Detección de la progresión de la retinopatía externa oculta zonal aguda (AZOOR) con angiografía por tomografía de coherencia óptica: a propósito de un caso / Detecting progression of acute zonal occult outer retinopathy (AZOOR) with optical coherence tomography angiography: A case report

Caso clínico Mujer de 48años con escotomas superotemporales persistentes y fotopsias de 2meses de evolución y zonas despigmentadas en retina de ambos ojos con patrón trizonal en imagen multimodal. La resonancia magnética cerebral, la tomografía por emisión de positrones, los anticuerpos antirretinianos y los marcadores inmunológicos, infecciosos y tumorales fueron negativos, por lo que se diagnosticó retinopatía externa oculta zonal aguda. La paciente fue tratada con adalimumab. Sin embargo, 19meses después los síntomas aumentaron y se detectó progresión en la angiografía por tomografía de coherencia óptica, así como también en la prueba de campo visual Humphrey y el electrorretinograma, por lo que se agregó micofenolato de mofetilo, mostrando mejoría y estabilización de la enfermedad durante un seguimiento de 4años. Discusión La angiografía por tomografía de coherencia óptica puede ser una herramienta potencial para monitorizar la progresión y la respuesta al tratamiento además de otras modalidades de imagen en la retinopatía externa oculta zonal aguda, y la combinación de adalimumab y micofenolato puede ser útil en la enfermedad recurrente (AU)

Clinical case A 48-year-old woman with persistent superotemporal scotomas and photopsias for 2months, and depigmented zones in the retina of both eyes with a trizonal pattern on multimodal imaging. Brain magnetic resonance imaging, positron emission tomography, antiretinal antibodies and immunological, infectious and tumor markers tests were negative, thus acute zonal occult outer retinopathy was diagnosed. Patient was treated with adalimumab. Nevertheless, 19months later symptoms increased, and progression was detected on optic coherence tomography angiography, as well as in Humphrey visual field test and electroretinogram, thus, mycophenolate mofetil was added showing improvement and stabilization of the disease in a 4-year follow-up. Discussion Optic coherence tomography angiography may be a potential tool to monitor progression and response to treatment in addition to other imaging modalities in acute zonal occult outer retinopathy, and the combination of adalimumab and mycophenolate may be useful in recurrent disease (AU)

Detecting progression of acute zonal occult outer retinopathy (AZOOR) with optical coherence tomography angiography: A case report.

CLINICAL CASE: A 48-year-old woman with persistent superotemporal scotomas and photopsias for 2 months, and depigmented zones in the retina of both eyes with a trizonal pattern on multimodal imaging. Brain magnetic resonance imaging, positron emission tomography, antiretinal antibodies, immunological, infectious and tumor markers tests were negative, thus acute zonal occult outer retinopathy was diagnosed. Patient was treated with adalimumab. Nevertheless, 19 months later symptoms increased, and progression was detected on optic coherence tomography angiography, as well as in Humphrey visual field test and electroretinogram, thus, mycophenolate mofetil was added showing improvement and stabilization of the disease in a 4-year follow-up. DISCUSSION: Optic coherence tomography angiography may be a potential tool to monitor progression and response to treatment in addition to other imaging modalities in acute zonal occult outer retinopathy, and the combination of adalimumab and mycophenolate may be useful in recurrent disease.

Consensus document on allergic conjunctivitis (DECA).

Allergic conjunctivitis (AC) is an inflammatory disease of the conjunctiva caused mainly by an IgE-mediated mechanism. It is the most common type of ocular allergy. Despite being the most benign form of conjunctivitis, AC has a considerable effect on patient quality of life, reduces work productivity, and increases health care costs. No consensus has been reached on its classification, diagnosis, or treatment. Consequently, the literature provides little information on its natural history, epidemiological data are scarce, and it is often difficult to ascertain its true morbidity. The main objective of the Consensus Document on Allergic Conjunctivitis (Documento dE Consenso sobre Conjuntivitis Alérgica [DECA]), which was drafted by an expert panel from the Spanish Society of Allergology and Spanish Society of Ophthalmology, was to reach agreement on basic criteria that could prove useful for both specialists and primary care physicians and facilitate the diagnosis, classification, and treatment of AC. This document is the first of its kind to describe and analyze aspects of AC that could make it possible to control symptoms.

