1. Retinoschisis
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2. Case 1 46/F C.C.) Blurred vision(OS) for 6months
Past history) 1993 Excimer laser(OS) at local clinic

3. Ocular exam VA OD (0.8 x -5.50Ds : -2.00Dc Ax 180)
OS (0.8 x -3.25Ds : -1.50Dc Ax 170)
IOP OD/OS 13/12 mmHg
Lid OU no swelling
Conj. OU not injected
Cornea OU clear
AC OU deep & cell (-)
Pupil OU round & nl sized, LR(+/+)
Lens OU mild cortical opacity

4. Fundus photography OD Tigroid appearance, flat post pole
Slit lamp exam
The elevation of the retina within the posterior staphyloma without a macular hole
May have a microcystic appearance in the area
OD Tigroid appearance, flat post pole
OS Suspicious elevation of the fovea, tigroid appearance, PVD(+)
Axial length OD/OS / 29.54

5. Foveal detachment (OS)
Retina split into a thick inner layer and a thin outer layer

6. Diagnosis & plan Diagnosis) Plan) R/O myopic retinoschisis(OS)
R/O RD(OS) – thick
R/O X-linked juvenile retinoschisis(OS) – female, age
R/O optic disc pit syndrome(OS) – optic disc aplasia or hypoplasia (-)
R/O macular edema(OS) – FAG leak
Plan)
FAG, VEP, ERG

7. FAG : no leak

8. VEP, ERG : Decreased rod response

9. Case 2 21/M C.C.) 우안 초점이 가려 보여요 onset) 1 year ago
Past history) Amblyopia(OS) since childhood
Muscle op. several years ago

10. Ocular exam VA OD 0.25(0.63 x +2.25Ds : -2.75Dc Ax 180)
OS 0.06(0.1 x +6.00Ds : -1.25Dc Ax 160)
IOP OD/OS 13/19 mmHg
Lid OU no swelling
Conj. OU not injected
Cornea OU clear
AC OU deep & cell (-)
Pupil OU round & nl sized, LR(+/+)
Lens OU clear

11. Fundus photography
Bilateral infero-temporal retinoschisis Chorioretinal atrophy of macula Veil-like membrane and fold protruded into vitreous Intraretinal pigmentation

12. Cyst-like maculopathy

13. Diagnosis & plan Diagnosis) R/O juvenile retinoschisis R/O RD Plan)
R/O retinitis pigmentosa
Plan)
Observation

15. Retinoschisis - Review

16. Retinoschisis Myopic retinoschisis X-linked Juvenile retinoschisis
Senile(acquired) retinoschisis

17. Myopic retinoschisis High myopia - pathological changes of the fundus
First reported in 1958, by Phillips
RD without retinal breaks in highly myopic eyes
Localized posterior RD over posterior staphyloma without a macular hole

18. Myopic retinoschisis : ocular features
Slit lamp
Elevation of the retina within the posterior staphyloma
Without a macular hole
Microcystic appearance in the area
OCT
Foveal detachment
Split into a thick inner layer and a thin outer layer
Posterior hyaloid exerts traction
Macular hole subsequently evolved in some cases
B-scan
Very thin, smooth, dome-shaped membrane
High spike 100%

19. Myopic retinoschisis Incidence : common in high myopia (9~34 %)
BCVA : ranged from 20/400 to 20/25 in most eyes
Pathogenesis
Anterior traction on the fovea caused by tangential contraction of the vitreous cortex
ERM from an eye with macular hole retinal detachment associated with high myopia

20. Myopic retinoschisis : differential diagnosisRD RD
Careful exam using OCT
Visual outcome can differ markedly
Optic disc pit syndrome
Excavations of the optic nerve head
Megalopapilla, optic disc aplasia, and hypoplasia, retinal colobomas
Macular edema
FAG : leak (+)
X-linked juvenile retinoschisis
Optic disc pit ME

