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Keratoconus and Fuchs' heterochromic iridocyclitis: a coincidence or a defect during embryogenesis?

PURPOSE: We aimed to discuss the possible role of developmental embryologic factors in neural crest cells in the aetiology of keratoconus and Fuchs' heterochromic iridocyclitis by presenting this case. CASE REPORT: We diagnosed bilateral keratoconus and unilateral Fuchs' heterochromic iridocyclitis in a 19 year old women complaining of progressively blurring vision in her left eye. We also examined most of her first and second degree relatives. One niece had FHI in addition to a choroidal nevus in the inferior temporal quadrant of her left eye. DISCUSSION: Regarding the common embryological origins of iris stroma, uveal melanocytes and corneal stroma, it might be worth considering that the combination of FHI and keratoconus is not coincidental. A role of embryologic factors in neural crest cells in the etiology of both diseases cannot be excluded.

Adult↗

Cataract surgery in Fuchs' heterochromic iridocyclitis.

PURPOSE: To assess the particularities of extracapsular cataract extraction and posterior chamber intraocular lens implantation in patients with Fuchs' heterochromic iridocyclitis. METHODS: Retrospective analysis of 24 extracapsular cataract operations performed from September 1991 to June 1994. Our technique-a slight modification of Blumenthal's technique-included a triplanar watertight small scleral incision, an envelope capsulotomy, nucleus hydrodissection and hydroexpression, anterior chamber maintainer use, thorough residual cortex removal through a side-port corneal incision and "in the bag" posterior chamber intraocular lens implantation. RESULTS: Mydriasis was slightly larger than 5 mm in 79.16% of the total group. Posterior capsule rupture with vitreous loss occurred in one and without in two patients. A transient rise in intraocular pressure was seen in seven patients and a mild anterior chamber reaction in almost all patients. No bleeding occurred during anterior chamber opening, or postoperative I.O.L. decentration or glaucoma. Best corrected postoperative visual acuity ranged from 5-10/10 in 21 patients (87.50%). CONCLUSIONS: Extracapsular cataract extraction in patients with Fuchs' heterochromic iridocyclitis does not currently present any difficulties compared with normal senile cataract. No major complications occurred and the final visual acuity was satisfactory.

Adult↗

[A case of chronic iridocyclitis in young girls--surgery for complicated cataract and effect of cyclosporin A].

PURPOSE: To report a case of chronic iridocyclitis in young girls and the effect of systemic cyclosporin A for inflammation following surgery for complicated cataract. CASE: A 6-year-old girl presented with white uveitis, complicated cataract, and band keratopathy in both eyes. We diagnosed her as having chronic iridocyclitis in young girls. Eight months later, the anterior chamber in the left eye became flat. Ultrasound biomicroscopy (UBM) showed the presence of cyclitic membrane and adhesion of the ciliary body to the lens capsule. Phacoemulsification with pars plana vitrectomy was used to remove the lens, anterior vitreous, and cyclitic membrane. Severe inflammation and hypotony developed after surgery and persisted after systemic and topical corticosteroids. Systemic cyclosporin A induced rapid resolution of inflammation. CONCLUSION: Systemic cyclosporin A was useful for postoperative inflammation in this case of uveitis of young girls.

Cataract Extraction↗

Results of extracapsular cataract extraction with intraocular lens implantation in eyes with uveitis and Fuchs' heterochromic iridocyclitis.

A total of 63 cataractous eyes of 48 patients (group 1) with uveitis and 12 eyes of 12 patients (group 2) with Fuchs' heterochromic iridocyclitis underwent extracapsular cataract extraction (ECCE) with capsular bag fixation of a posterior-chamber intraocular lens (PC OIL). A perioperative regimen of systemic dexamethasone (0.8-1.5 mg/kg) was initiated. This dose was tapered by 20 mg/day, reaching zero within 4-8 days. Intraoperatively, synechiolysis was required in 51 of 63 eyes in group 1 and a sector iridectomy was necessary in 42 eyes. A fibrinous reaction occurred in 7 eyes. During a mean follow-up period of 2 years, 7 eyes developed uveitis recurrences that were successfully treated with topical and systemic steroids in 4 eyes; the other 3 eyes developed cystoid macular edema. Overall, mean visual acuity improved from 20/120 to 20/50. Intraoperatively, 3 patients in group 2 developed a transient hemorrhage from atypical anterior-chamber-angle vessels. Postoperatively, 3 eyes showed a transient fibrinous reaction. The mean visual acuity improved from 20/240 to 20/30. In patients with Fuchs' heterochromic iridocyclitis, ECCE with PC IOL implantation renders good intermediate results. In other selected patients with uveitis, in-the-bag IOL implantation may be performed with an acceptable risk. As judged from their improved visual acuity, 57 of 63 eyes (90.5%) benefited from the procedure.

Adult↗

The iridocyclitis of early onset pauciarticular juvenile rheumatoid arthritis: outcome in immunogenetically characterized patients.

