White Dot Syndromes

Multiple Evanescent White Dot Syndrome (MEWDS)

Multiple evanescent white dot syndrome (MEWDS) is an acute inflammatory disorder in which patients present with unilateral, multiple, small, white spots at the level of the outer retina and retinal pigment epithelium. Patients typically are young, myopic, and female (75%) and present with symptoms of temporal field loss (enlarged blind spot), blurred vision, and photopsia, often following a flu-like illness. The disorder is typically self-limited with visual recovery over a few to several weeks. During the subacute phase white dots in the fovea may impart a granular appearance; old lesions may fade while new lesions develop in other areas. Vitreous cells and a mild papillophlebitis may occur. Fluorescein angiography (FA) shows early hyperfluorescent dots overlying larger spots (“wreath” pattern) corresponding to the white funduscopic lesions. With indocyanine green (ICG) angiography the dots and spots are hypofluorescent and with fundus autofluorescence the spots are hyperfluorescent and in each case more extensive than seen with color fundus photography or FA. With spectral domain optical coherence tomography (OCT) the spots correspond to loss or discontinuity of the inner segment ellipsoid band and the dots correspond to outer retinal hyper-reflective foci; these resolve briskly with resolution of the illness.

This patient with MEWDS has faint, small whitish lesions characteristic of the white spots noted in this condition. The fluorescein study reveals wreath-like punctate hyperfluorescent spots, which strongly stain in the late study. There is also slight staining of the optic nerve.

This patient with MEWDS has numerous punctate wreath-like hyperfluorescent spots throughout the posterior pole (left) . The spots were slightly larger and less dense in the near peripheral fundus (right) .

In this patient with MEWDS, widespread spots are larger and deeper in the fundus, which causes an alteration in the posterior blood ocular barrier, which is evident as leakage on the late-stage fluorescein angiogram. There is disc staining in this patient, a frequent finding in patients with this condition. Peripapillary atrophy and pigmentary disturbance may be seen in some patients (arrows) . Three weeks later, there is disappearance of the spots and improvement of the vision. Enlargement of the blind spot is seen after the acute manifestation resolves, as is common in patients with MEWDS; however, in some patients, it may only improve but not disappear completely, such as in this case.

This patient with MEWDS has prominently evident white spots. Note the distribution and variable sizes of the spots temporally (upper row, left) and superiorly (lower row, left) . The fluorescein angiogram shows punctate and multifocal hyperfluorescence (middle images) . Following resolution of the acute process, the fundus clears without any evidence of pigment epithelial or choroidal disturbance.

In this patient with MEWDS, spots are predominantly in the nasal posterior pole. They are more prominently evident on the red-free image (middle upper) . The fluorescein angiogram shows hyperfluorescence of some of the nasal peripapillary spots, and dilation of veins with some venular staining, and leakage at the optic nerve (middle lower) . Following resolution of the acute stage of the disease, there is a notable absence of pigmentary and atrophic chorioretinal abnormalities, except for two atrophic spots, most likely due to the inflammation of the pigment epithelium (arrows) . A mild optic neuritis seen in MEWDS may account for the blind spot enlargement.

These clinical photographs are examples of the variability of the white spots that are seen in MEWDS. Some are prominently evident as larger nummular retinal abnormalities (left) . Some are very faintly evident (middle) and, in other cases, there are just a few spots clustered in a small, zonal area (right) . New spots may appear as older lesions fade in a few to several days.

There is always some degree of associated foveal granularity. In this case the clinical findings are quite prominently evident in a patient of African descent, due to the pigmentation in the fundus. This change of the fovea may persist after the acute manifestations.

The ICG angiogram generally shows more hypofluorescent spots than clinically evident or seen on the fluorescein study. Surrounding the optic nerve, there is a collarette of hypofluorescent spots with confluency, accounting for enlargement of the blind spot described in these patients (right image) .