Assesment of fundus autofluorescence in serpiginous and serpiginous-like choroidopathy.

AIMS: The aim of this study was to correlate the activity status disclosed in fluorescein angiography (FA) and fundus autofluorescence (FAF) imaging, and the variations of FAF images in the evolution of serpiginous choroidopathy (SC) and serpiginous-like choroidopathy (SLC). METHODS: Prospective consecutive case series. Patients with SC or SLC were included from July 2009 to December 2010. All patients underwent FAF imaging (Spaide Autofluorescence Filters, Topcon TRC 50IX) and FA (Topcon TRC 50IX). RESULTS: Twelve patients (eight males, mean age 51.2 years) were included. Bilateral involvement in nine cases. Three different patterns of FAF images were present: active inflammation, transitional, and inactive inflammation. CONCLUSION: FAF may be a useful tool for following patients with SC and SLC. It is possible to reserve other invasive techniques, such as FA, for cases with suspicious activity disclosed by FAF imaging.

Adrenocortical adenoma and central serous chorioretinopathy: a rare association?

A 28-year-old man with bilateral central serous chorioretinopathy (CSCR) and body weight gain was diagnosed with Cushing's syndrome secondary to an adrenocortical adenoma. The patient had high levels of free cortisol and the tumor was confirmed by histopathology. After surgery, cortisol levels decreased and the CSCR spontaneously resolved. This case highlights the role of cortisol in the pathogenesis of CSCR. A diagnosis of endogenous Cushing's syndrome should be considered in patients with CSCR as an initial symptom.

Frosted branch angiitis diagnosed as neuro-behçet: a diagnostic and etiologic dilemma.

PURPOSE: To report a case of frosted branch angiitis (FBA) secondary to neuro-Behçet. METHODS: Description, diagnosis, angiogram imaging and follow-up of a 28-year-old female with FBA. RESULTS: 'Frosted branch angiitis' is a clinical term applied to three conditions: infiltration of vessels by malignant cells, and sheathing of vessels either secondary to an active disorder or subsequently to a previous inflammatory disease. Our patient's history of two optic neuropathies and the lack of demyelinating signs in neuroimaging made us consider FBA in the context of neuro-Behçet. CONCLUSION: Recognition of the category of FBA from the clinical signs is essential to establish the correct diagnosis and prescribe the appropriate treatment.

[Lack of concordance between dry eye syndrome questionnaires and diagnostic tests]. / Falta de concordancia entre los cuestionarios y las pruebas diagnósticas en el síndrome de ojo seco.

PURPOSE: To report the prevalence of dry eye syndrome (DES) in a subset of patients > 50 years old in Valladolid, Spain, calculate internal validity of two DES screening questionnaires, and correlate the results with DES diagnostic tests. METHODS: Patients > 50 years-old were randomly selected from the medical network census in Valladolid; they answered the modified McMonnies questionnaire (Q1) and the Ocular Surface Index questionnaire (Q2) and then underwent an ophthalmic evaluation which included: tear break-up time (TBUT), fluorescein and Rose Bengal staining, and Schirmer with anesthesia were performed. Descriptive analysis, test concordance analysis, and Q1 and Q2 reliability using ROC curves were performed. RESULTS: A total of 270 subjects (58.2% female; 41.8% male) with an average age of 64.5 years (95% CI: 63.3 - 65.7) were enrolled. The prevalence of DES based on the Schirmer-Tear break-up time tests combination was 24.2%. The mean Q1 score was 1.2, and 1.4 for Q2. Grittiness and discomfort in dry environments (28.9%) were the most common symptoms recorded for Q1. For Q2 "grittiness" (99.2%) and "burning sensation" (98.9%)were the most common symptoms. With the Schirmer-TBUT combination, DES prevalence in Valladolid was 24.2%, 45.8% with TBUT, and 45.6% with Schirmer test. Values for the areas under the ROC curve for Q1, Q2, and most objective tests were < 0.5, except for Q2 and TBUT (0.503). Cronbach alpha was > 0.7 for all Q1 and Q2 items. Statistically significant concordance was reported for Schirmer - TBUT (κ=0.14) and TBUT- fluorescein staining (κ=0.09); (P<0.05). CONCLUSIONS: Q1 and Q2 were unreliable for DES diagnosis and showed poor internal consistency. Fluorescein and Rose Bengal staining were the most concordant tests for DES diagnosis. Finally, there was no correlation between either screening questionnaire and objective DES diagnostic tests.

