[Placoid pigment epitheliopathy and serpiginous choroiditis]. / Épithéliopathie en plaques et choroïdite serpigineuse.

Placoid pigment epitheliopathy and serpiginous choroiditis are among the white dot retinal syndromes and possess similarities that can cause confusion between these two diseases. However, they are very different in terms of their progression and prognosis, which requires a diagnosis of certainty in order to better manage the patients with the diseases and identify potentially serious progressive complications. The clinical presentation, results of testing, differential diagnoses and treatment of these two pathologies are discussed in this article.

[White dot syndromes : Principles, diagnostics, and treatment]. / "White-dot-Syndrome" : Grundlagen, Diagnostik und Therapie.

The white dot syndromes include a group of diseases which are characterized by multiple yellowish-white foci in the outer retina, retinal pigment epithelium, and choroid. For clinicians and researchers alike they present significant diagnostic and therapeutic challenges. White dot syndromes include primary inflammatory choriocapillaropathies, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE)/acute multifocal ischemic choriocapillaropathy (AMIC), multiple evanescent white dot syndrome (MEWDS)/acute idiopathic blind spot enlargement (AIBSE), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), serpiginous choroiditis (SC), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Among the primary stromal choroiditis is birdshot retinochoroidopathy (BSRC); however, the pathogenesis of these disorders is largely unknown. Immunological reactions to previous viral infections with a genetic disposition seem to be a common denominator.

Similarities and differences between three different types of white dot syndrome and the therapeutic possibilities.

White dot syndromes consists a group of inflammatory eye diseases with an unknown etiology involving the external retina, retinal pigment epithelium, choroid or combinations of them. They affect one or both eyes, at the same time or not. White dot syndromes are often self-limited, with a variable prognosis, depending on the type of the disease. The purpose of this article was to look at the similarities and the differences between the different types of syndromes and the therapeutic possibilities existing at present. Abbreviations: WDS = White dot syndromes, MEWDS = Multiple Evanescent White Dot Syndrome, APMPPE = Acute Posterior Multifocal Placoid Pigment epitheliopathy, SC = serpiginous choroiditis RPE = retinal pigment epithelium.

Need for Quantitative Measurement Methods for Posterior Uveitis: Comparison of Dual FA/ICGA Angiography, EDI-OCT Choroidal Thickness and SUN Vitreous Haze Evaluation in Stromal Choroiditis.

BACKGROUND/PURPOSE: Quantitative methods for posterior uveitis are necessary for precise appraisal and follow-up of inflammation in practice and in clinical trials. The aim of this study was to assess fluorescein angiography (FA), indocanine green angiography (ICGA), and enhanced depth imaging optical coherence tomography choroidal thickness (EDI-OCT CT) in two stromal choroiditis entities, birdshot retinochoroiditis (BRC), and Vogt-Koyanagi-Harada disease (VKH), as well as to determine (1) disease patterns, (2) respective response to therapy, and (3) their potential utility in clinical trials in comparison to vitreous haze, the present standard outcome used in clinical trials. METHODS: This retrospective study included newly diagnosed patients with BRC and VKH, seen at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified using an established dual FA/ICGA scoring system for uveitis at presentation and on follow-up. FA/ICGA score ratios were compared between diseases to determine disease patterns. EDI-OCT CT was determined using a spectral domain instrument. Vitreous haze was determined using the SUN (Standardization of Uveitis Nomenclature) method. RESULTS: Among 1872 uveitis patients seen from 1995 to 2016, 8 newly diagnosed BRC patients (16 eyes) and 6 newly diagnosed VKH patients (12 eyes) had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA scores of 16.1 ± 7.0 vs. 4.6 ± 2.1 (p < 0.0001), respectively, while mean ICGA scores were similarly high in the two diseases, 18.9 ± 3.6 (BRC) vs. 20.8 ± 7.5 (VKH). After therapy, FA and ICGA scores decreased significantly for both entities (- 60% of FA score and 55% of ICGA score in BRC vs. - 72% of FA score and - 87% for ICGA score in VKH). EDI-OCT CT decreased significantly in the two entities. Vitreous haze was almost absent in VKH and low in BRC. CONCLUSION: Dual FA/ICGA scoring showed the diverse disease patterns of BRC and VKH; both the retina and choroid were involved at onset in BRC, whereas VKH was a pure choroidal disease with later spillover into the retina. Dual FA/ICGA allowed for the precise measurement of inflammation at onset and upon follow-up. EDI-OCT CT responded to therapy in both diseases but was found to be of limited use in this early/subacute disease phase because it lacked sensitivity to detect subclinical recurrences and was therefore only useful for long-term follow-up. Vitreous haze was low in both entities and thus useless as an inflammatory parameter.

