eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Uveitis, Fuchs Heterochromic: Treatment & Medication

Author: Mansoor Arif, MB, BS, Research Officer, Department of Ophthalmology, Aga Khan University Medical College
Coauthor(s): C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution; Ira G Wong, MD, MS, Associate Director, Clinical Affairs, Uveitis Service, The Francis I Proctor Foundation for Research in Ophthalmology, University of California at San Francisco; Clinical Professor, Department of Ophthalmology, University of California at San Francisco and Stanford University School of Medicine
Contributor Information and Disclosures

Updated: Dec 11, 2008

Treatment

Medical Care

In general, treatment is not necessary for patients with the typical low-grade inflammation. Symptomatic flare-ups may require short-term topical corticosteroids; however, long-term therapy is not indicated. Unlike other uveitides, topical steroids should not be used to eliminate cells from the anterior chamber as part of the cells and flare is contributed by the breakdown of the blood-aqueous barrier and leakage of inflammatory infiltrate.

Surgical Care

Surgical decisions are related to the development of cataracts and glaucoma in patients with Fuchs heterochromic iridocyclitis (FHI).14

  • Cataracts associated with Fuchs heterochromic uveitis26
    • Overall, the surgical outcome of patients with FHI is equivalent to patients with age-related cataracts. Patients with FHI tend to have better outcomes following cataract extraction than patients with other forms of uveitis.
    • A small group of patients with FHI are at a higher risk for complications. Risk factors for complications include the following:
      • Rubeosis irides that lead to hyphema
      • Glaucoma may be more difficult to control following surgery.
      • Severe iris atrophy with transillumination defects tends to have worse postoperative inflammation.
      • Dilation of the pupil may be difficult.
      • Phacoemulsification tends to have a better outcome in terms of complications when compared to cataracts removed by extracapsular cataract extraction.
    • In planning for cataract extraction in patients with FHI, consider the following:
      • Small incision surgery is recommended to reduce surgical trauma.
      • Clear corneal incision is preferred to avoid blood vessels in the anterior chamber angle.
      • Slow decompression of intraocular pressure is indicated to reduce the risk of hemorrhage from abnormal iris blood vessels.
      • Peripheral iridectomy is not indicated.
      • An acrylic intraocular lens implant is preferred over a silicone lens to decrease the amount of pigment adhering to the lens postoperatively and to prevent uveitis relapse. If possible, the lens should be placed within the capsular bag.
      • Topical prednisolone acetate 1%, 4 times per day, for several days before and after surgery may blunt the inflammation associated with procedure.
  • Glaucoma associated with Fuchs heterochromic iridocyclitis  
    • The incidence of glaucoma ranges from 15-59%. Fortunately, most secondary glaucoma associated with FHI can be controlled with antiglaucoma medications. In the severe case, where 2 or 3 types of antiglaucoma medications cannot achieve control, surgical treatment should be considered.
    • In planning glaucoma surgery in patients with FHI, consider the following:
      • Argon laser trabeculoplasty does not appear effective in improving the outflow where trabecular sclerosis and peripheral anterior synechiae are present.
      • Glaucoma filtering procedures in patients with FHI are less successful compared with that for patients with primary open-angle glaucoma. The intraocular inflammation may lead to bleb failure; therefore, strict control of the inflammation with topical steroids during the perioperative period may improve the surgical outcome. Use of antimetabolites also may improve surgical results.
      • Glaucoma drainage implants may improve the outcome of glaucoma surgery for patients with uveitic glaucoma. 
  • Management of symptomatic vitreous opacities: Vitrectomy is advisable for visually significant vitreous infiltrates. It successfully eliminates symptoms of floaters and is associated with a better visual outcome when compared to vitrectomy in other uveitides.

Medication

For the infrequent episodes when inflammation increases to a moderate level, the use of a topical ophthalmic corticosteroid solution or suspension for a short period of time is warranted. Cycloplegics are not generally required.

Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.


Prednisolone acetate suspension 1% (Pred Forte)

Criterion standard with a fine 1-3 µm suspension particle size. Generic preparations may be significantly less potent because of markedly larger particle sizes, approximately 10 µm in diameter. The larger particles decrease effective exposure of drug to ocular surfaces, reducing absorption and potency. Rebounds may occur when switching from proprietary to generic prednisolone acetate 1% suspension on a dose-for-dose basis.
Initial dosage is titrated according to the degree of inflammation. Therapeutic goal is to reduce the inflammation to the level prior to the flare-up.
Drops should be spaced at least 5 min apart to avoid dilution effects. Gentle eyelid closure or punctal occlusion can significantly decrease upper airway irritation and systemic absorption. A second drop given immediately after the initial drop does not increase potency because the conjunctival cul-de-sac only holds about 10 microliters, and each drop contains 40 microliters.

Adult

1 gtt q2h day 1, then taper frequency over following days based on clinical response

Pediatric

Administer as in adults

Documented hypersensitivity; viral, fungal, or tubercular infections

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate)

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Have analgesic, anti-inflammatory, and antipyretic activities. Their mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms also may exist, such as inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, and various cell membrane functions.

Topical formulations provide reasonable anti-inflammatory therapeutic effect without the major adverse effects of steroids. Although very potent analgesics, NSAID drops are generally far less potent than steroids.