[Influence of axial length in refractive outcome after cataract surgery]. / Influencia de la longitud axial en el resultado refractivo tras cirugía de catarata.

PURPOSE: To analyse the influence of axial length (AL) and age on refractive outcome after cataract surgery in terms of uncorrected visual acuity (UCVA), best corrected visual acuity (BCVA) and subjective refraction. METHODS: A retrospective review of 171 consecutive cases of uncomplicated cataract surgery was done. The refractive outcome was analysed (UCVA, BCVA and postoperative retraction) according AL before surgery (AL < 22 mm, between 22 and 25 mm and AL > 25 mm) and age (< 40, between 40-60 years and > 60 years). RESULTS: After surgery mean UCVA was 0.6 ± 0.33 Diopters (D) and mean BCVA was 0.93 ± 0.23 D. Mean refractive outcome was 0.89 ± 0.78 D. There were no significant differences in post-operative UCVA, BCVA and refraction between the three age groups. There were statistically significant differences (p = 0.004) in UCVA between the three AL groups. The group with AL between 22 and 25 mm had better UCVA. Mean refractive outcome was -0.95 ± 1.91 D in the group with AL < 22 mm, -0.36 ± 0.88 D in the group with AL between 22 and 25 mm and 0.23 ± 1.15 D in the group with AL > 25 mm. CONCLUSIONS: AL influences refractive outcome and UCVA after cataract surgery. Eyes with AL < 22 mm have a worse refractive outcome.

[Ocular presentation in Wegener granulomatosis]. / Presentación ocular de la granulomatosis de Wegener.

CASE REPORT: A 71 year-old female presented with bilateral conjunctivitis, scleritis and peripheral ulcerative keratitis. Ancillary tests showed presence of anti-neutrophil cytoplasmic antibodies, with diffuse granular cytoplasmic immunofluorescence staining (ANCAc). Conjunctival biopsy showed necrotizing vasculitis in small vessels. Diagnosis of Wegener Granulomatosis (WG) was established and upper respiratory affection detected. Treatment with megadoses of cyclophosphamide and corticoids was initiated and remission of the disease was obtained. DISCUSSION: WG is a potentially lethal systemic vasculitis in which ocular involvement may be the first clinical presentation. Scleritis and peripheral ulcerative keratitis indicate systemic involvement, highlighting the importance of prompt diagnosis and treatment.

[Ocular Behçet disease. Retrospective study]. / Enfermedad de Behçet ocular. Estudio retrospectivo.

OBJECTIVE: To study clinical features, visual prognosis and response to immunosuppressive drugs in patients with ocular Behçet Disease (BD). PATIENTS AND METHODS: clinical data of 17 patients (33 eyes) were retrospectively reviewed from May 1996 to May 2003. RESULTS: The male/female ratio was 12/5. The commonest manifestations were oral ulceration (100%) and skin lesions (88%). Anterior uveitis was present in 59%, posterior uveitis was in 76% and panuveitis was in 88.1%. Visual acuity improved in 11 (33.3%) eyes, remind stable in 18 (54.5%) eyes and four (12.1%) eyes lost two or more lines. Main drug therapy consisted of colchicine and cyclosporine (59%). Seven patients had received three drugs simultaneously. CONCLUSIONS: BD can lead to an irreversible visual loss; however, the visual prognosis is good (85%) if immunosuppressor treatment is implemented.

