Acute Multifocal Placoid Pigment Epitheliopathy Research Articles

Panretinal acute multifocal placoid pigment epitheliopathy: a novel posterior uveitis syndrome with HLA-A3 and HLA-C7 association.

BackgroundThe purpose of this study is to report a previously undescribed clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) but with an atypical, panretinal and diffuse presentation in young patients with an HLA-A3 and HLA-C7 association. We describe a cluster of three, young, healthy patients who experienced acute visual loss, aged 16 through 27 years exhibiting an unusual clinical entity over an 8-month period.FindingsOur patients demonstrated a unique presentation with acute retinal lesions similar to APMPPE but had widespread presentation of multiple lesions in the peripheral retina. All three of our patients exhibited an acute loss of vision, two of them bilaterally. Ophthalmoscopy and fundus photography demonstrated a diffuse distribution of lesions located extensively throughout the retina. On fluorescein angiography, the lesions showed a characteristic ‘blocking early and staining late’ pattern similar to APMPPE. The average duration of activity was 6 weeks (range 4 to 8 weeks), and there were no recurrences and good visual prognosis. HLA-A3 and HLA-C7 was noted in 100% of the patients. Ocular coherence tomography during the acute stage in one patient demonstrated thickening at the RPE layer.ConclusionsTo our knowledge, this cluster of young patients represents a previously undescribed clinical entity, with clinical features similar to APMPPE, relentless placoid chorioretinitis and ampiginous. Due to the diffuse distributions of the active lesions, the acute clinical course without recurrences, good visual prognosis, and HLA-A3/C7 association, we believe it to be distinct from these other white dot syndromes. All three patients experienced preceding viral-like prodrome which, when combined with major histocompatibility commonalities, may predispose these individuals to an immune response. We call this entity panretinal acute multifocal placoid pigment epitheliopathy or PAMPPE.

Imaging in vitreoretinal diagnostics

Imaging in vitreoretinal diagnostics

Indocyanine green angiography in posterior uveitis.

Literature review for indocyanine green angiography and evaluate the role of indocyanine green angiogram (ICGA) in patients with posterior uveitis seen at a tertiary referral eye care centre. Detailed review of the literature on ICGA was performed. Retrospective review of medical records of patients with posterior uveitis and dual fundus and ICGA was done after institutional board approval. Eighteen patients (26 eyes) had serpiginous choroiditis out of which 12 patients had active choroiditis and six patients had healed choroiditis, six patients (12 eyes) had ampiginous choroiditis, six patients (12 eyes) had acute multifocal posterior placoid pigment epitheliopathy, eight patients (10 eyes) had multifocal choroiditis, four patients (eight eyes) had presumed ocular histoplasmosis syndrome, four patients (eight eyes) had presumed tuberculous choroiditis, two patients (four eyes) had multiple evanescent white dot syndrome and two patients (four eyes) had Vogt Koyanagi Harada (VKH) syndrome. The most characteristic feature noted on ICGA was the presence of different patterns of hypofluorescent dark spots, which were present at different stages of the angiogram. ICGA provides the clinician with a powerful adjunctive tool in choroidal inflammatory disorders. It is not meant to replace already proven modalities such as the fluorescein angiography, but it can provide additional information that is useful in establishing a more definitive diagnosis in inflammatory chorioretinal diseases associated with multiple spots. It still needs to be determined if ICGA can prove to be a follow up parameter to evaluate disease progression.

Spatio-temporal understanding of the pathology of acute posterior multifocal placoid pigment epitheliopathy

We report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and the associated spatio-temporal pathologic findings of this disease. A 29-year-old woman with bilateral recurrent APMPPE. The patient complained of bilateral blurred vision. The best-corrected visual acuity was 20/13 bilaterally. Slit-lamp examination revealed bilateral, multiple, yellowish-white, flat, placoid lesions over the posterior fundus. A hyperreflective area between the outer nuclear layer (ONL), and the photoreceptor inner and outer segments (IS/OS) visible on the optical coherence tomographic images and a hyperfluorescent area identified on the late-phase fluorescein angiographic images were spatio-temporally consistent with the presence of the placoid lesions. The pattern of juxtafoveal hyperfluorescent spots was accompanied by multiple hypofluorescent, wedge-shaped lesions. The hypofluorescent lesions visible on the indocyanine green angiographic (ICGA) images were irregular in shape and intensity. APMPPE was diagnosed, and the patient was treated with an initial 30 mg dose of oral prednisolone, followed by a 3-week taper. The lesions recurred in an area where hypofluorescent lesions had not occurred previously, while the previous hypofluorescent spots resolved completely without the hyperfluorescence associated with vascular remodeling. These findings suggest that the placoid appearance of APMPPE may correspond to edema between the ONL and the photoreceptor IS/OS and that the hypofluorescent lesions characteristic of this condition visible on ICGA images might reflect infiltration and subsequent blockage of choroidal vasculature-derived fluorescence.

