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Optometry & Vision Science:
Case Report

Multiple Evanescent White Dot Syndrome and Acute Zonal Occult Outer Retinopathies

LOMBARDO, JAMES OD, FAAO

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Author Information

Northwest Eye Surgeons, Seattle, Washington

Received September 17, 2002;

revision received March 27, 2003.

James Lombardo

Northwest Eye Surgeons

10330 Meridian Ave. North, Suite 370

Seattle, WA 98133

e-mail: jlombardo@nweyes.com

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Abstract

Background. Multiple evanescent white dot syndrome (MEWDS) is a rare, acute, multifocal retinitis located in the posterior pole. Patients with MEWDS may report photopsia, central scotoma, or decreased vision. Prodromal viral-like symptoms may be associated with this syndrome, but its etiology remains unknown. The condition is self limiting with a favorable prognosis for visual recovery within several weeks.

Case Report. A young, myopic, female patient presented with painless photopsia, scotoma, and subsequent vision loss secondary to MEWDS. The clinical appearance, differential diagnosis, and management of MEWDS are discussed.

Discussion. Careful ophthalmoscopic and angiographic interpretation along with complete history taking are the keys to diagnosing MEWDS. Ancillary testing including electroretinogram, early receptor potential, and visual fields aid in the differential diagnosis. Once the diagnosis has been established, patient reassurance is appropriate in the management of this condition.

Multiple evanescent white dot syndrome (MEWDS) was originally described in 1984 by Jampol et al. 1 The disease is characterized as a rare, acute, unilateral, multifocal retintitis, typically affecting young women. The fundus presentation typically includes numerous perifoveal white dots scattered primarily in the posterior pole and just beyond the major arcades. A granular foveal appearance is also evident in the affected eye. Symptoms of afflicted patients include visual acuity decrease, scotoma, and photopsia. Systemic history may be significant for prodromal flu-like symptoms including low-grade fever or upper respiratory infection. Approximately 50% of patients in the MEWDS case series of Jampol et al. 1 had prodromal flu-like symptoms.

Despite the possible association with antecedent upper respiratory infection, infectious etiologies for MEWDS have not been proven. Infectious and immunologic theories have been proposed. Literature review reveals anecdotal reports of select MEWDS patients with elevated total serum IgG and IgM, 2 recent hepatitis A 3 and hepatitis B 4 vaccinations, recent varicella infection, 5 associated increased levels of protein in the cerebrospinal fluid, 6 and a strong association with the HLA-B51 haplotype. 7 Although the etiology remains elusive, relevant examination and ancillary testing for possible MEWDS includes ophthalmoscopy, fluorescein angiography, Amsler grid, visual fields, and electrodiagnostics. Currently, no formal protocol for medical therapy exists. Once the diagnosis has been established, the mainstay of management includes clinical follow-up and patient reassurance. Fortunately, the disease is self limiting, and the prognosis is good for complete recovery within several weeks.

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CASE REPORT

A 29-year-old white woman was referred to the eye clinic with a 2- to 3-week history of painless visual disturbance in her right eye. She described a paracentral photopsia in the right eye. Ocular history was significant for previous retinal detachment and scleral buckle repair of her left eye 1 year earlier. The patient denied having other ocular abnormalities, inflammations, injuries, or surgeries. She wore myopic spectacles measuring −8.75 DS in the right eye and −9.75 DS in the left eye. She had suffered no recent ocular trauma to either eye. Family history was unremarkable for ocular disease, including retinal detachment.

At the time of examination, the patient was not taking medications, and she reported no history of medical allergies. Her general history was significant for a resolved low-grade fever and cough, which she had experienced 1 month before her ocular symptoms. She denied excess stress or pregnancy. She denied use of recreational drugs, alcohol, and smoking.

