Multimodal Imaging of APMPPE, Related Disorders

Recognizing the distinctive features of each placoid disorder is critical for accurate and timely diagnosis and management.

By Meena George, MD, PhD, Pamela Golchet, MD, K. Bailey Freund, MD, and David Sarraf, MD, Los Angeles
2/10/2014

Acute posterior multifocal placoid pigment epitheliopathy and serpiginous choroiditis are two well-defined members of the white spot syndromes with characteristic placoid lesions. Variant placoid entities have been described that resemble these two better-known disorders and include macular serpiginous choroiditis, tubercular serpiginoid choroiditis, relentless placoid chorioretinitis and persistent placoid maculopathy. Although the conditions are rare, recognizing the distinctive features of each is critical for accurate, timely diagnosis and management and, thus, for optimizing visual prognosis. In this review, we summarize the clinical presentation, diagnosis and management as guided by multimodal imaging for these variant placoid white spot syndromes.

APMPPE

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) was first described by J. Donald M. Gass, MD, in 1968.1 It typically presents in young adults with bilateral vision loss and may be preceded by a viral illness. Presenting symptoms may include photopsia, decreased vision, paracentral scotoma or metamorphopsia. There is no gender or ethnic predilection. On fundus examination, APMPPE is characterized by randomly scattered, flat multifocal creamy white or yellow plaques at the level of the retinal pigment epithelium with indistinct margins. Plaques are typically located in the macula but may also involve the peripheral quadrants (See Figure 1A). New lesions can develop, and as such, lesions of differing age may be present at any given time.

On fluorescein angiography, active lesions demonstrate early hypofluorescence (See Figure 1B) and late staining (See Figure 1C). Inactive lesions show hyperfluorescence corresponding to window defects from retinal pigment epithelium atrophy. Indocyanine green angiography shows hypofluorescence of active and healed lesions.

Figure 1. Acute posterior multifocal placoid pigment epitheliopathy. A) Color fundus photograph showing placoid lesions in the macula and periphery of the left eye. B and C) Fluorescein angiogram of the same patient three months prior demonstrating early hypofluorescence (B) and late staining (C) of the lesions. D) SD-OCT showing lesion with irregular thickening of the retinal pigment epithelium and patchy loss of the ellipsoid zone and RPE layers. E) On fundus autofluorescence, active lesions are hypoautofluorescent due to a blocking effect by the outer retinal lesions, but with resolution develop a hyperautofluorescent rim thought to be secondary to altered RPE metabolism and lipofuscin deposition.
Recent analyses of APMPPE lesions using spectral-domain optical coherence tomography demonstrate various stages of retinal morphologic changes.2,3 At onset, the placoid lesions appear as prominent, dome-shaped elevations of the ellipsoid zone (EZ) band, with hyper-reflective material and subretinal fluid accumulation.4 Over the course of the disease, the dome-shaped lesion flattens, the EZ thickens, the outer nuclear layer shows hyperreflectivity followed by thinning, and the RPE thickens (See Figure 1D).5 At three months, there is partial restoration of the outer macula, reconstitution of the EZ band and minimal residual RPE irregularity.2 These findings of outer retinal pathology in the absence of inner retinal abnormalities indicate that the choroid likely plays a role in this disease.

Fundus autofluorescence in the acute phase shows hypoautofluorescence of lesions. This hypoautofluorescence may be due to a blocking effect by the outer retinal lesions versus RPE edema or direct RPE damage with decreased lipofuscin production. After resolution, lesions may demonstrate hyperautofluorescence due to deposition of lipofuscin or altered metabolism of affected RPE cells (See Figure 1E),6 but often the hypoautofluorescence persists. Further histological studies are necessary to determine the true pathogenesis and whether the primary site of damage is the outer retina and the RPE versus choroidal hypoperfusion leading to RPE and outer retinal damage.

Active plaques resolve spontaneously over a course of approximately two to four weeks, often with mottling of the RPE but without atrophy of the choroid. Visual prognosis is usually excellent, with most patients recovering their baseline visual acuity, although prognosis is less favorable when there is foveal involvement. There are uncommon cases of severe vision loss from significant RPE alterations in the fovea or due to complications from choroidal neovascularization. Recurrences are rare.

