Tears of the retinal pigment epithelium (RPE) Case Presentation

1. PresentedBy:
Dr. MAB
Dr. ABJ
Dr. KSD
Case presentation

2. A male patient, 76 years old
Main complaints : DOV in the right eye since 1 year
back .
Past Ocular History: OU Cataract surgery
done
VA: OD 3/60,N36 6/24 with PH
OS 6/9,N6
IOP: OD: 18 mmHg OS: 20 mmHg
Anterior segment was WNL
OU:Pseudophakia

3. fundus:

4. FFA:
a large feeder
net with
temporal
leakage
s/o mature
vessels

5. OCT Before 1st injection

6. • Action Plan:
– OD: Ranibizumab injection under TA
OD: 6 Ranibizumab injection under TA last
one on 22.05.2015
The Right eye show good response for the
injection and doing well now
The following OCT tests done during follow
up between injections and show good response
for injection:

7. OD Post 1st injection OD Post 2nd injection
OD Post 3rd injection OD Post 4th injection

8. Action Plan:
To be watch
• OD Post 5th injection
OD Post 4th Inj. & Before 5th Inj.
• OD Post 6th injection

9. On 9.7.2015
The patient came for regular follow up without any ocular complaints
VA:
OD 6/18,N24
OS 6/6,N6
IOP:
OD:15 mmHg
OS:16 mmHg
Anterior segment was WNL
OU: Psuedophakia

10. Fundus:
.
OD: Stable
OS: Has SRF at the fovea with increase
in PED size

11. OCT:

12. OS: Speckled fluorscence with a
single area of increased
hyperfluorescence ST to the fovea
AF:

13. FFA:
OS:Speckledfluorscencewitha
singleareaofincreased
hyperfluorescenceSTtothefovea

14. • Action Plan:
– OS: Ranibizumab injection under TA
• OS: Ranibizumab injection under TA monotherapy given on
14.07.2015
• On 15.07.2015
OS: 1st Day post injection Accentrix Fundus Show RPE RIP + (small
sparing fovea)
• On 18.07.2015
OS: 3rd Day post injection Accentrix Fundus Show RPE RIP +
progressing

16. On 30.07.2015

17. On 2015-08-31
The patient felt Vision dropped since three months
C/O metamorphopsia in left eye
Right eye stable
VA:
OD 6/18,N18
OS 6/9,N8  BCVA 6/9, N6
IOP:
OD: 14 mmHg
OS:14 mmHg
Anterior segment was WNL

18. Fundus :

20. OCT:

22. RPE tear: In era of anti VEGF
agents

23. • First described by Hoskin et al
• Varied etiologies
• Trauma, CSC, Angioid streak, Myopia
• AMD
• PCV
• RAP
Hoskin A, Bird AC, Sehmi K. Tears of detached retinal pigment epithelium. Br J Ophthalmol 1981;65(6):417-422.

24. • Most commonly associated with neovascular
AMD
• Can be spontaneous or associated with
treatment
• Anti VEGF, PDT, Laser photocogaulation
• Incidence spontaneous tear rate 10-12 %
• Bilateral incidence 53%
Chuang EL, Bird AC. The pathogenesis of tears of the retinal pigment epithelium. Am J Ophthalmol
1988;105:285–290.

25. Theories proposed
• Increased intra PED hydrostatic pressure due
to enlargement
• Tangential forces on posterior surface of
detached RPE
Stretched RPE- blow out tear
Goldstein BG, Pavan PR. Blow-outs in the retinal pigment epithelium. Br J Ophthalmol 1987;71:676–681

26. RPE tear post anti VEGF injections
• Bevacizumab, Ranibizumab,
Pegaptinib,Afliberept
• Overall incidence 5-19.7%
• Average number of injections before RPE tear 1.3
• Duration of appearance
• Earliest 1st day postoperative in our patient
(Unpublished data)
• Literature 11 days after initial injection (Range 11
days to 46.3 weeks)
Chang LK, Sarraf D. Tears of the retinal pigment epithelium: an old problem in a new era. Retina 2007;27:523-534
M Gutfleisch et al. Long-term visual outcome of pigment epithelial tears in association with anti-VEGF therapy
of pigment epithelial detachment in AMD. Eye 2011;25:1181–86

27. • IOP shifts post anti VEGF injection
• Interruption of tight junction maintenance
post anti VEGF
• Vitreomacular traction
• CTGF VEGF imbalance post anti VEGF
Nagiel A, Freund KB, Spaide RF, Munch IC, Larsen M, Sarraf D. Mechanism of retinal pigment epithelium tear formation following intravitreal
anti-vascular endothelial growth factor therapy revealed by spectral-domain optical coherence tomography. Am J Ophthalmol 2013;156(5):981-988.