Multifocal Choroiditis and Panuveitis - difficulties in diagnosis and treatment.

We present the case of a 49-year-old patient who was treated with Aripriprazole in context of Paranoid Schizophrenia. The patient had a history of numerous Panuveitis recurrences for the left eye, which led to a marked decrease of the vision VA-NLP and was diagnosed with Multifocal Choroiditis and Panuveitis for the right eye. The examination revealed VA of 20/ 200 for right eye, keratic precipitates, and vitritis. Fundus aspect of the right eye showed multiple discrete, ovoid, yellowish-grey lesions at the posterior pole and periphery, optic disc oedema was present. The Human leukocyte antigen typing of group A, ancillary investigation (OCT, Angiofluorography, B-mode ultrasonography) and fundus examination confirmed the diagnosis of Multifocal Choroiditis and Panuveitis but we did not exclude antipsychotic-related chorioretinopathy or a Birdshot-like Syndrome.

[Birdshot retinochoroidopathy: Key messages]. / Rétinochoroïdopathie de type Birdshot : les éléments clés.

Birdshot retinochoroidopathy (BRC) is a potentially blinding posterior autoimmune uveitis that affects Caucasian patients in their fifties. Strongly associated with the HLA A 29 phenotype, BRC is characterized by the presence of suggestive choroidal lesions on the fundus, but their apparition can be delayed. Visual acuity is not a good descriptive factor of visual function. Ancillary tests can therefore help establish the diagnosis and allow proper follow-up of BRC patients: optical coherence tomography can reveal either oedema or atrophy of the macula. Fluorescein and infracyanine green angiograms can show signs of inflammatory activity and give further diagnostic clues. Electroretinograms and visual fields can be useful for the diagnosis and follow-up. BCR patients should be examined periodically and undergo exhaustive clinical and paraclinical tests to insure the best prognosis. Corticosteroids should be associated with immunosuppressive treatments as early as possible in the presence of factors that are predictive of the worse prognoses.

[Macular serpiginous choroiditis complicated by macular hole]. / COROIDITA SERPIGINOASA MACULARA COMPLICATA CU GAURA MACULARA.

Macular serpiginouschoroiditis is a rare variant of serpiginous choroiditis characterized by a severe recurrent inflammation of both central choroid and retinal pigment epithelium. Visual prognosis is severe due to subsequent distruction of retinal structures. Permanent central visual loss is the consequence of retinal pigment epithelium hyper or hypoplasia and/or subretinal neovascularization leading to fibrous scarring. This article reports the unusual case of rapid development of a macular hole soon after the onset of characteristic clinical features. Despite anti-inflammatory treatment and successful macular hole surgery the visual function remained significantly impaired by secondary central retinal pigment epithelium changes.

Serpiginous choroiditis and acute retinal necrosis occurring in the same patient.

We describe a rare association of serpiginous choroiditis with necrotizing retinitis having clinical features of acute retinal necrosis (ARN). A 23-year-old male developed ARN in the fellow eye while he was on tapering doses of immunosuppressive medications for unilateral serpiginous choroiditis. The association may represent a common viral etiology of the two diseases or may be due to the development of ARN due to general state of iatrogenic immunosuppression. This report also highlights the importance of a detailed evaluation of both the eyes on regular follow-up visits in the patients receiving iatrogenic immunosuppression.

