Alessandro Mantovani

Natural evolution of fundus autofluorescence findings in multiple evanescent white dot syndrome: a long-term follow-up.

Purpose: The purpose of the study was to investigate the natural evolution of fundus autofluorescence (FAF) findings in eyes with multiple evanescent white dot syndrome. Methods: This was a retrospective, observational case series of nine eyes of eight consecutive patients with multiple evanescent white dot syndrome who underwent color fundus photographs, fluorescein and indocyanine green angiography, and FAF photography in two referral practices. Results: The mean follow-up was 8.6 months (range, 3-14 months). In the acute/ subacute phase, FAF showed 1) hypoautofluorescent areas, ≤50 μm in size, mostly concentrated around the optic disk and posterior pole; and 2) areas of increased autofluorescence usually found in correspondence to the white dots seen ophthalmoscopically. During the follow-up period, some of the hypoautofluorescent areas faded away, others persisted; the areas originally showing increased autofluorescence variably tended to: 1) become smaller and more demarcated; 2) retract centripetally becoming small hyper-autofluorescent areas surrounded by an hypoautofluorescent halo; 3) turn into areas of decreased autofluorescence; or 4) disappear without becoming hypofluorescent. Conclusion: Multiple evanescent white dot syndrome represents a unique model to study the natural evolution of FAF findings in chorioretinal affections, from the acute phase to resolution; FAF findings, evaluated along with fluorescein and indocyanine green angiography features, can expand our understanding about retinal pigment epithelium and retinal involvement in this rare chorioretinal disorder.

Tuberculosis-related choriocapillaritis (multifocal–serpiginous choroiditis): follow-up and precise monitoring of therapy by indocyanine green angiography

To report the case of a patient initially diagnosed with acute posterior multifocal placoid pigment epitheliopathy (APMPPE), characterized by relentless evolution despite high-dose steroid therapy. An interferon-gamma release assay (IGRA) indicated a diagnosis of suspected tuberculous choriocapillaritis and the disease responded only to massive inflammation suppressive therapy and antibiotic therapy. Case report. Review of clinical features and investigational procedures. Smoldering relentless evolution and subsequent arrest of progression could be precisely monitored by indocyanine green angiography (ICGA). The patient did not recover after standard anti-tubercolosis (TB) therapy combined with corticosteroid. A fourth antibiotic had to be added in order to stop the progression of the retinal disease. In each case of choriocapillaritis such as APMPPE an infectious cause including TB has to be excluded making IGRA tests unavoidable. As the main structure involved is the choriocapillaris the most precise follow-up or monitoring is obtained with ICGA.

Interobserver Agreement in Scoring of Dual Fluorescein and ICG Inflammatory Angiographic Signs for the Grading of Posterior Segment Inflammation

Purpose: To test interobserver agreement in the fluorescein/indocyanine green angiography (FA/ICGA) scoring system for uveitis. Methods: Four observers scored 32 dual FA/ICGAs. Spearman rank correlation was used to analyze correlation between pairs of observers in scores assigned to angiographic signs. Kappa statistics were used to test agreement between pairs of observers in comparative total FA and ICGA scores. Results: The authors found a significant correlation between pairs of observers in scores assigned to all FA signs and all except one ICGA sign. The only discordant sign was early stromal vessel hyperfluoresence on ICGA. There was a moderate to substantial agreement between pairs of observers in comparative FA/ICGA total scores. Conclusions: The level of agreement between uveitis specialists in scoring of dual FA/ICGA indicates that the scoring system tested in this study will be useful for clinical studies of uveitis. Increased experience with this system may further improve its reproducibility.

Use of indocyanine green angiography in uveitis.

