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New England Eye Centre Grand Rounds Emma C. Massicotte, MD April 5, 2001
New England Eye Centre  Grand Rounds <ul><li>A 42 year old white female was referred to the New England Eye Centre cornea ...
New England Eye Centre  Grand Rounds <ul><li>Past medical history: None </li></ul><ul><li>Past ocular history </li></ul><u...
New England Eye Centre  Grand Rounds <ul><li>Social history </li></ul><ul><ul><li>Executive Assistant at a large company <...
New England Eye Centre  Grand Rounds <ul><li>Examination </li></ul><ul><ul><li>Best corrected acuity was 20/20 OD and 20/2...
New England Eye Centre  Grand Rounds
New England Eye Centre  Grand Rounds <ul><li>Examination </li></ul><ul><ul><li>There was mild right upper lid ptosis. </li...
New England Eye Centre  Grand Rounds <ul><li>Patient was reassured that her right eye looked good postoperatively.  </li><...
New England Eye Centre  Grand Rounds <ul><li>Upon examination there was increased anterior chamber inflammation with 2+ ce...
New England Eye Centre Grand Rounds <ul><li>Anterior Segment OD </li></ul>
New England Eye Centre Grand Rounds <ul><li>Examination revealed persistent 1+ cell and flare in the anterior chamber OD a...
New England Eye Centre  Grand Rounds <ul><li>What is the differential diagnosis? </li></ul>
New England Eye Centre Grand Rounds <ul><li>Differential Diagnosis of heterochromia (where involved iris is darker than no...
New England Eye Centre Grand Rounds <ul><li>Differential Diagnosis: causes of posterior subcapsular cataract </li></ul><ul...
New England Eye Centre Grand Rounds <ul><li>The patient was diagnosed with Fuchs’ Heterochromic Iridocyclitis. </li></ul><...
New England Eye Centre Grand Rounds <ul><li>Fuchs’ Heterochromic Iridocyclitis (FHI) </li></ul><ul><ul><li>chronic low-gra...
New England Eye Centre Grand Rounds - Clinical Signs <ul><li>heterochromia (75-90% ) </li></ul><ul><ul><li>most often hypo...
New England Eye Centre Grand Rounds <ul><li>Histopathologic appearance of the iris:  From: Ophthalmic Pathology and Intrao...
New England Eye Centre Grand Rounds - Clinical signs <ul><li>other iris features </li></ul><ul><ul><li>flattened iris text...
New England Eye Centre Grand Rounds – Clinical signs <ul><li>Iridocyclitis: </li></ul><ul><ul><li>low grade anterior chamb...
New England Eye Centre Grand Rounds – Symptoms <ul><li>decreased vision  30-52% </li></ul><ul><li>floaters  26-33% </li></...
New England Eye Centre Grand Rounds - Pathogenesis <ul><li>unknown - controversial, early theories include: </li></ul><ul>...
New England Eye Centre Grand Rounds – Treatment <ul><li>Iridocyclitis: </li></ul><ul><ul><li>corticosteroid use is limited...
New England Eye Centre Grand Rounds - Treatment <ul><li>Cataract </li></ul><ul><ul><li>In the late 1960’s cataract extract...
New England Eye Centre Grand Rounds – Treatment <ul><li>Considerations for FHI cataract extraction: </li></ul><ul><ul><li>...
New England Eye Centre Grand Rounds – Conclusion <ul><li>A 42 year-old female was diagnosed with Fuchs’ heterochromic irid...
New England Eye Centre Grand Rounds - References <ul><ul><li>Jones, NP: Fuchs’ heterochromic uveitis: A reappraisal of the...
New England Eye Centre Grand Rounds – References <ul><ul><li>Ophthalmic Pathology and Intraocular Tumors, American Academy...
New England Eye Centre Grand Rounds – References <ul><ul><li>Jones NP. Fuchs’ Heterochromic Uveitis: An Update. Survey of ...