Ocular examination revealed best-corrected visual acuities of 20/20 in the right eye and 20/20 in the left eye. Monocular finger count visual fields were unrestricted for each eye. Amsler grid testing was normal for each eye. Pupils were equally reactive to bright and dim illumination with no evidence of afferent pupillary defect. Biomicroscopic evaluation of both anterior segments revealed healthy lids and lashes, clear corneas, deep and quiet anterior chambers, and nonpathologic irides. Dilated examination revealed clear crystalline lenses in both eyes. The vitreous media was clear in both eyes with a longstanding vitreous detachment in the left eye only. The left retina was attached with a high scleral buckle evident 360°. No new breaks were noted posterior to the buckle. The right fundus demonstrated a small area of lattice with vitreoretinal traction at 12 o’clock, adjacent to the vitreous base. Scleral depression demonstrated no retinal holes or tears in the right eye. No macular abnormalities were noted. Vitreoretinal traction was considered as a possible cause of the patient’s symptoms. The symptoms of retinal detachment were reviewed with the patient, and she was advised to return the clinic should they arise. Otherwise, the patient was to be followed in 1 month.

The patient returned for follow-up 1 week later, reporting “missing vision” in her right eye in addition to scintillations. Best-corrected visual acuity of the right eye now measured 20/30, and the left eye remained at 20/20. Amsler grid confirmed a cecocentral scotoma consistent with the patient’s symptoms (Fig. 1). Pupil testing revealed normal responses in both eyes without afferent pupillary defect. Biomicroscopic evaluation of both anterior segments continued to be unremarkable. Fundus evaluation of the left eye was also unchanged. Fundus evaluation of the right eye revealed a cluster of small, scattered white dots in the deep retina of the posterior pole, temporal to the fovea and within the major vascular arcades (Fig. 2). Few vitreous cells were now visible in the right eye only. There was no evidence of subretinal fluid or edema. Subsequent fluorescein angiography demonstrated early- and late-phase hyperfluorescence of the white dots (Fig. 3). Late-phase staining of the right optic nerve was also evident (Fig. 4). The tentative diagnosis was MEWDS. Automated perimetry and electrodiagnostic testing were ordered but later declined by the patient. The patient was followed for 2 months, during which vision returned to 20/20 with complete clinical resolution of retinal lesions in the right eye.

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Figure 2
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Figure 3
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Figure 4
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DISCUSSION

MEWDS is an uncommon, acute, unilateral retinopathy affecting young adults. The condition is typically benign, self limiting, and rarely recurrent. It may be associated with symptoms of transient vision decrease, photopsia, or scotoma. Described originally in 1984, 1 the condition manifests as white dots visualized in the deep retina or retinal pigment epithelium (RPE) of the posterior pole. Foveal granularity may also be visible. Making the correct diagnosis of MEWDS may be difficult due to the subtle, rapidly resolving, or “evanescent” retinal findings and vision changes. 8, 9

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Epidemiology

MEWDS is uncommon and has no racial or regional predilection. Cases have been reported worldwide, including the United States, Europe, Japan, and China. 10 Young adults are primarily affected, with up to 75% being female. The average age of involvement reported initially was 28 years, with a range of 17 to 38 years. 1 More recently, a 64-year-old male and a 67-year-old female have been reported to spontaneously recuperate from a MEWDS presentation. 11 Furthermore, in 1998, MEWDS was diagnosed in a 10-year-old boy. 12 Although MEWDS is typically unilateral and nonrecurrent, bilateral and recurrent cases have been reported with less frequency. 5, 13–18

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Etiology

At this time, the cause of MEWDS is undetermined. An infectious syndrome has been suspected because the condition is often preceded by a flu-like illness, as was the case with our patient. Also supporting an infectious etiology is an anecdotal report of high levels of total serum IgG and IgM during an acute case of MEWDS. 2 MEWDS was reported after a varicella infection, 5 and a case was also reported in association with increased levels of protein in the cerebrospinal fluid. 6

Baglivo et al. 4 reported a 23-year-old woman who developed MEWDS within 24 h of a hepatitis B vaccination. Fine et al. 3 recently reported a case of MEWDS developing in a 30-year old woman 13 days after a hepatitis A vaccination. Because these vaccines contain inactivated virus particles, the authors suspected an immune response mechanism in the pathogenesis of MEWDS.

Borruat et al. 7 found a 3.7-fold increased frequency of HLA-B51 in patients affected by MEWDS, supporting the possibility that MEWDS could be associated with an autoimmune or environmental antigen-driven reaction. However, due to the limited number of patients in their study (nine), these results need to be confirmed in a larger patient population before HLA subtyping can be recommended to rule out MEWDS as a differential diagnosis.

