Two patients presented complaints of visual acuity worsening in one eye. During examination they showed optical coherence tomography (OCT) and clinical findings indistingishable from myopic foveoschisis, although both had minimal refractive errors. One of the patients was followed without surgery until the disease progressed and the visual acuity decreased. The second patient presented with lamellar macular hole associated with the foveoschisis. Both patients underwent pars plana vitrectomy with pre-retinal membranes peeling and intra-vitreal gas infusion and both developed full-thickness macular hole after surgery. The second patient underwent a second surgery with peeling of the internal limiting membrane (ILM) and with successful macular hole closure. OCT was performed at the initial and follow-up visits. In conclusion, foveoschisis may develop in eyes without high myopia. The causative factors, OCT findings and surgical outcomes are very similar to myopic foveoschisis.
Foveoschisis Without High Myopia
From New England Eye Center/Department of Ophthalmology/Tufts Unversity School of Medicine, Boston, Massachusetts.
Supported in part by a Research to Prevent Blindness Challenge Grant to the New England Eye Center/Department of Ophthalmology, Tufts University School of Medicine.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Jay S. Duker, MD, 800 Washington St., Box 450, Boston, MA, 02111.
Accepted: December 12, 2008
Posted Online: March 09, 2010
Myopic foveoschisis is a relatively common complication in highly myopic patients. It has been estimated to affect 9% to 34% of patients with high myopia and posterior staphyloma.1,2 Several factors, such as rigidity of the retinal vessels, the presence of epiretinal membrane (ERM), posterior staphyloma formation, and the rigidity of the ILM are hypothesized to contribute to the formation of the myopic foveoschisis.
Takano and Kishi1 first described the OCT findings in myopic foveoschisis and noted that the retina was divided into a thick inner layer and a thin outer layer in the area surrounding the foveal pit, and, in most cases, there was also a retinal detachment limited to the fovea.
We report two cases of foveoschisis that on clinical examination and OCT were indistinguishable from cases of myopic foveoschisis. However, in these cases, the refractive error was minimal.
A 78-year-old man complained of vision loss in the left eye for 3 years. The best-corrected visual acuity was 20/30 in right eye (OD) and 20/60 in the left eye (OS), with a refractive error of −0.25 spherical diopters in both eyes. Anterior segment examination revealed mild nuclear sclerosis in the right lens and posterior chamber intraocular lens in the OS. In the posterior pole, the OD was normal. The OS showed hyaloidal traction along the inferior arcade vessels resulting in a localized tractional retinal detachment. OCT disclosed a foveal detachment associated with the splitting of the outer retina in the macular area along with the associated vitreoretinal traction (Fig. 1A).
Figure 1. Case 1. Color Fundus Photography (initial Visit) Shows the Presence of an Elevated Retina Inferiorly to the Macula. Vertical Line Represents OCT Scanning Through the Macula. (A) The OCT Scan Disclosed a Foveal Detachment Associated to the Splitting of the Outer Retina and Vitreomacular Traction in the Inferior Macula at Initial Visit. (B) 4 Months After Initial Visit: Worsening of the Retinal Traction and Development of a Lamellar Macular Hole. (C) 4 Months After Surgery: Resolution of the Foveoschisis and the Presence of a Full-Thickness Macular Hole.
After 4 months, the visual acuity in the OS diminished to 20/80 and OCT revealed a lamellar macular hole with worsening of the macular traction (Fig. 1B).
After 8 months from the first visit, the patient returned complaining of worsening of visual acuity in the OS. Visual acuity was counting fingers (CF) at 2 feet. A pars plana vitrectomy with peeling of the hyaloid, removal of pre-retinal membranes and intraocular gas injection was performed.
Four months after the surgery, the visual acuity improved to 20/200. The OCT showed improvement of the foveoschisis, but a full-thickness macular hole was present (Fig. 1C).
A 62-year-old woman complained of visual loss and metamorphopsia in the OD for one year. The best-corrected visual acuity was 20/50 in the OD and 20/20 in the OS. Her refractive error was +2.00 and +2.75 spherical diapters equivalent in the OD and OS respectively. The anterior segment examination showed mild cataract in both eyes. In the posterior pole of the OD, a thick ERM with retinal striae and macular edema was visible. The OS was completely normal. The ultrahigh resolution OCT (UHR-OCT) showed the ERM associated with vitreomacular traction, lamellar macular hole and splitting of the outer retina in the macular area (Fig. 2A), consistent with foveoschisis.
Figure 2. Case 2. Red-Free Fundus Photography at Initial Visit Shows a Thick Epiretinal Membrane, Retinal Striae and Macular Edema. The Line Represents the UHR-OCT Scanning Through the Macula. The UHR-OCT (A) Shows the Presence of the Epiretinal Membrane, the Vitreomacular Traction, Two Cystic Spaces in the Inner Retina, the Lamellar Macular Hole and the Splitting of the Outer Retina. The Photoreceptor Inner and Outer Segment (IS/OS) Layer is Intact in the Foveal Area (arrow). UHR-OCT Scan 20 Days After the Surgery (B) Shows a Full-Thickness Macular Hole. Note that the Splitting of the Outer Retina is Still Present. The ILM Can be Visualized in the Temporal Side of the Macula (arrow).