In most cases, APMPPE lesions can be observed and will resolve without any intervention. However, several reports have linked APMPPE to central nervous system vasculitis, with manifestations ranging from headaches to venous sinus thrombosis.7 This combination of APMPPE with neurologic manifestations occurs more frequently in males and very rarely can have dire consequences, including death. Treatment recommendations in such cases include IV corticosteroids followed by a slow oral taper in combination with an immunosuppressant.

Serpiginous Choroiditis

In contrast to the typically short-lived course of APMPPE, serpiginous choroiditis is characterized by bilateral, progressive and often recurrent inflammation of the choroid and outer retina.8 It usually presents in healthy, white, middle-aged adults, with a slight male predilection.9Although typically a bilateral disease, patients often present with unilateral activity and decrease in vision once the fovea is affected. Other common symptoms include small scotomas or metamorphopsia.

On examination, the anterior chamber is generally quiet, whereas up to 50 percent of patients may exhibit fine cells in the vitreous. Peripapillary gray-white or yellowish serpentine lesions that extend centrifugally are the classic finding by ophthalmoscopy (See Figures 2A and 2B). Recurrences are contiguous with previous lesions and often assume a pseudopodal pattern. Chorioretinal atrophy in a jigsaw-puzzle configuration may ensue (See Figure 2A). Due to the gradual extension of these geographic (serpiginous) zones of chorioretinal and RPE infiltration and then atrophy, serpiginous choroiditis has previously been referred to as “geographic helicoid peripapillary choroidopathy”10 and “geographic choroidopathy,”11 among other nomenclature.

On FA, acute serpiginous lesions demonstrate early hypofluorescence (See Figure 2C) likely due to both choriocapillaris nonperfusion and blockage from RPE and outer retinal edema. Progressive hyperfluorescence at lesion margins may represent intact choriocapillaris. Over time, there is staining of the acute lesions (See Figure 2D). ICGA shows early and late hypofluorescence of lesions, which appear larger than the corresponding lesions on examination or FA. Recurrences are common at the edges of prior atrophic scars and can occur after long periods of quiescence (even after several years). In the majority of patients the disease recurs, often several times.12

SD-OCT shows hyperreflectivity of the outer retina13 and RPE with minimal distortion of the inner retinal layers.14 Upon healing, lesions demonstrate loss of the RPE, photoreceptor outer segments, and EZ band with choroidal hyperreflectivity.14

FAF can be used to follow the course and demarcate the disease.15,16 Active inflammation appears hyperautofluorescent, while older inactive lesions appear hypoautofluorescent. A hypoautofluorescent halo that surrounds all edges of active hyperautofluorescent lesions serves as a transitional stage between active and inactive inflammation. Inactive inflammation shows dark, hypoautofluorescent lesions with sharp borders without any hyperautofluorescence at lesion edges.

With involvement of the macula, central vision is often significantly compromised. An important complication of serpiginous choroiditis is the development of choroidal neovascularization, reported to occur in about 13 to 20 percent of cases.17,18 Other complications described include cystoid macular edema, pigment epithelial detachments and branch retinal vein occlusions.19,20

Figure 2. Classic serpiginous choroiditis. A and B) Color fundus photographs of the right and left eyes showing yellowish peripapillary serpentine lesions. The right eye (A) displays jigsaw-like chorioretinal atrophy with pigmentary changes that occurs after active lesions resolve. The left eye (B) shows active serpiginous choroiditis. On FA, active lesions (left eye) demonstrate early hypofluorescence (C) and late staining (d).
 