Abnormal electroretinogram and early receptor potential studies in some MEWDS patients support the possibility of a metabolic disturbance of the RPE-photoreceptor complex. 10, 19 Noting that MEWDS affects predominantly healthy young women, many of whom are taking birth control pills, Gass and Hamed 20 have questioned the role of hormonal status as a predisposing factor.

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Symptoms and Signs

MEWDS typically presents with reduced visual acuity in the range of 20/20 to 20/300 (Table 1). Patients may also complain of scotoma, photopsia, and dyschromatopsia. 1, 10 Gray-white dots, approximately 100 to 200 μm in size, are located in the perifoveal area, extending sparsely beyond the major vascular arcades. 10, 21, 22 These white spots are often small, ill-defined, and potentially easy to overlook. Often, an orange or yellow macular granularity is visible. A transient, afferent pupil defect has been reported, but not uniformly, in the literature. Vitreous cells are often visible in approximately 50% of MEWDS cases. 20 Occasionally, venous sheathing and optic nerve edema are noted. Choroidal neovascularization has been described in rare, recurrent or consecutive cases of MEWDS. 6, 17, 23 Two recent papers have documented clinically evident, evanescent, circumpapillary white lesions preceding the typical MEWDS findings. 24, 25 Up to 50% of patients with MEWDS report an antecedent flu-like illness. 1, 22 Symptoms include headache, 8 low-grade fever, chills, nonspecific upper respiratory infection, 12, 26 rhinorrhea, and fatigue. 1, 11, 27

Table 1
Table 1
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Visual Field Defects and Optic Neuropathy

The principal visual field defects associated with MEWDS include enlarged blind spot and central or cecocentral scotoma. 8, 10, 22 The visual field may demonstrate marked enlargement of the blind spot irrespective of optic disc size or the distribution of the clinically evident lesions. 13 There have been various suggestions as to the cause of visual field defects associated with MEWDS. These include optic nerve disorders, retinal lesions, and choroidopathy. 28

In their original paper, Jampol et al. 1 described various cases of MEWDS with disc edema. Some cases presented with funduscopically visible disc edema, whereas other cases were associated with healthy-appearing nerves with late-phase staining of the discs on fluorescein angiography. Our patient presented in this latter manner. Afferent pupillary defects were occasionally observed, occurring with delayed onset and eventual resolution during the course of the disease. In their 1990 MEWDS series, Dodwell et al. 15 postulated that optic nerve pathology was causally related to visual field defects, afferent pupillary defects, color vision anomalies, and retention of good central vision. Contradictory reports by Gass 22 assert that there is minimal evidence that damage to the retinal ganglion cell layer and optic nerve are responsible for visual field loss. Ikeda et al. 28 used indocyanine green (ICG) angiography to demonstrate that the visual field defects in MEWDS were possibly caused by retinal dysfunction secondary to inflammatory changes. Also utilizing ICG angiography, Yen and Rosenfeld 29 suggested a pathology of the peripapillary choroidal circulation as cause for transient visual field loss in MEWDS.

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Fluorescein Angiography

Fluorescein angiography demonstrates early and late punctate hyperfluorescence of the gray-white lesions, often in a cluster or wreath-shaped pattern. 22 Disc capillary leakage may also be apparent. ICG angiography has demonstrated early and late hypofluorescent lesions, which are more numerous than those visualized with fluorescein angiography. These lesions are between 50 to 500 μm in size and typically resolve in 4 to 6 weeks. These spots have been considered to represent infiltrative or inflammatory lesions of the RPE, photoreceptors, and choroid. 8, 22, 28, 30

ICG has been shown to demarcate these classic hypofluorescent lesions weeks and months after visual acuity, fundus view, and fluorescein angiography have returned to normal. 13, 29, 31 The authors conclude that these persistent ICG findings may suggest an inflammation or other disease process in the choriocapillaris or precapillary arterioles. Persistent subjective blind spot and associated objective ICG peripapillary hypofluorescence have also been noted by Schelfhout et al. 27 These authors postulate that choroidal involvement may be more extensive than indicated by fundus evaluation or fluorescein angiography.

