Management of coexistent choroidal neovascular membrane and macular hole with serous detachment in a case of pathological myopia: challenges and dilemmas

Abstract

Coexistent choroidal neovascular membrane and macular hole with serous retinal detachment in pathological myopia is a rare entity and may be quite challenging to manage. We present such a case managed with bevacizumab injection and vitrectomy. Although the retina was attached at the final follow-up, the visual outcome was poor, due to non-closure of hole.

Background

Choroidal neovascular membrane (CNVM) and macular holes (MHs) are complications associated with pathological myopia.¹ However, coexistence of both the conditions in the same patient is rare and the management may involve unique challenges and dilemmas, which have been documented previously.² Most of the previous reports of management of coexistent CNVM and MH have documented generally poor outcomes, with no consensus regarding treatment. We hereby report a case of pathological myopia with a CNVM which developed a MH with serous retinal detachment (SRD) later and was managed accordingly.

Case presentation

A 75-year-old man with bilateral pseudophakia presented to our clinic with problems of floaters in the right eye (OD). He had been operated for left eye (OS) retinal detachment in 2014 with silicone oil endotamponade in situ and was on treatment with glaucoma drops for silicone oil-induced glaucoma. His best corrected visual acuity (BCVA) was 6/6 OD with a correction of −1.00 D and only perception of light OS. Axial lengths were 31.76 OD and 32.08 OS.

Investigations

Spectral-domain optical coherence tomography (SD-OCT) OD showed a lamellar MH (LMH) with a posterior staphyloma (figure 1A). Patient was advised observation for 6 months. He returned after 5 months with a problem of diminution of vision OD with BCVA of 6/18 since 1 week. SD-OCT showed subfoveal hyper-reflective irregular lesion arising from the retinal pigment epithelium (RPE) which was heterogeneous in appearance, without any subretinal fluid (SRF) or haemorrhage; however, cystic changes were present perifoveally (figure 1B).

Figure 1.

(A) Right eye spectral-domain optical coherence tomography showing a lamellar macular hole inside a posterior staphyloma. (B) Right eye showing choroidal neovascular membrane with overlying cystic changes present.

Treatment and outcome

A diagnosis of a myopic CNVM was made and the patient underwent intravitreal injection of bevacizumab. After 1 month of follow-up, patient had BCVA of 6/36 OD and SD-OCT showed a large SRD with a full-thickness MH (FTMH; figure 2A). The minimum diameter of the hole appeared to be 220 µm. SD-OCT could not capture the CNVM accurately because of shadowing from the SRD. Fluorescein angiography (FA) showed a large area of blocked fluorescence corresponding to the neurosensory detachment (NSD), with an area of staining corresponding to scarred CNVM (figure 2B). Patient underwent 23-gauge pars plana vitrectomy along with internal limiting membrane (ILM) peeling and C3F8 gas endotamponade. After 15 days of endotamponade, OCT showed non-closure of MH (minimum diameter 470 µm) with persistent SRD (figure 2C). Hence, a resurgery was performed with injection of silicone oil for tamponade and after 1 month, BCVA was 6/60. OCT showed open MH with underlying attached retina and scarred perifoveal CNVM. However, after 3 months, OCT showed macular redetachment, for which a silicone oil exchange surgery was performed. After 6 months, patient had a BCVA of hand movements.

Figure 2.

(A) Full-thickness macular hole with serous detachment. (B) Fundus fluorescein angiography of right eye showing blocked fluorescence from serous detachment and staining of choroidal neovascular membrane (CNVM). (C) Unsettled right eye posterior pole under C3F8 endotamponade. (D) Flat neuroretina and retinal pigment epithelium with scarred CNVM with open macular hole status after 7 months.

Follow-up

At the seventh month and final follow-up, patient returned with BCVA of 1/60; the neurosensory retina and RPE appeared flattened and the MH stayed open with silicone oil in situ (figure 2D).

Discussion

Choroidal neovascularisation in an eye with pathological myopia can be rarely associated with MH. Only a few such cases have been reported with coexisting MH and CNV in the same eye and treated as such (table 1).

Table 1.

Previous reports of the occurrence of macular holes (MHs) coexistent with choroidal neovascular membrane (CNVM)

	Condition	Risk for CNVM	Treatment given	Final visual acuity
Smith et al3	Idiopathic MH later developed CNVM	AMD	Laser photocoagulation	FCCF
Elsing et al 8	Idiopathic MH later developed CNVM	AMD	Vitrectomy/submacular surgery for CNVM removal/laser photocoagulation	20/100 to HMCF
Recchia and Williams9	Idiopathic MH with coexistent CNVM	AMD	PDT with MH surgery	20/50
Singh and Kaiser10	Stage 1 MH developed CNVM and later converted to FTMH	AMD	Macugen, 6 months later MH surgery	20/32
Chen et al2	MH, later developed CNVM	Pathological myopia	PDT followed by MH surgery	20/200
Current study	CNVM, later developed MH	Pathological myopia	Bevacizumab followed by MH surgery	20/1000

AMD, age-related macular degeneration; CNVM, choroidal neovascular membrane; FCCF, fingers counting close to face; FTMH, full-thickness macular hole; HMCF, hand movements close to face; PDT, photodynamic therapy.

