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Presented by: 
Dr. Mohammad Abdullah Bawtag 
Sankara Nethralay a– Chennai, India 
2014
History of Pathological Myopia
Myopia- New Latin …… was derived from the original Greek word “mŭopia” … 
contracting or closing the eye. 
- 138–201 Galen...
Terminologies of Pathological Myopia
Pathological myopia 
Degenerative myopia 
Malignant myopia 
High degree myopia 
Progressive myopia 
Magna myopia
Definitions of Pathological Myopia
Clinically- refractive error > -6 D. 
Duke-Elder - Myopia with degenerative changes 
especially in the post. segment. 
Tok...
Prevalence of Pathological Myopia
Country % Country % 
Myopia 
Some Asian countries 70–90% Industrialized -West 10%–25% 
Taiwan 84% Africa 10–20% 
Industria...
Interesting facts 
Lengthening of the post. segment of the eye commences only during 
the period of active growth. The eye...
Pathogenesis of Pathological Myopia
Etiology of Myopia is as diverse and controversial as one 
can imagine. Everything in medicine has been blamed as a 
cause...
Mechanical theories - distension of normal sclera - Increased IOP 
caused by the action of EOMs or IOMs or by insidious ch...
Classification of Myopia
Type of Class. Classes of Myopia 
Cause Axil Myopia 
Refractive Myopia ( Curvature & Index ) 
Clinical Entity Simple myopi...
High Myopia is classified in a simple manner as: 
i) Simple ii) pathological 
Simple Myopia - not progressive, good vision...
Risk factors
Risk factors Description 
Race & ethnicity Asians 
Age Middle aged (working life) or younger 
Gender Female 
Social group ...
Genetic factors
Family studies and twin studies have revealed the heritability of myopia since the 
1960s. 
In familial studies and twin s...
Manifestations of Pathological Myopia 
Anatomical Manifestations 
Functional Manifestations 
Ocular Manifestations
Anatomical Manifestations 
Corneal astigmatism 
Deep AC 
Angle iris processes 
Zonular dehiscences 
Vitreous syneresis 
La...
Functional Manifestations 
Suboptimal binocularity 
Image minification 
Anisometropic amblyopia 
Subnormal visual acuity 
...
Ocular Manifestations 
-Strabismus:exophoria/exotropia 
-Cataract. 
-Glaucoma.. pigmentary / normal-tension glaucoma 
-Tig...
Complications of Pathological Myopia 
This review aims to provide an overview on some of the important 
complications asso...
Vitreous degeneration 
 Syneresis 
 Vitreous liquefaction, fibril aggregation & condensation 
 Associated with floaters...
Liquefaction of the 
vitreous gel 
Hole in the posterior 
hyaloid membrane 
Fluid tru defect into 
retrohyaloid space 
Vit...
•PVD with gel collapse 
Without vitreous hage, 4% develop retinal breaks 
With vitreous hage, 20% develop breaks 
PVD w...
Symptomatic PVD 
Approx 10-15 % 
Retinal breaks at first 
assessment 
Approx 90 % 
uncomlicated at first 
assessment 
High...
Ultrasound picture showing PVD. 
Note that the vitreous is still attached 
at the optic disc and the ora serrata.
Vitreous changes in PM 
 Vitreous liquefaction 
 Early PVD 
Presence of CPVD 
Years PM control 
20- 39 27.8% 
40-59 43% ...
Myopic Foveoschisis 
 Prevalence – 9% to 34% 
 Pathogenesis : 
1. Attachment of Contracted vitreous cortex to retinal su...
 Natural history: 
Varied course with diverse visual outcomes- stable to development of 
macular holes 
Eyes with anterio...
Macular hole 
Myopic macular hole may occur, but the exact mechanism is 
unknown. 
Whether attenuation of the neural retin...
Various surgical procedures have been performed for macular hole 
with or without RD and they include : 
 PPV with gas or...
Myopic macular chorioretinopathy 
 DEF: is a rare, genetic eye disorder that causes vision loss. 
 Grading(shih et al) 
...
Peripheral retinal degenerations & RRD 
 “Lattice degeneration is a common retinal degeneration.” 