Algorithm of Choroiditis

Choroiditis may present as focal or multifocal lesions, and could be due to infectious or noninfectious etiology. Focal choroidal lesions are commonly caused by toxoplasma infection, tuberculosis, sarcoidosis or serpiginous choroiditis. Multifocal choroiditis can be due to infective etiology, such as tuberculosis, or any autoimmune choroiditis, such as ampiginous choroiditis or acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Diffuse choroiditis may be correlated to panuveitis, such as Vogt-Koyanagi-Harada (VKH) syndrome or sympathetic ophthalmia. Other choroiditis may present as opportunistic infections in immunocompromised patients. Careful clinical examinations and tailored investigations would be helpful in making a prompt diagnosis of these different entities. Due to varied manifestations, diagnosis of choroiditis is challenging. This article will review the diagnostic approach of choroiditis based on clinical presentations, investigations and ancillary tests.

A Case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) and Crohnʼs Disease

A Case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) and Crohnʼs Disease

Intravitreal ranibizumab for choroidal neovascularization secondary to acute multifocal posterior placoid pigment epitheliopathy

Intravitreal ranibizumab for choroidal neovascularization secondary to acute multifocal posterior placoid pigment epitheliopathy

High-Definition OCT Findings in Acute Posterior Multifocal Placoid Pigment Epitheliopathy

The authors report a case of acute posterior multifocal placoid pigment epitheliopathy evaluated by autofluorescence, fluorescein angiography, indocyanine green angiography, high-definition optical coherence tomography, and microperimetry in both the acute and the post-acute phase. Based on the integrated findings, the authors believe that acute posterior multifocal placoid pigment epitheliopathy is an inflammatory disease that primarily affects the choroid and that the retinal pigment epithelium is secondarily involved in the course of the disease.

Mitoxantrone Therapy for Acute Posterior Multifocal Placoid Pigment Epitheliopathy with Cerebral Vasculitis

Purpose. To report favorable outcome of a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) associated with cerebral vasculitis after treatment with immunosuppressive therapy by mitoxantrone. Design. Single case report. Method. A 22-year-old man presented with acute isolated bilateral loss of vision revealing APMPPE. Corticosteroid therapy was initiated and visual acuity gradually improved. Seventeen days later, visual function deteriorated again, associated with flu-like syndrome and severe headaches. A relapse of APMPPE was diagnosed, complicated with lymphocytic meningitis and cerebral ischemia. Intravenous therapy with mitoxantrone was performed in combination with methylprednisolone. Results. Headaches disappeared in a few days whereas visual acuity gradually improved and stabilized at 20/40 in the right eye and 20/32 in the left eye. No adverse event was observed. Clinical improvement was confirmed by magnetic resonance imaging. Conclusion. Cerebral vasculitis is the most severe complication of the extraocular manifestations of APMPEE. This diagnosis should be evoked when severe headaches or behavior disorder are associated with APMPEE.

White-Dot-Syndrom

The white dot syndromes comprise a group of diseases with a suspected immunological background, which show no systemic manifestations. The characteristic inflammatory changes of the choroid and the retinal pigment epithelium are typically yellow-white foci beneath the retina. Diseases belonging to the white dot syndromes which will be discussed in this article are acute multifocal placoid pigment epitheliopathy (AMPPE), multiple evanescent white dot syndrome (MEWDS), birdshot retinochoroidopathy (BSRC), multifocal choroiditis with panuveitis (MFC/MCP), punctuate inner choroidopathy (PIC), acute zonal occult outer retinopathy (AZOOR) and serpiginous choroiditis, Neither the trigger mechanism nor the pathogenetic development is known with certainty for any of these diseases. Immunological reactions to previous viral infections coupled with a genetic predisposition seem to be a common denominator. Transitions between the individual diseases have also been described.