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Electrodiagnostics

Decreased electroretinography a waves and irregular early receptor potential have been associated with MEWDS. 19 Early receptor potential regeneration times are prolonged. These findings suggest photoreceptor involvement. Oh et al. 32 demonstrated that multifocal electroretinography revealed pigment epithelial damage to the retina in patients with MEWDS and patients with multifocal choroiditis. Whereas multifocal choroiditis patients suffer a persistent diffuse depression of multifocal electroretinography, MEWDS patients demonstrate a greater focal loss initially, followed by near full recovery. Electrooculography studies of a few patients revealed mildly abnormal ratios, also indicative of pigment epithelial impairment. 33

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Differential Diagnosis

The differential diagnosis of MEWDS includes acute retinal pigment epitheliitis, acute posterior multifocal placoid pigment epitheliopathy, birdshot retinochoroidopathy, diffuse unilateral subacute neuroretinitis, acute macular neuroretinopathy (AMN), multifocal choroiditis variants, pseudo-presumed ocular histoplasmosis (P-PHOS) and punctate inner choroidopathy (PIC), acute idiopathic blind spot enlargement syndrome (AIBSE), acute annular outer retinopathy (AAOR), and acute zonal occult outer retinopathy (AZOOR) (Table 2). 1, 8, 10, 34–37 Primary intraocular lymphoma must also be ruled out when making the differential diagnosis in older patients. 38

Table 2
Table 2
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Acute retinal pigment epitheliitis is also a self-limiting, nontreatable retinal disease of young to middle-age persons. This condition manifests in the macula as hyperpigmented clusters within a white halo, and the lesions are not uniform in color throughout as in MEWDS. The fluorescein angiography is unique in that it yields hypofluorescent lesions centrally with hyperfluorescent haloes. 39

Acute posterior multifocal placoid pigment epitheliopathy is a self-limiting condition affecting young men and women. Uveitis is often present, and the lesions are typically bilateral with a creamy color. Acute posterior multifocal placoid pigment epitheliopathy lesions are larger and associated with early angiographic hypofluorescence as opposed to the hyperfluorescence demonstrated by MEWDS lesions. More pigment disruption results from acute posterior multifocal placoid pigment epitheliopathy. Visual recovery usually occurs over a 6-month period. 40

Birdshot retinochoroidopathy affects an older population and is associated with multiple creamy spots in the midperipheral fundus. Its spectrum also includes chronic iritis and vitritis, optic disc edema, vasculitis and cystoid macular edema, distinguishing it from MEWDS. Its course tends to be bilateral and chronic. Patients may test positive for HLA-A29. The vision is usually unaffected until the development of cystoid macular edema. Steroids may be effective treatment. 41

Diffuse unilateral subacute neuroretinitis is caused by an intraocular nematode usually in young persons. In addition to retinal pigment changes and occasional white dots, the disease is prolonged with progressive visual field loss and optic atrophy. 42

AMN is described as red-orange wedge-shaped or petaloid, oval, or round lesions confined to the macula only. The lesions differ from those in MEWDS based on their color, size, and shape. 20 In 1989, Gass and Hamed 20 presented two patients with features common to both MEWDS and AMN. He suggested an overlap of these conditions.

Two variants of multifocal choroiditis are considered differential diagnosis of MEWDS: P-PHOS and PIC. Both are associated with enlarged blind spots.

Multifocal choroiditis with panuveitis, also referred to as P-PHOS, 43–45 affects young females with a sudden vision loss, photopsia, or scotoma. It presents bilaterally with small, multiple yellow-white lesions occurring in the posterior pole, periphery, or both. The lesions become punched-out chorioretinal scars when inactivated. Peripapillary atrophy and choroidal neovascularization may develop. The condition is frequently recurrent. The lesions may respond to steroids or immunomodulators. 43–46

