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Posner-Schlossman Syndrome

Posner-Schlossman Syndrome. Bianka Sobolewska, MD Manfred Zierhut, MD Centre of Ophthalmology University of Tuebingen , Germany. Ocular History. 49 year old healthy white women 1999: OD recurrent anterior uveitis IOP up to 60 mmHg no response to oral aciclovir.

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Posner-Schlossman Syndrome

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  1. Posner-Schlossman Syndrome Bianka Sobolewska, MD Manfred Zierhut, MD Centre of Ophthalmology University of Tuebingen, Germany

  2. OcularHistory • 49 yearoldhealthywhitewomen • 1999: OD • recurrentanterioruveitis • IOP upto 60 mmHg • noresponseto oral aciclovir

  3. April 2010: First Presentation • VA: OD 20/63, OS 20/20 • IOP: OD 38 mmHg, OS 17 mmHg • OD: non-granulomatouscentral KPs, irispigmentdefect, anteriorchambercells 1+, posteriorsubcapsularcataract, theiridocorneal angle open with pigment • OS: regular • Fundus: • OD/OS: regular with physiologicexcavationoftheoptic nerve

  4. non-granulomatouscentral KPs April 2010: First Presentation

  5. First Presentation - Diagnostics • negative anteriorchamber fluid analysis(PCR) for • CMV • Epstein-Barr virus • herpessimplexvirus • varicellazostervirus • all othertests negative: serologyforsyphilis, borreliosisand Bartonella, Quantiferontest, ANA, ANCA, chest CT

  6. Diagnosis • CMV- indducedanterioruveitis • based on: • Clinical findings with classical Posner-Schlossman Syndrome behaviourofintraocularpressure • Missingresponsetoacyclovir

  7. Treatment • topicalantiglaucomatosa • prednisoloneeyedrops (3x/day) • systemicvalganciclovir • 3 weeks: 900 mg b.i.d. • followedby 450 mg b.i.d.

  8. Follow up – After 1-6 MonthsMai-October 2010 • norecurrencesincethestartofvalganciclovirtherapy • VA: OD 20/1000 • IOP: OD 16-17 mmHg • onlytwoold KPs, nocells, posteriorsubcapsularcataract • therapy: • reductionoftopicaltherapy • systemicvalganciclovir 450 mg b.i.d • clearcornealphacoemulsification 11/2010

  9. Follow up – After 7 MonthsOctober 2011 • OD: 20/20 • norecurrence • terminationofvalganciclovirtherapy

  10. Follow up – After 11 MonthsMarch 2012 • recurrence with IOP of 40 mmHg, • anteriorchambercells 1+ • therapy • valganciclovir (450 mg 2x/day • topicalrimexolone (4x/day) • topicalganciclovir (5x/day) • acetacolamide (2x125 mg/day)

  11. Follow-up – After 1-2 YearsApril 2012 – April 2013 • IOP 14-17 mmHg • norecurrence • reductionoftopicaltherapy • terminationof oral therapy in 04-2013

  12. Follow-up – After 28 MonthsAugust 2013 • recurrence with IOP of 60 mmHg • acetacolamide 2x250 mg/day • starttherapy with leflunomide 20mg/d • topicaltherapy with • antiglaucomatosa, • prednisolone 3x/day • ganciclovir 3x/day

  13. Follow-up – After 29 MonthsSeptember 2013 • twicerecurrenceswith IOP of 60 mmHg • stopofleflunomide (headache, nausea) • topicaltherapywith • antiglaucomatosa • prednisolone 3x/day • ganciclovir 3x/day

  14. Follow-up – After 30- 42 MonthsOctober 2013-October 2014 • norecurrences • reductionoftopicaltherapy

  15. Final Ocular Investigation – After 42 MonthsOctober 2014 • VA: 0.7 • anteriorchambernocells • opticdisc: physiologicalexcavation • notopicaltreatmentbesidesarteficialtears

  16. Final Diagnosis • Possner-Schlossman Syndrome (PSS) • Differential diagnosis • Herpeticanterioruveitis (HSV, VZV) • Fuchs` uveitis

  17. Problems • rare disease • oftenmisdiagnosed • negative AC tap (PCR) does not exclude PSS • uncleartreatmentregimenwhen AC tapis negative • recurrences after terminationofsystemicvalganciclovirtherapyoftenreported

  18. Conclusion – CMV anterior Uveitis • Clinical signscanmimicother viral uveitisdisorders • When AC tapis negative but theclinicalsignsare suggestive for PSS, systemicacyclovirineffective: systemicvalganciclovirisrecommended • In caseofrecurrences, oral valganciclovirtherapywith additional topicalganciclovircanberepeated • In long-lasting undiagnosed PSS treatedonly with aciclovir, theresponsetovalgancilovirseems limited

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