Diclofenac (Voltaren)

Inhibits prostaglandin synthesis by decreasing activity of enzyme cyclooxygenase, which, in turn, decreases formation of prostaglandin precursors. May facilitate outflow of aqueous humor and decrease vascular permeability.

Adult

1 gtt into affected eye qid; continue for a maximum of 2 wk

Pediatric

Not established

Additive effect with systemic NSAIDs may occur

Documented hypersensitivity; avoid during pregnancy

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Corneal thinning may occur


Ketorolac (Acular)

Inhibits prostaglandin synthesis by decreasing activity of the enzyme cyclooxygenase, resulting in decreased formation of prostaglandin precursors, which, in turn, results in reduced inflammation.

Adult

1 gtt into affected eye qid for up to 2 wk

Pediatric

Not established

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Perform ophthalmologic studies in patients who develop eye complaints during therapy; discontinue therapy if changes are noted; changes may include blurred or diminished vision, corneal deposits and retinal disturbances, scotomata, changes in color vision, and macula degeneration

More on Uveitis, Fuchs Heterochromic

Overview: Uveitis, Fuchs Heterochromic
Differential Diagnoses & Workup: Uveitis, Fuchs Heterochromic
Treatment & Medication: Uveitis, Fuchs Heterochromic
Follow-up: Uveitis, Fuchs Heterochromic
References

References

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  2. Jones NP. Fuchs Heterochromic Uveitis: A reappraisal of the clinical spectrum. Eye. 1991;5 (Pt 6):649-61. [Medline].

  3. Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. I. Clinical characteristics of the syndrome. Surv Ophthalmol. May-Jun 1973;17(6):394-457. [Medline].

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  6. Jurkunas UV, Bitar MS, Rawe IM. Co-localization of Increased Transforming Growth Factor Beta Induced Protein (TGFBIp) and Clusterin Expression in Guttae of Fuchs Endothelial Corneal Dystrophy Patients. Invest Ophthalmol Vis Sci. Nov 14 2008;[Medline].

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  15. Liesgang TJ. Fuchs uveitis syndrome. In: Pepose JS, Holland GS, Wilhelmus KR, eds. Ocular Infection & Immunity. 1995:495-506.

  16. Loewenfeld IE, Thompson HS. Fuchs heterochromic cyclitis: A critical review of the literature. II. Etiology and mechanisms. Surv Ophthalmol. 1973;18:2-61.

  17. Sungur G, Hazirolan D, Duman S, et al. Fuchs' heterochromic uveitis associated with retinal break or dialysis. Can J Ophthalmol. Feb 2008;43(1):109-10. [Medline].

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  19. de Groot-Mijnes JD, ten Dam-van Loon NH, Weersink AJ, et al. [Relationship between rubella virus and Fuchs heterochromic uveitis; 2 patients]. Ned Tijdschr Geneeskd. Nov 24 2007;151(47):2631-4. [Medline].

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  22. Teyssot N, Cassoux N, Lehoang P, et al. Fuchs heterochromic cyclitis and ocular toxocariasis. Am J Ophthalmol. May 2005;139(5):915-6. [Medline].

  23. Murray PI, Hoekzema R, van Haren MA, et al. Aqueous humor interleukin-6 levels in uveitis. Invest Ophthalmol Vis Sci. May 1990;31(5):917-20. [Medline].

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Further Reading

Keywords

Fuchs heterochromic uveitis, Fuchs' heterochromic uveitis, Fuchs heterochromic iridocyclitis, Fuchs' heterochromic iridocyclitis, FHI, Fuchs' heterochromic cyclitis, Fuchs' uveitis syndrome

Contributor Information and Disclosures

Author

Mansoor Arif, MB, BS, Research Officer, Department of Ophthalmology, Aga Khan University Medical College
Disclosure: Nothing to disclose.

Coauthor(s)

C Stephen Foster, MD, FACS, FACR, FAAO, Clinical Professor of Ophthalmology, Harvard Medical School; Consulting Staff, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary; Founder and President, Ocular Immunology and Uveitis Foundation, Massachusetts Eye Research and Surgery Institution
C Stephen Foster, MD, FACS, FACR, FAAO is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Association of Immunologists, American College of Rheumatology, American College of Surgeons, American Federation for Clinical Research, American Medical Association, American Society for Microbiology, American Uveitis Society, Association for Research in Vision and Ophthalmology, Massachusetts Medical Society, Royal Society of Medicine, and Sigma Xi
Disclosure: Nothing to disclose.

Ira G Wong, MD, MS, Associate Director, Clinical Affairs, Uveitis Service, The Francis I Proctor Foundation for Research in Ophthalmology, University of California at San Francisco; Clinical Professor, Department of Ophthalmology, University of California at San Francisco and Stanford University School of Medicine
Ira G Wong, MD, MS is a member of the following medical societies: American Academy of Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

John D Sheppard Jr, MD, MMSc, Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Program Director, Ophthalmology Residency Training, Eastern Virginia Medical School; President, Virginia Eye Consultants
John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

R Christopher Walton, MD, Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, Assistant Dean for Graduate Medical Education, University of Tennessee College of Medicine; Consulting Staff, Regional Medical Center, Memphis Veterans Affairs Medical Center, St Jude Children's Research Hospital
R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society
Disclosure: Nothing to disclose.

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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