In a cohort of 72 patients with iridocyclitis (iritis) and early onset pauciarticular juvenile rheumatoid arthritis (EOPA-JRA) the course of the eye disease was matched with ocular outcome. Chronicity of inflammation (greater than 6 months/episode) was correlated with complications of eye disease that caused impairment of vision. HLA antigens in these patients were compared with the HLA antigens in a cohort of 77 patients with EOPA-JRA in whom iridocyclitis had failed to develop over a followup of 5 years or longer. HLA-DR5 (11) was correlated with the presence of eye disease, and HLA-DR1 with its absence; HLA-DRw8, which strongly predisposes to EOPA-JRA, was neutral with respect to eye disease.

Arthritis, Juvenile↗

Longitudinal analysis of HLA associated risks for iridocyclitis in juvenile rheumatoid arthritis.

The risk of iridocyclitis in children with early onset pauciarticular juvenile rheumatoid arthritis (EOPA-JRA) has been shown to be associated with certain HLA haplotypes. Our report contains an actuarial analysis, using one-year intervals, of 161 subjects and estimates haplotype specific risks. Individuals who possess the major susceptibility haplotype HLA-DR5 (11) developed eye disease earlier and with a greater frequency than did those with the protective HLA-DR1 haplotype. Highly significant differences were found between the resulting life-table curves for HLA-DR5 and HLA-DR1 positive subjects (p = 0.00003). These time oriented risk estimates may aid clinicians in determining more precisely the probability of iridocyclitis throughout the course of the disease in children with EOPA-JRA.

Adolescent↗

Subtypes of HLA-DRB1*03, *08, *11, *12, *13 and *14 in early onset pauciarticular juvenile chronic arthritis (EOPA) with and without iridocyclitis.

A set of 200 patients with early onset pauciarticular juvenile chronic arthritis (EOPA-JCA) from Munich (165) and Prague (35) was investigated for the subtypes of HLA-DRB1*03, *08, *11, *12, *13 and *14. In addition, the relationship of DRB1, DQA1, DQB1 and DPB1 alleles with iridocyclitis in patients with EOPA-JCA was investigated. Subtyping for DRB1*03 was not informative, as all DR3 positive patients and all except one of the controls possessed DRB1*0301. Thus, the role of DRB1*0302 could not be assessed. The subtypes for DRB1*12, *13, and *14 did not reveal any statistically significant difference between patients and controls. In contrast, the subtype DRB1*1104 was the one most strongly associated with EOPA-JCA (chi 2 31.2, p value < 10(-6)). It appears that the subtype DRB1*1103 may also be associated with EOPA-JCA. The association of EOPA-JCA with DR8 is almost exclusively due to the subtype *0801. For the other alleles *0802, *0803, and *0804 there is no evidence for or against involvement in JCA. The analysis of iridocyclitis in EOPA-JCA revealed that DRB1*1104 is not more frequent in patients with eye disease than in patients without eye disease. The presence of DRB1*01 appears to convey some protective effect against the occurrence of iridiocylitis in EOPA-JCA, as had been previously observed by Melin-Aldana et al.

Age of Onset↗

An intraocular foreign body masquerading as idiopathic chronic iridocyclitis.

The authors report a case of presumed idiopathic chronic iridocyclitis in which an intraocular foreign body (IOFB) was finally identified. There was no history of prior eye trauma. Ophthalmologic and laboratory evaluations were inconclusive. Seven months following unsuccessful medical treatment of the iritis and given the patient's occupational history, a skull x-ray was performed followed by computed tomography of the orbits. A metallic IOFB was identified in the pars plicata and was surgically removed with resolution of the inflammation. The possibility of an IOFB should always be entertained in idiopathic iridocyclitis refractory to medical treatment, even when there is no history of ocular trauma.

Adult↗

Rubeosis in Fuchs heterochromic iridocyclitis.

A patient had Fuchs heterochromic iridocyclitis of approximately 17 years' duration. Histologically, keratic precipitates, iris atrophy, rubeosis iridis, discontinuous rubeosis of the anterior chamber angle, a chronic nongranulomatous iridocyclitis, and trabeculitis were noted. The cause of the glaucoma probably is a combination of rubeosis of the anterior chamber angle and trabeculitis.

Anterior Chamber↗

Herpetic iridocyclitis.

Twenty patients with presumptive herpetic iridocyclitis without active corneal inflammation received either topical idoxuridine 0.5% or acyclovir 3% ointment. The uveitis of all ten patients treated with acyclovir resolved within one to eight weeks. Four of ten patients treated with idoxuridine worsened but subsequently responded to topical prednisolone 0.3%. The pathogenesis of herpetic iridocyclitis is discussed with a literature review.

Acyclovir↗

Iridocyclitis associated with juvenile rheumatoid arthritis.

In a study of 45 patients, those with monarticular and pauciarticular juvenile rheumatoid arthritis, and with antinuclear antibodies, were likely also to have chronic, persistent iridocyclitis. In cases where iridocyclitis was present, topical mydriatics were used to prevent formation of posterior synechiae. Topical corticosteroids were used but discontinued if the uveitis failed to respond. Despite the generally poor prognosis for the vision of these patients, surgery for complicated cataract was often successful.

