Glaucoma in Fuchs' heterochromic cyclitis associated with congenital Horner's syndrome.

Abstract

SUMMARY We report a retrospective study of five patients with monocular Fuchs' heterochromic cyclitis associated with an ipsilateral Horner's syndrome. The minimum follow-up was 10 years. The presenting findings were cyclitis in three of the patients and heterochromia iridis associated with blepharoptosis in the other two. The major factors affecting all five patients were cataract and glaucoma. The intraocular pressure was uncontrolled even with maximal therapy, and antiglaucomatous surgery was performed in all cases. A short period of good postoperative control was followed by an intractable ocular hypertension, causing loss of useful vision in all patients. The remarkable combination of Horner's syndrome with glaucoma and their interaction is discussed.Fuchs' heterochromic cyclitis (FHC) is a commoner ocular disorder than has generally been recognised. f It is frequently overlooked when the heterochromia is slight or when all its clinical features are not present at the same time.One of the most serious complications of the disease is glaucoma, which has been observed in 18*6% of the studied cases.6 Although there is no unanimity on the prognosis for the glaucoma in this syndrome,357 recent communications"" suggest that it usually becomes resistant to medical therapy, and surgery is only moderately successful.The purpose of this report is to describe the manifestations of unilateral FHC associated with Horner's syndrome and complicated by ipsilateral glaucoma in five patients. To the best of our knowledge, no similar cases have been previously reported. We discuss the significance of the association and the implications regarding the unfavourable course of the glaucoma in these patients.Case reports CASE 1A 53-year-old male was first seen in May 1970 because of a progressive decrease in visual acuity,

Surgical Denervation of Ocular Sympathetic Afferents Decreases Local Transforming Growth Factor-β and Abolishes Immune Privilege

“…[13][14][15][16][17] In these patients the stimulus for inflammation might be an otherwise innocuous viral eye infection, which in the absence of ocular immune privilege unleashes an inflammatory response that ultimately be- Figure 4. Removal of the SCG results in reduced TGF-␤ levels in the AC.…”

“…12 This view is supported by clinico-pathological observations that patients with aberrant or absent sympathetic innervation of eye structures (eg, Horner syndrome) can develop a chronic inflammatory condition of the anterior chamber (AC) known as Fuch's iridocyclitis. [13][14][15][16][17] Although it has been hypothesized that this condition is caused by a propensity for certain intraocular viral infections, 18 -20 another potential explanation for this condition is that local mechanisms of immune privilege are compromised by the absence of sympathetic signals to ocular structures. Traditionally ocular immune privilege is thought to be mediated by classical immunosuppressive molecules such as TGF-␤, Fas ligand, thrombospondin-1, and neuropeptides such as ␣-melanocortin stimulating hormone (␣-MSH), vasoactive intestinal peptide (VSP), calcitonin gene-related peptide (CGRP).…”

Glaucoma in Fuchs' heterochromic uveitis: Aetiology, management and outcome

“…6 The response to that surgery has been reported as being reliable 12 or unre liable. 6 The relationship of the glaucoma to cataract surgery,11.13-15 or to other intraocular procedures16 has been a matter for concern. In this series of 27 patients with glaucoma , 15 have undergone cataract surgery.…”

“…8-11 trabeculitis9 and steroid induction. 6 The management of the glaucoma has been considered difficult , with drainage surgery being frequently necessary. 3.…”

Usher syndrome associated with Fuchs’ heterochromic uveitis

“…It is an uncommon condition, with estimates of incidence of about 0.2 (per 100,000) in the general population [1,2] and ranging between 1.5% to 4.5% of uveitis cases [3,4]. It has been described in association with multiple diseases including retinitis pigmentosa (RP) [5][6][7][8]. Development of FHU in patients with RP is uncommon, and since the first case was described by Winkler in 1940 [9] only 14 additional cases have been reported [3,[10][11][12][13][14][15].…”