Coroiditis serpiginosa en paciente con carcinoma de cérvix uterino / Serpiginous choroiditis in a patient with uterine cervix carcinoma

Caso clínico: Se presenta el caso clínico de una paciente de 70 años con coroiditis serpiginosa y carcinoma de cérvix uterino. Discusión: La etiología de la coroiditis serpiginosa es desconocida pero lesiones similares han sido descritas en asociación con lupus eritematoso sistémico, linfoma no Hodgkin, enfermedad de Crohn, sarcoidosis, tuberculosis, infección por virus del herpes, hepatitis autoinmune y carcinoma pulmonar(AU)

Case report: We report the case of a 70-year-old patient with serpiginous choroiditis and uterine cervix carcinoma. Discussion: The etiology of serpiginous choroiditis is unknown, but similar lesions have been described in association with systemic lupus erythematosus, non-Hodgkin lymphoma, Crohn's disease, sarcoidosis, tuberculosis, herpes virus infection, autoimmune hepatitis and lung carcinoma(AU)

[Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)--a therapeutic dilemma]. / Ostra tylna wieloogniskowa plackowata epiteliopatia barwnikowa--dylematy terapeutyczne.

PURPOSE: To present a rare idiopathic inflammation of the posterior segment of the eye - acute posterior multifocal placoid pigment epitheliopathy (APMPPE). MATERIAL AND METHODS: A 17 years old male patient was referred to the Department of Ophthalmology of Medical College, Jagiellonian University in Krakow with the diagnosis of bilateral choroiditis of unknown etiology. The patient underwent the basic ophthalmological examination followed by fluorescein (FA) and indocyanine green (ICGA) angiography. Laboratory testing for Lyme disease, sarcoidosis and syphilis, as well as genetic testing, to determine the presence of HLA antigen complex, were performed RESULTS: Based on the outcomes of performed investigations the definitive diagnosis of APMPPE was established. Systemic steroidotherapy was initiated due to rapid progression of the inflammatory changes, progression of the central visual field changes and profound visual acuity deterioration. CONCLUSIONS: APMPPE is a rare, inflammatory and idiopathic eye disease with no clear indications for pharmacological therapy. However decision about necessity and sort of pharmacological therapy should be taken under consideration individually for each patient. ostra tylna wieloogniskowa plackowata epiteliopatia barwnikowa, steroidoterapia. acute posterior multifocal placoid pigment epitheliopathy, steroidotherapy.

Comparison of patients with ocular tuberculosis in the 1990s and the 2000s.

PURPOSE: To compare clinical findings in patients with ocular tuberculosis experienced during two different decades. METHODS: Thirty-four patients with ocular tuberculosis were divided into two groups: a 1990s group (n = 18) and a 2000s group (n = 16), according to the dates of their first outpatient visit. The clinical profiles of the two groups were then compared. RESULTS: More cases of the 1990s group had complications involving extraocular tuberculosis than those of the 2000s group. While various ophthalmic manifestations were observed clinically in the 1990s group, all retinal periphlebitis cases presented in the 2000s group. The proportion of patients who received antituberculous treatment was higher in the 1990s group, but the proportion who received oral corticosteroid therapy did not differ between the two periods. However, more patients underwent laser photocoagulation in the 2000s group. The percentage of eyes with final visual acuity better than 20/20 increased in the 2000s group. CONCLUSIONS: The clinical outcome of patients with ocular tuberculosis was improved in the 2000s group, which may be attributable to the increase in active use of laser photocoagulation therapy.

