Patients affected by myopia are not only heavily dependent on optical correction such as glasses or contact lenses, they also live with a lifelong increased risk of retinal complications such retinal detachment, myopic macular atrophy, vitreomacular interface disease, and myopic choroidal neovascularization (CNV). This is particularly true for those with high myopia (often considered as refractive error greater than -6.00) and/or pathological myopia (those with pathological posterior segment alterations from myopia).

Case Report

In this instance a 54-year-old female patient with -10.00 refractive error OU presented with a new onset of metamorphopsia OS for one week. Her best corrected visual acuity was 20/50 OS. On clinical examination, both eyes exhibited a tessellated macula as well as diffuse macular atrophy, seen as an ill-defined yellowing of the macula. Both eyes also had tilted discs and peripapillary atrophy, commonly seen in high myopia. The left eye had a dark-grey circular lesion inferior to the fovea, suspicious for a myopic CNV (Figure 1). 

Figure 1: Blue arrow points towards myopia CNV OS.

OCT was performed to further evaluate the macula. In the OS, there was a myopic contour of the macula, a thin choroid often seen in high myopia, a mild epimacular membrane, and a subretinal lesion of non-uniform moderate to high reflectivity consistent with a CNV (Figure 2). 

Figure 2: OCT through the lesion shows non uniform areas of reflectivity in the subretinal space (blue arrow) as well as subretinal fluid (red arrow) consistent with CNV.

OCT-A, although not absolutely necessary, was also performed to further evaluate the lesion. This testing confirmed presence of vascularity in the avascular zone consistent with CNV (Figure 3).

Figure 3: OCTA showing presence of vascularity in the avascular complex (blue arrow).

The patient was educated regarding the presence of CNV, and intravitreal anti-VEGF injection OS was recommended. The patient had monthly Avastin injections initially, followed by as needed injections for a year. She had significant improvement in visual symptoms with resolution of her metamorphopsia. Her visual acuity returned to 20/20 best corrected. Anatomically, the lesion also significantly improved on OCT (Figure 4). The patient has not received injections for over 2 years now, and the lesion remains stable. Continued frequent monitoring still remains necessary, and the patient is seen for examination with OCT every 4 months. 

Figure 4: Top image is pre-treatment, bottom image is 1 year following treatment with Avastin.

Discussion

Myopic CNV is a well-documented complication of myopia, particularly for those that are considered to be high myopes or those with pathological myopia. CNV may occur in upwards of 11% of those with pathological myopia.1  35% of patients who develop myopic CNV in one eye, will also develop it in the fellow eye.1

Those with acute or active CNV may complain of recent onset metamorphopsia, scotoma, or blurred vision. CNV lesions from myopia are often small (less than 1000 microns in size) and typically present as classic or type 2 lesions, which means the vascular network is above the RPE. On OCT, this will be seen as hyper-reflective material in the subretinal space. Subretinal fluid may be present, but is often minimal. There is less frequently a large pigment epithelial detachment, as is commonly seen in occult or type 1 lesions that are more typical of age-related macular degeneration (AMD). On clinical examination, there is often a darkly pigmented lesion present. Hemorrhage may also be present, but is typically minimal.

Active lesions are most often treated with intravitreal anti-VEGF. They tend to respond quickly to treatment and do not require treatment as frequently or continuously as those with CNV secondary to AMD. However, once lesions regress, they must continue to be monitored for reactivation.3

Myopic CNV left untreated will regress into an inactive scar, historically called Fuch’s spot.2 Even with treatment, vision recovery may be limited by submacular scar formation. The goal of early intervention is to limit this scar formation and preserve vision. Patients with myopic CNV may also have limited visual recovery due to formation or presence of macular atrophy.3

Final Takeaway

Patients with high myopia or pathological myopia must be monitored for myopic CNV. Fundus examination along with OCT and OCT-A imaging can lead to an accurate diagnosis. Early intervention with intravitreal anti-VEGF treatment helps to preserve vision.  

 


  1. Cheung CMG, Arnold JJ, Holz FG, et al. Myopic Choroidal Neovascularization: Review, Guidance, and Consensus Statement on Management. Ophthalmology. 2017;124(11):1690-1711. 
  2. Silva R. Myopic Maculopathy: A Review. Ophthalmologica. 2012;228(4):197-213. 
  3. Toto L, Di Antonio L, Costantino O, Mastropasqua R. Anti-VEGF Therapy in Myopic CNV. Curr Drug Targets. 2021;22(9):1054-1063.