Figure 3. Tubercular serpiginoid choroiditis. A) Yellowish choroidal lesion in the inferior macula on initial presentation of tubercular serpiginoid choroiditis in a 42-year-old Indian male. The lesion demonstrates early hypofluorescence (B) and late staining (C). Over the course of two to three months, the patient developed several new lesions along with extension of existing choroidal lesions in a serpiginoid pattern (D). Wide-field autofluorescent montage demonstrating the extension of the old lesion and the presence of new foci of choroidal lesions (E). Wide-field fundus autofluorescence (F) reveals hypoautofluorescent inactive lesions as well as lesions with a stippled pattern of hypo- and hyperautofluorescence, suggestive of lesions that are still active but in the early phases of healing. The patient was treated with a four-drug TB regimen, oral prednisone and ultimately periocular and intraocular steroids in order to prevent further progression.
Various strategies have been attempted to treat acute episodes of serpiginous choroiditis and to prevent recurrences. The mainstay of treatment is oral (or periocular) corticosteroids. Patients may relapse or recur when tapered to a dose less than 15 milligrams per day of per oral prednisone.9 Intravitreal corticosteroid implants may be more effective in preventing recurrences. Immunomodulatory agents including cyclosporine A, azathioprine and mycophenolate mofetil have been employed to prevent recurrences, with mixed reports of success. Additional strategies attempted are “triple-agent therapy,” which includes cyclosporine A, azathioprine and prednisolone, as well as alkylating agents (e.g., cyclophosphamide or chlorambucil) in extreme cases, although caution must be used given the potentially severe side-effects of these drugs.9,11 Laser photocoagulation and, recently, anti-VEGF agents have been used to treat CNV associated with serpiginous choroiditis.18,19,21
 
Macular Serpiginous Choroiditis

A variant of serpiginous choroiditis termed “macular serpiginous choroiditis” was first described by Robert A. Hardy, MD, and Howard Schatz, MD, in 198710 and shares a number of features similar to classic peripapillary serpiginous choroiditis. However, in the latter entity, lesions begin around the nerve and recur centrifugally toward the macula, whereas macular serpiginous lesions commence in the macula and recur in a pseudopodal pattern centripetally towards the nerve.22 FA and ICGA findings are essentially the same and treatment strategies are similar.23 Because of its onset in the central macular region, visual acuity deteriorates early on and permanent vision loss is more profound and difficult to treat. Macular serpiginous choroiditis can also be complicated by CNV, further worsening the visual prognosis.22

Tubercular Serpiginoid Choroiditis

Included in the differential diagnosis of the placoid entities, particularly in areas of endemic tuberculosis, such as India, is tubercular serpiginoid choroiditis. It presents in either of two ways: discrete, noncontiguous multifocal choroiditis that later becomes diffuse and contiguous with an active advancing edge resembling classic serpiginous choroiditis (See Figure 3); or as a diffuse plaque-like choroiditis with amoeboid spread.24,25 Aqueous and vitreous humor aspirates from two reported cases were positive for Mycobacterium tuberculosis by polymerase-chain reaction analysis.26

In a retrospective study by Reema Bansal, MD, and colleagues, of 105 patients with confirmed TB by positive tuberculin skin testing or QuantiFERON-TB gold testing, more than 70 percent of cases were male, with a mean age of 33 years. The majority (80 percent) of patients presented with vitritis and over 60 percent had bilateral disease. Lesions were present in both the posterior pole and the periphery. In contrast to classic serpiginous choroiditis, lesions were usually not contiguous with the optic disc (>85 percent). Patients treated with antitubercular therapy (four-drug regimen) and oral corticosteroids demonstrated good responses. The addition of antitubercular treatment reduced recurrences whereas those treated with oral steroids alone had a 75 percent recurrence rate. Lesions were followed using autofluorescence to monitor response to therapy.24 Active lesions showed ill-defined hyperautofluorescence with an amorphous appearance. In the early phase of healing, a thin surrounding rim of hypoautofluorescence with a central stippled pattern developed; on complete resolution, lesions were uniformly hypoautofluorescent (See Figure 3F). With treatment, the fovea was spared in 75 percent of patients, including those with macular involvement. Up to 3.5 percent of patients developed CNV.

