Spontaneous separation of epiretinal membrane associated with retinal hemangioblastoma: a case report with 10-year follow-up

Abstract

Purpose

To report a case with a spontaneous separation of a secondary epiretinal membrane (ERM) associated with a retinal hemangioblastoma, and its long-term follow-up after laser photocoagulation (PC) for the hemangioblastoma.

Observations

A 38-year-old man presented with a one-month history of decreased vision in his left eye (20/32) and was diagnosed with an ERM secondary to a retinal hemangioblastoma. Although PC was initially planned, he requested that the treatment be postponed. One month later, our examination showed a spontaneous separation of the ERM with a posterior vitreous detachment. His visual acuity improved from 20/32 to 20/20. PC was performed on the feeding vessels and hemangioblastoma later. We have followed this patient for 12 years, and his visual acuity is currently 20/16 without other complications or a recurrence of the ERM.

Conclusions and importance

A spontaneous separation of a secondary ERM can occur before any treatment with a rapid functional and anatomical recovery. Our findings indicate that clinicians need to assess the treatment options for secondly ERMs associated with a hemangioblastoma.

Highlights

• •Findings in a case of spontaneous separation of epiretinal membrane before treatment.

• •A rapid visual recovery and anatomical restoration after spontaneous separation.

• •No recurrence of the epiretinal membrane occurred during a 10-year follow-up period.

1. Introduction

An epiretinal membrane (ERM) is a pathological membrane-like tissue formed at the vitreoretinal interface that can lead to visual impairment and metamorphopsia. An ERM can be classified as either idiopathic or secondary ERM,¹ though vitrectomy is the standard treatment for both types. A spontaneous separation of an ERM occurs in only 2.1–3 % of ERM cases,^2,3 and it occurs especially following laser photocoagulation (PC) or cryotherapy treatment.4, 5, 6, 7 To the best of our knowledge, there are no reports on the long-term outcomes after a spontaneous separation of an ERM.

We report our findings in a case of a spontaneous separation of a secondary ERM associated with a retinal hemangioblastoma without any intervention. The patient's visual acuity remained stable for at least 10 years thereafter.

2. Case report

A 38-year-old man with no prior ocular or systemic medical history was examined at a local clinic with vision impairment in his left eye. He was diagnosed with an ERM and referred to our hospital. At the initial visit, his best-corrected visual acuity (BCVA) was 20/20 in the right eye and 20/32 in the left eye. Fundus examination of the left eye showed a grayish-white ERM spreading from the macula to the optic disc with tortuous vessels (Fig. 1a). Optical coherence tomographic (OCT) imaging showed a thick ERM spreading from the macula to the optic disc with retinal thickening and macular deformation (Fig. 1b). No posterior vitreous detachment (PVD) was observed. There was also a 1.5-disc-diameter reddish-orange mass located in the superotemporal quadrant of the retina. The mass was located away from the ERM (Fig. 1c). Early phase fluorescein angiography revealed hyperfluorescent leakages around the peripheral mass with feeding and draining vessels (Fig. 1d). Brain magnetic resonance imaging showed no sign of central nervous system hemangioblastomas, and there was no family history of von Hippel-Lindau (VHL) syndrome. The patient declined genetic testing. There were no significant abnormal findings in the right eye. Thus, he was diagnosed with a unilateral secondary ERM associated with a retinal hemangioblastoma.

Fig. 1.

Fundus photograph and optical coherence tomographic (OCT) images of a 38-year-old man with reduced vision in his left eye (20/32) at the initial examination

(a): Fundus photograph shows an ERM that extends from the macula to the optic disc (white arrows).

(b): Optical coherence tomographic (OCT) image shows the thick ERM and a deformation of the macula.

(c): Fundus photograph showing a 1.5-disc-diameter orange-reddish mass in the super-temporal quadrant area.

(d): Early phase fluorescein angiogram showing a hyperfluorescent leakage (dot arrow) in the elevated lesion. Two feeding vessels (black arrows) and one draining vessel (black arrowhead) can also be seen.

ERM: epiretinal membrane, OCT: optical coherence tomography. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Initially, we planned PC of the hemangioblastoma to reduce its activity and a subsequent pars plana vitrectomy. However, the patient strongly requested to postpone the treatment because of social circumstances, and we planned to follow him with careful observations without any treatment. One month after the initial visit, fundus examinations revealed a spontaneous separation of the ERM from the retina with the formation of a PVD. The BCVA was improved to 20/20, and the foveal morphology was immediately improved (Fig. 2a and b).

Fig. 2.

Fundus images of the same patient during the follow-up period

(a): OCT image at 1 month after the initial visit shows a separation of the ERM (white arrows). A hyaloid membrane can also be seen because of the posterior vitreous detachment (white arrowheads).

(b): The macular structure is restored.

(c): The hemangioblastoma has regressed with a reduction of the exudative changes five months after the laser photocoagulation.

(d): Fluorescein angiography also shows a regression of the leakage (dot arrow).