Treatment of Cystoid Macular Edema Due to Acute Posterior Multifocal Placoid Pigment Epitheliopathy

Purpose: To report a case of unilateral late-presenting acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with CME evaluated before and after administration of IVTA. Method: A 29-year-old male diagnosed as APMPPE with CME was treated with IVTA. Results: Fundus examination revealed CME and sequelae APMPPE lesions without involvement of posterior pole. FA and OCT findings were consistent with CME. After IVTA (4 mg/0.1 mL) administration; BCVA increased from 20/28 to 20/20, mean retinal thickness decreased in OCT, and mean sensitivity in microperimetry increased from 15.7 to 17.2 dB. Conclusion: In this case report, IVTA improved macular anatomy, visual acuity, and macular sensitivity.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy After Cerebral Sinus Thrombosis

A 19-year old woman presenting with headaches, focal neurological deficits, and seizures, diagnosed with sagittal sinus venous thrombosis (SSVT). On recovery she developed blurry vision due to acute posterior multifocal placoid pigment epitheliopathy (APMPPE). APMPPE, an inflammatory chorioretinal disease, presents as visual impairment that precedes accompanying neurological manifestations. The association of APMPPE with SSVT is rare, and APMPPE occurring after SSVT has not been previously reported. Awareness of this possibility might facilitate management of future cases. Our observation suggests a link between APMPPE and SSVT especially among young stroke patients who develop visual symptoms not readily explained by cerebral pathology.

A Young Japanese Man With Relentless Placoid Chorioretinitis

Jones and colleagues described a clinical entity they called relentless placoid chorioretinitis, characterized by features similar to both acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and serpiginouschoroiditis, but with atypical features for either of these diseases. 1 Here we describe a young Japanese man with what we believe fits their description of relentless placoid chorioretinitis.

Optical coherence tomography findings in a case of an acute multifocal posterior placoid pigment epitheliopathy (AMPP).

To report the optical coherence tomography (OCT) findings in a case of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). A 26-year-old woman was seen for a mild loss of visual acuity in both eyes. The fundus examination revealed yellow-white placoid lesions in the posterior pole suggesting AMPPPE. Fluorescein and indocyanine green (ICG) angiography and optical coherence tomography (OCT) were performed 2 days, 9 days, and 1 month after the first examination. In the acute phases the OCT revealed a mild hyperreflective area above the retinal pigmented epithelium (RPE) in the photoreceptor layer. In the later phases the OCT scan revealed a nodular hyperreflective lesion on the plane of the RPE with mild underlying backscattering. In AMPPPE, the OCT demonstrates hyperreflective lesions that may indicate inflammatory tissue and inflammatory cells or the presence of ischemic edema in the outer retinal layers. (Eur J Ophthalmol 2005; 15: 143-7).

Optical Coherence Tomography Findings in a Case of Acute Multifocal Posterior Placoid Pigment Epitheliopathy (AMPPPE)

To report the optical coherence tomography (OCT) findings in a case of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). Case report. A 26-year-old woman was seen for a mild loss of visual acuity in both eyes. The fundus examination revealed yellow-white placoid lesions in the posterior pole suggesting AMPPPE. Fluorescein and indocyanine green (ICG) angiography and optical coherence tomography (OCT) were performed 2 days, 9 days, and 1 month after the first examination. In the acute phases the OCT revealed a mild hyperreflective area above the retinal pigmented epithelium (RPE) in the photoreceptor layer. In the later phases the OCT scan revealed a nodular hyperreflective lesion on the plane of the RPE with mild underlying backscattering. In AMPPPE, the OCT demonstrates hyperreflective lesions that may indicate inflammatory tissue and inflammatory cells or the presence of ischemic edema in the outer retinal layers.

Atypical Serpiginous Chorioditis Treated by Posterior Subtenon Methylprednisolone

A 37 year-old Asian man with an infection of the upper respiratory tract experienced sudden visual changes suggestive of acute multifocal posterior placoid pigment epitheliopathy (AMPPPE). Extensive examination showed this to be serpiginous choroidopathy that was treated successfully with a single subtenon injection methylprednisolone. It is suggested that this method of treatment needs further investigation and evaluation.

Épithéliopathie en plaque et mauvais pronostic visuel : à propos d’un cas

We report a case of a fifty-year-old women suffering from acute multifocal posterior placoid pigment epitheliopathy (AMPPPE), very atypical in terms of clinical appearance and course. From this observation, we provide a progress report on the usual course of AMPPPE as well as atypical features of this affection through reports in the literature; finally we detail disorders ressembling both AMPPPE and serpiginous choroidopathy, the main differential diagnosis.