Interestingly, 14 percent of patients were reported to have progression of disease after initiation of antitubercular therapy.24,25 Some have proposed that tubercular serpiginoid choroiditis is an immune-mediated hypersensitivity reaction to the acid-fast bacilli sequestrated in the RPE and that dying bacilli release proteins that can paradoxically aggravate the immune-response and initially worsen the choroiditis, a type of Jarisch-Herxheimer reaction.24,25 An alternative possibility is a delayed effect of antitubercular therapy with worsening of the underlying disease by the corticosteroids. However, this subset of patients responded to increased oral corticosteroids or the addition of immunosuppressive therapy, supporting the former hypothesis. Thus, identifying the tubercular variant of serpiginous choroiditis is critical for initiation of appropriate antibiotics in combination with oral steroid therapy and one should not terminate antitubercular treatment even in the setting of initial paradoxical worsening.

Relentless Placoid Chorioretinitis

Relentless placoid chorioretinitis (RPC) is an entity that has features resembling both APMPPE and serpiginous choroiditis, but with an atypical retinal distribution and clinical time course.27 The hallmark of this disease is the development of numerous new lesions during the disease course, which may span up to two years, eventually resulting in 50 to hundreds of lesions. Peripheral lesions can precede or occur simultaneously with posterior involvement and recurrences do not necessarily initiate at the edges of prior lesions (as in serpiginous). Because of its shared features with APMPPE and serpiginous choroiditis, this entity was termed “ampiginous” by Robert B. Nussenblatt, MD.9,28

RPC presents in young patients, usually with a sudden decrease in vision and/or metamorphopsia with no history of a viral prodrome. An anterior chamber reaction or vitritis may be present.14,29 Patients exhibit small, white, placoid lesions at the level of the RPE both anterior and posterior to the equator. Some of these lesions develop pigmented chorioretinal atrophy within weeks while others have a drawn out course of persistent activity or new growth. Angiographic findings are similar to those of APMPPE and serpiginous choroidopathy.27,28,30 Only one case of RPC with OCT imaging31 and a different case with FAF imaging16 have been reported. On time-domain OCT, the active stage of the disease demonstrated hyperreflectivity of the inner and outer retinal layers with subfoveal fluid accumulation and a pigment epithelial detachment. With quiescence, normal foveal anatomy was reestablished.31 FAF revealed extensive confluent areas of marked hypoautofluorescence corresponding to areas of chorioretinal atrophy.16

Vision can decrease significantly in RPC, especially with foveal involvement. However, if treated with prolonged systemic corticosteroids, most patients can recover most of their prior vision.27 In the absence of treatment with systemic steroids, final visual acuity tends to be compromised with prolongation of disease course.28 Complications of RPC include CNV, subretinal fibrosis, subretinal fluid and epiretinal membrane formation.

Persistent Placoid Maculopathy

Persistent placoid maculopathy (PPM) was first described by Pamela Golchet, MD, and colleagues in 2006.32,33 It resembles macular serpiginous choroiditis in its early involvement of the macula and predominantly affects middle-aged Caucasian males. Unlike in macular serpiginous choroiditis, patients with PPM initially present with only mild visual symptoms.

Anterior chamber inflammation or vitritis is typically absent.32,33 On ophthalmoscopy, jigsaw-pattern, whitish placoid lesions centered around the fovea that are not contiguous with the optic disc (See Figure 4A) are noted. Unlike serpiginous or macular serpiginous choroiditis, PPM lesions have a longer time course and gradually fade over months to years without the development of new lesions. FA demonstrates early hypofluorescent plaques that partially fill in late phases of the study (See Figures 4B and 4C). ICGA shows hypofluorescent plaques throughout the study (See Figure 4D). SD-OCT shows retinal thinning with photoreceptor and RPE disruption. Once the disease is stable, SD-OCT shows well-defined areas of photoreceptor loss, collapse of the outer plexiform layer and thinning of the choriocapillaris.34 In the presence of CNV, intraretinal fluid or neurosensory detachment may be present.35 FAF shows a speckled pattern of hyper- and hypo-autofluorescence in the macula.34