The fluorescein angiogram in this patient with MEWDS shows punctate wreath-like hyperfluorescent dots, some of which overlie larger hyperfluorescent spots. These “dots and spots” reside at the level of the outer retina and ellipsoid, respectively, on the basis of recent en-face imaging studies.

The ICG angiogram of this patient with MEWDS shows numerous deep retinal lesions with some confluence. Involvement of the peripapillary area is associated with blind-spot enlargement.

This patient has acute MEWDS with enlargement of the blind spot. There are scattered white spots throughout the posterior fundus with minimal abnormalities noted with FA (middle) . A corresponding ICG angiogram (right) shows widespread, hypofluorescent spots.

This patient with MEWDS has white spots throughout the fundus. They are larger than the fine dots that are often associated with this disorder. These lesions are hyperfluorescent with fluorescein angiogram ( left ) but are best visualized as hypofluorescent lesions with late phase ICG angiogram ( right ).

This patient with MEWDS has scattered white lesions throughout the fundus, particularly around the disc and in the nasal periphery. The fluorescein angiogram shows hyperfluorescence of the lesions and staining of the optic disc due to a mild papillitis. The late phase ICG study shows numerous hypofluorescent lesions throughout the fundus and confluent peripapillary hypofluorescence that is very characteristic of MEWDS and correlates with an enlarged blind spot.

This patient with MEWDS has a variant of the disease, which presents with widespread small lesions or “dots” and deeper lesions or “spots.” The clinical photos show numerous white spots. There are larger lesions located in the peripheral fundus. The fluorescein angiogram (second row) shows numerous wreath-like hyperfluorescent dots corresponding to the retinal lesions. The late phase ICG angiograms show an increased number of lesions including many dots and more confluent spots (bottom row, second image and bottom row far right image) .

This young male patient presented with photopsias in the right eye. Color fundus montage demonstrates white spots throughout the posterior pole (top left) . Widefield fundus autofluorescence shows more hyperfluorescent spots than can be identified on color photography (top right) . Spectral domain optical coherence tomography (SD-OCT) illustrates a disrupted ellipsoid zone (second row, left) . Recently en-face OCT imaging has localized the spots to the ellipsoid level and the dots to the outer nuclear layer. Note loss of the inner segment ellipsoid corresponding to the hyporeflective spots (second row) and punctate hyper-reflective foci in the outer nuclear layer (ONL) corresponding to the dots (third row) .

Follow-up autofluorescence (top) and SD-OCT (bottom) illustrates complete resolution of the hyperautofluorescent spots and normalization of the ellipsoid zone band.

Color fundus montage of this patient with MEWDS illustrates white spots scattered throughout the posterior pole and into the midperiphery. Note the granular appearance of the fovea (bottom left) . Follow-up at 6 weeks shows resolution of the white spots and persistent foveal granularity (bottom right) .

Multifocal Choroiditis (MFC) (Punctate Inner Choroidopathy (PIC), Multifocal Choroiditis and Panuveitis (MCP), Idiopathic Progressive Subretinal Fibrosis Syndrome)

Punctate inner choroidopathy (PIC) and MFC are related, if not identical, entities. Both tend to affect young females (<75%) who are often myopic. These patients develop focal areas of inflammation in the deep retina and choroid that progress into punched-out, atrophic, and pigmented chorioretinal scars. The acute lesions are typically multiple, bilateral, and yellow-white or grayish in appearance. Occasionally, there may be an overlying neurosensory detachment. When these inflammatory spots are small and confined to the posterior pole with minimal vitreous reaction, the entity is typically referred to as PIC. More diffuse disease with larger lesions and associated panuveitis is referred to as MFC. These eyes may present with peripapillary fibrosis and linear clusters of lesions in the peripheral fundus forming curvilinear or concentric streaks (Schlaegel’s line) similar to those seen in the presumed ocular histoplasmosis syndrome (POHS). The presence of uveitis, most commonly anterior and vitreous cells, distinguishes MFC from POHS. Like POHS, both PIC and MFC are frequently associated with secondary choroidal neovascularization (CNV), which can lead to subretinal fibrosis. Rarely, this subretinal fibrosis can be extensive and progressive, in which case it is referred to as the idiopathic progressive subretinal fibrosis syndrome.