 1. Epidemiology 
 8...
Lattice degeneration - predispose to RRD 
Retinal tears - posterior and lateral margins of the lattice 
degeneration 
Role...
In eyes with RD, laser photocoagulation alone is insufficient to treat 
the condition and V-R surgery is required. 
Surgic...
Myopic RD 
• Incidence of RD in general population range between 
0.005 and 0.01 % . 
• RD occurs far more frequently in p...
Syneresis of the 
central vitreous 
Traction caused 
by spontaneous 
or PVD 
RETINAL TEAR
CNV in Pathological Myopia 
Among various lesions associated with high myopia, macular CNV 
is one of the most vision thre...
- Develops from laquer cracks. 
- Smaller, less exudation. 
- Type 1 (severe myopic degeneration)- Leakage does not 
exten...
The mechanism of CNV formation in myopic CNV is still 
unclear. 
 A possible explanation includes, certainly, the induced...
Treatment of myopic CNV 
More recently, the use of anti-VEGF agents 
The most commonly used currently is PDT with 
vertepo...
Features of choroid in PM 
 Stretched choroid without additional vasculature 
 Thinner choroid 
 Choriocapillaries and ...
Lacquer cracks 
Spontaneous ruptures in the Bruch's membrane . 
Small hages may develop within the lacquer cracks. 
Lacque...
Post. Staphyloma
post. staphyloma (ectasia) 
Equatorial staphyloma with scleral dehiscence - STQ. 
Visual loss is most often due to macular...
Curtin classified the staphylomas into ten categories. The first five were simpler 
configurations, while the last five we...
Tesselated Fundus 
 Hypoplasia of the RPE following axial elongation reduces 
the pigment, allowing the choroidal vessels...
References
 Ohno-Matsui K, Yoshida T, Futagami S, Yasuzumi K, Shimada N, Kojima A, et al. Patchy 
atrophy and lacquer cracks predisp...
 Pukhrai Rishi, … et al …..Photodynamic monotherapy or combination treatment with 
intravitreal triamcinolone acetonide, ...
Pathological myopia 01.03.2014
Pathological myopia 01.03.2014
Pathological myopia 01.03.2014
Pathological myopia 01.03.2014
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Pathological myopia 01.03.2014

  1. 1. Presented by: Dr. Mohammad Abdullah Bawtag Sankara Nethralay a– Chennai, India 2014
  2. 2. History of Pathological Myopia
  3. 3. Myopia- New Latin …… was derived from the original Greek word “mŭopia” … contracting or closing the eye. - 138–201 Galen was the first to use the term myopia PM- 1988 Takashi Tokoro …Definition of pathologic myopia Staphyloma - is a pathognomic feature of PM - 1801 Antonio Scarpa First anatomical description of posterior staphyloma, but did not make the link to myopia - 1856 Carl Ferdinand von Arlt First connected staphyloma and myopic refraction - 1977 Brian J. Curtin Classification scheme for staphyloma
  4. 4. Terminologies of Pathological Myopia
  5. 5. Pathological myopia Degenerative myopia Malignant myopia High degree myopia Progressive myopia Magna myopia
  6. 6. Definitions of Pathological Myopia
  7. 7. Clinically- refractive error > -6 D. Duke-Elder - Myopia with degenerative changes especially in the post. segment. Tokoro - Myopia caused by pathological axial elongation. A more specific - Myopic retinopathy, refers to the degeneration of chorioretinal tissue ass. with axial elongation of the eye.
  8. 8. Prevalence of Pathological Myopia
  9. 9. Country % Country % Myopia Some Asian countries 70–90% Industrialized -West 10%–25% Taiwan 84% Africa 10–20% Industrialized - East 60%–80% India 6.9% Europe and the US 30–40% PM Asian 9–21% Most countries 1–4% Spain 9.6% USA 2% Singapore 9.1% Bangladeshi 1.8% Japan 8% Czechoslovakia 1% Northern China 4.1% Egypt 0.2% High myopia affects 27%-33% of all myopic eyes in Asia.
