Lens and Anterior Uvea

This chapter illustrates photos of clinical signs seen in uveitis and interesting cases of lens pathologies. Anterior uveitis is the inflammation of the iris and the ciliary body. Anterior uveitis can be idiopathic, isolated, or associated with systemic diseases. The clinical findings observed in anterior uveitis include keratic precipitates, inflammatory cells and flare in anterior chamber, hypopyon, rarely hyphema, miosis, iris nodules and atrophy, synechiae, and band keratopathy in chronic cases (shown in corneal degenerations chapter). The inflammation in anterior uveitis is almost always immune. Treatment includes steroid eye drops, cycloplegic drops, sub-Tenon steroid injections when cystoid macular edema is present. Chronic macular edema can be treated with intravitreal Triamcinolone injection and Dexamethasone implants. In cases of refractory anterior uveitis or associated immune systemic diseases, immunomodulatory treatment or biologic agents are prescribed.

Blau syndrome: a case report from Palestine

Background This case study documents the first familial case of Blau syndrome (BS) in Palestine characterized with mutation in CARD15/NOD2. Case presentation Eighteen years old female was initially misdiagnosed with Juvenile idiopathic arthritis (JIA). The patient had been on steroids and methotrexate treatment for the last 16 years, but did not respond well to treatment. Initial examination at Saint John of Jerusalem Eye Hospital Group clinic showed bilateral intermediate uveitis with camptodactyly. The patient’s sister (aged 19 years) had bilateral intermediate uveitis and camptodactyly. Both eyes of their father had signs of old posterior uveitis. Father’s left eye showed 360 degrees posterior synechia, mature cataract with old Keratic precipitates (KPs). He also had camptodactyly. The patient was referred to pediatric rheumatologist to rule out sarcoidosis. Lung CT scan showed bronchiectasis, genetic consultation followed. Complete eye examination, full history, refraction, and Optical coherence tomography (oct) were done. Systemic and topical steroid therapy could not control the ocular inflammation. The family then was referred to a geneticist. Genetic analyses showed that the proband and all three family members had an R334q mutation in the CARD15/Nod2 gene. Conclusions BS should be considered in the differential diagnosis of childhood uveitis, especially in low and middle income countries where it is misdiagnosed in many cases, which delay appropriate diagnosis and thus control. Genetic analysis of the CARD15/Nod2 gene is helpful in the diagnosis. Steroids alone are not enough to control the disease, other immunosuppressants and biologics are needed.

Corneal Edema with Anterior Uveitis after Exposure to the Sap of Euphorbia trigona: A Case Report

Although a few cases of dermatitis or keratitis caused by exposure to the sap of <i>Euphorbia trigona</i> have been reported, we present a rare case of transient corneal endothelial dysfunction following exposure to the sap, resulting in corneal edema. A woman in her 70s complained of reduced vision, redness, and teariness in her left eye 2 days after exposure to the sap of <i>E</i>. <i>trigona</i> at home. Upon examination, hyperemia, serious corneal edema, and anterior uveitis with hypopyon were observed in her left eye, without corneal epithelial defects or keratic precipitates. The best-corrected visual acuity (BCVA) was 2.0 (logarithm of the minimum angle of resolution), and the measured central corneal thickness (CCT) was 812 µm. The patient was treated with topical instillation of 1.5% levofloxacin and 0.1% dexamethasone to reduce intraocular inflammation and corneal edema. Three weeks later, the BCVA reached 0, the CCT was 519 μm, and the corneal endothelial cell density was 3,233 cells/mm². Six months after the injury, the patient had good visual acuity, and the cornea was completely transparent. No recurrence of corneal edema or anterior uveitis was observed. Exposure to the sap of <i>E</i>. <i>trigona</i> can lead to severe corneal edema with anterior uveitis, impairing visual acuity. Taking precautions to prevent the exposure of the eye to the sap of this plant is crucial.