These patients have multiple chorioretinal inflammatory spots in the posterior pole. Sometimes they are associated with cells in the posterior vitreous or even an exudative detachment (arrows) . Evidence of previous fibrovascular scarring may be seen in some cases (middle row, left) . A more accurate number of the lesions is detectable with fundus autofluorescence (lower right) .

Courtesy of Dr. James Folk

This case demonstrates the acute and resolved forms of MFC in the same patient. Note the whitish lesions in the acute phase (arrows) and the more hyperpigmented, well-defined lesions in the healed stage. The fluorescein angiogram shows staining and even leakage of the acute lesions in the posterior pole (middle image) .

This myopic patient shows the acute lesions of MFC (left) . These acute inflammatory lesions resolve into chorioretinal scars upon follow-up (right, arrows) . The white atrophic lesions in the posterior pole are present within thinned retina associated with the myopic staphyloma.

This patient has MFC and secondary CNV (arrow) . The fluorescein angiogram shows punctate hyperfluorescence of the CNV inferior to the fovea and optic disc leakage. Optic disc inflammation is an important feature of MFC not associated with presumed ocular histoplasmosis syndrome. The right eye developed peripapillary atrophy and pigmentation following acute inflammation (right) .

This patient has MFC complicated by active CNV (arrows), noted with color fundus photography and with FA (left and middle images) . Note that the 2 neovascular lesions are classic (i.e. type 2) with FA showing an early well-defined lacy appearance and a pigment ring of blockage. An ICG angiogram shows widespread multifocal spots throughout the posterior fundus (upper right) and in the periphery multifocal hypofluorescent lesions and more acute hyperfluorescent lesions ( bottom images ) are noted.

The late-phase ICG study of a patient with active MFC and an enlarged blind spot of the left eye demonstrates multiple large hypofluorescent lesions extending toward the periphery and confluence around the temporal border of the optic nerve (arrows) . Oral prednisone therapy was administered for 6 weeks. Another late-phase ICG study 6 months following treatment demonstrates complete resolution of the hypofluorescence in the macula and around the optic nerve. Vitritis and visual field changes also resolved. Pre-existing peripapillary atrophy remains.

This patient had CNV that was ablated with laser photocoagulation (arrow) . He presented 2 years later complaining of an enlarged blind spot in the same left eye. A fluorescein angiogram showed staining of the laser scar and multiple new hypo- and hyperfluorescent spots. The ICG angiogram illustrates hypofluorescence surrounding the nerve and the chorioretinal scar and widespread areas of hypofluorescent lesions in the choroid consistent with recurrent MFC in a patient with a previous episode of MFC. Note the peculiar filamentous extensions around the macular scars, as seen on the angiograms, that are characteristic of MFC. This lesion may be related to hypervascularity induced by the antecedent inflammation.

During a quiescent stage of MFC, the fundus may contain numerous chorioretinal scars in the peripheral fundus with a variable distribution. There may be peripapillary atrophy or macular scarring (arrow) . Each of these cases had peripapillary atrophy. In the periphery, one or more curvilinear or concentric lines of chorioretinal scars may be appreciated (Schlaegel’s line) that are characteristic of MCP and POHS (lower image) .

These patients have manifestations of MFC in its quiescent stage. The montage shows concentric pigmentary scars encircling the globe. Note the presence of pigment hyperplasia in the nasal periphery. The photo on the left shows a macular hole with detachment (arrows) . This may be the result of vitreoretinal traction. The macular images on the right show fibrovascular scarring due to antecedent CNV.

These cases of MFC show the variation in shapes and patterns of the curvilinear or concentric postinflammatory scars referred to as Schlaegel’s lines.