28. Clinical features
• Abrupt sudden onset loss of vision
• Clinically, a well demarcated area of bare choroid visible
adjacent to hyperpigmented area, which is retracted,
redundant retina
• Temporal edge of PED most commonly affected
• Often accompanied by subretinal hemorrhages,exudation or
break through vitreous hemorrhage
• Initial course, good prognosis
• Long term follow up- progressive visual loss
• Depends of foveal involvement
• Foveal involvement incidence range 23-75%
•Chang LK, Sarraf D. Tears of the retinal pigment epithelium: an old problem in a new era. Retina 2007;27(5):523-534.
•Gamulescu MA, Framme C, Sachs H. RPE-rip after intravitreal bevacizumab (Avastin) treatment for vascularised PED
secondary to AMD. Graefes Arch Clin Exp Ophthalmol 2007; 245:1037–40.

29. • RPE tears graded based on the greatest length
in the vector direction of the tear and
involvement of the fovea using FA analysis, a
measurement of greatest linear diameter
[millimeter] was obtained
Sarraf D, Reddy S, Chiang A, Yu F, Jain A. A new grading system for retinal pigment
epithelial tears. Retina 2010;30(7):1039-1045.

30. • Prospective study
• Incidence of RPE tear -14%
• RPE tear + PED height >550μ-31%
• RPE tear + PED height > 550μ + ring sign on
FFA/Grade 1 tear- 67%
Sarraf D, Chan C, Rahimy E, Abraham P. Prospective evaluation of the incidence and risk factors for the development of
RPE tears after high- and low-dose ranibizumab therapy. Retina 2013;33(8):1551-1557.

31. AMD v/s PCV
• More common in AMD – 3.5 % v/s 0.62% in PCV
• Pathogenesis differs
• Element of FVPED in AMD
– Anti VEGF causes fibrotic contraction ripping overlying RPE
• Large serosanguinous PED in PCV
– Vascular complexes in PCV may not contract enough
– AntiVEGF reduces leakage, but shrinkage of polypoidal
dilatations hardly affected.
– Adhesions of PCV components to RPE might be weak
– Micro rips (7.1%) at margin of PED reduces intra PED
pressure and thereby frank RPE tear
•Shin et al. Pigment epithelial tears after ranibizumab injection in polypoidal choroidal vasculopathy and typical age-related
macular degeneration. Graefes Arch Clin Exp Ophthalmol DOI 10.1007/s00417-015-2977-3
•Musashi K, Tsujikawa A, Hirami Y, et al. Microrips of the retinal pigment epithelium in polypoidal choroidal vasculopathy.
Am J Ophthalmol 2007;143(5):883-885.

32. FFA,ICG and OCT
• FFA-Hyperfluoresecence in area of bare choroid and
hypofluoresence in area of retracted and elevated RPE flap
• No leak in area of bare choroid-Atrophy of choriocapillaris
• ICG- Normal choroidal fluorescence in area of bare choroid
and varying degrees of hyperfluoresence in area of retracted
RPE
• OCT- Interrruption of hyperreflective RPE layer with
elevated or scrolled edege of torned RPE flap
• Three configurations of retracted RPE-Dome shaped,
pleated, tent like
• Increased reflectivity in area of bare choroid
•Arroyo JG, Schatz H, McDonald R, Johnson RN. Indocyanine green videoangiography after acute retinal pigment epithelial
tears in age-related macular degeneration. Am J Ophthalmol 1997;123:377–385.
•Giovannini A, Amato G, Mariotti C, Scassellati-Sforzolini B. Optical coherence tomography in the assessment of retinal
pigment epithelial tear. Retina 2000;20:37–40.

33. Prognostic indicators
• Pre injection PED height >400 μ
• PED height predicts RPE tear risk with 85%
sensitivity and 92% specificity
• GLD of PED- 5 mm
• PED duration <4.5 months- Predicts RPE tear risk
with 77% sensitivity and 98% specificity
• Additional prognostic factor-Fibrovascular
scarring and atrophy in RPE free area
• Fibrovascular-poorer prognosis
•Chan et al. Optical coherence tomography–measured pigment epithelial detachment height as a predictor for retinal pigment epithelial tears
associated with intravitreal bevacizumab injections. Retina 2010;30:203–11.
•Doguizi and Ozdek. Pigment epithelial tears associated with anti-VEGF therapy. Incidence, long-term visual outcome, and relationship with
pigment epithelial detachment in age-related macular degeneration. Retina 2014 ;34:1156–62.
•Sarraf D, Chan C, Rahimy E, Abraham P. Prospective evaluation of the incidence and risk factors for the development of RPE tears after high- and
low-dose ranibizumab therapy. Retina 2013;33(8):1551-1557.

34. • Does anti VEGF cause RPE tear?
• Why it is more common in AMD?
• What are the risk factors? Height and GLD
• What is the prognosis?
• What is the further course of treatment?