Ophthalmic Surgery, Lasers and Imaging Retina

CASE REPORT 

Unilateral Macular Retinoschisis with Stellate Foveal Appearance in Two Females with Myopia

Ugo Menchini, MD; Rosario Brancato, MD; Gianni Virgili, MD; Luisa Pierro, MD

Abstract

Abstract. Stellate foveal retinoschisis is considered the hallmark of X-linked retinoschisis. We describe the finding of unilateral retinoschisis with stellate foveal appearance in 2 females with myopia who had no evidence of familial disease. Optical coherence tomography (OCT) and fluorescein angiography were obtained in both cases. Neither patient had a family history of low vision, night blindness, or retinal detachment. Visual acuity in the affected eyes was 20/50 and 20/25, respectively. Both fellow eyes had normal fundi, except for mild myopic changes. With OCT, widespread retinal splitting was detected at the posterior pole in the affected eyes, whereas the fellow eyes were normal. In one case, OCT showed that foveal retinoschisis represented the macular involvement of a flat inferior retinoschisis, although this was not clinically apparent. Macular retinoschisis with stellate foveal appearance may rarely be associated with pathologic myopia. OCT was useful to establish the true extension of these macular changes. [Ophthalmic Surg Lasers 2000;31:229232]

Abstract

Abstract. Stellate foveal retinoschisis is considered the hallmark of X-linked retinoschisis. We describe the finding of unilateral retinoschisis with stellate foveal appearance in 2 females with myopia who had no evidence of familial disease. Optical coherence tomography (OCT) and fluorescein angiography were obtained in both cases. Neither patient had a family history of low vision, night blindness, or retinal detachment. Visual acuity in the affected eyes was 20/50 and 20/25, respectively. Both fellow eyes had normal fundi, except for mild myopic changes. With OCT, widespread retinal splitting was detected at the posterior pole in the affected eyes, whereas the fellow eyes were normal. In one case, OCT showed that foveal retinoschisis represented the macular involvement of a flat inferior retinoschisis, although this was not clinically apparent. Macular retinoschisis with stellate foveal appearance may rarely be associated with pathologic myopia. OCT was useful to establish the true extension of these macular changes. [Ophthalmic Surg Lasers 2000;31:229232]

Figure 1. Red free (A, B) and fluorescein angiography (C, D) of the foveal region of Patient 1 . Foveal stellate retinoschisis is seen in the left eye (B), whereas the right macula is normal (A); fluorescein angiography shows no leakage of dye (C, D).

Figure 1. Red free (A, B) and fluorescein angiography (C, D) of the foveal region of Patient 1 . Foveal stellate retinoschisis is seen in the left eye (B), whereas the right macula is normal (A); fluorescein angiography shows no leakage of dye (C, D).

INTRODUCTION

Several macular changes may be associated with myopia, such as lacquer cracks, choroidal neovascularization, macular hole1 as well as peripheral retinal changes such as retinoschisis or retinal detachment. Foveal retinoschisis was believed to be the hallmark of X-Iinked juvenile retinoschisis,2 although macular schisis-like changes may be associated with various inheritable or acquired diseases2'3; bilateral sporadic retinoschisis in a young woman has also been reported,2 as well as one pedigree with familial retinoschisis in female patients.4 We describe the cases of two females with myopia affected by unilateral macular retinoschisis with stellate foveal appearance.

CASE REPORTS

The 2 patients underwent fluorescein angiography (Topcon IMAGEnet H 1 024,Tokyo, Japan) and optical coherence tomography (OCT, Humphrey Instruments, Zeiss machines, San Leandro, CA).

Figure 2. Red-free image (A) of the left fundus of Patient 1 . In (A), arrowheads indicate tiny retinal microvascular anomalies that show no leakage with fluorescein angiography (B, arrows). The oblique line in (A) represents the OCT scan line seen in (C), which shows splitting of the outer retinal layers consistent with macular retinoschisis, communicating with a flat inferior retinoschisis that presented cavities in the nerve fiber layer as well. A 1.5 mm horizontal scan through the fovea (D) clearly shows outer layer schisis-cavities with bridging elements.

