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Elschnig’s spots in the acute and remission stages in preeclampsia: spectral-domain optical coherence tomographic features

Abstract

Purpose

Preeclampsia is a multisystem disorder defined as new onset of hypertension and proteinuria during the second half of pregnancy. Serous retinal detachment (SRD) is a known complication of preeclampsia caused by choroidal ischemia and subsequent disruption of blood-retinal barrier. Poor perfusion of the choriocapillaris causes Elschnig’s spots. We aim to describe distinctive optical coherence tomography (OCT) features of a cluster of Elschnig’s spots in a patient with severe preeclampsia during the acute and remission stages. We also present a summary of OCT features of preeclampsia patients with SRD.

Methods

Descriptive case report.

Results

V-shaped adhesions were observed at the location of each of the Elschnig’s spots, the center of which showed disruption of the outer retinal bands (external limiting membrane, inner segment/outer segment junction layer), and the V-shaped outer parts remained tethered to retinal pigment epithelium (RPE)-Bruch’s complex by hyperreflective exudate on the surface of RPE. These features parallel the histopathologic features described earlier. In an experimental model, Elschnig’s spots showed a gradient of RPE damage from the center to the periphery, where RPE was necrotic in the center and edematous in the periphery. Three months later, spectral-domain OCT (SD-OCT) showed focal RPE elevations at the location of each of the Elschnig’s spots.

Conclusions

We illustrate the distinctive SD-OCT features of a cluster of Elschnig’s spots in the acute and chronic stages of preeclampsia and demonstrate the correlation with the previously described histopathologic features. Despite the favorable visual outcome, irreversible tissue damage may occur, necessitating prompt diagnosis and immediate therapeutic intervention.

Eur J Ophthalmol 2015; 25(5): e84 - e87

Article Type: CASE REPORT

Article Subject: Retina MED

DOI:10.5301/ejo.5000586

Authors

Ala’a AlTalbishi, Samer Khateb, Radgonde Amer

Article History

Disclosures

Financial support: No financial support was received for this submission.
Conflict of interest: The authors have no conflict of interest.

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Introduction

Preeclampsia is a multisystem disorder defined as new onset of hypertension and proteinuria during the second half of pregnancy (1). It affects between 2% and 8% of pregnancies worldwide. It may be associated with cardiovascular, hematologic, hepatic, renal, neurologic, and fetal complications. Eclampsia is life-threatening, characterized by the appearance of tonic-clonic seizures.

Ocular complications that have been described in association with preeclampsia/eclampsia include hypertensive retinopathy, retinal artery and vein occlusion, hypertensive choroidopathy, choroidal infarcts, serous retinal detachment, ischemic optic neuropathy, optic atrophy, Purtscher-like retinopathy, and cortical blindness (1).

Serous retinal detachment (SRD) as a complication of preeclampsia was first described by von Graefe (2) in 1855. Kishi et al (3) suggested that it may be caused by choroidal ischemia. As ischemic retinal pigment epithelium (RPE) becomes edematous and necrotic, the outer blood-retinal barrier (BRB) becomes disrupted, allowing leakage of fluid from the choroid into the subretinal space and forming serous detachments. Outer BRB function is usually rapidly restored by normalization of blood pressure and spontaneous resolution of SRD with recovery of vision is the rule.

Elschnig’s spots are a known manifestation of this condition. They are ischemic infarcts of RPE and choroid. What are the spectral-domain optical coherence tomography (SC-OCT) features of Elschnig’s spots in preeclampsia? What are the residual features once the acute phase resolves? We aim to report the SD-OCT features of Elschnig’s spots in a patient with severe preeclampsia during the acute and remission stages. We also present a summary of OCT features of preeclampsia patients with SRD (Tab. I).

optical coherence tomographic features of patients with serous retinal detachment secondary to preeclampsia