21. Myopic retinoschisis : treatment
Pars plana vitrectomy
Posterior vitreous separation
Removal of ILM
Gas tamponade, in eyes with macular hole RD
After vitrectomy, retinoschisis gradually decreases and completely disappears in several months
Visual outcomes
Without a macular hole >> with macular hole
Better than 20/50 : ≥ 50%
Spontaneous resolution after spontaneous PVD

22. 53/M -15. 0 diopters and an axial length of 29
53/M diopters and an axial length of 29.4 mm Shallow RD and retinoschisis without a macular hole 6 months after ppV+ILM peeling+gas tamponade : reattached

23. 2. X-linked juvenile retinoschisis
“Congenital hereditary retinoschisis” , “Congenital vascular veil”
Rare, prevalence 1:120,000
Develops early in life and is probably present at birth
Presenting symptoms
Poor vision, strabismus, and nystagmus
Onset in the first decade of life in most patients
Transmitted as an X-linked recessive trait (occurring almost in males)
Rare females
Turner syndrome (45, X genotype)
Possibly a related autosomal disease
Retinoschisis gene (RS1) : Xp22, retinoschisin

24. X-linked juvenile retinoschisis – ocular features
Presentation
Bilateral infero-temporal retinoschisis
Cyst-like stellate maculopathy
Typical finding as age
In young : stellate striation of macula
In older : atrophic changes of underlying RPE
In periphery
Veil-like membrane and fold protruded into vitreous
Others
Blood vessel change : sheathing, dendritic and falciform degeneration
End-stage : atrophic macula, diffuse chorioretinal degeneration, intraretinal pigmentation
Mimic cystoid macular edema
FAG is different : no leakage that is characteristic of CME

25. X-linked juvenile retinoschisis
Stellate maculopathy
Vitreous veils

26. Retinoschisis in the temporal inferior quadrant

27. X-linked juvenile retinoschisis – ocular features
Progression
Usually only very slowly progressive
Rapid during first 5 yrs, and then slows
Become stationary till 20 yrs of age
Increased height of schisis cavity
Vitreous traction
Play a role in progression

28. X-linked juvenile retinoschisis – Diagnosis
Indirect ophthalmoscope
Domelike elevation of very thin layer of retinal tissue containing blood vessel
Biomicroscopy : Aids DDx for retinoschisis and retinal detachment
No vitreous detachment
Very thin detachment
Transparent layer
Absence of fluid shift
M-OCT
Valuable
Wide hyporeflective space that split the neurosensory retina into thin outer layer and thick inner retinal layer
ERG
Selective b-wave reduction
Slit DDx
Retinoschisis는 보통 vitreous가 떨어져있지 않다.
Retinoschisis는 바깥에 떨어져 보이는 부분이 매우 얇아서 망막박리와는 다르게 보이며 박리된 망막과 달리 물결치는 듯한 양상을 보이지 않는다.
Retinoschisis는 머리를 움직임에 따라 shift되지 않는다.
*OCT finding
: Wide hyporeflective space with vertical palisades that split the neurosensory retina into thin outer layer and thick inner retinal layer

29. X-linked juvenile retinoschisis – DDxRD
Acquired (senile) retinoschisis
Retinitis pigmentosa
Sickle cell retinopathy
ROP
Enhanced S cone syndrome

30. X-linked juvenile retinoschisis - treatment
Indication of surgery
Retina is detached
Vitreous hemorrhage is present
Macula is obscured by schisis
How to treat?
Pars plana vitrectomy
Creation of posterior vitreous separation
Removal of internal limiting membrane
Internal tamponade
Topical dorzolamide : to reduce macular cystic cavity

31. 3. Senile retinoschisis Uncommon
Splitting of the neurosensory retina, at the outer plexiform later
Men = Women
Myopia (-), adult, FAG leak(-)
Asymptimatic, rarely progressive, benign
Indication for prophylactic therapy
Significant progression of subretinal fluid toward the post pole

32. Reference RETINA, 4th edition
X-linked retinoschisis maculopathy treated with topical dorzolamide, and relationship to genotype. (EYE 2011 Jul;25(7))
Acquired senile retinoschisis of the peripheral retina imaged by spectral domain optical coherence tomography: a case report. (Eur J Ophthalmol 2010;20(6))