ATYPICAL PRESENTATIONs OF Ocular toxocariasis

1. ATYPICAL PRESENTATIONs OF Ocular toxocariasis Juan Gabriel Santiago, MD Carmen Santos, MD University of Puerto Rico Department of Ophthalmology

2. Toxocara species • One of the most common parasitic uveitis • Toxocara canis • Intestinal parasite of dogs • Humans are not natural host • Ingestion of ova from soil and undercooked foods • Handling infected puppies or lactating female • Ocular disease of children and young adults • Systemic illness – Visceral larva migrans

3. Ocular Toxocariasis • Usually unilateral • Three typical clinical presentations • Chronic endophthalmitis • Posterior retinal granuloma • Peripheral retinal granuloma

4. Chronic Endophthalmitis • Young children • 1 - 9 years • Leukocoria • White and quiet eye • Reduced vision • Cataract • Hypopyon • Posterior synechiae • Retinal detachment

5. Posterior Retinal Granuloma • Older children • 4 - 14 years • ↓ vision and granuloma in posterior pole • White retinal or subretinal mass in macular or peripapillary area, usually dark center • Overlying degree of vitritis • Adjacent retina may be edematous, with localized serous RD

6. Peripheral Retinal Granuloma • Children, adolescents and young adults • 6 - 40 years • White inflammatory mass usually asymptomatic • May involve pars plana and ciliary body • Traction falciform fold from the mass toward the optic disk may be present

7. Examples of atypical presentation of ocular toxocariasis

8. Atypical Case #1 • 15 y/o male referred for sudden, severe visual loss in OS of 2 weeks duration • ROS - controlled BA • Had taken 80 mg prednisone X 2 days • Hx of neuroretinitis OD 3 years before • Retinal infiltrates • Optic nerve swelling • Extensive ID work-up negative • No treatment given

9. Atypical Case #1 • Visual acuity • OD - 20/70 • OS - HM • Optic nerve atrophy OD • OS - Severe posterior segment inflammation • Optic nerve swelling • Retinal infiltrates/macular star • Vasculitis

10. Atypical Case #1

11. Atypical Case #1 • Presumptive diagnosis • Neuroretinitis • Treatment • Systemic steroids – 80 mg continued • Posterior subtenon kenalog (PSTK) • Work-up

12. PPD - Neg VDRL - Neg FTA-ABS - Neg TORCH Toxoplasma - Neg HSV titers - Pos CMV- Neg Cat scratch - Neg Lyme disease - Neg MRI - Neg CXR - Neg ACE - WNL Lysozyme - Neg ANCA tests- Neg Sed Rate - 6 Atypical Case #1

13. Patient improved with steroid tx Steroids tapered very slowly over 10 months Granuloma formation Atypical Case #1

14. Last dose 2.5mg QOD x 3 months stopped on 9/24/03 Visual acuity - 20/50 Atypical Case #1

15. Severe reactivation 1 month later VA - CF Severe vitritis Granuloma larger Treatment Prednisone 80mg Cleocin 300mg QID Atypical Case #1

16. Improvement with tx Vitrectomy and vitreous biopsy recommended at BPEI Toxoplasma titers - Neg Atypical Case #1

17. Atypical Case #1

18. Vitrectomy and vitreous evaluation done at BPEI Vitreous positive for TOXOCARA by PCR Granuloma was excised Distended retina resulted in retinal hole VA- 20/400 Atypical Case #1 2-25-2004

19. Steroid tx tapered Retinal hole bigger Surrounding RD Repaired but the retina re-detached Repaired with silicone oil Poor visual outcome Atypical Case #1

20. Atypical Case #1 • Atypical presentation • Older age than usual • Bilateral disease • Posterior pole granuloma and endophthalmitic form • Prolonged and aggressive inflammatory course • Severe reactivation despite very slow tapering of steroids (10 months)

21. Atypical Case #2 • 66 y/o woman with hx of type II DM, HTN, and arthritis presented with progressive vision loss in her left eye. • Hx of having taken care of 2 puppies prior to onset of symptoms. Denied exposure to cats. • Visual acuity OS – 20/200 • Findings (Oct/2003): Peripapillary retinitis with granuloma-like lesion left eye

22. Atypical Case #2 • Presumptive diagnosis • Toxoplasmaperipapillary retinitis • Treatment • Antibiotics • Steroids • Workup

23. Atypical Case #2 • Workup • ToxoplasmaIgM Neg • ToxoplasmaIgG  Pos • BartonellaIgG (Cat scratch)  Pos • ACE  WNL • Lysozyme  Neg • CXR  Neg

24. Atypical Case #2 • Initial treatment was modified to cover Toxoplasma and Bartonella • Vibramycin • Rifampin • Daraprim • Prednisone • Inactive lesion several months later (April/2004)

25. Atypical Case #2 • November/2004 • Preretinal membrane • Surgery being considered • Improved VA - 20/400

26. Atypical Case #2 • May/2005 • Reactivation of lesion • VA – CF • Increased membrane formation • Vitreous cells +3 • Granuloma looked more defined • Toxoplasmatx restarted

27. Atypical Case #2 • September/2005 • No improvement with treatment • VA – HM • Sent to BPEI for PCR

28. Atypical Case #2 • BPEI Workup • Anterior chamber tap • Toxoplasma Neg • Bartonella  Neg • ToxocaraIgG (ELISA)  Pos • Serology • ToxoplasmaIgG  Pos • Bartonella  Neg • Syphilis  Neg • ToxocaraIgG  Neg

29. Atypical Case #2

30. Atypical Case #2 • Treatment at BPEI • Mebendazole – 5 day course trial • PSTK • Surgery #1 • PPV / MP / relaxing retinectomy / SO placement • Surgery #2 – 2 weeks later • SBR / PPL / 360º relaxing retinectomy / SO placement • Poor visual acuity despite treatment • VA – HM • Continued to form membranes with re-detachment of retina and VA of bare LP

31. Atypical Case #2 • Toxocariasis should be entertained in adults with active granulomatous inflammation and a suggestive hx. • Probably this 66 y/o patient is one of the oldest described to have active ocular toxocariasis. • Importance of ELISA or PCR testing of intraocular fluids for ocular toxocariasis, even if systemic antibodies are negative.

32. Summary and Conclusion • Think of toxocara infection whenever a granulomatous process is seen in the retina. • Diagnosis was missed in case 1 due to suggestive bilateral disease and unusual presentation. • Diagnosis was missed in case 2 due to old age of patient. • Both cases illustrate the importance of intraocular fluid testing, especially PCR, in cases of retinitis in which diagnosis is uncertain.

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