These two patients with MFC demonstrate type 2 neovascularization, the most common subtype in this disorder. A fibrotic scar surrounded by atrophy occurred in the patient on the left following photocoagulation in the left eye. The patient on the right has regressed CNV in each eye with surrounding chorioretinal atrophy.

This montage shows MFC in a quiescent stage. There is severe atrophy in the macular region, as well as pigment epithelial hyperplastic and atrophic change from the inflammatory stages of the disease.

This young female patient presented with photopsias in the left eye. Widefield autofluorescence illustrates small discreet hyperfluorescent spots in the nasal periphery of the left eye (top right) consistent with PIC. She was started on a course of oral NSAIDS and returned 1 month later. The spots are resolved in the left eye (middle right) , but new spots appeared in the temporal periphery of the right eye (middle left) . After 2 months on NSAIDS she returned again and the spots are resolved in both eyes (bottom row) .

OCT angiography of the right eye of the same patient illustrates a choroidal neovascular membrane in the macula.

This patient with MFC presented with an acute flare and new choroidal infiltrates (top row, arrows) . The lesion blocks in early phase fluorescein angiogram and stains in the late phase (top row, second and third images) . SD-OCT illustrates a choroidal infiltrate extending into the outer retina. The patient was initiated on oral steroid therapy with significant improvement (second row) . After tapering steroids, however, the infiltrate recurred (bottom row) .

This patient with MFC presented with new infiltrates in the posterior pole well identified with color fundus photography (top, left) . The lesions stain late on fluorescein angiogram, and block on ICG angiogram (top right and second row left) . SD-OCT at presentation illustrates choroidal infiltrates and thickening with overlying subretinal fluid. Color fundus photos taken 15 days after initiation of treatment show consolidation of the white infiltrates (bottom left) . SD-OCT at follow-up shows a significantly reduced choroidal thickness and resolution of the subretinal fluid (bottom right) .

Acute Zonal Occult Outer Retinopathy (AZOOR)

Acute zonal occult outer retinopathy (AZOOR) is an idiopathic inflammatory disorder usually affecting young healthy women who develop photopsia and acute progressive visual field loss in one or both eyes due to damage of broad zones of the outer retina. The field abnormality typically begins as enlargement of the blind spot, often described with movements of colors within the scotoma. Funduscopy upon initial presentation may be normal with the exception of mild vitritis. However, in later stages, retinal pigment epithelium (RPE) atrophy, pigment clumping, and arterial attenuation may develop. Approximately one-third of patients develop recurrent disease. Electroretinogram (ERG) testing, autofluorescent imaging, fluorescein and ICG angiography, and OCT localize the abnormality to the photoreceptor–RPE complex. Recently a trizonal appearance on autofluorescence, ICG angiography, and SD-OCT has been described as a defining feature of AZOOR. On SD-OCT this is characterized by normal retina outside of the AZOOR lesion (zone 1) and subretinal drusenoid deposits (zone 2) and RPE and choroidal atrophy (zone 3) within the lesion. On autofluorescence and ICG angiography this trizonal pattern is characterized by a hyperfluorescent line demarcating the AZOOR lesion adjacent to normal retina (zone 1), speckled hyperfluorescence within the AZOOR lesion (zone 2), and hypofluorescence corresponding to choroidal atrophy within the lesion (zone 3).

This patient had bilateral AZOOR. The findings were relatively stable in the right eye compared to the left. Note the well-demarcated annular border at the junction between involved and uninvolved retina (arrows) in the right eye. The middle row shows the progressive area of peripapillary inflammation in the fellow left eye at baseline and 3 years later. Five years later, there was a large zonal area also noted in the inferior fundus (bottom row, left). Seven years later, there was diffuse atrophic and pigment epithelial degenerative disease in the fundus ( bottom right ). The fundus autofluorescent montage and magnified image show a characteristic hyperautofluorescent flare at the margins of the zonal defects (lower left) and the aforementioned trizonal pattern of disease.