Zoledronate-induced acute anterior uveitis: a three-case report and brief review of literature

Purpose This three-case report aims to highlight the ocular adverse effects induced by bisphosphonate therapy and to call clinicians’ attention. Methods Three cases of acute anterior uveitis secondary to the initial dose of zoledronate infusion were reported with focus on their symptoms, treatment regimens, and outcomes. A review of published reports provided a basis for discussion. Results Three cases of acute anterior uveitis were either bilateral or unilateral. They demonstrated typical manifestations of bisphosphonate-induced acute anterior uveitis such as eye pain, blurred vision, conjunctival and ciliary hyperemia, keratic precipitates, and flare in the anterior chamber. After topical corticosteroid-containing comprehensive treatments, these symptoms resolved completely without any vision loss and long-term sequelae. Conclusions Acute anterior uveitis may be part of the acute phase reaction induced by zoledronate. Patients should be informed of its symptoms in advance and be monitored closely during and after administration. Clinicians should have a good awareness of the zoledronate-associated acute anterior uveitis and to treat it in a prompt and appropriate manner.

Diagnosis of cytomegalovirus corneal endotheliitis using surgically removed Descemet’s membrane and endothelium despite negative results with aqueous humor PCR: a case report

Background Cytomegalovirus (CMV) has been known to cause unilateral corneal endotheliitis with keratic precipitates and localized corneal edema, iridocyclitis, and secondary glaucoma. CMV endotheliitis is diagnosed based on clinical manifestations and viral examination using qualitative polymerase chain reaction (PCR) of the aqueous humor. Case presentation An 80-year-old woman was referred to our department for bullous keratopathy. Pigmented keratic precipitates were found in the right eye without significant anterior chamber inflammation. After 8 months there was inflammation relapse with mutton fat keratic precipitates and PCR on aqueous humor was performed, with negative results for CMV, herpes simplex virus, and varicella zoster virus. Keratic precipitates disappeared with steroid instillation, and Descemet-stripping automated endothelial keratoplasty (DSAEK) was performed for the right eye. CMV-DNA was positive at 6.0 × 102 copies/ GAPDH 105 copies in real time PCR of corneal endothelial specimen removed during DSAEK with negative results for all the other human herpes viruses. After diagnosis of CMV corneal endotheliitis, treatment with systemic and topical ganciclovir was initiated and there was resolution of symptoms. No recurrence of iridocyclitis or corneal endotheliitis was observed at 6 months follow up. Conclusions This case report suggests that PCR should be performed using the endothelium removed during DSAEK for bullous keratopathy of an unknown cause, even if PCR for aqueous humor yields negative results.

When HIV Immunodeficiency and Heterochromia Confuse the Issue: Recurrent Zoster Uveitis Mistaken for Fuchs’ Uveitis

Purpose: We report a case with iris heterochromia misdiagnosed as Fuchs’ uveitis which finally turned out to be a unilateral zoster uveitis in an HIV-positive patient. Case Report: A 45-year old patient was seen for a recurrent right anterior uveitis treated with prednisolone 1% drops BID. The iris of the right eye was hypochromic and atrophic and several small granulomatous keratic precipitates (KPs) were present. After discontinuation of corticosteroid drops, severe uveitis developed with mutton-fat KPs, and laser flare photometry (LFP) increased from 20 to 50.3 ph/ms. He had presented with right zoster ophthalmicus two years earlier and HIV-serology revealed to be positive. Conclusion: Iris heterochromia is not a good disease-defining criterion for Fuch’s uveitis even when typical KPs are present and can lead to misdiagnosis. More reliable criteria including stellate KPs, low LFP values, absence of synechiae, vitreitis, and disc hyperfluorescence, all absent in this case, should be sought to confirm or exclude the diagnosis.