Figure 2. Red-free image (A) of the left fundus of Patient 1 . In (A), arrowheads indicate tiny retinal microvascular anomalies that show no leakage with fluorescein angiography (B, arrows). The oblique line in (A) represents the OCT scan line seen in (C), which shows splitting of the outer retinal layers consistent with macular retinoschisis, communicating with a flat inferior retinoschisis that presented cavities in the nerve fiber layer as well. A 1.5 mm horizontal scan through the fovea (D) clearly shows outer layer schisis-cavities with bridging elements.

Case 1. A 34-year-old woman was diagnosed having macular changes in her left eye during a routine examination. Visual acuity was 20/20 with -7 sph -0.75 Hz 180 RE and 20/25 -9 sph -1,50 Hz 180 LE and she was asymptomatic. Both eyes had myopic fundus and optic discs. Her left eye showed stellate foveal retinoschisis (Fgure 1) and flat midperipheral retinoschisis with tiny overlying microvascular anomalies in the superior and inferior quadrants (Figure 2); with biomicroscopy, the inferior retinoschisis seemed to reach the inferior temporal vascular arcade. The right macula, as well as the periphery, was normal (Figure 1). With fluorescein angiography, no leakage of dye was observed in the macula (Figure 1), as well as from the midperipheral microvascular anomalies seen in the left eye (Figure 2). Optical coherence tomography showed splitting of the outer retinal layers in the left macula (Figure 2), and it also showed that the macular retinoschisis represented the extension of the midperipheral inferior retinoschisis. The left vitreous was attached on echographic examination. Fifteen months later, ophthalmoscopic, fluorescein angiographic, and OCT features were unchanged, and visual acuity was stable. Her brother was highly myopic and he did not have anomalous vitreoretinal findings on clinical and OCT examination.

Case 2. A 44-year-old woman complained of decreased vision and metamorphopsias in her left eye since four months. Her visual acuity was 20/25 -5-50 sph -0.75 Hz 170 RE and 20/50 -5-50 sph -0.75 Hz 150 LE. Her left eye showed stellate foveal retinoschisis with biomicroscopy (Figure 3); no other vitreoretinal changes were observed in either eye, except for myopic changes. OCT was performed five months later and showed widespread retinoschisis throughout the left posterior pole (Figure 4) and a normal right macula. Fluorescein angiographie and echographic findings were normal. Twenty months after the first examination, best corrected visual acuity was 20/25 RE and 20/80 LE and her fundus findings were unchanged.

Figure 3. Red-free image of the left fundus of Patient 2. Stellate foveal retinoschisis is observed.

Figure 3. Red-free image of the left fundus of Patient 2. Stellate foveal retinoschisis is observed.

DISCUSSION

To the best of our knowledge this is the first report of unilateral macular retinoschisis with stellate foveal appearance in myopia. Although X-linked retinoschisis can be ruled out in our patients because they were females and did not have a family history, we cannot exclude rare forms of familial retinoschisis with different inheritance2,3; however, all of these reported cases were bilateral and a positive family history was elicited. One sporadic, bilateral case has been reported by Gass2 in a nonmyopic young woman with negative family history for eye disease. Goldmann-Favre syndrome, a recessively inherited dystrophy associated with macular retinoschisis, is usually diagnosed at an earlier age and is associated with severe functional and chorioretinal damage during adulthood.2

Figure 4. Red-free image (A) of Patient 2 taken on the same day and displays the scan line of the OCT seen in (B). This horizontal scan through the posterior pole shows outer retinoschisis with some inner retinal cavities. A possible interruption of the inner retinal layers is seen at the fovea.

Figure 4. Red-free image (A) of Patient 2 taken on the same day and displays the scan line of the OCT seen in (B). This horizontal scan through the posterior pole shows outer retinoschisis with some inner retinal cavities. A possible interruption of the inner retinal layers is seen at the fovea.