Administration, Topical↗

Lepromatous iridocyclitis diagnosed by anterior chamber paracentesis.

A 34-year-old man with a three-month history of intraocular inflammation after ocular trauma with a fir branch, had an acute unilateral fulminant iridocyclitis. The iris had a thick, gray, cheesy membrane composed of nodular lepromata. The patient denied a history of Hansen's disease, despite the dermatologic and facial features that suggested the diagnosis. Anterior chamber paracentesis and scleral nodule biopsy demonstrated Mycobacterium lepra. The iridocyclitis resolved after treatment with dapsone, corticosteroids, and rifampin.

Adult↗

A case of chronic iridocyclitis in young girls. Surgery for complicated cataract and effect of cyclosporin A

Purpose: To report a case of chronic iridocyclitis in young girls and the effect of systemic cyclosporin A for inflammation following surgery for complicated cataract.Case: A 6-year-old girl presented with white uveitis, complicated cataract, and band keratopathy in both eyes. We diagnosed her as having chronic iridocyclitis in young girls. Eight months later, the anterior chamber in the left eye became flat. Ultrasound biomicroscopy showed the presence of cyclitic membrane and adhesion of the ciliary body to the lens capsule. Phacoemulsification with pars plana vitrectomy was used to remove the lens, anterior vitreous, and cyclitic membrane. Severe inflammation and hypotony developed after surgery and persisted after systemic and topical corticosteroids. Systemic cyclosporin A induced rapid resolution of inflammation.Conclusion: Systemic cyclosporin A was useful for postoperative inflammation in this case of uveitis of young girls.

Journal Article↗

Corneal endothelial cell density in iridocyclitis.

The endothelial cells of both eyes of 60 unilateral iridocyclitic patients were photographed with a specular microscope to establish the possible effect of iridocyclitis on corneal endothelial cells. Chronic severe iridocyclitis with mutton-fat keratic precipitates (KP) lowered the central endothelial cell count. Five patients out of the seven with mutton-fat KPs had a distinctly lower central cell density in the affected eye. In the remaining patients, no significant difference in cell densities could be demonstrated between affected and healthy eyes. Neither the inflammatory process itself nor the round white KPs had a deleterious effect on the central corneal endothelial cell densities.

Adolescent↗

Iridocyclitis in black Americans: association with HLA B8 suggests an autoimmune aetiology.

Histocompatibility (HLA) testing was performed on a total of 171 black Americans, 129 controls, and 42 patients with iridocyclitis. None of the patients had a history of rheumatological disease. The phenotype frequency of HLA B8 was greatly increased among patients than the control group (exact p = 0.0010). Most striking was the almost identical phenotypic frequency of B27 found in patients and in the control group (exact p = 0.07087). No HLA DR antigen in the patient group was found to be significantly raised when compared with controls. The HLA B8 associated diseases was seen more in females, was bilateral and nongranulomatous, had no systemic disease associated with it, and left patients with visual handicap. The HLA B8 antigen has been associated with various entities of presumed autoimmune origin, and these data suggest that iridocyclitis, at least in blacks, may be of similar aetiology.

Adolescent↗

Metastatic carcinoma of the iris simulating iridocyclitis.

A 46-year-old woman with a history of breast carcinoma and no known metastatic disease presented with iridocyclitis and secondary glaucoma. Intraocular inflammation and pressure elevation persisted despite standard medical therapy, and paracentesis was performed. Cytological examination of the aspirate revealed adenocarcinoma. Subsequent studies disclosed no evidence of extraocular metastasis. Two courses of radiation therapy to the involved eye resulted in a dramatic reduction in intraocular inflammation and allowed temporary control of the intraocular pressure. Ultimately, however, progressive glaucoma necessitated enucleation. This case confirms previous statements that iridocyclitis may be the initial clinical manifestation of metastatic malignancy. In addition, this report emphasises the importance of paracentesis in the diagnostic evaluation of selected cases of anterior uveitis of unknown aetiology.

Adenocarcinoma↗

Antibodies to peptidoglycan in juvenile onset ankylosing spondylitis and pauciarticular onset juvenile arthritis associated with chronic iridocyclitis.

Using an indirect enzyme linked immunosorbent assay, antibodies of the IgG class to Streptococcus pyogenes group A peptidoglycan-polysaccharide polymers (PG-GSP) were measured in the sera of 37 patients with juvenile onset ankylosing spondylitis (JAS), 22 with pauciarticular onset juvenile arthritis associated with chronic iridocyclitis and 20 healthy children. Mean antibody activity, measured in arbitrary units, was of 184 U in JAS and 250 U in chronic iridocyclitis (p = NS). The results in both groups differed significantly from those found in healthy children (p less than 0.001 and p less than 0.01, respectively). This suggests either a possible role of bacterial cell wall products in the pathogenesis of these disorders or a state of immune reactivity to PG-GSP in these children.

Antibodies↗