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Heterochromia

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  1. 1. New England Eye Centre Grand Rounds Emma C. Massicotte, MD April 5, 2001
  2. 2. New England Eye Centre Grand Rounds <ul><li>A 42 year old white female was referred to the New England Eye Centre cornea service for a second opinion. </li></ul><ul><li>The patient complained that since cataract surgery OD three weeks prior her upper lid was drooping, her iris was darker in colour and she was seeing floaters, flickers of light and halos around lights at night. </li></ul><ul><li>The patient was very unhappy with the outcome of her surgery and wanted her posterior chamber intraocular lens implant (PCIOL) removed. </li></ul>
  3. 3. New England Eye Centre Grand Rounds <ul><li>Past medical history: None </li></ul><ul><li>Past ocular history </li></ul><ul><ul><li>s/p uncomplicated phacoemulsification with PCIOL implantation for posterior subcapsular cataract (3 weeks prior to presentation) </li></ul></ul><ul><li>Systemic medications: None </li></ul><ul><li>Ocular medications </li></ul><ul><ul><li>ketorolac OD qd </li></ul></ul><ul><ul><li>prednisolone acetate 1% OD qd </li></ul></ul><ul><li>Allergies: none known </li></ul>
  4. 4. New England Eye Centre Grand Rounds <ul><li>Social history </li></ul><ul><ul><li>Executive Assistant at a large company </li></ul></ul><ul><ul><li>has smoked 1/2 pack of cigarettes a day X 20 years </li></ul></ul><ul><ul><li>no ETOH </li></ul></ul><ul><li>Family history </li></ul><ul><ul><li>denied eye diseases or medical problems in immediate family </li></ul></ul>
  5. 5. New England Eye Centre Grand Rounds <ul><li>Examination </li></ul><ul><ul><li>Best corrected acuity was 20/20 OD and 20/20 +2 OS. </li></ul></ul><ul><ul><li>Manifest refraction: -1.75 +0.50 X 65 OD, -0.50 +0.50 X35 OS </li></ul></ul><ul><ul><li>Pupils were 5 mm OD, 4 mm OS, reactive OU without APD. </li></ul></ul><ul><ul><li>Intraocular pressures by applanation tonometry were 15 mm Hg OD and 13 mm Hg OS. </li></ul></ul><ul><ul><li>Extraocular movements were full OU </li></ul></ul>
  6. 6. New England Eye Centre Grand Rounds
  7. 7. New England Eye Centre Grand Rounds <ul><li>Examination </li></ul><ul><ul><li>There was mild right upper lid ptosis. </li></ul></ul><ul><ul><li>The conjunctiva were white and quiet OU. </li></ul></ul><ul><ul><li>The iris OD was slightly darker in colour than OS. </li></ul></ul><ul><ul><li>The anterior chamber OD was deep with 1-2+ cells. The anterior chamber OS was deep and quiet. </li></ul></ul><ul><ul><li>The PCIOL OD was in good position with trace posterior capsular haze. The crystalline lens OS was clear. </li></ul></ul><ul><ul><li>Dilated fundus exam was normal OU. </li></ul></ul>
  8. 8. New England Eye Centre Grand Rounds <ul><li>Patient was reassured that her right eye looked good postoperatively. </li></ul><ul><li>She was continued on prednisolone acetate 1% OD qd and ketorolac OD qd each for one more week. </li></ul><ul><li>She returned 2.5 weeks later with the same complaints of right upper lid droop, floaters, darker right iris and larger pupil OD. </li></ul><ul><li>She had no ocular discomfort or photophobia. </li></ul>