Levels of mucin gene expression in normal human conjunctival epithelium in vivo.

PURPOSE: Conjunctival impression cytology (CIC) samples were used to determine the mean and normal range of mRNA levels of human MUC1, MUC2, MUC4, MUC5AC, and MUC7 mucin genes. METHODS: Real time PCR was performed to determine normal mRNA levels in CIC samples of 24 male and 19 female healthy donors. Correlation coefficients between gene expression levels were obtained. RESULTS: All five mucin genes were expressed in the CIC samples. MUC1 and MUC4 were present at the highest level and MUC2 was at the lowest. There were no gender differences. Significant positive correlations existed between MUC2 and MUC4 and between MUC2 and MUC7 levels. CONCLUSIONS: Normal levels and ranges of mRNAs for MUC1, MUC2, MUC4, MUC5AC and MUC7 conjunctival mucin genes have been established for the first time. These data may serve as the normal threshold values for future comparisons in different experimental and pathological conditions involving the ocular surface.

[Normal human conjunctival epithelium expresses MUC13, MUC15, MUC16 and MUC17 mucin genes]. / Expresión de los genes de mucinas MUC13, MUC15, MUC16 y MUC17 en el epitelio conjuntival normal humano in vivo.

PURPOSE: The ocular surface epithelia express at least five mucin genes of the total of 17 human mucin genes that have been identified so far. This study was designed to determine the expression profile of mucin genes in conjunctival impression cytology (CIC) samples from healthy subjects. METHODS: Two polyethersulfone filters were applied to the superior conjunctiva of both eyes from eight healthy donors. Polymerase chain reaction (PCR) was performed using isolated and retrotranscripted total RNA obtained from the CIC samples to study the expression of all known human mucin genes. Following amplification, PCR products were electrophoresed on 1.5% agarose gel and stained with ethidium bromide to confirm that only a single band was obtained when amplifying all cDNAs with the convenient primers. RESULTS: Transcripts of the previously reported conjunctival mucin genes MUC1, MUC2, MUC4, MUC5AC, and MUC7 were detected in all samples. In addition, transcripts of MUC13, MUC15, MUC16 and MUC17 mucin genes also were detected. Amplified products by conventional PCR showed the expected amplicon size. Transcripts of MUC3A, MUC3B, MUC5B, MUC6, MUC8, MUC11, and MUC12 mucin genes were not detected. CONCLUSION: The expression of four additional mucin mRNA (MUC13, MUC15, MUC16, and MUC17) has been proved in human conjunctival epithelium from healthy donors for the first time. The function of these genes remains to be further elucidated.

Evaluation of lectin staining in the diagnosis of fungal keratitis in an experimental rabbit model.

PURPOSE: To assess the sensitivity, specificity, and reliability of peroxidase labeled lectin staining in the diagnosis of fungal keratitis in an experimental rabbit model. METHODS: Fungal keratitis by Candida albicans, Aspergillus fumigatus, and Fusarium solani was induced in rabbits. WGA-peroxidase staining of 660 corneal sections was performed. Fungal staining was evaluated independently by two observers. The test sensitivity, specificity, and reliability indexes were calculated. RESULTS: The sensitivity of the lectin staining test for Candida albicans was 100% (95% CI: 93.51-100.00), and specificity was 100% (95% CI: 93.51-100.00). The sensitivity of the test for Aspergillus fumigatus was 96.36% (95% CI: 86.46-99.35), and specificity was 100% (95% CI: 93.51-100.00). The sensitivity of the test for Fusarium solani was 96.36% (95% CI: 86.46-99.35) and specificity was 96.15% (95% CI: 85.74-99.31). There was also a high degree of test-retest and inter-rater concordance for all three fungi tested. The test-retest k reliability indexes were 0.9455, 0.9636, and 0.8879, for Candida albicans, Aspergillus fumigatus, and Fusarium solani, respectively. The inter-rater k reliability indexes were 0.9636, 0.9818, and 0.9252, for Candida albicans, Aspergillus fumigatus, and Fusarium solani, respectively. CONCLUSIONS: WGA-peroxidase staining is a very sensitive, specific, and reliable test for the identification of fungi in an experimental rabbit model of fungal keratitis.

