Retinoschisis is a splitting within the neurosensory layer of the retina that frequently occurs in the inferotemporal quadrant. It is usually asymptomatic but can potentially cause visual field defects. On examination, it appears as a well-circumscribed, dome-shaped elevation of the inner retina. OCT shows a break between the retinal layers. While often stable, it can occasionally lead to retinal detachment or holes. Treatment may be needed if complications occur.

1. Retinoschisis
MOHAMMAD RIYAJ ALI
Consultant Optometrist

2. Retinoschisis
•Retinoschisis is an eye disease characterized by the abnormal splitting
of the retina's neurosensory layers, usually in the outer plexiform layer.
Most common forms are asymptomatic, some rarer forms result in a
loss of vision in the corresponding visual field.
•Retinoschisis is a splitting within the neurosensory layer of the retina.
This condition frequently occurs in the inferotemporal quadrant. A
cross-section echogram can display a membranous structure in the far
periphery that has a convex border facing the vitreous.
• The splitting of the retinal layers can occur in different layers of the
retina and can sometimes involve more than one layer . Inner and outer
retinal holes can occur in the retinoschisis

3. •Senile Retinoschisis is a microcystoid degeneration of the
neurosensory retina, with splitting at the outer plexiform layer of the
retina.
•Retinoschisis is a thin immobile membrane with convex and smooth
configuration usually on temporal retina of a hyperopic patient
•Peripapillary and macular retinoschisis as well as serous retinal
detachment are often associated with optic nerve head pits,
colobomas, X-linked macular schisis and myopia..

4. •Peripheral splitting of the retinal nerve fiber layer, most commonly
between inner nuclear and outer plexiform layer.
Clinical features:
•Symptoms: mostly asymptomatic but may be associated with large
peripheral visual field defect in advanced cases.
Signs:
•Usually bilateral, located inferotemporally and very-slowly
progressive.
•Well-circumscribed, dome-shaped elevation of the inner retina
which extends anteriorly towards the ora serrata.
•The retinal surface usually appears normal but may frequently
have sheathed vessels and whitish snowflake opacities.
•May be complicated by retinal breaks, hole formation on either
the inner or outer retinal layer, retinal detachment or macular
compromise if the schisis advances posteriorly.

5. Image of typical inferotemporal location of senile retinoschisis.

6. Diagnostic procedures
You can check for an absolute scotoma with indirect ophthalmoscope
perimetry: hold a scleral depressor on the observer's side of the
condensing lens and document whether patient can see the depressor's
shadow
Laser will blanch retinoschisis but not a rhegmatogenous retinal
detachment (RRD)
Retinoschisis will not re-appose with scleral depression (no subretinal
fluid to move out)
OCT shows a break in the outer plexiform layer (OPL) in retinoschisis and
not detachment of retina from RPE (retinal detachment)
May be difficult to distinguish from longstanding retinal detachment
(RD): A smooth or bullous elevation is also seen in retinal detachments,
but characteristics for a (longer standing) retinal detachment are RPE
alterations and demarcation lines

11. •Affects only males
•Female carriers have normal vision and are normal on ophthalmic
examination
•Age of onset is in the first decade
•Histopathologically, there is a split between the nerve fiber and ganglion cell
layer
Clinical features:
Symptoms: decreased central vision (20/25 - 20/50) and slowly progressive
Signs:
•Presence of perifoveal microcystic changes with spoke wheel-like
plications of the overlying ILM, often referred to as stellate maculopathy
which are better seen on red-free fundus photography
•Peripheral retinal schisis occurs in 50% of cases
•The most common location for this peripheral schisis is the
inferotemporal quadrant
Potential findings at later ages as the condition progresses may include
RPE changes, macular hole, retinal dragging with vitreous strands,
complicated with vitreous hemorrhage and rhegmatogenous retinal
detachment

12. Ancillary Test:
Fluorescein angiographic reveals similar pattern to that of cystoid
macular edema, but without leakage of dye in the late phase
Visual field testing shows an absolute scotoma that corresponds to
the areas of peripheral schisis
ERG shows refuced photopic and scotopic b-wave
Management:
Observation, since this is a slowly progressive condition where
patients maintain their visual acuity for a long period of time. Surgical
management is necessary if complications such as retinal
detachment, vitreous hemorrhage or macular hole should occur

15. B SCAN:The height of this round mound of typically does not decrease on indentation or
pressure (versus retinal detachment the height of which reduces on indentation).

16. OCT 5-line raster scan in the mid-peripheral retina over a retinoschisis. The retinoschisis involved
multiple retinal layers. There is no subretinal fluid or retinal detachment.

17. OCT showing retinoschisis (white arrow) and neurosensory retinal detachment (red arrow) OD.

18. OCT of the retinal nerve fiber
layer showing significant
thinning OU. Note that the
temporal quadrant OD
appears thickened due to the
macular schisis and
neurosensory macular
detachment

21. Myopic macular retinoschisis. (A) Horizontal optical coherence tomography scan across the cen
fovea shows macular retinoschisis accompanying macular retinal detachment. (B) Six months a
vitrectomy, the macular retinoschisis as well as retinal detachment is resolved.

22. FIG. 1A and B. (A) Color photograph of bullous retinoschisis located in the superotemporal
retinal periphery. (B) OCT obtained with the scanning plane orthogonal to elevated retina, while
still including a portion of flat retina (see yellow line, Fig. 1A). Note that in the area where the
retina is most elevated, ORLs are visible overlying the RPE, wh

23. FIG. 2A and B. (A) Color
photograph of bullous
retinoschisis located in the
inferotemporal retinal
periphery. (B) OCT was
obtained with the scanning
plane orthogonal to the
elevated retina, while still
including a portion of flat
retina (see yellow line, Fig.
2A). ORLs are visible
overlying the RPE, which
defines schisis on OCT.