This color montage shows peripapillary AZOOR with a clearly delineated margin bordering the atrophic region with the normal retina. The larger montage shows a well-demarcated inactive nasal region remarkable for atrophy and pigmentation and a large active zone of progressive inflammation extending from the superior macula and bordered by a characteristic outer annular ring (arrows) .

This is an asymptomatic patient that was diagnosed with AZOOR on routine evaluation. When tested, there was field loss corresponding to the multizonal abnormalities. Note the trizonal pattern of involvement with autofluorescence. The margins of the lesion show a flare of hyperautofluorescence typical of AZOOR. As the acute zonal areas become quiescent, the hyperautofluorescent margins become more normal.

This patient has bilateral symmetric AZOOR. The color photograph of each eye shows peripapillary atrophy. The atrophic area is delineated on OCT imaging by the absence of the inner segment ellipsoid, beginning from the nasal aspect of the fovea (lower left, arrows) . The ICG study reveals a trizonal abnormality. The black area corresponds to absence of choriocapillaris and the gray area represents atrophy of the pigment epithelium adjacent to normal RPE. At the border of abnormal and normal pigment epithelium, there may be a distinct area of fundus hyperautofluorescence, which is actually not present in this patient, although flares of hyperautofluorescence extend from the junction between atrophic pigment epithelium and normal pigment epithelium (arrows, middle row right) .

These images show the variability of AZOOR with atrophic zones present at different locations in the fundus. Note the sparing of the fovea associated with good visual acuity in the top row case. The patient in the middle row experienced photopsia and progressive field loss over several years, but then remained stable for the subsequent 11 years. The lower row shows various peripheral changes in AZOOR, including perivascular inflammatory sheathing (arrows) , atrophy, and hyperplastic pigment epithelial migration into the retina.

This male patient was first diagnosed with AZOOR at the age of 70. Note the delineating annulus or margin of chorioretinal degeneration superior to the nerve. The high-resolution ICG and autofluorescent montage sharply demarcate the zone of peripapillary degeneration. There is a smaller lesion inferiorly (arrow) with an annulus or delineating margin.

The OCT shows the basis for the trizonal pattern. The following can be seen: choriocapillaris atrophy in the immediate juxtapapillary area; adjacent RPE and photoreceptor degeneration with reticular-like drusenoid deposition; and a contiguous normal photoreceptor–RPE–choriocapillaris complex.

This patient had field loss and enlargement of the blind spot. There were no clinical findings evident. The only abnormality was the high-resolution OCT that showed an absence of photoreceptors, which correlated with the field loss. Note the sharp junction between normal and abnormal inner segment ellipsoid.

The fundus image at presentation is on the left, and the follow-up image several years later is on the right. This patient with AZOOR experienced diffuse subretinal fibrosis, not the characteristic pigmentation and atrophy, in the affected macula.

This patient with AZOOR experienced diffuse progressive photoreceptor and pigment epithelial degeneration.

This patient developed recurrent episodes of floaters and blurry vision. Color fundus photos show linear white lines in the parafoveal zones. Late phase fluorescein angiograms (bottom) show late hyperfluorescent lesions. The partial annular pattern of the lesions suggested acute annular outer retinopathy, which is a variant of AZOOR.

Images courtesy of Amani Fawzi, MD

Autofluorescence images of these three patients with AZOOR demonstrate the trizonal pattern characteristic of the disease, as described in the text above.

This patient initially presented with creamy, plaque-like peripapillary lesions seen here on color fundus photography (top row) . Six years later, autofluorescence imaging demonstrates progression in both eyes and the trizonal autofluorescence pattern characteristic of AZOOR (second row) . Progression continued, and 13 years after initial presentation widefield color fundus photos show widespread atrophy of the retina and RPE (third row) . Widefield autofluorescence imaging illustrate an island of temporal sparing, but with surrounding active disease demonstrated by the hyperautofluorescent edge adjacent to the remaining unaffected retina (bottom row) .