[In vitro toxicity of non-preserved artificial-tear formulations]. / Lágrimas artificiales sin conservantes: estudio comparativo in vitro.

PURPOSE: To analyse the putative toxic effect of three commercially available non-preserved artificial tear formulations on in vitro human conjunctival cells. MATERIAL AND METHOD: A conjunctival human epithelial cell line was exposed to Cellufresh, Oculotect and Acuolens formulations during 1, 3 and 24 hours. Cytotoxicity was measured by calculating the percentage of cell viability examination and scanning electron microscopy (SEM). Controls underwent exposure to supplement free DMEM-F12 (negative control) and exposure to 0.005% benzalkonium chloride solution (positive control). RESULTS: Cell viability after 1 or 3 hours incubation with Cellufresh and Oculotect was similar to that obtained for negative controls. With Acuolens incubation however, cell viability showed significant reduction after 3 and 24 hours compared to control. SEM showed that Cellufresh and Oculotect exposed cells presented similar behavior to control cells. All three cell lines presented evidence of cellular surface alteration after incubation for 1 or 3 hours compared to controls, Acuolens showing the highest rate of alterations in exposed cells and an additional increment in cell loss was observed. CONCLUSION: In the present study, non preserved artificial tears formulations showed a different degree in their in vitro toxicity, Acuolens being more toxic than Cellufresh or Oculotect.

Effect of hyaluronic acid on corneal haze in a photorefractive keratectomy experimental model.

PURPOSE: To evaluate the ability of topical hyaluronic acid to decrease corneal opacity after excimer laser photorefractive keratectomy (PRK) in hens. METHODS: Twenty-four white hens underwent bilateral 193-nm excimer laser PRK to correct -9.00 D of myopia. One eye received postoperative treatment with topical 1% hyaluronic acid six times daily for 3 days; the other eye received phosphate buffered saline. Slit-lamp evaluation by a masked observer was performed for 6 months after PRK, and electron microscopy was carried out at the end of the study. RESULTS: There were no significant differences in postoperative haze between the eyes treated with hyaluronic acid and those treated with phosphate buffered saline. CONCLUSION: Topical administration of hyaluronic acid had no effect on the development of corneal haze following PRK in hens.

Pars planitis: epidemiology, treatment, and association with multiple sclerosis.

During retrospective and prospective studies, we attempted to determine the clinical characteristics, treatment, and visual outcome of patients with pars planitis and to evaluate the association between pars planitis and multiple sclerosis (MS). The retrospective study included 44 patients with pars planitis, who had been examined between October 1986 and January 1999. We analyzed age, sex, visual acuity (VA), median follow-up time, and medical and surgical treatments. The prospective study, which included 21 consecutive patients with pars planitis, was performed to determine the presence of MS. In the retrospective study, the mean patient age was 22.4 years (SD +/- 11.5) and the median follow-up was 34.9 months (SD +/- 27.2). Complications included macular edema (47.7%), vitreous opacities (38.6%), papillitis (38.6%), vasculitis (36.4%), and cataract (20.5%). Forty patients (90.9%) had a final bilateral VA better than 20/40. In the prospective study, magnetic resonance imaging (MRI) was performed. Demyelinating lesions were found in 10 (47.6%) of the 21 patients and relapsing-remitting clinically definite MS was diagnosed in seven (33.3%). With the exception of age, no significantly statistical differences were observed when the visual prognosis and the clinical and epidemiologic characteristics were compared between the two groups of patients with and without associated MS; a diagnosis of MS was more frequently made in patients over 25 years of age. With appropriate treatment, patients with pars planitis have a good visual prognosis. Because the presence of demyelinating lesions seems to be high among patients with pars planitis, MRI should be considered, especially in patients over 25 years of age.