In contrast to true presumed ocular histoplasmosis, which presents without anterior or posterior chamber inflammation, P-PHOS always presents with vitritis and may present with anterior uveitis in half of all cases. Patients with P-PHOS have no predilection for HLA-DR2 or positive histoplasmin skin test as do P-HOS patients. 43 PIC was described by Watzke et al. 47 as a disease of young, myopic, female patients complaining of blurred vision, photopsia, and paracentral scotoma. The condition presents with small, yellow-white punctate lesions in the posterior pole at the level of inner choroid. The lesions may be associated with small, overlying serous detachments. The lesions may give rise to atrophic scars and delayed pigmentation and choroidal neovascularization. Vitreous cells are not associated with this condition. Patients with PIC who are acutely symptomatic can sometimes outline scotomas corresponding to the choroidal lesions. 48 Patients with POHS are typically asymptomatic unless choroidal neovascularization develops. One patient in the initial PIC series 47 underwent electroretinography testing without reported abnormalities.

AIBSE is associated with acute enlarged blind spots without disc edema. Fletcher et al., 49 using multifocal electroretinography, postulated that this was a disease of retinal dysfunction. Vision typically recovers spontaneously and there are no retinal lesions associated with this entity. Callanan and Gass 35 proposed that given the transient nature of its associated lesions, MEWDS may be confused with AIBSE or, indeed, may be part of the same disease entity.

Hamed et al. 50 note that AIBSE has many features common to MEWDS, including photopsia, scotoma, a predominance among young women, electroretinographic abnormalities, resolution in months, and occasional recurrences and bilaterality. Due to the subtle, fleeting, and evanescent findings of MEWDS, the authors postulate that such findings may have actually existed, then resolved before initial evaluation in the original AIBSE series. 49 Despite subjective symptoms of scintillations or scotoma, patients retaining adequate central vision may delay seeking ocular examination until after their objective findings have resolved. For this reason, the authors propose that AIBSE may be a subset of MEWDS, presenting weeks after initial symptoms.

Contradicting opinions have also been published. In 1991, Singh et al. 36 presented a 10-patient series of patients with acute blind spot enlargement. At least five patients could not be classified with a known disease entity or fundus abnormality. These authors assert that MEWDS is a subset of AIBSE.

Watzke and Shults 51 recently presented a 25-patient, retrospective case series that challenged the notion of AIBES as an isolated disease. Within their patient group, they found overlapping presentations of MEWDS, AMN, AZOOR, multifocal choroiditis, and AIBSE. All patients in the study reported blind spots and scintillations. These patients were followed from 1 year and 7 months to 15 years and 6 months. The authors concluded that if a patient with an acute, symptomatic blind spot is examined within 2 weeks of onset, signs of the forementioned chorioretinal diseases will usually be present. The authors assert that the time interval between symptom onset and ocular examination is crucial because beyond a 2-week interval, transient or evanescent presentations such as disc congestion, disc staining, peripapillary retinitis, foveal changes, and peripheral retinal spots may not be present.

Three recent papers 52–54 describe cases of acute annular outer retinopathy. They separately presented a total of six patients with a concentric enlargement of a white, annular outer retinal ring located in the peripapillary area. Loss of retinal transparency and blood vessel narrowing were also evident within the ring. Unlike previous reports of circumpapillary chorioretinopathy preceding MEWDS findings, 24, 25 acute annular outer retinopathy presented with the white ring only, with no evidence of subsequent white dots in the fundus. This objective finding corresponded to subjective visual field loss. The annular ring spontaneously resolved in all cases with or without steroid treatment. Gass and Stern 52 proposed that the ring could be immune-related after a previous viral infection. This condition, along with others, is thought to be a variant of AZOOR according to Gass.

In 1993, Gass 55 reported a series of 13 patients, mostly women, with visual field loss and photopsia. These cases were associated with one or more large zones of outer retinal function, minimal fundus changes, and electroretinographic abnormalities in one or both eyes during initial evaluation. Delayed arteriolar narrowing and depigmentation of the retinal pigment epithelium developed during the course of the disease. Late-onset, segmental bone-spicule pigmentation occasionally developed in the overlying retina of permanently affected fundus. Associated visual field defects included enlarged blind spot and superotemporal defects associated with late fundus changes. Rather than attribute this constellation of findings to a new disorder, Gass placed them within a spectrum of disease.