(e): Fundus image at 12 years after the treatment shows floating ERM particles in the vitreous cavity (black arrowhead).

(f): OCT image shows a normal macular structure.

ERM: epiretinal membrane, OCT: optical coherence tomography.

Six months after the initial visit, the patient agreed to undergo PC. PC was performed on the feeding vessels and the hemangioblastoma. The retinal hemangioblastoma regressed with no exudative changes (Fig. 2c and d). The patient was followed for 12 years after the PC without any complications, including a recurrence of the ERM, and OCT imaging demonstrated normalization of macular structure (Fig. 2e and f). No additional treatments were performed, and his BCVA remained at 20/16 during this 12-year follow-up period.

3. Discussion

The initial treatment strategy for our case was PC for the hemangioblastoma followed by ERM peeling with vitrectomy. However, the ERM spontaneously separated with rapid recovery before PC. Although recurrence of an ERM after a spontaneous separation can occur,⁸ our patient has been followed for 12 years without any complications. This case highlights the following four aspects: an immediate functional and anatomical recovery, a possible condition of “acute macular pucker,” the suspected mechanism of the ERM separation, and the differential diagnosis.

First, our case supports the notion that secondary ERMs can spontaneously separate, leading to rapid recovery of both visual function and macular morphology. To identify comparable cases of spontaneous separation of secondary ERMs, we reviewed literature published in the last 20 years reporting all three parameters—visual acuity, OCT findings, and follow-up duration prior to separation—and identified 11 reports covering 14 eyes.^6,9, 10, 11, 12, 13, 14, 15, 16, 17, 18 After ERM separation, visual acuity ranged from partial improvement to 20/20, and macular morphology improved from residual changes to normal foveal structure. Separation occurred within 1 month to 1 year from the initial visit. In our case, spontaneous separation occurred within 1 month, with recovery to 20/20 and near-normal macular structure. This immediate course compares favorably to prior cases and suggests that initial observation may be an option for secondary ERMs.

Second, this case may represent an “acute macular pucker,” a rapidly developing secondary ERM caused by retinal tears or detachments.¹⁹ Such membranes can cause acute visual loss but are associated with rapid visual recovery once traction is relieved. In our patient, visual acuity declined within a month and improved rapidly after the spontaneous separation of ERM with PVD, consistent with the previous report of early surgical peeling in acute cases.¹⁹ Although histologic confirmation was unavailable in our case, the clinical course—characterized by a rapid onset and recovery—mirrors the descriptions of acute ERMs with pigmented cells. While the ERM of our case was secondary to a retinal hemangioblastoma rather than a retinal tear or detachment, and a vitrectomy was not performed, the acute clinical course is consistent with this possible subgroup.

Third, in our case, PVD was considered the primary factor leading to spontaneous separation of ERM. Previous reports have identified both PVD and ERM contractility as key mechanisms for spontaneous release.^2,3 In our patient, no signs of PVD were observed at the initial visit, but PVD was evident at the time of ERM separation, supporting the causal relationship. Because the interval between presentation and separation was only one month, no serial fundus or OCT images were available to confirm whether membrane contraction played a role. In secondary ERMs, spontaneous separation has often been reported after laser or cryotherapy to the primary lesion,4, 5, 6, 7 possibly owing to inflammation or vitreoretinal interface changes induced by treatment. However, in our case, separation occurred before any therapeutic intervention, making treatment-related mechanisms unlikely.

Finally, the differential diagnosis of peripheral retinal vascular tumors can be challenging. In our case, we initially diagnosed the lesion as a retinal hemangioblastoma based on several characteristic features, including an orange-red, well-circumscribed, dome-shaped mass, and clearly identifiable feeding and draining vessels.^20,21 However, genetic testing was not performed; hence, VHL syndrome could not be definitively excluded. In addition, some of these features—particularly the relatively modest vessel dilation—can also be seen in vasoproliferative tumors (VPTs) of the retina.²² When lesions are solitary and there is no known systemic association, such as VHL, differentiation can be difficult. Nevertheless, the overall clinical profile in this case was consistent with retinal hemangioblastoma. This case highlights the diagnostic challenge in assessing peripheral retinal vascular tumors and underscores the importance of careful, multimodal evaluation.

4. Conclusions

An ERM associated with retinal hemangioblastoma can spontaneously separate from the retina. The rapid functional and anatomical recovery and the long-time stable outcome emphasizes the importance of individualized treatment planning.

CRediT authorship contribution statement

Eriko Inamura: Writing – original draft, Visualization, Investigation, Data curation. Yusuke Takeda: Writing – review & editing, Writing – original draft, Visualization, Investigation, Conceptualization. Hidetoshi Yamashita: Writing – review & editing, Writing – original draft, Supervision, Conceptualization. Masahiko Sugimoto: Writing – review & editing, Supervision, Conceptualization.

Patient's consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

Authorship

All authors attest that they meet the ICMJE criteria for authorship.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Declaration of competing interest

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.