Widefield color photographs of this patient with AZOOR illustrates subtle areas of pigment changes in the posterior poles of both eyes (top row) . Widefield autofluorescence demonstrates the typical trizonal pattern in both eyes characteristic of this disease (second row) . Follow-up widefield autofluorescence of the left eye 4 months after presentation shows progression of the lesions (bottom) .

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

APMPPE is a syndrome of multiple, plaque-like, creamy lesions at the level of the RPE that typically affects young healthy men and women in the second and third decade of life. Patients develop rapid visual loss that may be associated with central or paracentral scotomas, photopsia, and metamorphopsia. Most cases are bilateral and the second eye is involved within a few days; however, delayed involvement of the second eye by several weeks can occur. Approximately one-third of the patients report a flu-like syndrome, particularly headaches preceding the visual symptoms. The characteristic clinical finding is the presence of multiple, yellow-white, placoid lesions at the level of RPE, located primarily in the posterior pole. New lesions may develop more peripherally. The size of the lesions varies, but they are usually less than one disc diameter. Associated ocular findings include mild vitritis, papillitis, retinal vasculitis, exudative retinal detachment, and retinal neovascularization and hemorrhage. The active lesions begin to resolve within a few days after the onset of the symptoms and are replaced by RPE atrophy and hyperpigmentation. As the old lesions fade, new active lesions may appear. The visual acuity may return to near normal, but patients may experience prolonged recovery associated with persistent scotomas and more uncommonly even severe vision loss. Rarely stroke and even death have been reported due to central nervous system vasculitis.

This patient with APMPPE presented with a solitary deep whitish-yellow lesion near the macula. Four days later, the lesion enlarged and a satellite lesion developed (arrow) . Two weeks after presentation, there is further progression. Three months after presentation, the resolved lesions appeared atrophic and hyperpigmented.

Note the creamy white lesions at the level of the RPE in this patient with APMPPE. Although most cases of APMPPE resolve with a good prognosis, some patients may develop central hyperpigmentation with a poor visual prognosis, as occurred in this case.

This patient with APMPPE shows a few larger lesions in the posterior pole of the left eye. Note the creamy-colored placoid abnormalities with geographic variation. There are also a few smaller spots in the superior paramacular region. The early fluorescein angiogram shows hypofluorescence from blockage versus a perfusion abnormality of the inner choroid with late staining of the lesions. The fundus autofluorescence shows hyperautofluorescence corresponding to the more recent lesions and hypoautofluorescence matching the healed lesions.

This is a patient with APMPPE and typical fluorescein angiographic (FA) findings of early hypofluorescence and late staining of the lesions. The FA shows visibility of the choroidal circulation in the superior macula, corresponding to window defect or transmitted choroidal fluorescence (arrow) . This is a subacute lesion in which the pigment epithelium has become atrophic.

This patient has APMPPE with typical FA findings of early hypofluorescence (middle) and late staining (right) of the acute lesions. Early hypofluorescence may be attributed to RPE blockage versus choroidal ischemia. More recent evidence with OCT angiography indicates that inner choroidal ischemia is the etiology of APMPPE and related placoid disorders.

Courtesy of Dr. Howard Schatz

These are additional patients with APMPPE that demonstrate variation in the distribution and confluency of the acute lesions.

Top and lower right images courtesy of Dr. Frank Holz

In addition to angiography, fundus autofluorescence (top right) may be useful in determining the precise localization and disease activity of the lesions. Montage photography is also helpful to identify all lesions. Atrophy and pigment degeneration may develop and are associated with a poor visual outcome ( middle right ). Note the diffuse lesion acutely involving the macula (bottom left and right) and confirmed with ICG angiography.

This patient with APMPPE shows acute bilateral geographic macular lesions with a mild papillitis associated with disc staining in the left eye. Spontaneous resolution ensued without recurrence over many years. A presentation such as this must be differentiated from serpiginous choroidopathy, granulomatous disease, and syphilis.