  9. 9. New England Eye Centre Grand Rounds <ul><li>Upon examination there was increased anterior chamber inflammation with 2+ cells and 2+ flare OD. There were also 1+ anterior vitreous cells OD. </li></ul><ul><li>The remainder of the exam, including dilated fundus exam was unchanged. </li></ul><ul><li>She was restarted on prednisolone acetate 1% OD q2 hours while awake and ketorolac OD qid. </li></ul><ul><li>She returned 2 weeks later, admitting at that time she was using prednisolone acetate 1% bid only. </li></ul>
  10. 10. New England Eye Centre Grand Rounds <ul><li>Anterior Segment OD </li></ul>
  11. 11. New England Eye Centre Grand Rounds <ul><li>Examination revealed persistent 1+ cell and flare in the anterior chamber OD and 1+ vitreous cells. </li></ul><ul><li>New fine keratic precipitates were present on the inferior corneal endothelial surface OD. </li></ul><ul><li>There was trace pigment on the anterior capsule OD. </li></ul><ul><li>She was kept on Prednisolone acetate 1% OD q 2 hours while awake and ketorolac OD qid. </li></ul><ul><li>Two weeks later (2.5 months after cataract extraction), the low grade inflammation remained and the keratic precipitates were no longer present. </li></ul>
  12. 12. New England Eye Centre Grand Rounds <ul><li>What is the differential diagnosis? </li></ul>
  13. 13. New England Eye Centre Grand Rounds <ul><li>Differential Diagnosis of heterochromia (where involved iris is darker than normal) </li></ul><ul><ul><li>Fuchs’ heterochromic iridocyclitis (less common than involved iris being lighter in color) </li></ul></ul><ul><ul><li>ocular melanocytosis </li></ul></ul><ul><ul><li>Hemosiderosis </li></ul></ul><ul><ul><li>Siderosis </li></ul></ul><ul><ul><li>retained intraocular foreign body </li></ul></ul><ul><ul><li>ocular malignant melanoma </li></ul></ul><ul><ul><li>Leukemia, lymphoma </li></ul></ul>
  14. 14. New England Eye Centre Grand Rounds <ul><li>Differential Diagnosis: causes of posterior subcapsular cataract </li></ul><ul><ul><li>Age </li></ul></ul><ul><ul><li>Trauma </li></ul></ul><ul><ul><li>Inflammation </li></ul></ul><ul><ul><li>use of steroids </li></ul></ul><ul><ul><li>ionising radiation </li></ul></ul>
  15. 15. New England Eye Centre Grand Rounds <ul><li>The patient was diagnosed with Fuchs’ Heterochromic Iridocyclitis. </li></ul><ul><li>The prednisolone acetate 1% was slowly tapered over 3 months. Follow up exam (6 months after cataract extraction) showed persistent low grade (1+ cells) anterior chamber inflammation. </li></ul>
  16. 16. New England Eye Centre Grand Rounds <ul><li>Fuchs’ Heterochromic Iridocyclitis (FHI) </li></ul><ul><ul><li>chronic low-grade inflammatory condition characterized by heterochromia, cataract, keratic precipitates, vitreous opacities, and glaucoma. </li></ul></ul><ul><ul><li>usually unilateral (90-95%) </li></ul></ul><ul><ul><li>accounts for 2-5% of uveitis cases </li></ul></ul><ul><ul><li>no gender or ethnic predisposition but may be under-diagnosed in African-Americans because heterochromia is difficult to detect in dark irides </li></ul></ul><ul><ul><li>age of diagnosis between 20-60 years, mean age 40 </li></ul></ul>
  17. 17. New England Eye Centre Grand Rounds - Clinical Signs <ul><li>heterochromia (75-90% ) </li></ul><ul><ul><li>most often hypochromia due to loss of pigment from the anterior border layer and stroma. </li></ul></ul><ul><ul><li>rarely (as in this case), a blue-eyed patient may display hyperchromia as atrophy of overlying iris reveals the darker iris pigment epithelium. </li></ul></ul>
  18. 18. New England Eye Centre Grand Rounds <ul><li>Histopathologic appearance of the iris: From: Ophthalmic Pathology and Intraocular Tumors, American Academy of Ophthalmology, Basic and Clinical Science Course 1999-2000 </li></ul>
