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Retinoschisis

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#Retinoschicis_diagnosis
#Retinoschicis_OCT_BScan

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Retinoschisis

  1. 1. Retinoschisis MOHAMMAD RIYAJ ALI Consultant Optometrist
  2. 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. 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. 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. 5. Image of typical inferotemporal location of senile retinoschisis.
  6. 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
  7. 7. •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
  8. 8. 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
  9. 9. 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).
  10. 10. 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.
  11. 11. OCT showing retinoschisis (white arrow) and neurosensory retinal detachment (red arrow) OD.
  12. 12. 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
  13. 13. 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.
  14. 14. 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
  15. 15. 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.
  16. 16. FIG. 1. X-linked schisis with OCT. Note splitting of both inner and outer retina with no evidence of retinal detachment.
  17. 17. 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
  18. 18. Combined retinoschisis and retinal detachment. Both areas are distinctly visualized on each side of the transition zone (yellow arrow).
  19. 19. 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
  20. 20. Wide-angle OCT imaging the retina from ora to ora. Bullous retinoschisis is visible temporally
  21. 21. 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-
  22. 22. 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).
  23. 23. 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-
  24. 24. 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).
  25. 25. 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.
  26. 26. 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.
  27. 27. 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
  28. 28. 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.
  29. 29. 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.
  30. 30. 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

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