The histopathology in this patient who had APMPPE and died of cerebral vasculitis, shows a choroidal granuloma beneath the RPE with focal disruption of the monocellular tissue. The choriocapillaris was spared. Systemically, the patient had granulomatous vasculitis and multinucleated giant cells in the large cerebral arteries.

This case of resolved APMPPE shows a variation in the healing process. Comparatively minimal atrophy and sparing of the fovea are noted in the right eye (left) while widespread fibrous metaplastic and pigmentary degeneration are identified in the fellow eye.

Courtesy of Dr. Dimitrios Karagiannis

Note the widespread and confluent posterior and peripheral lesions that are acute in the first case (top row) and resolved with atrophy and pigmentary scarring in the second case (bottom row) .

Bottom row courtesy of Dr. Mark Blumenkranz

This patient with APMPPE had associated optic nerve edema and venous stasis bilaterally that was more severe in the right eye. At presentation, there were a few retinal hemorrhages in the left eye during the acute stage of the disease. The right eye then developed frank central retinal vein occlusion (CRVO) with scattered hemorrhages (bottom left) throughout the fundus that block on the fluorescein angiogram (bottom middle) . The hemorrhages in the left eye resolved with less venous engorgement and tortuosity (bottom right image) . This case illustrates CRVO in each eye associated with APMPPE.

A choroidal segmental inflammatory process may rarely be seen in the peripheral fundus of patients with APMPPE as illustrated here. This segmental choroidal vasculitis may not be coincidental because panvasculitis is known to occur in APMPPE. It may also be indicative of an inflammatory process in the choroid as a primary mechanism for the pathogenesis of the disease.

Color fundus montage of this patient with APMPPE illustrates multiple yellow creamy plaque-like lesions scattered throughout the posterior pole and midperiphery of both eyes. Fluorescein angiogram shows early blockage and late staining of the lesions. Autofluorescence of the posterior pole of the right eye at presentation demonstrates hyperautofluorescence of the lesions. Healed lesions at follow-up illustrate hypoautofluorescence. SD-OCT illustrates disruption of the ellipsoid zone and hyperreflective lesions tracking through the outer retina and into the outer plexiform layer of Henle.

Images courtesy of Lee Jampol, MD

Widefield color fundus photos (top left), widefield fundus autofluorescence (top right) and widefield fluorescein angiogram (middle row) in this patient with APMPPE demonstrates more lesions than can be detected with fluorescein angiogram versus color and autofluorescence imaging. These lesions block in the early frames of the angiogram and stain in the late phase. The early blocking may be the result of choroidal inflammation, which prevents normal filling of the choroidal vasculature.

Color fundus photos (top and third row, left) autofluorescence (top and third row, right) and fluorescein angiogram (second and bottom rows) in this patient with APMPPE also demonstrates that more lesions can be seen on fluorescein angiogram than can be seen on autofluorescence, which is likely due to the choroidal location of the lesions. These lesions block in the early frames of the angiogram (second and bottom row, left).

SD-OCT of this patient with APMPPE at 18 months before his acute presentation (left column) is essentially normal. The arrows in the bottom left image denote a normal choriocapillaris. The patient then presented with new visual disturbances and SD-OCT revealed hyper-reflective lesions in the outer retina and intraretinal fluid (middle column). Note the thickening of the choriocapillaris that develops 6 days after presentation (right column).

This 68-year-old man presented with eye pain and a floater in his left eye. Fluorescein angiogram of the right and left eyes illustrates macular lesions that block early and stain late (first and second row) . SD-OCT images of the right and left eyes show irregular elevations at the level of the RPE with overlying disruption of the ellipsoid band and a thickened choroid. The patient was started on oral steroids but presented to the emergency room complaining of decreased coordination shortly after. MRI of the brain illustrates hyperintense lesions scattered throughout the brain, but was not suggestive of cerebral vasculitis. After initiation of further immunosuppressive therapy his symptoms improved.

