A case of spikes and specks

1. A case of spikes and specks Grand Ward Round 18 October 2007

2. History • 65/Chinese/male • First presented Jul 05 with LE pain, left-sided headache • Examination • VA 6/9 • Ac shallow, cells 2+. Gonio: closed angles bilaterally • Mid dilated pupil. “Moth-eaten” appearance of iris noted. ( L RAPD documented later) • NS 1+ • IOP 62mmHg • CDR 0.3 RE, 0.7LE

3. History • Impression: • L Acute on chronic ACG • R PAC • Treated medically • IOP subseq 35 • Underwent R/L PI next day

4. Progress • From Jul 05 to Aug 07 • Had 6 episodes of IOP spikes • AC inflammation (cells 1-2+), KPs noted during each spike • Responded well to steroids and anti-glaucoma therapy • Episodes usually occurred when off steroids or on tailing dose (BD) • Maintained on • Gutt Timolol 0.5% bd LE • Gutt alphagan bd LE  changed to Gutt Travatan May 06 (?reaction to alphagan) • Increase in CDR, stable L nasal step on HVF. VA 6/21  6/15

6. Thoughts?

7. Differentials • Uveitis and glaucoma • While most uveitic conditions can lead to glaucoma • Those a/w acute rise in IOP: • Herpetic uveitis • HSV • VZV • CMV • Posner-Schlossman Syndrome • Fuch’s heterochromic iridocyclitis

8. Progress • Another episode of high IOP (40mmHg) in Sept 2007 • L AC tap performed • Tetraplex PCR • Positive for CMV. CMV DNA 1.4E+06 copies/mL • HSV, VZV, Toxoplasma neg

9. Progress • L phaco/IOL/ Ahmed tube/MMC 10/10/07 with intravitreal ganciclovir • IOP not controlled despite 3 anti-glaucoma meds, control of inflammation with steroids • CDR 0.8 – 0.9 • Cataract with deteriorating VA (6/45) • At last visit • IOP 9mmHg • VA 6/30  6/21

10. Discussion • Differentials of hypertensive iridocyclitis • When is investigation (AC paracentesis) indicated? • How to treat CMV uveitis?

12. Features • Described in 1948 by Posner and Schlossman • “Glaucomatocylitic crisis” characterised by self-limited recurrent episodes of markedly elevated IOP with mild AC inflammation • IOP elevation out of proportion to degree of AC inflammation • Usually in adults 20-50 yrs • Previously thought to be idiopathic, but postulated aetiologies include • Abnormal vascular process • Autonomic defect • Infective: HSV, CMV

13. Features • Chronic unilateral (bilateral in 10%) iridocyclitis • Classic triad of • Iris heterochromia • KPs • Cataract • Low grade inflammation which does not usu req Rx • Postulated aetiology • Adrenergic dysfunction • Infective cause: link between ocular toxoplasmosis and FHI • Immunologic theories

15. CMV and the eye • CMV retinitis in immunocompromised hosts • HIV/AIDs, immunosuppressive drugs • Increasing evidence for CMV as cause of hypertensive iritis in immunocompetent patients • Local experience • Case report by S Teoh: Patient with PSS and incr IOP, aqueous PCR positivefor CMV Teoh SB, Thean L, Koay E. Cytomegalovirus in aetiology of Posner-Schlossman syndrome: evidence from quantitative polymerase chain reaction. Eye 2005 Dec; 19 (12): 1338-40 • Case series by Chee SP: 12 immunocompetent pt with corneal endothelitis and incr IOP. AC tap in 11/12 +ve for CMV DNA. Chee SP, Bascal K et al. Corneal endothelitis associated with evidence of cytomegalovirus infection. Ophthalmology 2007 Apr; 114(4): 798-803. • Several other case reports and case series Van Boxtel LA et al. Cytomegalovirus as a cause of anterior uveitis in immunocompetent patients. Ophthalmology 2007; 114(7): 1358-62 De Shryer I et al. Diagnosis and treatment of cytomegalovirus iridocyclitis without retinal necrosis. Br J Ophthalmol 2006;90: 852-5.

16. CMV and the eye • However, role of CMV in hypertensive iritis has been questioned • Most of the cases reported received local immunosuppressive therapy (e.g. steroids) prior to AC tap • CMV detected in aqueous may be a consequence of that • CMV may not be the aetiologic agent • Latent CMV is present in monocytes which transit through ocular tissues

17. Investigation of hypertensive iritis • When to tap? • No consensus • But probably reasonable to tap when high rate of recurrence, poor response to therapy, visual cx or deterioration • To tap or not to tap? • AC paracentesis is an intraocular procedure • Risks: cataract, endophthalmitis • Evidence that AC paracentesis generally safe • 361 patients underwent diagnostic tap • No sight-threatening side effects e.g. cataract, endophthalmitis, keratitis • 72 pt examined within 30min after tap: 5 had small hyphaema Van der Lelik A, Rothova A. Diagnostic anterior chamber paracentesis in uveitis: s safe procedure? Br J Ophthalmol 1997; 81 (11): 976-9

18. Treatment of CMV uveitis • Treatment of infective component • Intravitreal ganciclovir4 • Systemic ganciclovir 1,5 • Oral Valganciclovir 1-2 • Foscarnet 1 • Treatment of complications • Glaucoma: anti-glaucoma meds or surgery • Treatment of inflammation • Topical steroids • De Shryer I et al. Diagnosis and treatment of cytomegalovirus iridocyclitis without retinal necrosis. Br J Ophthalmol 2006;90: 852-5. • Van Boxtel LA et al. Cytomegalovirus as a cause of anterior uveitis in immunocompetent patients. Ophthalmology 2007; 114(7): 1358-62 • Mietz H et al. Ganciclovir for the treatment of anterior uveitis. Graefes Arch Clin Exp Ophthalmol 2000 Nov; 238(11): 905-9. • Chung RS, Chua CN. Intravitreal ganciclovir injections in aqueous cytomegalovirus DNA positive hypertensive iritis. Eye 2006 Sep; 20(9): 1080 • Chee SP, Bascal K et al. Corneal endothelitis associated with evidence of cytomegalovirus infection. Ophthalmology 2007 Apr; 114(4): 798-803.

19. Stellate KPs • Seen in AC inflammation due to • Fuch’s heterochromic iridocyclitis • Herpetic uveitis • Toxoplasmosis • Not diagnostic, but useful for differential diagnoses • What are they? • Fibrin deposition around inflammatory cells Walter KA, Coulter VL, Palay DA et al. Corneal endothelial deposits in patients with cytomegalovirus retinitis. Am J Ophthalmol 1996;232: 391-396 Pillai CT et al. Evaluation of corneal endotheluim and kertic precipitates by specular microscopy in anterior uveitis. Br J Ophthalmol 2000; 84:1367-1371