Hepatitis autoinmune en un paciente con coroiditis serpiginosa / Autoimmune hepatitis in a patient with serpiginous choroiditis

Caso clínico: Presentamos el caso clínico de una paciente afecta de coroiditis serpiginosa que presentó un cuadro de hepatitis autoinmune. Discusión: La etiología de la coroiditis serpiginosa permanece desconocida. Se ha postulado una anormal respuesta de sistema inmunitario como mecanismo. Este caso muestra la asociación de la coroiditis serpiginosa con un proceso de tipo autoinmune

Case report: We report the case of a patient with serpiginous choroiditis who developed an autoinmune hepatitis. Discussion: The etiology of serpiginous choroiditis remains uncertain. An immune-mediated mechanism has been reported. This case shows an association between serpiginous choroiditis and autoimmune processes

[Acute posterior multifocal placoid pigment epitheliopathy, serpiginous and multifocal choroiditis: etiological and therapeutic management]. / Epithéliopathies en plaques, choroïdites serpigineuses et multifocales: analyse étiologique et prise en charge thérapeutique.

PURPOSE: To highlight the importance of an extensive medical work-up in serpiginous and multifocal choroiditis, and acute posterior multifocal placoid pigment epitheliopathy before therapeutic management. PATIENTS AND METHODS: Records of patients referred to our department, between January 2000 and January 2002, for the diagnostic and therapeutic management of choroiditis or acute posterior multifocal placoid pigment epitheliopathy were retrospectively reviewed. All patients had a complete ophthalmologic examination, fluorescein and infrared angiographies. An extensive work-up was performed in order to exclude an infectious etiology. RESULTS: Fourteen patients were included (six cases of serpiginous choroiditis, four cases of multifocal choroiditis, and four cases of APMPPE). The mean age was 42.1 years and the sex ratio was 9: 5. Six patients presented with a history of tuberculosis in the family or with a tuberculosis primary infection. Toxoplasmic retinochoroiditis was confirmed in one case by a positive PCR applied to the aqueous humor. In the serpiginous choroiditis group, two patients have been treated with antituberculous drugs, one of whom was initially resistant to immunosuppressive regimens. In the acute posterior multifocal placoid pigment epitheliopathy group, one patient was treated with antituberculous drugs and another received antibiotics. CONCLUSION: An infectious agent may be associated with this group of clinical presentations. All patients presenting with severe forms of ocular inflammation, who resist to corticosteroids or immunosuppressive regimens, must undergo an extensive infectious work-up in order to propose a specific treatment.

Coroiditis serpinginosa en pacientes con carcinoma pulmonar / Serpenginous choroiditis in a patient with lung carcinoma

Caso clínico: Presentamos el caso clínico de un paciente varón de 66 años con lesiones coroideas de tipo serpinginoso y carcinoma pulmonar en fase terminal.Discusión: La coroiditis serpinginosa es una enfermedad idiopática, si bien se han descrito lesiones similares asociadas a tuberculosis, sarcoidosis, linfoma sistémico, infección por herpes virus y enfermedad de Crohn. La coexistencia de lesiones coroideas de tipo serpinginoso y un carcinoma pulmonar no ha sido descrita hasta el momento en la literatura (AU)

Case report: We report the case of a 66-year-old patient with serpiginous-like choroiditis and terminal epidermoid lung carcinoma.Discussion: Serpiginous choroiditis is an idiopathic disease, although similar lesions have been described in association with tuberculosis, sarcoidosis, systemic lymphoma, herpes virus infection and Crohn´s disease. The coexistence of serpiginous-like choroiditis and a lung carcinoma has not previously been described (AU)

[Indocyanine green angiography in chorioretinal inflammatory diseases]. / Valoración de enfermedades inflamatorias coriorretinianas con angiografía verde indocianina.