Figure 4. Persistent placoid maculopathy. A) Color fundus photograph showing white plaque-like lesion in the macula of the right eye. The patient’s best-corrected visual acuity was 20/70 on presentation and declined significantly to 20/400 due to CNV. The white macular lesion becomes fainter over time on serial fundoscopy. B) Early-phase FA shows hypofluorescent plaques with punctate areas of hyperfluorescence. C) Late-phase FA demonstrates multiple punctate spots of hyperfluorescence with partial filling of the hypofluorescent regions. In this particular case, there is almost complete resolution of the hypofluorescent regions. Early hyperfluorescent spots were associated with leakage and indicate choroidal neovascularization. D) Persistent hypofluorescence on indocyanine green angiography. The choroid remained hypofluorescent in the area of the lesion for a period lasting more than 14 months.
Despite macular involvement, visual acuity is only mildly affected in PPM, with good prognosis for visual recovery unless RPE atrophy or CNV develops. While development of RPE atrophy is rare, CNV occurs at a much higher incidence in PPM than any of the previously described placoid diseases (only three eyes of 16 total eyes reported thus far did not develop CNV).32-34 Treatment with oral or periocular corticosteroids and cyclosporine have resulted in improvement in visual acuity prior to complications with CNV.32-35 In one case, chronic immunosuppression was initiated early in the diagnosis of PPM and no CNV developed in either eye at one-year follow-up.34 Treatment strategies for PPM-related CNV prior to the age of anti-VEGF agents have included sub-Tenon’s and intravitreal triamcinolone acetonide, oral prednisone, laser photocoagulation, photodynamic therapy and submacular surgery, with limited success and subsequent formation of disciform scarring in the majority of cases.32,33 There is one reported case of PPM-related CNV treated with intravitreal bevacizumab with good visual outcome followed over a period of two years.35

A summary of the clinical and angiographic characteristics of the spectrum of placoid white spot syndromes is provided in Table 1. In 2002, Nadia Bouchenaki, MD, and colleagues described these disorders with characteristic hypofluorescence on ICGA as indicating choriocapillaris nonperfusion and classified them as inflammatory choriocapillaropathies.30 With the aid of newer spectral-domain OCT studies, it appears there is also outer retinal involvement, and exactly where the site of primary insult resides still remains unclear. Future studies looking at enhanced depth imaging of the choroid may provide greater insight and understanding of the pathogenesis of placoid WSS. The etiology of WSS remains unknown, although the increased prevalence of systemic autoimmunity in patients with WSS and their relatives suggests WSS occurs in families with a genetic predisposition toward autoimmunity.36

Recognizing the different entities among the spectrum of the white-spot syndromes is important for the general ophthalmologist and especially for the retina specialist. Among the placoid WSS, APMPPE is the most common and benign with a viral prodrome and multiple creamy lesions that often fade within a few weeks as visual acuity recovers. Visual prognosis is more ominous in cases of serpiginous choroiditis, which endangers the fovea and carries a high risk of recurrences. The majority of patients with this particular placoid lesion permanently lose central vision in at least one eye. Relentless placoid chorioretinitis presents with numerous smaller lesions both anterior and posterior to the equator, with a prolonged clinical course and frequent relapses over months to years. With treatment, there is often only minimal sustained vision loss. Finally, persistent placoid maculopathy presents as a placoid lesion in the macula that persists for a time course of months to years; although there is only a mild decrease in visual acuity, it carries a high risk of choroidal neovascularization that may result in significant loss of central vision. Obtaining imaging studies including fundus photos, FA, ICG angiography, SD-OCT and FAF is critical to establishing the correct diagnosis and guiding management. Close clinical follow-up and providing timely treatment when indicated are paramount in preventing or at least minimizing permanent vision loss.   REVIEW


Dr. George is in his final year of residency at the Jules Stein Eye Institute, UCLA, Los Angeles. Contact him at: meenageorge@gmail.com. Dr. Sarraf is a clinical professor of ophthalmology in the Retinal Disorders and Ophthalmic Genetics Division at Jules Stein. Contact him at dsarraf@ucla.edu or (310) 794-9921. Dr. Golchet practices at Southern California Permanente Medical Group in Woodland Hills, Calif. Dr Freund is a clinical professor of ophthalmology at NYU School of Medicine and practices at Vitreous Retina Macula Consultants of New York.



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