Number Name of first author (year of publication) Age at presentation,y Time when SRD developed Eye involved OCT features at presentation Type of OCT machine used Time to resolution OCT findings al last follow-up visit
ELM = external limiting membrane; IRF = intraretinal fluid; IS = inner segment; OS = outer segment; PED = pigment epithelial detachment; RPE = retinal pigment epithelium; SD-OCT = spectral-domain optical coherence tomography; SRD = serous retinal detachment; SRF = subretinal fluid; wk = week; mo = month.
1 Theodossiadis (2002) 24 10 days postdelivery Both Diffuse IRF Time-domain 8 mo Residual RPE focal thickenings
2 Androudi (2007) 33 At 28th week of pregnancy Both SRF and IRF in a schisis-like pattern Time-domain Not available Not available
3 Song (2013) 19 6 days postdelivery Both SRF, PED, wavy structure possibly fibrin between photoreceptor-ellipsoid zone and RPE-outer segment line SD-OCT 4 wk Resolution of SRD, PED, and wavy structure; unclear photoreceptor-ellipsoid zone in some areas
4 Querques (2014) 38 2 days postdelivery Both SRF, focal RPE elevations, increased choroidal thickness SD-OCT 2 wk Resolution of SRD, persistence of focal RPE elevations, choroidal thickness decreased peripapillary and remained unchanged subfoveally
5 Pastore (2012) 26 At 32nd week of pregnancy Both SRF, IRF, hyperreflective membrane of ELM+IS+OS irregular and undulated SD-OCT 6 mo Restoration of normal retinal anatomy
6 Somfai (2006) 3 patients: 26, 28, 27 5, 10, 4 days postdelivery, respectively Both, left, both respectively SRF Time-domain 5-10 wk; 6, 10, 5 wk, respectively In first patient, right eye resolving SRF, left eye normal macula; In second and third patients total resolution of SRD
7 Thorsrud (2009) 24 1 day postdelivery Both SRF Time-domain 6 wk Total resolution of SRD
8 Neudorfer (2014) 3 eyes of 2 patients, ages not available Not available All eyes had peripapillary SRF and irregularity at the photoreceptor and RPE level, 2 eyes had subfoveal SRF, 1 eye had RPE lesion (Elschnig spot); no choroidal involvement was noted Time-domain and SD-OCT Not available for one patient, and 6 mo for 2nd patient One small focal hyperreflective lesion beneath the photoreceptor level at the location of the former RPE lesion (Elschnig’s spot)

Case report

A healthy 26-year-old primigravida presented with headache, new-onset systemic hypertension (160/110 mm Hg), and mild thrombocytopenia at 24 weeks of gestation. There was intrauterine growth retardation. She was diagnosed with severe preeclampsia. Prompt treatment with antihypertensive medications was instituted. Under spinal anesthesia, she underwent urgent low segment transverse cesarean section. Six days postpartum, she complained of blurring of vision of both eyes. On examination, visual acuity was 6/60 in the right eye and 6/9 in the left eye. There was no relative afferent pupillary defect (RAPD). Slit-lamp biomicroscopy revealed normal anterior segments and anterior vitreous. Intraocular pressures were normal. On funduscopy, optic nerves and retinal vessels looked normal but in the posterior poles, there was a localized area of SRD involving the right macular area and just temporal to the left macular area (Fig. 1). Discrete subretinal round-oval lesions compatible with Elschnig’s spots were observed. Fluorescein angiography showed diffuse subretinal leakage in late phases (Fig. 1). The SD-OCT revealed SRD in the posterior poles of both eyes (Fig. 2) and also peripapillary in left eye (both nasal and temporal to optic disc). Several foci of hyperreflective exudates were observed subretinally (Fig. 2). In addition, V-shaped adhesions were observed at the location of each of the Elschnig’s spots, the center of which showed disruption of the outer retinal bands (external limiting membrane and the inner segment/outer segment junction layer), and the V-shaped outer parts being tethered to the RPE-Bruch’s complex by a hyperreflective exudate on the surface of RPE. Inner retinal layers were uninvolved. Two weeks later, vision was fully regained (6/6 in each eye) with total resolution of SRD. The SD-OCT done 3 months later (Fig. 3) showed fine focal RPE thickening at the location of previous Elschnig’s spots. Visual fields were normal.