PURPOSE: To determine the role of indocyanine green angiography (ICG) in handling chorioretinal inflammatory disorders. METHOD: We present several posterior uveitis whose diagnosis and therapeutic approach was facilitated with ICG. RESULTS: ICG is useful in the differential diagnosis of <>. In serpiginous choroiditis, the edges and the progression of the inflammatory areas are clearly delineated. In infectious uveitis such as toxoplasmosis, ICG determines the presence or absence of active lesions. There are granulomatous diseases such as sarcoidosis where some areas of choroidal inflammation that cannot be detected with other methods are shown by ICG. Moreover, ICG can detect areas of subretinal neovascularization in some choroidal pathologies such as polypoidal choroidal vasculopathy. CONCLUSIONS: ICG appears to be a useful and complementary tool in the clinical interpretation of posterior uveitis.

Indocyanine green-mediated photothrombosis combined with intravitreal triamcinolone for the treatment of choroidal neovascularization in serpiginous choroiditis.

PURPOSE: To report a case of peripapillary choroidal neovascularization (CNV) complicating serpiginous choroiditis that was treated by a single indocyanine green (ICG)-mediated photothrombosis session combined to intravitreous triamcinolone acetonide (TA) injection. METHODS: Interventional case report. A 48-year-old patient with peripapillary CNV was submitted to a laser-dye-mediated technique that uses ICG and low-intensity 810-nm light for continuous laser application; TA was then injected into the vitreous cavity 1 hour later, and prospective evaluation with fluorescein and ICG angiography as well as optical coherence tomography (OCT) was performed. RESULTS: At 2 weeks after treatment, best-corrected visual acuity improved from 20/200 to 20/50, with further improvement to 20/20-1 in the subsequent 10 weeks. Absence of fluorescein leakage from the CNV and OCT evidence of resolved retinal oedema was observed at that time. Clinical stabilization was maintained up to 1 year of follow-up. There was no significant complication related to the procedure. CONCLUSION: Combined ICG-mediated photothrombosis and intravitreous TA induced rapid and significant visual acuity recovery in this particular case of peripapillary CNV complicating serpiginous choroiditis. Accordingly, angiographic and OCT findings demonstrated neovascular lesion regression and restoration of the macular architecture.

New treatments for serpiginous choroiditis.

Serpiginous choroiditis is a bilateral, inflammatory condition of the inner choroid and overlying retinal pigment epithelium with a distinctive retinal distribution. Although rare, serpiginous choroiditis deserves attention because it can lead to permanent loss of vision due to foveal involvement by inflammation or choroidal neovascularization from frequent recurrences. This article reviews the recent ophthalmic literature published on the management of serpiginous choroiditis.

Coriopatía serpiginosa: descripción de un caso clínico / Choroidopathia serpiginosa: description of a clinical case

Choroiditis serpiginosa is an infrequent disorder, which is chronic, progressive, bilateral, and recurrent; in primary form it affects the pigmented epithelium of the retina and the choriocapillary layer within the eye. It typically begins juxtapapillary and progresses to extend centrifugally to the optic disc, usually affecting the macula and thus decreasing visual acuity. It's etiology is unknown and it debutes in acute form in adult patients, without preference for sex. It's treatment includes the use of systemic corticosteroids, non steroidal anti-inflammatory drug (NSAID), and immunosuppressant agents. In this paper we document the clinical case of a diabetic, 61 year old man, having the disease in an inactive phase with serious visual sequelae.

Coroiditis serpiginosa es un desorden poco frecuente, crónico, progresivo, bilateral y recurrente que efecta en forma primaria al epitelio pigmentario de la retina y la coriocapilar. Típicamente comienza yuxtapapilar y su progresión se extiende en forma centrífuga al disco óptico, afectando usualmente la mácula, disminuyendo así la agudeza visual. Su etiología es desconocida y debuta en forma aguda en pacientes de edad madura sin predilección por sexo. El tratamiento incluye el uso de corticoesteroides sistémicos, antiinflamatorios no esteroidales (AINES) y agentes inmunosupresores. Presentamos el caso clínico de un hombre diabético de 61 años con enfermedad en fase inactiva con secuelas visuales serias.