Investigational imaging of the choroid was limited with only rough information obtained by echography before indocyanine green angiography (ICGA) became available in the 1990s. In posterior uveitis the choroid is involved at least as often, if not more often, than the retina, meaning that, in a large proportion of cases, information was lacking on an entire compartment involved in the inflammatory process. Indeed there are only few conditions such as the Behçet uveitis where significant choroidal involvement can be excluded a priori. In a large proportion of conditions the inflammatory lesion process involves the choroid. Either it starts primarily in the choroid such as in Vogt-Koyanagi-Harada (VKH) disease, birdshot chorioretinitis (BCR), and the primary inflammatory choriocapillaropathies (PICCPs) including, among others, multiple evanescent white dot syndrome (MEWDS) and multifocal choroiditis (MFC), or it can involve the choroid as one of the possible sites of inflammation such as in ocular sarcoidosis or tuberculosis. Choroidal inflammatory involvement, mostly inaccessible to classical imaging such as fluorescein angiography (FA), is largely occult and therefore missed unless ICGA is performed. New types of optical coherence tomography (OCT) devices allow one to measure choroidal thickness as a whole, but cannot identify choroidal stromal inflammatory foci nor do they allow to analyze morphopathologic changes in the thin choriocapillaris. Given the quality of information obtained by ICGA, mostly not available by other imaging techniques, it makes no doubt that ICGA is one of the major investigational imaging modalities in posterior uveitis.

Interpretation of fundus autofluorescence changes in choriocapillaritis: a multi-modality imaging study

PurposeWe aimed to describe imaging findings in primary inflammatory choriocapillaropathies (PICCPs) after a photobleaching process.Methods2Images from six consecutive cases of patients affected by PICCPs (four with multiple evanescent white dot syndrome and two with multifocal choroiditis) were reviewed. Patients underwent fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and spectral-domain optical coherence tomography (SD-OCT) by means of the Spectralis HRA (Heidelberg Engineering, Heidelberg, Germany). FAF images were acquired at the beginning of the examination in partially dark-adapted conditions followed by light adapted conditions.ResultsDuring the active phase of the diseases, all patients showed areas of increased FAF that became isoautofluorescent after photobleaching. Simultaneously with increased FAF, the ICGA showed typical hypofluorescent dark areas that were more evident in the late phase. SD-OCT showed disruptions in the hyper-reflective band at the ellipsoid zone.ConclusionsFAF and SD-OCT are complementary imaging techniques that show alterations in the outer retina of patients affected by PICCPs.

Sickle cell-hemoglobin C retinopathy: transient obstruction of retinal and choroidal circulations and transient drying out of retinal neovessels

We present a case of sickle cell-hemoglobin C disease that presented acute retinal and choroidal peripheral non-perfusion on the base of chronic microvascular obstruction, which transiently closed retinal neovessels.

Multiple Evanescent White Dot Syndrome: A Multimodal Imaging Study of Foveal Granularity

ABSTRACT Purpose: To describe multiple evanescent white dot syndrome (MEWDS)-related foveal granularity features on different imaging modalities. Methods: Clinical and multi-imaging files from five patients affected by MEWDS were reviewed. Each image set included: blue fundus autofluorescence (BAF), near-infrared fundus autofluorescence (NIR-FAF), spectral-domain optical coherence tomography (SD-OCT), fluorescein (FA), and indocyanine green (ICGA) angiography. Foveal granularity features were analyzed and described for each technique at baseline and follow-up. Results: At baseline, 4/5 patients did not show white dots at funduscopic examination, but all subjects demonstrated hypo-fluorescent lesions on ICGA and areas of increased BAF. In all patients, foveal granularity was detectable on NIR-FAF as an irregular hypo-fluorescent area, persisting during follow-up visits. The corresponding SD-OCT scans revealed outer retinal layers’ disruption resolving overtime. Conclusions: Foveal granularity can be the sole presenting sign of MEWDS. NIR-FAF and SD-OCT should be considered as non-invasive investigations in the detection of MEWDS-related foveal granularity.

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) like other inflammatory entities of the choriocapillaris was included in the purely descriptive pot-pourri group of white dot syndromes, a classification that nowadays has no reason to be maintained. As for many fundus diseases, it was J. Donald M. Gass who first described the disease in 1968. He called the disease acute posterior multifocal placoid pigment epitheliopathy (APMPPE) because he thought that the disease was due to an acute cellular response on the part of the retinal pigment epithelium (RPE) to a local noxious agent. In 1971, van Buskirk et al. suggested choriocapillaris perfusion as the underlying disorder. In 1972 and again in 1977 and 1983, Deutman and colleagues, based on the choriocapillaris nonperfusion seen on early FA frames, indicated that it was the choriocapillaris rather than the RPE that was primarily involved, and they suggested to rename the disease as “acute multifocal ischaemic choriocapillaropathy” (AMIC). With the aid of indocyanine green angiography (ICGA), Dhaliwal et al. identified choriocapillaris nonperfusion as the primary event in APMPPE.