Gass noted that these patients shared common features with those of MEWDS, AIBSE, AMN, and P-PHOS. Common to his 13-patient series and these four conditions were the following: a preponderance of women, acute loss of large zones of outer retinal function, electroretinographic abnormalities, and minimal corresponding fundus changes. Gass stated that the female predilection suggests autoimmune involvement in the pathogenesis of these disorders. He later developed a new classification of acute zonal occult outer retinopathies to encompass these similar disorders (Table 3). 56

Table 3
Table 3
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Gass and others have suggested that MEWDS, AIBSE, AMN, and P-POHS, PIC, and AZOOR may be variations of one disease entity. 20, 35–37, 55, 56 Alternatively, Jampol and Wiredu 57 note that there may be many overlapping findings that happen to be common to many chorioretinal syndromes with different etiologies. Because the etiologies for these reported syndromes are unknown, both theories remain plausible at this time.

Shah et al. 38 recently reported on three older patients, aged 53 (two patients) and 58, with primary intraocular lymphoma, presenting monocularly with white fundus lesions similar to those found in MEWDS. Although MEWDS is occasionally found in this age group, it is very rare. 11 The resolution and return of the lesions was unique to this sample. Therefore, the authors suggest including primary intraocular lymphoma in the differential diagnosis in this age group. Diagnostic vitreous biopsy, magnetic resonance imaging of the brain and orbits, and lumbar puncture may be used to rule out this rare entity.

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Treatment

Because MEWDS is typically self limiting with good visual recovery, no treatment is currently indicated for the acute stage of the disease. Laser photocoagulation of rarely seen choroidal neovascularization may be appropriate in select patients. Future research may also demonstrate a possible role for photodynamic therapy to treat neovascularization associated with MEWDS.

There has been one reported case of MEWDS responsiveness to cyclosporine therapy. 58 However, this case was atypical in its bilateral simultaneous and recurrent presentation. The 46-year-old woman described in the report suffered nine episodes of MEWDS over a 7-year period. She suffered no recurrences during an interim 2-year period while treated with 5 mg/kg/24 h cyclosporine tapered gradually to 3 mg/kg/24 h. Exacerbation was experienced only when further tapering was attempted. During the 3 years after therapy cessation, she experienced four recurrent episodes. Assuming that the diagnosis was consistent with MEWDS, the treatment success may support an autoimmune origin linked to cellular immunity.

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Prognosis

Visual restoration and resolution of clinical findings of MEWDS are usually noted after 6 to 10 weeks. There have been reports of window defects in the fundus replacing the resolved white lesions. Persistent enlargement of the blind spot on visual field testing has also been reported. This may cause diagnostic difficulty for patients who were not examined during the acute stages of the illness. 13

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CONCLUSIONS

Our patient was interesting in that her initial presentation suggested possible vitreoretinal traction as the cause of photopsia. She presented initially without retinal MEWDS lesions or vitreous cells. It is possible that these findings were initially overlooked given their subtle nature or that the lesions became more evident as the disease process progressed. Her history of previous retinal detachment in the fellow eye and accompanying lattice in her affected eye were, in retrospect, “red herrings” in terms of the underlying pathology relating to her photopsia.

This case underscores the importance of timely follow-up and serial ophthalmoscopy when making the differential diagnosis of scotoma and photopsia without fundus lesions. When fundus findings are few or absent, electrodiagnostic testing in conjunction with visual fields may be useful to rule out primary optic nerve disease and the need for ancillary neurologic evaluation.

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ACKNOWLEDGMENTS

I thank Robert Gander, OD, (Lenscrafters) and Sam Barloon, MD, (Retinal Specialist, Northwest Eye Surgeons) for allowing me to share in the care of this most interesting patient. Additionally, I thank Denise Hess and D. J. Matthews (Southern California College of Optometry) for their time and resourcefulness. Finally, I thank Jim Foltz (Retinal Photographer, Northwest Eye Surgeons) for his time and expertise.

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Keywords:

evanescent; electroretinogram; early receptor potential; hepatitis; multiple evanescent white dot syndrome; acute zonal occult outer retinopathy

© 2003 American Academy of Optometry

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