  19. 19. New England Eye Centre Grand Rounds - Clinical signs <ul><li>other iris features </li></ul><ul><ul><li>flattened iris texture, especially near pupil </li></ul></ul><ul><ul><li>Pupil occasionally enlarged </li></ul></ul><ul><ul><li>iris nodules </li></ul></ul><ul><ul><li>posterior synechiae are rare, except after cataract surgery </li></ul></ul><ul><ul><li>prominence of normal iris vessels due to stromal atrophy </li></ul></ul><ul><ul><li>fine, filamentous “rubeotic vessels” (6-22%). Likely responsible for “Amsler’s sign” - limited hyphaema which may occur on paracentesis of the anterior chamber in patients with FHI. </li></ul></ul>
  20. 20. New England Eye Centre Grand Rounds – Clinical signs <ul><li>Iridocyclitis: </li></ul><ul><ul><li>low grade anterior chamber reaction (50%) </li></ul></ul><ul><ul><li>vitreous cells or opacity (66% - 84%) </li></ul></ul><ul><ul><li>keratic precipitates (83-96%) </li></ul></ul><ul><li>Posterior subcapsular cataracts </li></ul><ul><ul><li>detected in 80-90% of patients followed for mean of 9 years </li></ul></ul><ul><li>Glaucoma </li></ul><ul><ul><li>between 26-59%, usually chronic open angle </li></ul></ul><ul><ul><li>other mechanisms: peripheral anterior synechiae, rubeosis, lens-induced angle closure, recurrent hyphaema, steroid response </li></ul></ul>
  21. 21. New England Eye Centre Grand Rounds – Symptoms <ul><li>decreased vision 30-52% </li></ul><ul><li>floaters 26-33% </li></ul><ul><li>discomfort 13-19% </li></ul><ul><li>conjunctival injection 11 % </li></ul><ul><li>no symptoms 13 % </li></ul>
  22. 22. New England Eye Centre Grand Rounds - Pathogenesis <ul><li>unknown - controversial, early theories include: </li></ul><ul><ul><li>sympathetic dysfunction, hereditary factors, intrauterine toxins, trauma, maternal illness, infectious, and lens induced inflammation </li></ul></ul><ul><ul><li>ocular toxoplasmosis ? </li></ul></ul><ul><ul><li>reported prevalence of toxoplasmosis-like lesions in FHI range from 7.5% to 65% </li></ul></ul><ul><ul><li>conflicting studies - needs further investigation </li></ul></ul><ul><li>immunologic mechanism? </li></ul><ul><ul><li>aqueous humour analysis in FHI shows increased levels of IgG </li></ul></ul><ul><ul><li>immune complexes, and IL-6. immune response trigger unknown </li></ul></ul>
  23. 23. New England Eye Centre Grand Rounds – Treatment <ul><li>Iridocyclitis: </li></ul><ul><ul><li>corticosteroid use is limited, inflammation usually persists </li></ul></ul><ul><li>Glaucoma </li></ul><ul><ul><li>most serious complication </li></ul></ul><ul><ul><li>In a large series by Jones, 63% of FHI glaucoma was medically managed, 22% required trabeculectomy and 4 eyes enucleated. </li></ul></ul><ul><ul><li>trabeculectomies have increased risk of bleb failure. </li></ul></ul><ul><ul><li>ALT response has been anecdotally reported as poor </li></ul></ul><ul><li>Vitreous opacities </li></ul><ul><ul><li>vitrectomy may be considered if visually significant </li></ul></ul>
  24. 24. New England Eye Centre Grand Rounds - Treatment <ul><li>Cataract </li></ul><ul><ul><li>In the late 1960’s cataract extraction was reported to be associated with more frequent complications (glaucoma, postoperative intraocular haemorrhage, vitreous opacificaton) in FHI cataracts than in normal senile cataracts. (note: mostly intracapsular technique) </li></ul></ul><ul><ul><li>More recent studies using newer surgical techniques (extracapsular, PCIOL) report a more favourable outcome </li></ul></ul>
