucomatocyclitic crises occurring in both eyes. Am J Ophthalmol 1956; 41:1056-1059. 
  3. Sangha, SS. Posner Schlossman Syndrome. Ophthalmology 2002; 109:409.
  4. Subramanian D, Kayarkar V. Trabeculectomy in the management of Posner-Schlossman Syndrome. Ophthalmic Surg Lasers 2002; 33:321-322. 
  5. Kass MA, Becker B, Kolker AE. Glaucomatocyclitic crisis and primary open-angle glaucoma. Am J Ophthalmol. 1973; 75:668–673.
  6. Raitta C, Vannas A. Glaucomatocyclitic crisis. Arch Ophthalmol 1977; 95:608–612. 
  7. Varma R, Katz LJ, Spaeth GL. Surgical treatment of acute glaucomatocyclitic crisis in a patient with primary open-angle glaucoma. Am J Ophthalmol 1988; 105:99–100.
  8. Jap A, Sivakumar M, Chee SP. Is Posner-Schlossman syndrome benign? Ophthalmology 2001; 108:913–918.
  9. Teoh SB, Thean L, Koay E. Cytomegalovirus in aetiology of Posner-Schlossman syndrome: evidence from quantitative polymerase chain reaction. Eye (Lond) 2005; 19:1338–1340. 
  10. Bloch-Michel E, Dussaix E, Cerqueti P, Patarin D. Possible role of cytomegalo-virus infection in the etiology of the Posner-Schlossmann syndrome. Int Ophthalmol 1987; 11:95–96.
  11. Shazly TA, Aljajeh M, Latina MA. Posner-Schlossman glaucomatocyclitic crisis. Semin Ophthalmol 2011; 26:282-4. 
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Madhu Bhoot, Suneeta Dubey, Saptarshi MukherjeeBilateral Posner Schlossman Syndrome with Underlying Open Angle Glaucoma.DJO 2019;29:54-58
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Madhu Bhoot, Suneeta Dubey, Saptarshi MukherjeeBilateral Posner Schlossman Syndrome with Underlying Open Angle Glaucoma.DJO [serial online] 2019[cited 2019 Feb 20];29:54-58. Available from: http://www.djo.org.in/articles/29/3/Bilateral-Posner-Schlossman-Syndrome-with-Underlying-Open-Angle-Glaucoma.html
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