24. FIG. 1. X-linked schisis with OCT. Note splitting of both inner and outer retina with no
evidence of retinal detachment.

25. FIG. 3A and B. (A) Transition zone from flat retina to shallow retinoschisis is illustrated. (B)
More of the area of retinoschisis is shown, which widens from left to right (nasal to
temporal). Connecting elements, thought to be Müller's cells, are visible, and outer retinal
layers are present overlying the RPE

26. Combined retinoschisis and retinal detachment. Both areas are distinctly visualized on each
side of the transition zone (yellow arrow).

27. A) Peripheral retinoschisis includes multiple planes of separation. (B) The peripheral
retinoschisis communicates posteriorly to involve the macula, where the plane of separation is
easier to determine between the outer plexiform layer and outer nuclear layer

28. Wide-angle OCT imaging the retina from ora to ora. Bullous retinoschisis is visible temporally

29. Clinically, retinoschisis is commonly located inferotemporally and tends to be bilateral but
asymmetric (Figure 1A). It is dome-shaped with uniform convexity and without the
corrugations often seen in RRD. The corrugations are not always appreciated in chronic RRD
(Figure 2A). The inner layer has a “beaten metal” or pitted appearance, and minute,
glistening, yellow-white surface dots can be seen. There are no retinal tears, vitreous pigment
cells, or demarcation lines as might be seen in RRD.1-

30. FIG1.Widefield fundus photo (Optos) of retinoschisis (arrows; A). SD-OCT (Carl Zeiss
Meditec) of posterior edge of retinoschisis cavity demonstrating split in
neurosensory retina with formation of inner and outer leaves (B).

31. Clinically, retinoschisis is commonly located inferotemporally and tends to be
bilateral but asymmetric (Figure 1A). It is dome-shaped with uniform convexity
and without the corrugations often seen in RRD. The corrugations are not always
appreciated in chronic RRD (Figure 2A). The inner layer has a “beaten metal” or
pitted appearance, and minute, glistening, yellow-white surface dots can be seen.
There are no retinal tears, vitreous pigment cells, or demarcation lines as might be
seen in RRD.1-

32. Figure 2. Widefield fundus photo of chronic retinal detachment with
demarcation line marked by arrows (A). SD-OCT of the posterior edge of the
retinoschisis cavity demonstrating complete separation of the retina from
the RPE (B).

33. Figure 3. Widefield fundus photo of retinoschisis associated with frank RRD. Large outer retinal
hole is marked by black arrows, and inner retinal break is marked by red arrow.

36. Spectral domain optical coherence tomography (OCT) of the right macula demonstrating
the vertical inter-bridging strands and cystic degeneration of the outer retina typical of
myopic retinoschisis.

38. Figure 1 Wide-field imaging and spectral domain optical coherence tomography (SD-OCT) of
the three patterns of outer retinal holes corresponding to isolated retinoschisis and schisis-
detachment. (A–C) Isolated retinoschisis (pattern I). (A) Wide-field photo (Optos) of isolated
retinoschisis and outer retinal hole. The edge of the retinoschisis and outer retinal hole are
marked by arrows. (B) SD-OCT (Carl Zeiss Meditec) of outer retinal hole with both edges of
the hole down and attached to retinal pigment epithelium (RPE). (C) SD-OCT of the posterior
edge of the retinoschisis cavity with splitting of neurosensory retina. (D–G) Schisis-
detachment (pattern II). (D) Wide-field photo of schisis-detachment and outer retinal hole.
The edge of the schisis-detachment and outer retinal break are marked by arrows. (E–F) SD-
OCT of outer retinal hole with one edge elevated from RPE (E) and the other edge down and
attached to RPE (F). (G) SD-OCT of the posterior edge of the schisis-detachment with
elevation of neurosensory retina from RPE. (H) Schisis-detachment (pattern III). SD-OCT of
outer retinal hole with both edges elevated from RPE. (I–K) Schisis-detachment with
breakthrough distal to outer retinal hole with both edges attached to RPE. (I) Wide-field
photo of schisis-detachment with breakthrough distal to outer retinal hole. (J) SD-OCT of
schisis-detachment with breakthrough distal to outer retinal hole with both edges attached to
RPE. (K) SD-OCT of the posterior edge of the schisis-detachment with elevation of
neurosensory retina from RPE

39. ERG is one of the most clinically useful tests for XLRS. The dark-adapted ERG shows
reduced b-wave amplitude, while the a-wave frequently remains normal . This ERG
configuration is termed an electronegative response because the positive-going b-wave
fails to return to above baseline from the negative-going a-wave (Fig. 3). Sixty percent of
XLRS eyes had the “electronegative waveform” in a cohort of 68 affected men . As the b-
wave originates from activity of depolarizing bipolar cells that lie postsynaptic to the rods ,
this implies primary involvement of the inner retina in XLRS.

40. FIGURE 3. Scotopic full-field ERG traces from an affected XLRS male (45 years old),
harboring a c354del1- ins18 mutation of the retinoschisis (RS1) gene. XLRS affected male
shows typical ERG with b-wave reduction but a-wave preservation in dark-adapted
recordings, compared with a normal individual. The arrow indicates the characteristic
“electronegative” waveform with b-wave smaller than the a-wave.

45. Retinoschisis usually doesn't require treatment aside from glasses to
improve your vision. However, some children with X-linked
retinoschisis may have bleeding in their eye. This can be treated with
either laser therapy or cryosurgery. In rare cases, children may
need surgery to stop the bleeding