Upper panel: Fundus red-free images show serous retinal detachment in both eyes. Discrete subretinal round-oval lesions compatible with Elschnig’s spots are observed in the left eye. Lower panel: Fluorescein angiogram shows diffuse subretinal leakage in late phases in both eyes.

Spectral-domain optical coherence tomography shows subretinal fluid and several foci of ­hyperreflective exudates subretinally (arrows, upper panel). In addition, V-shaped adhesions are seen at the location of each of the Elschnig’s spots, the center of which show disruption of the external limiting membrane and the inner segment/outer segment junction layer whereas the outer parts remain tethered to retinal pigment epithelium (RPE)–Bruch’s complex by a hyperreflective deposit on the RPE surface. Inner retinal layers were uninvolved.

Spectral-domain optical coherence tomography images of both eyes 3 months later show total resolution of subretinal fluid and focal retinal pigment epithelium elevations at the location of previous Elschnig’s spots.

Discussion

Hypertensive choroidopathy is a well-known manifestation of accelerated hypertension observed in preeclampsia/eclampsia. Serous retinal detachment and Elschnig’s spots are known manifestations of this condition. The SD-OCT features of this case parallel the histopathologic changes that were previously reported in experimental models. Occlusion of terminal choroidal arterioles and choriocapillaris was described; they were filled with fibrinous exudate and desquamated endothelial cells (3). The RPE was seen to get necrotic. Elschnig’s spots showed a gradient of RPE damage from the center to the periphery where RPE was totally necrotic in the center and edematous in the periphery; such a histopathologic finding could provide a thorough interpretation for the V-shaped adhesions seen in our patient here. The necrotic RPE cells in the center of Elschnig’s spots elicited a copious fibrinous reaction to which the outer retinal bands remained tethered producing the V-shaped adhesions whereas the subretinal fluid caused separation between the neurosensory retina and RPE in the remaining part affected by the hypertensive choroidopathy. In these experimental models, neurosensory retina layers were not involved as seen in the case here. Complete resolution of SRD was ­observed 2 weeks later and the patient regained excellent vision (6/6 in each eye) with no ocular complaints. Visual fields performed later did not reveal any focal defect. The SD-OCT done 3 months later showed fine foci of thickening along the inner surface of Bruch’s membrane. Kishi et al (3) categorized the pathologic changes of hypertensive choroidopathy into acute ischemic, chronic occlusive, and chronic reparative, where in the latter stage, which developed 4-12 months after the initial insult, the reattached retina showed diffuse patchy RPE depigmentation and subretinal gliosis. Thus, these minute elliptical thickenings between RPE and Bruch’s membrane could reflect chronic tissue modulation developing months following the first appearance of Elschnig’s spots.

Few reports in the literature describe OCT features in preeclampsia (4-5-6-7-8-9-10-11). Most reports describe occurrence of subretinal fluid, less commonly pigment epithelial detachment and intraretinal fluid in the acute stage of the disease. Focal RPE elevations were noted in the resolution stage by Theodossiadis et al (8), Querques et al (11), and Neurdorfer et al (4). Herein we illustrate the distinctive identical SD-OCT features of a cluster of Elschnig’s spots in the acute and chronic stages of the disease and demonstrate the correlation with the previously described histopathologic features.

Despite the favorable visual outcome in patients with SRD secondary to hypertensive choroidopathy, irreversible tissue damage and necrosis may occur in the outer retinal and choroidal layers, necessitating prompt diagnosis and immediate therapeutic intervention. Advances in OCT technology have enhanced our understanding of the different retinal pathologies and SD-OCT with its enhanced sensitivity and specificity detects fine in vivo morphologic changes in retinal microstructure thus improving the diagnostic and decision-making abilities of ophthalmologists.

Disclosures

Financial support: No financial support was received for this submission.
Conflict of interest: The authors have no conflict of interest.
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Authors

Affiliations

  • Department of Ophthalmology, Hadassah-Hebrew University Medical Center, Jerusalem - Israel

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