Indocyanine green angiography in chorioretinal diseases: indications and interpretation: An evidence-based update

Topic/purpose To assess the clinical usefulness and relevance of indocyanine green angiography (ICG) in the investigation of chorioretinal disorders and assess specifically in what conditions it may add useful information to that obtained using standard fluorescein angiography. Clinical relevance Many publications on ICG have appeared in recent years touting its use in ophthalmology. These publications have led to increasing use of this technique and to its application in numerous retinal diseases in which the fluorescein angiographic findings have been thoroughly described. Methods/literature reviewed During this systematic literature review, we identified and reviewed a total of 376 articles, from among which we selected 92 that we considered most relevant to our purpose of evaluating published evidence as to the efficacy of ICG. We excluded many articles with weak study designs and those that simply duplicated previously published information. Our literature search used PubMed and was confined to articles in English or that included an English abstract. Results Our systematic review suggests that ICG has relatively few specific indications for use as justified by previously published peer-reviewed studies. In keeping with the requirements for this Journal’s evidence-based articles, we have divided our clinical recommendations for the use of ICG into three categories: (A) strongly recommended and supported by strong evidence; (B) recommended with moderately strong supporting evidence; (C) not recommended at present because supported only by anecdotal or group consensus evidence. A. We highly recommended ICG for (1) identification of polypoidal choroidal vasculopathy, (2) occult choroidal neovascularization, (3) neovascularization associated with pigment epithelial detachments, and (4) recurrent choroidal neovascular membranes. These are all conditions in which ICG contributes to the identification of lesions that may be treatable. B. We recommend ICG with some enthusiasm for identifying feeder vessels in age-related macular degeneration, choroidal neovascular membranes, chronic central serous retinopathy, multiple evanescent white dot syndrome, vasculitis, acute multifocal placoid pigment epitheliopathy, Vogt-Koyanagi-Harada syndrome, macular lesions associated with angioid streaks, and birdshot retinopathy. In all these conditions, ICG may help establish a diagnosis and provide some useful guidance for therapy. C. At present, we do not recommend ICG for scleritis and posterior scleritis, drusen differentiation, Behçet’s disease, or sarcoidosis, because it has not been demonstrated to add useful clinical information. Conclusions ICG, although now a well established technique, has clear advantage over fluorescein angiography in relatively few chorioretinal disorders. It has, however, contributed to the understanding of pathologic processes in many ocular diseases. As yet, no published randomized controlled clinical trials show any benefit to the use of ICG in the management of any specific ocular disease.

Acute multifocal placoid pigment epitheliopathy-like lesion as an early presentation of subacute sclerosing panencephalitis

PurposeTo report a case of acute multifocal placoid pigment epitheliopathy (AMPPE)-like lesion as an early presentation of subacute sclerosing panencephalitis (SSPE). DesignObservational case report. MethodsA 21-year-old woman presented with a profound loss of vision in the left eye. The initial clinical picture was characterized by a whitish infiltration of the macular area and papillary edema in the left eye. Six days later multiple chorioretinal infiltrations were present in the peripheral fundus of the left eye, which led to the diagnosis of AMPPE. ResultsDuring the following year, a gradual change in her personality developed. The diagnosis of SSPE was made by cerebrospinal fluid examination. Her clinical condition deteriorated rapidly, and she died. ConclusionsAMPPE can be accompanied by central nervous system involvement. We wish to emphasize the importance of considering the diagnosis of SSPE in cases of AMPPE.

Serous retinal detachment with swelling of the optic disc after a bee sting

Vasculitis (with cerebral involvement) papillitis, Guillain-Barré syndrome, and other neuro-ophthalmological diseases have been described in connection with bee toxins. We observed an unusual case of serous retinal detachment with bilateral disc swelling five weeks after a bee sting. Highly elevated titers against bee toxins and slightly elevated circulating immune complexes were found and we therefore proposed a serum sickness immune reaction. As other affections of the outer blood retinal barrier, e.g. acute multifocal placoid pigment epitheliopathy, are thought to be due to an immune complex vasculitis of the choriocapillaris, we suggest that bee toxins cause serous retinal detachment due to global breakdown of the outer blood retinal barrier.