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Year : 1990  |  Volume : 38  |  Issue : 4  |  Page : 159-161

Clinico-pathologic correlates in Fuch's heterochromic iridiocyclitis-An iris angiographic study

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029, India

Correspondence Address:
L Verma
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110 029
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Source of Support: None, Conflict of Interest: None

PMID: 2086464

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Iris Angiography in a case of Fuchs' heterochromic iridocyclitis (FHI) is described. The finding of iris angiography and hylainzed iris vessels on histopathology suggested ischemic etiology of FHI. This is the first report on Iris Angiography in a pigmented eye with Fuchs' heterochromic iridocyclitis.

How to cite this article:
Verma L, Arora R. Clinico-pathologic correlates in Fuch's heterochromic iridiocyclitis-An iris angiographic study. Indian J Ophthalmol 1990;38:159-61

How to cite this URL:
Verma L, Arora R. Clinico-pathologic correlates in Fuch's heterochromic iridiocyclitis-An iris angiographic study. Indian J Ophthalmol [serial online] 1990 [cited 2023 Dec 1];38:159-61. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1990/38/4/159/25511

  Introduction Top

Fuchs' Heterochromic Iridocyclitis (FHI) occurs in quiet eyes and is almost always unilateral. It is the most misdiagnosed form of uveitis. [1] Various essential criteria and associated findings have been described which help in diagnosis of FHI. [2] Iris angiographic findings in FHI have been described from Western Countries. [4] How­ever, to the best of our knowledge, no report exists in literature regarding iris angiography in FHI from this subcontinent. In this communication, we are reporting angiographic and histological features in a patient of FHI.

  Case report Top

A 52-year-old woman presented to us with gradually progressive, painless diminution of vision i , the right eye of two years duration. There was no history of trauma, redness, photophobia or coloured haloes. She was a diabetic controlled on dietary therapy. Rest of the history regarding any other systemic illness was unremarkable.

On examination, the patient was of average built. Detailed examination of cardiovascular. respiratory and gastrointestinal systems did not reveal any abnormality.

Ocular examination revealed visual acuity of hand movement with an accurate projection in the right eye and 6/6 in the left eye. Examination of the left eye was essentially normal. Examination of right lid, eyeball, adenexa and conjunctiva was within normal limits. Cor­nea was normal in size, shape and transparency. Multi­ple, small - medium sized keratic precipitates (KP's) were seen diffusely distributed at the back of the right cornea. Few of the KP's showed brown pigmentation. Anterior chamber did not reveal any flare or cells. Right iris was slightly lighter in colour than the left with multiple transillumination defects. Pupil was 3 mm in size, round, central, with brisk reactions. Lens was totally opaque and in its normal position. Fundus examination was not possible in the right eye. Applanation intraocular pres­sure was 18 mm of Hg in both the eyes.

Systemic investigations done included blood sugar profile, urine examination. Blood sugar values were 107 mg% fasting and 142 mg% 2-hour post prandial. Urine was 1 +for sugar.

Ocular investigations included specular microscopy, ultrasonography, iris angiography, diurnal variation, out­flow facility (C-value) and Goldman perimetry. Values of diurnal variation and C-value were within normal limits in both the eyes. Field charting of left eye gave normal curves. Specular microscopy of the right eye revealed a cell count of 1300 cells per square millimeter with evidence of polymegathism, pleomorphism and guttate changes. Multiple keratic precipitates were also picked up. Corneal endothelial cell count in left eye was 2000 cells/sq mm without any abnormality. Contact A-Scan (Kretztechnik) of the right eye showed anechoic posterior segment. Iris fluorescein angiography of the right eye showed non-filling of temporal half of iris, multiple hyperfluorescent spots in the nasal mid­peripheral iris and peripupillary leak [Figure - 1]a. The peripupillary leak increased in the late phase [Figure - 1] b. In the left eye radial iris vasculature was not demonstrable. There was no evidence of any leak in the left eye.

The patient underwent routine extracapsular cataract extraction in right eve. Multiple, small hemorrhaaes were noted from angle area at 4, 6 and 8 O'clock areas during surgery. A peripheral iridectomy was done at 10 0; clock and iris tissue sent for histopathological ex­amination. Postoperatively, the patient had moderate degree of uveitis and secondary glaucoma which was controlled with topical betamethasone (0.1%) 2 hourly and timolal maleate 0.5% twice a day. Indirect ophthal­moscopic examination of the right eye revealed clear media with no posterior segment abnormality. Best cor­rected distant visual acuity, 2 week postoperatively was 6/9. Histopathological examination of iris specimen showed minimal mononuclear infilterate with hyalinized vessels [Figure - 2].

  Discussion Top

The presence of small, evenly distributed keratic precipitates, minimal anterior chamber reaction, heterochromia, no synechia, characteristic transil­lumination defects established clinical diagnosis of Fuch's heterochromic iridocyclitis in our patient.