  25. 25. New England Eye Centre Grand Rounds – Treatment <ul><li>Considerations for FHI cataract extraction: </li></ul><ul><ul><li>Amsler sign: reported in 10-75% of patients </li></ul></ul><ul><ul><li>postoperative inflammation may be worse than in general population </li></ul></ul><ul><ul><li>patients may become more aware of vitreous opacities postoperatively </li></ul></ul><ul><ul><li>beware transient rise intraocular pressure postoperatively </li></ul></ul><ul><ul><li>CME is uncommon in comparison to other types of uveitis. </li></ul></ul><ul><ul><li>PCIOLs have low rate of lens-related complications, ACIOL and iris-fixated lenses should be avoided. </li></ul></ul>
  26. 26. New England Eye Centre Grand Rounds – Conclusion <ul><li>A 42 year-old female was diagnosed with Fuchs’ heterochromic iridocyclitis after presenting with hyperchromia, keratic precipitates, and persistent mild anterior chamber and anterior vitreous reaction following posterior subcapsular cataract extraction </li></ul>
  27. 27. New England Eye Centre Grand Rounds - References <ul><ul><li>Jones, NP: Fuchs’ heterochromic uveitis: A reappraisal of the clinical spectrum. Eye 5:649, 1991 </li></ul></ul><ul><ul><li>Liesegang TJ: Clinical features and prognosis in Fuchs’ uveitis syndrome. Arch Ophthalmol 100:1622, 1982 </li></ul></ul><ul><ul><li>Rutzen, AR and Raizman MB: Fuchs’ Heterochromic Iridocyclitis: In: Principles and Practice of Ophthalmology, Albert and Jakobeic, chapter 96, page 1294-1306, 2000 </li></ul></ul><ul><ul><li>Tabbut BR, Tessler HH, Williams D: Fuchs’ heterochromic iridocyclitis in blacks. Arch Ophthalmol 106: 1688, 1988 </li></ul></ul><ul><ul><li>Jones NP: Glaucoma in Fuchs' heterochromic uveitis: Aetiology, management and outcome. Eye 5:662, 1991 </li></ul></ul>
  28. 28. New England Eye Centre Grand Rounds – References <ul><ul><li>Ophthalmic Pathology and Intraocular Tumors, American Academy of Ophthalmology, Basic and Clinical Science Course 1999-2000, Section 4, page 154 </li></ul></ul><ul><ul><li>Waters FM et al. Vitrectomy for vitreous opacification in Fuchs’ heterochromic uveitis. Eye (2000) 14, 216-218 </li></ul></ul><ul><ul><li>La hey E, de Jong PTVM, Kijlstra A. Fuchs’ heterochromic cyclitis: review of the literature on the pathogenetic mechanisms. British J of Ophthalmol 1994: 78: 307-312 </li></ul></ul><ul><ul><li>Jakeman CM et al. Cataract Surgery with Intraocular Lens Implantation in Fuchs’ Heterochromic Cyclitis. Eye (1990) 4, 543-547 </li></ul></ul>
  29. 29. New England Eye Centre Grand Rounds – References <ul><ul><li>Jones NP. Fuchs’ Heterochromic Uveitis: An Update. Survey of Ophthalmol. 1993, 37:4, 253-272 </li></ul></ul><ul><ul><li>Schwab IR. Fuchs’ Heterochromic Iridocycliltis. International Ophthalmology Clinics, 30:4: 252-256, 1990 </li></ul></ul><ul><ul><li>Dernouchamps JP et al, Immune complexes in the aqueous humour and serum. Am J Ophthalmol 84:24, 1977 </li></ul></ul><ul><ul><li>Murray PI et al. Analysis of aqueous humor immunoglobulin G in uveitis by enzyme-linked immunosorbent assay, isoelectric focusing, and immunoblotting. Invest Ophthalmol Vis Sci 31:2129, 1990. </li></ul></ul><ul><ul><li>Murray PI et al. Aqueous humor interleukin-6 levels in uveitis. Invest Ophthalmol Vis Sci31:917, 1990 </li></ul></ul>
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