Few previous reports have documented the development of CNVM in pre-existing MH eyes, and authors have hypothesised that this may possibly be due to circulation of chronic inflammatory mediators in the SRF.^{2 3} However, other factors like iatrogenic surgical injury, excessive light exposure and prolonged endotamponade have also been believed to cause RPE damage, thereby leading to CNVM.

This report is unique, since the MH followed the formation of CNVM. According to Shimada et al, highly myopic eyes with atrophic CNVM have a high chance of developing MHs (14%), because of loss of RPE and outer retinal layers in the area of the CNVM scar.^{1 4} Not all myopic MHs are associated with a SRD and if present, may be because of chorioretinal atrophic changes and tractional forces inside a posterior staphyloma.

Our patient, a high myope, initially presented with foveoschisis and LMH (signs of myopic maculopathy), which converted into FTMH during the period of management of the CNVM. As seen in our case, in the active stage, the myopic CNVM may appear as a highly reflective dome-like elevation above the RPE on OCT and may or may not be associated with intraretinal or sub-retinal fluid. OCT also helps in identifying any associated macular pathology like MH or foveoschisis. Such patients also warrant close monitoring with OCT, as foveal pathologies may evolve over a small amount of time. Fluid leakage from the CNVM or vascular endothelial growth factor (VEGF) secreted from CNVM might have led to the serous detachment. However, fundus FA suggested that the CNVM had regressed after the anti-VEGF injection, and was not directly causative of the SRF. The intravitreal injection itself might also have promoted vitreoretinal interface traction leading to the conversion to FTMH.

Myopic CNVM is an important cause of irreversible vision loss in pathological myopia. Various treatment modalities like laser photocoagulation, photodynamic therapy and anti-VEGF injections have been described.⁵ Anti-VEGF agents have been extensively used in the management of CNVM in diseases like age-related macular degeneration and pathological myopia.⁶

Myopic MHs with retinal detachment are very difficult to treat. Different modalities like pars plana vitrectomy and macular buckling surgery have been described, although having poor success rates.⁷ The few coexistent reported cases had quite variable results, with a high chance of poor prognosis (table 1).

Our case is different from the previous cases in that after the confirmation of the diagnosis of CNVM, we injected bevacizumab intravitreally for treating the lesion. The CNVM seemed to regress with bevacizumab, however, followed by the development of FTMH with SRD, for which a pars plana vitrectomy with membrane peeling had to be performed. There is no consensus regarding the ideal management of such cases; however, we believe that each case needs to be dealt with in an individualised manner.

Singh et al described one such case of a MH with SRD, which developed following treatment of a CNVM with multiple pegaptanib sodium injections over 6 months. With prompt vitrectomy, hole closure was achieved, with good visual outcomes.⁶ However, in our case, although the MH developed after a month of intravitreal bevacizumab injection for CNVM, and surgery was done immediately, hole closure was not achieved till the last follow-up. The most important difference between Singh et al’s case and ours is that our patient had pathological myopia with posterior staphyloma, which predisposed the patient to a poorer prognosis, whereas the previously reported case was a non-myopic patient having macular degeneration. Pathological myopes suffer from vitreomacular as well as transverse traction due to taut ILM, which probably has led to the final non-closure of the MH in our case. Contraction of the scarring CNVM may be an additional factor which might have led to the non-closure of MH in our case. In a previous case report, Chen et al reported surgical closure of MH in a pathological myope with a CNVM; in their case, MH was already present before the formation of CNVM, and most probably idiopathic.²

To summarise, coexistent myopic CNVM and MH may have poor anatomical and functional prognosis due to disruption of neuroretinal layers and RPE. OCT serves as an important tool for closely following up such patients during treatment.

Learning points.

• Pathological myopia may be associated with multiple macular complications at the same time.

• Management of simultaneous choroidal neovascularisation and macular hole may be difficult.

• After vitrectomy, it is difficult to titrate dose of anti-VEGF drugs given for choroidal neovascularisation, because of lowering of vitreous half-life.

• Even after proper management, simultaneous macular hole and choroidal neovascular membrane generally have poor prognosis.