Fuchs' heterochromic iridiocyclitis (FHI) is a unilateral lesion often misdiagnosed. It occurs in males and females in their third or fourth decades and accounts for 2% of all cases of uveitis. [1] It iss an important entity to consider, first, because it is often not recognised and second, because when recognised it is often over­treated. [1],[2],[3]

Fluorescein angiography has an important role in dis­ease entities like neovascularization of iris, iris tumours, diabetic retinopathy and iris ischemia. [4] Iris angiography has recently been employed in Fuchs' heterochromic iridocyclitis. [4],[8] These studies have been done in blue irides patients.

There is no report of iris angiography in brown colored iris with Fuchs' heterochromic iridocyclitis. It has been reported that Fluorescein angiography of iris is not pos­sible in pigmented irides because the pigment situated in the anterior stromal layer of iris hinders with the visualization of vessels. [4] The left eye of our patient showed no radial vascular pattern of iris. This substan­tiates the observation made by previous workers. 4,5 Age has been incriminated as an influencing factor in iris angiography, in lightly colored races. Ten to thirty percent of persons above the age of 50 years show peripupillary leakage from iris as a nonspecific ageing change. [4] Such age related fluorescein leakage from iris could not be picked up in the left eye (normal eye) of our brown iridic patient. In the right eye of our patient, hyperfluorescent spots in the nasal half of iris and dense peripupillary leak was seen [Figure - 1] a, b. This points towards a vascular insult to the iris vessels in FHI. The angiographic findings in the right eye of our patient further prove that although normal iris angiogram is difficult to obtain, the functional integrity of iris vascula­ture can be studied in darkly pigmented eyes.

The etiology of heterochromic iridocyclitis is a matter of dispute. Previously considered causes implicating abiotrophy, abnormal autonomic nervous system, micro-organism or some developmental anomaly have not been held because of insufficient and inconclusive evidence. [4] The Iris Angiographic findings [Figure - 1] a, b in the right eye of our patient and the demonstration of hyalinized iris vessels on histopathology [Figure - 2] sup­ports ischemic etiology of FHI. Similar observations have been made by previous workers. [4],[5],[7],[8] Hyalinized aterioles in iris stroma have been reported on pathologic studies from FHI patients. [7],[8] However, no report exists where an iris angiography has been performed in a pigmented iris with Fuchs' heterochromic iridiocyclitis. Our findings of sectorial nonfilling of radial vessels of iris and dense peripupillary leakage [Figure - 1] a,b are in conformity with the iris angiographic findings reported in light colored irides. [4] These findings also support the clinical impression that the inflammatory component of Fuchs' disease though mild is not confined to the ciliary body. In addition, the ischemic component of the anterior segment in FHI may well explain the ap­pearance of vessels in the angle, the tendency to bleed (occurence of a small filiform hemorrhage into the anterior chamber on diagnostic paracentesis, transient hyphema after cataract surgery) and the rubeotic reac­tion seen in terminal cases.

  References Top

Smith, R.E., Nozik, R.M. Uveitis, a clinical approach to Diagnosis and Management. The Williams and Wilkins, Baltimore, pp 185 - 187, 1983.  Back to cited text no. 1
Tabbut, B.R., Tessler, H.H., Williams, D. Fuchs Heterochromic Iridocyclitis in Blacks. Arch Ophthalmol, 106: 1688 - 1690, 1988.  Back to cited text no. 2
Lowenfeld, I.E., Thompson, H.S. Fuchs' Heterochromic cyclitis : A critical review of the literature : Clinical characteristics of the syndrome. Surv Ophthalmol, 17: 394 - 457, 1973.  Back to cited text no. 3
Kottow, M.H. Anterior Segment Fluorescein Angiography, The Williams and Wilkins Company. Baltimore, Maryland. Intraocular Inflammation, pp 120 - 121, 1978.  Back to cited text no. 4
Bergstorm, T.J., Rath, M., Martonyl, C.L.. Pigmented Iris Angiography. Acta Ophthalmol, 94 : 1180 - 1182. 1976.  Back to cited text no. 5
Lowenfeld, I.E., Thompson, H.S. Fuchs' Heterochromic cyclitis : A critical review of the literature: II, Etiology and Mechanisms. Surv Ophthalmol, 18 :2.61,1973.  Back to cited text no. 6
Kimura, S.T. Fuchs' Syndrome of Heterochromic Iridocyclitis in brown eyed patients. Trans Am Ophthalmol Soc, 76 : 76 - 78, 1978.  Back to cited text no. 7
Goldberg, M.F., Erozan, Y.S., Duke, J.R., Frost, J. K. Cytopathological and Histopathological aspects of Fuchs' Heterochromic iridocyclitis. Arch. Oph­thalmol, 74 : 604 - 609, 1965.  Back to cited text no. 8


  [Figure - 1], [Figure - 2]


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