Ocular Toxocariasis Masquerading as Toxoplasmosis

PurposeTo report panuveitis due to Toxocara, with positive Toxoplasma serology. ObservationA nine-year-old boy presented with a panuveitis, intermittent headaches, and a visual acuity of 20/400 in his left eye. Slit lamp examination showed anterior chamber cells and flare without keratic precipitates (KPs), vitreous cells and veils, and optic disc edema with a blurred fundus view. Initial lab values were positive for toxoplasmosis. Topical cycloplegic and steroid eye drops, in addition to trimethoprim/sulfamethoxazole (TMP/SMX) treatment, was initiated. Four days later, oral corticosteroids were started. As inflammation cleared, the fundus examination showed an inferior tractional detachment, leading to an ultimate diagnosis of ocular Toxocariasis.Conclusion and ImportanceHigh false positives on Toxoplasma serology and diffuse vitritis from toxocariasis limiting retinal visualization may confuse the initial diagnosis.

Relationship between the higher inflammatory cytokines level in the aqueous humor of Fuchs uveitis syndrome and the presence of cataract

Background This study aims to compare the levels of intraocular cytokines between Fuchs uveitis syndrome (FUS) eyes and the senile cataract eyes. The association between inflammatory cytokine levels and cataract severity in FUS is evaluated to find the possible mechanism of cataract in FUS eyes. Methods A retrospective study of 28 eyes with FUS was performed. Auxiliary examinations were performed, including ophthalmic examinations, laser flare-cell photometry, and levels of inflammatory cytokines in the aqueous humor were measured. The control group included 25 eyes with senile cataract. Data on the aqueous humor inflammatory cytokines were compared between the two groups. The association between the aqueous humor cytokine levels and severity of posterior subcapsular cataract was assessed. Results There were 28 eyes with FUS in 27 patients. Unilateral involvement was noted in 26 patients (96.30%). Stellate keratic precipitates (KPs) were noted in 16 eyes (57.14%). Heterochromia was observed in 21.43% of affected eyes. Posterior subcapsular cataract (PSC) was observed in 16 of the 28 eyes. Eyes with FUS had significantly higher aqueous humor (AH) cytokine levels (VEGF, bFGF, IL-6, IL-8 and IL-10) compared with the control eyes (P < 0.05). There was a statistically significant positive correlation between the severity of cataract and IL-6 and IL-8 levels in the AH (τ = 0.664 and 0.634, respectively; P = 0.001, P = 0.002, respectively). Conclusions Expression of VEGF, bFGF, IL-6, IL-8 and IL-10 in the AH of FUS patients was significantly higher than in senile cataract eyes, and the aqueous humor levels of IL-6 and IL-8 were significantly positively associated with the severity of posterior subcapsular cataract. Our results imply that an inflammation mechanism may be involved in the early development of cataract in FUS.

An Atypical Presentation of Sympathetic Ophthalmia in an Intact Globe Following Mechanical Fall: A Case Report and Literature Review

Purpose: To describe an atypical case of sympathetic ophthalmia presenting after blunt trauma causing disinsertion of the iris in an intact globe. Methods: Case report. Results: A 71-year-old lady presented to the Emergency Department following a mechanical fall. On examination, she was noted to have periocular haematoma, subconjunctival haemorrhage, hyphaema, and vitreous haemorrhage in the left eye, but there was no evidence of globe rupture. The presenting visual acuity was 6/18. As the hyphaema and vitreous haemorrhage settled, a complete loss of the iris was noted with normal fundus. She was re-admitted a month later under the medical team with urinary tract infection and reduced vision in both eyes. On examination, there was mild conjunctival injection, keratic precipitates, anterior chamber flare, 180-degree posterior synechiae, and vitritis with no fundal view of the right eye. She was diagnosed with sympathetic ophthalmia and was treated with topical and systemic corticosteroid. Her vision improved gradually with treatment and was stable at 6/6 on the right (sympathising) eye and 6/9 on the left (excited) eye at final follow-up. Conclusion: Sympathetic ophthalmia may result from non-penetrating ocular trauma. Comprehensive history of mechanism of injury and ophthalmic examination is essential so that prompt treatment can be given to improve the visual prognosis of affected patients.