Images courtesy of Nicole Benitah, MD

Serpiginous Choroiditis

Serpiginous choroiditis is a rare, usually bilateral, recurrent inflammatory chorioretinitis that affects middle-aged men or women. It remains an idiopathic entity; however, recently, many reports have demonstrated an association with tuberculosis exposure in some cases. The acute, grayish-white, subretinal lesions typically originate in the peripapillary region and localize to the outer retina, RPE, and choroid. Over time, there is gradual progression away from the nerve in a helicoid or serpiginous manner, often toward the macula. Chronic lesions show pigmentary changes and fibrous atrophy. New lesions often originate from the margin of older lesions as finger-like extensions. Occasionally, the disorder may originate in the macula, in which case it may be referred to as “macular serpiginous.” CNV is a common complication of serpiginous choroid­itis. A vitritis is seen in approximately one-third of cases. Retinal vasculitis and branch retinal artery and vein occlusions have been reported in some cases.

This patient has serpiginous choroidopathy. Note the pigmentary changes consistent with chronic serpiginous disease. Also note the fluffy whitish-yellow lesion superiorly that represents an active recurrence (arrow) .

This patient shows atrophy and fibrosis originating from the peripapillary area in a serpiginous-like pattern. Note the acute fluffy white lesion at the inferior border of the lesion (arrow in the left image) , which represents a recurrence. Approximately 2 months later, the acute lesion has resolved with atrophy and scarring, and additional acute lesions have developed (arrows in the right image) .

Courtesy of Dr. Stuart L. Fine

This patient with serpiginous choroidopathy demonstrates an acute lesion (arrows) near the central macula. There are multiple foci of chorioretinal scarring. Note the satellite or “skip” lesions (arrowheads) , which are common in this disorder.

The chronic stage of serpiginous choroidopathy with atrophic and pigmented chorioretinal scars can be seen in this patient. Note that the choroidal vasculature can be observed because of the overlying atrophy.

Serpiginous choroidopathy may begin anywhere. This patient has solitary serpiginous choroiditis that started in the macular region (macular serpiginous).

Courtesy of Dr. Maurice Rabb, University of Illinois at Chicago

This patient with serpiginous choroidopathy shows the characteristic geographic, progressive, serpiginoid extension of the process from the disc into the macula and beyond in each eye. There are skip lesions in the periphery of the right eye (left image, arrows) .

These are two additional patients with serpiginous choroidopathy that show a variation in the geometric pattern, commonly referred to as “jigsaw” in nature.

This patient illustrates recurrent serpiginous choroidopathy surrounding a central primary lesion that had healed. The fluorescein angiogram shows staining of the original lesion (middle) . An ICG angiogram shows additional multifocal areas of choroidal staining that could represent quiescent lesions (right) .

In this patient with acute serpiginous choroidopathy, there is a geographic lesion noted with color fundus photography (left) and FA (middle) . The ICG study shows additional multifocal areas of staining in the temporal region of the fellow left eye that were not evident clinically or with FA. These foci may represent occult lesions that have not yet become active.

This patient with serpiginous choroidopathy initially showed a peripapillary lesion in each eye. Over a period of several years, he experienced chronic and/or recurrent acute attacks with extension of the serpiginoid atrophy into the macular region of each eye. He subsequently developed secondary CNV (arrows) in each eye, which is not uncommon in this disorder. The fundus autofluorescence more clearly delineates the hypofluorescent atrophic pattern and multiple skip lesions throughout the fundus.

This patient has serpiginous choroidopathy with multiple large atrophic lesions in the right eye and a single substantial atrophic lesion in the left eye. There is fibrous scarring and pigmentary degeneration in each eye. Fundus autofluorescence shows the sharp demarcation line between the hypofluorescent atrophic lesions and normal retina in this disease.

These patients with serpiginous choroidopathy demonstrate the severe pigmentary and atrophic degeneration and fibrous scarring that may occur in this inflammatory disorder.

Jul 30, 2019 | Posted by in OPHTHALMOLOGY | Comments Off on Inflammation

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