Optical coherence tomography allows noncontact in vivo cross sectional imaging of eye tissues with a maximal longitudinal resolution of 10 urn.5,6 Recently, Azzolini et al have reported OCT findings in males with X-linked retinoschisis; they found a macular cleavage plane not only in the nerve fiber layer, but also in the outer retinal layers.7 With OCT, we found outer retinal splitting and inner retinal cavities at the posterior pole in our patients; in one of them, foveal retinoschisis represented the macular extension of a larger peripheral flat retinoschisis, a fact that was not clinically apparent.

Radial perifoveal striae have been observed in some cases around a foveal cyst by Folk et al in aged nonmyopic patients.8 Eight out of 23 eyes with foveal cysts developed a macular hole within 4-to-44 months in their series. Examination with a retinal thickness analyzer revealed splitting or schisis in the middle layers of the retina in 4 patients. The authors believed that foveal cysts are caused by traction on or at the margin of the edges of the fovea.8 The 2 young, females with myopia reported by us differ from the cases reported by Folk et al because they neither showed a foveal cyst, nor developed a macular hole.8 Since we could not detect ophthalmoscopic or OCT findings indicating vitreous traction, we cannot know if vitreous traction rather than intraretinal degenerative changes might have been the cause of unilateral retinoschisis in the affected eyes. Although no macular hole developed in our patients during a follow-up of 15 and 20 months, respectively, a longer observation period may be required to assess if foveal retinoschisis represents a premacular hole condition in myopes.

One of our patients (Case 1) also showed midperipheral retinoschisis with tiny overiying microvascular abnormalities. Retinal vascular dilation, nonperfusion, and even new vessels have been observed in senile, acquired,9 or juvenile retinoschisis10; the authors postulated that edema and displacement of the retina caused damage to microcirculation. Similarly, the midperipheral microvascular anomalies seen in our patients may have been the result of chronic retinoschisis.

The cases reported herein show that macular retinoschisis with stellate foveal appearance may be included among the vitreoretinal changes complicating myopia. OCT was useful to establish the true extension of these changes.

REFERENCES

1. Gass JDM. Myopic choroidal degeneration. In: Gass JDM, ed. Stereoscopic atlas of macular diseases: Diagnosis and treatment. VbI 1 . 4th ed. St. Louis, Mo: CV Mosby, 1997:126-129.

2. Gass JDM. Heredodystrophic disorders affecting the pigment epithelium and retina. In: Gass JDM, ed. Stereoscopic atlas of macular diseases: Diagnosis and treatment. Vol I. 4th ed. St. Louis, Mo: CV Mosby, 1997:372-377.

3. Madjarov B, Hilton GF, Brinton DA, Lee SS. A new classification of the retinoschisis. Retina. 1995; 15:282285.

4. Shimazaki J, Matsuhashi M. Familial retonoschisis in female patients. Documenta Ophthalmologica. I987;65:393-400.

5. Hee MR, Izatt JA, Swanson EA, Huang D, Schuman JS, Lin CP, et al. Optical coherence tomography of the human retina. Arch Ophthalmol. 1995;113:325-32.

6. Puliafito CA, Hee MR, Lin CP, Reichel E, Schuman JS, Duker JS, et al. Imaging of macular diseases with optical coherence tomography. Ophthalmology. 1995;102:217-29.

7. Azzolini C, Pierre L, Codenotti M, Brancate R. OCT images and surgery of juvenile macular retinoschisis. Eur J OphthalmoL 1997:7:196-200.

8. Folk JC, Boldt HC, Keenum DG. Foveal cysts. A premacular hole condition associated with vitreous traction. Arch OphthalmoL 1998;116:1177-1183.

9. Campo, RV, Reeser FH, Flindall RJ. Vascular leakage, neovascularization, and vitreous hemorrhage in senile bullous retinoscrusis. Am J Ophthalmol, 1983;95:826832.

10. Brancate R, Menchiní U. Retinoschisis maculaire idiopathique du sujet jeune associé a des neovaisseaux preretiniens et prepapillaires. J Fr Ophtalmol. 1984;7:685-688.

10.3928/1542-8877-20000501-13

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