Conjunctival biopsy in the diagnosis of ocular sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease of unknown etiology that can affect almost every organ in the body, particularly the lungs, skin, eyes, and thoracic lymph nodes. A definitive diagnosis of sarcoidosis requires that a biopsy be performed. A specimen can be obtained from any affected ocular structure, including conjunctiva, lacrimal gland, eyelid skin, and orbit. Among them, conjunctival biopsy has been suggested as a sensible and safe procedure for confirming suspected sarcoidosis. We describe three patients in whom ocular symptoms were the sole initial manifestations of sarcoidosis and who were diagnosed based on the results of a conjunctival biopsy. We also discuss the efficacy of and indications for conjunctival biopsy.

[Usefulness of conjunctival biopsy as diagnostic technique]. / Utilidad de la biopsia conjuntival como técnica diagnóstica.

PURPOSE: The conjuctival biopsy is described as a useful technique in the diagnosis of some chronic conjunctivitis, with low specific signs and with poor response to the conventional treatment. Furthermore it is quite important in the diagnosis of conjunctival diseases with potential dangerous treatment. PATIENTS AND METHODS: Three clinical cases are described. Case 1: A 37 years old woman with a chronic lesion of conjuctiva and the suspicion of blepharoconjunctivitis. There was no improvement with treatment. Case 2: A 58 years old woman with a chronic conjunctivitis and follicular reaction. Case 3: A 66 years old man who suffers from a cicatricial conjunctivis in both eyes with corneal lesion in one eye. A conjunctival biopsy was perfomed and studied under light microscopy and immunohistochemistry. There is a description of the technique. RESULTS: The results of every clinical case are described. In the first case the biopsy established the diagnosis of conjunctival lymphoma. In the second, the biopsy showed the appearance of granulomas and a diagnosis of conjunctival sarcoidosis was performed. In the third case a diagnosis of ocular cicatricial pemphigoid was established. CONCLUSION: Conjunctival biopsy is a useful and effective technique in the diagnosis of chronic conjunctivitis.

Fuchs' heterochromic iridocyclitis: a review of 26 cases.

UNLABELLED: Fuchs' heterochromic iridocyclitis (FHI), an unusual form of uveitis of unknown etiology, is frequently misdiagnosed. PURPOSE: To report the analysis of 26 patients (27 eyes) with FHI who were diagnosed and observed over a period of 1-7 years (mean: 3.42 +/- 2.2 years). METHODS: Ocular and systemic examinations were performed on all patients. RESULTS: The most common presenting symptom was visual deterioration (42.3%); one patient was affected bilaterally. Characteristic keratic precipitates (100%), cataracts (77.8%), and heterochromia (70.4%) were the major signs. Eleven eyes (40.7%) required cataract surgery. At the initial examination, four eyes (14.8%) had glaucoma, and no new cases of glaucoma developed during the follow-up period. One eye required filtration surgery. Vitrectomy was performed in two eyes (7.4%) because of vitreous opacities. Most patients (73.0%) did not require active treatment; pre- and postoperative anti-inflammatory treatment for cataract extraction was performed successfully to minimize the risk of inflammation. No severe uveitis was seen in any patient after surgery, but visual acuity did not improve greatly after cataract extraction; 54.5% eyes had visual acuity better than 20/40 before surgery, and 45.4% postoperatively. CONCLUSION: Posterior capsule opacification, glaucoma, and vitreous opacity were the major obstacles to visual rehabilitation after cataract surgery in patients with this type of uveitis.