Fuchs' Heterochromic Iridocyclitis

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Fuchs' heterochromic iridocyclitis (FHI) is a chronic, low grade, usually unilateral, ocular inflammatory disease with a good visual prognosis.

Disease Entity[edit | edit source]

Fuchs’ heterochromic iridocyclitis 364.21 Disease synonyms: Fuchs’ heterochromic cyclitis, Fuchs’ uveitis syndrome, Fuchs’ heterochromic uveitis

Disease[edit | edit source]

Fuchs’ heterochromic iridocyclitis is an unusual form of chronic, low grade anterior uveitis with variable clinical appearance. Clinical criteria for establishing the diagnosis of FHI has not yet been internationally accepted.

Etiology[edit | edit source]

Unclear. Associations with ocular toxoplasmosis, herpes simplex virus, rubella virus and CMV infections have been suggested. Other associated diseases include ocular trauma, retinitis pigmentosa, Horner syndrome, Status disraphicus and hemifacial atrophy aka Parry-Romberg syndrome.

Risk Factors[edit | edit source]

None known

General Pathology[edit | edit source]

The iris shows a decrease in the number of stromal melanocytes with patchy depigmentation of the posterior layer. The ciliary body shows stromal fibrosis with muscular atrophy.

Pathophysiology[edit | edit source]

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Primary prevention[edit | edit source]

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Diagnosis[edit | edit source]

Clinical diagnosis of exclusion based on history and physical findings. Laboratory testing is of no value.

History[edit | edit source]

Floaters and visual deterioration.

Physical examination[edit | edit source]

• Heterochromia with typically the lighter iris indicating the eye of involvement. This generalization varies depending on the intensity of anterior stromal atrophy, iris color, and amount of pigment in the iris pigmented epithelium. • Diffuse iris stromal atrophy with variable pigment epithelial layer atrophy • Iris nodules occur in 20% of cases localized to either the pupillary margin (koeppe) or on the surface of the iris (busacca) • Small white stellate KP diffusely scattered over endothelium • Anterior chamber inflammation with possible spill over into anterior vitreous (66%) • Fine vessels over the iris and crossing the trabecular meshwork that may result in hyphema after anterior chamber paracentesis, surgery, mydriasis, ocular trauma, gonioscopy, applanation tonometry of even spontaneously (Amsler’s sign). • Lack of anterior synechiae

Signs[edit | edit source]

Heterochromia, low grade anterior uveitis, cataract

Symptoms[edit | edit source]

Range from none to mild blurry vision, floaters and ocular discomfort

Clinical diagnosis[edit | edit source]

By history and clinical examination significant for the findings mentioned above.

Diagnostic procedures[edit | edit source]

Anterior chamber paracentesis is considered safe and aqueous humor antibody analysis is useful when the diagnosis cannot be determined based on clinical findings alone. Detection for rubella-specific antibodies in the aqueous humor of patients suspected to have Fuchs is not specific, but absence would make the diagnosis highly unlikely

Laboratory test[edit | edit source]

There are no specific serological tests for FHI but one must consider Sarcoidosis, Syphilis and TB when appropriate

Differential diagnosis[edit | edit source]

¥ Glaucomatocyclitic crisis aka Posner-Schlossman syndrome ¥ Phacolytic glaucoma ¥ Sympathetic heterochromia ¥ Heterochromia due to Duane’s syndrome or Waardenburg syndrome ¥ Neovascular glaucoma ¥ Herpes keratouveitis


Management[edit | edit source]

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General treatment[edit | edit source]

¥ Few cases require therapy because the anterior chamber inflammation is low grade and minimally responsive to ocular corticosteroids.


Medical therapy[edit | edit source]

¥ Occasional and short-term usage of topical corticosteroids may be indicated if a symptomatic exacerbation of uveitis occurs. ¥ Cycloplegics are seldom required ¥ Glaucoma associated with Fuchs’ is often responsive to medical therapy.

Medical follow up[edit | edit source]

Should be monitored at regular intervals for progression of cataract and development of glaucoma.

Surgery[edit | edit source]

¥ Intraocular inflammation should be reasonably well controlled prior to cataract surgery but it is not necessary to achieve complete absence of cell and flare. A reasonable perioperative treatment might include topical corticosteroids six times daily for 7 days prior to surgery. If there is a history of herpes simplex virus in the eye, the patient should receive a systemic antiviral during the corticosteroid prophylaxis period. The surgical procedure of choice is phacoemulsification with implantation of an intraocular acrylic lens in the capsular bag. At the completion of surgery, a subconjunctival injection of corticosteroids and antibiotics should be given and intensive topical corticosteroid and mydriatic therapy initiated. ¥ Secondary glaucoma may require surgical intervention if unable to medically manage IOP. ¥ Trabeculectomy with intraoperative mitomycin C is a reasonable first choice . Combined cataract extraction and glaucoma surgery may lead to increased failure rate. If possible, the cataract surgery should be performed first. Glaucoma drainage devices may afford a better prognosis.

Surgical follow up[edit | edit source]

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Complications[edit | edit source]

¥ Glaucoma, typically chronic open-angle glaucoma, complicates in 9-59% of cases. Mechanisms include trabeculitis, neovascularization of the iris stroma and angle, steroid treatment, and cataract surgery. ¥ Cataracts, most commonly posterior subcapsular, occur in 80% of patients and are often present at the time of initial diagnosis. They usually also form in the setting of corticosteroid therapy. ¥ Glaucoma as a result of cataract extraction and IOL implementation occurring from 3-35%. ¥ Cystoid macular edema occurring after cataract surgery ¥ Vitreous debris resulting in symptomatic floaters in 12-50%. ¥ Significant vitreous opacification following uncomplicated cataract surgery may be corrected with pars plana vitrectomy. ¥ Post-operative hyphema due to abnormal blood vessels

Prognosis[edit | edit source]

Good with most patients maintaining a visual acuity of 20/40 or better in the affected eye.


Additional Resources[edit | edit source]

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References[edit | edit source]

• Basic and Clinical Science Course. Intraocular inflammation and Uveitis. p 132-134 • Bonifioli AA, Curi AL, Orefice F. Fuchs’ heterochromic cyclitis. Semin Ophthalmol 2005; Jul-Sep; 20(3): 143-6 • Velilla S, Dios E, Herreras JM, Calonge M. Fuchs’ heterochromic iridocyclitis: a review of 26 cases. Ocul Immunol Inflamm. 2001 Sep;9(3):169-75.  • Tejwani S, Murthy S, Sangwan VS. Cataract extraction outcomes in patients with Fuchs’ heterochromic cylitis. J Cataract Refract Surg. 2006 Oct;32(10):1678-82. • Kimura SJ, Hogan MJ, Thygeson P. Fuchs’ syndrome of heterochromic cyclitis. AMA Arch Ophthalmol 1955; 54:179-186