The patient underwent a pars plana vitrectomy with peeling of the hyaloid, removal of the ERM, and gas injection. 20 days after the surgery, the patient returned complaining of abrupt drop in the vision. Her visual acuity was CF at 5 feet and the fundoscopy revealed a full thickness macular hole. The UHR-OCT showed the ILM in the temporal side of the macular hole (Fig. 2B). A repeat pars plana vitrectomy was performed, the ILM was peeled and a gas bubble was injected.
Two months after the second surgery, the best-corrected visual acuity in the OD was 20/300 and the UHR-OCT showed the closed macular hole, persistence of splitting of the outer retina and discontinuation of the photoreceptor inner and outer segments in the foveal area. These conditions are not seen in the Stratus OCT (Figs. 3A and 3B). 1 year after the second surgery, the visual acuity was still 20/300 and the OCT showed improvement of the splitting of outer retina and thinning of the foveal area (Fig. 3C).
Figure 3. Case 2. (A, B) Both Stratus OCT and UHR-OCT Demonstrated, Two Months After Second Surgery, the Closure of the Macular Hole and the Splitting of the Outer Retina Temporal to the Fovea, but Stratus OCT Failed to Demonstrate the IS/OS Layer Defect Under the Fovea, This Is Seen only in the UHR-OCT Scan (arrows). (C) Stratus OCT Scanning of the Macula 1 Year After the Second Surgery Showing Improvement of the Splitting of the Outer Retina and Thinning of the Foveal Area.
Since Takano and Kishi1 described the first OCT findings of macular detachment and foveoschisis in highly myopic patients, several other papers described similar findings.2,3 In others papers the presence of a concurrent or subsequent macular hole was noted. To the best of our knowledge, this is the first report of foveoschisis in patients without myopia of any degree. Clinical examination and OCT findings were indistinguishable from cases of myopic foveoschisis.
Other forms of macular schisis, as X-linked retinoschisis and Goldmann–Favre syndrome, could be ruled out because of the lack of family history and the age of diagnosis, which is usually younger in those cases. Also, in cases of X-linked retinoschisis, the cleavage plane shown by OCT includes the outer retinal layers and the nerve fiber layer.4 Our patients presented splitting only in the outer retina.
Shimada et al.5 showed that 4 of 8 cases with myopic foveoschisis developed complications (macular hole formation and retinal detachment without macular hole) during a follow-up of 24 to 60 months, and suggested that it might be a progressive condition with the complications appearing to be related to the presence of vitreoretinal traction. Although our patients had minimal refractive errors, both presented with significantly vitreoretinal traction. One of the patients (Case 1) also presented with foveal retinal detachment, which worsened during the follow-up. The other patient presented with lamellar macular hole associated with the foveoschisis.
Both cases presented here developed full thickness macular hole after the first surgery, and one of the patients underwent a second pars plana vitrectomy (Case 2) with peeling of the ILM and successful closure of the macular hole. Kobayashi and Kishi6 presented a series of nine patients with myopic foveoschisis that underwent pars plana vitrectomy and peeling of the ILM for the treatment of foveal retinal detachment. In one case, the surgeon was not able to peel the ILM, and the patient developed a full-thickness macular hole in the post-operative period. Gaucher et al.7 also described the surgical treatment of 10 myopic foveoschisis associated with foveal retinal detachment, but without peeling of the ILM. 3 of these patients developed full-thickness macular hole after surgery. These findings might suggest that in non-myopic foveoschisis, as in the myopic case, peeling of all pre-retinal membranes (including the ILM) could be important for a good post-operative result.
We presented two cases of foveoschisis in patients with minimal refractive errors with clinical and OCT findings indistinguishable from myopic foveoschisis. The progression of the disease and surgical outcomes in our patients were also very similar to those described for foveoschisis in highly myopic eyes in the literature.
- Takano M, Kishi S. Foveal retinoschisis and retinal detachment in severely myopic eyes with posterior staphyloma. Am J Ophthalmol. 1999;128:472–476. doi:10.1016/S0002-9394(99)00186-5 [CrossRef]
- Baba T, Ohno-Matsui K, Futagami S, et al. Prevalence and characteristics of foveal retinal detachment without macular hole in high myopia. Am J Ophthalmol. 2003;135:338–342. doi:10.1016/S0002-9394(02)01937-2 [CrossRef]
- Benhamou N, Massin P, Haouchine B, et al. Macular retinoschisis in highly myopic eyes. Am J Ophthalmol. 2002;133:794–800. doi:10.1016/S0002-9394(02)01394-6 [CrossRef]
- Azzolini C, Pierro L, Codenotti M, Brancato R. OCT images and surgery of juvenile macular retinoschisis. Eur J Ophthalmol. 1997;7: 196–200.
- Shimada N, Ohno-Matsui K, Baba T, et al. Natural course of macular retinoschisis in highly myopic eyes without macular holes or retinal detachment. Am J Ophthalmol. 2006;142:497–500. doi:10.1016/j.ajo.2006.03.048 [CrossRef]
- Kobayashi H, Kishi S. Vitreous surgery for highly myopic eyes with foveal detachment and retinoschisis. Ophthalmololgy. 2003;110: 1702–1707. doi:10.1016/S0161-6420(03)00714-0 [CrossRef]
- Gaucher D, Haouchine B, Tadayoni R, et al. Long-term follow-up of high myopic foveoschisis: natural course and surgical outcome. Am J Ophthalmol. 2007;143:455–462. doi:10.1016/j.ajo.2006.10.053 [CrossRef]