  10. 10. Interesting facts Lengthening of the post. segment of the eye commences only during the period of active growth. The eye and the brain show precocious growth at the age of 4 years; the brain is 84% and the eye 78% and the rest of the body 21%. After this, both the eye and the brain increase slowly while the body grows more rapidly. However, when axial myopia continues to progress, it is interpreted as a precocious growth which has failed to get arrested…………….!!!!!!!!!! We do not as yet know what this influence is.
  11. 11. Pathogenesis of Pathological Myopia
  12. 12. Etiology of Myopia is as diverse and controversial as one can imagine. Everything in medicine has been blamed as a cause of Myopia. Two types of theories are put forward: 1) Mechanical and Environmental 2) Biological
  13. 13. Mechanical theories - distension of normal sclera - Increased IOP caused by the action of EOMs or IOMs or by insidious chronic glaucoma. Others theories : weakening of the sclera - venous congestion, inflammation or dietary deficiency.
  14. 14. Classification of Myopia
  15. 15. Type of Class. Classes of Myopia Cause Axil Myopia Refractive Myopia ( Curvature & Index ) Clinical Entity Simple myopia Nocturnal myopia Pseudomyopia Degenerative myopia Induced myopia Degree Low myopia (<-3.00 D) Medium myopia (-3.00 D - -6.00 D) High myopia (>-6.00 D) Age of Onset Congenital myopia (present at birth and persisting through infancy) Youth-onset myopia (<20 years of age) Early adult-onset myopia (20-40 years of age) Late adult-onset myopia (>40 years of age)
  16. 16. High Myopia is classified in a simple manner as: i) Simple ii) pathological Simple Myopia - not progressive, good vision- optical correction. Pathological Myopia - changes in the posterior segment, lengthening of AP axis of the globe.
  17. 17. Risk factors
  18. 18. Risk factors Description Race & ethnicity Asians Age Middle aged (working life) or younger Gender Female Social group Children(Asian) professional working adults Geography Industrialised/developed nations Lifestyle Time spent outdoors Education High level of education/academic achievement Occupation Near work indoors (e.g. lawyers, physicians, microscopists and editors) Familial inheritance (parental refraction) Genetic
  19. 19. Genetic factors
  20. 20. Family studies and twin studies have revealed the heritability of myopia since the 1960s. In familial studies and twin studies, linkage analysis using microsatellite markers has identified 19 loci for myopia: MYP1 to MYP19. AD High Myopia AR High Myopia X-Linked High Myopia Common Myopia MYP1 MYP13 MYP2 MYP18 MYP3 MYP4 MYP5 MYP11 MYP12 MYP15 MYP16 MYP17 MYP19 MYP7 MYP8 MYP9 MYP10 MYP14 MYP17
  21. 21. Manifestations of Pathological Myopia Anatomical Manifestations Functional Manifestations Ocular Manifestations
  22. 22. Anatomical Manifestations Corneal astigmatism Deep AC Angle iris processes Zonular dehiscences Vitreous syneresis Lattice retinal degeneration Scleral expansion and thinning ↓ Ocular rigidity ↑ AL Tilted disc Peripapillary detachment in PM Temporal crescent or halo atrophy Macular lacquer cracks Pigment epithelial thinning Choroidal attenuation Foveal retinoschisis Post. staphyloma
  23. 23. Functional Manifestations Suboptimal binocularity Image minification Anisometropic amblyopia Subnormal visual acuity Visual field defects Impaired dark adaptation Abnormal color discrimination
  24. 24. Ocular Manifestations -Strabismus:exophoria/exotropia -Cataract. -Glaucoma.. pigmentary / normal-tension glaucoma -Tigroid, or blond fundus, with choroidal visible underneath -Tilted optic nerve with peripapillary atrophy -Peripapillary detachment -Chororetinal atrophy -PVD -RD -Lacquer cracks -Lattice degeneration (spontaneous breaks in Bruch's membrane) -Cobblestone degeneration -Fuch's spot (RPE hyperplasia in response to CNV) -Scleral thinning -Peripheral retinal holes -Macular holes causing RD -CNV
  25. 25. Complications of Pathological Myopia This review aims to provide an overview on some of the important complications associated with PM. Vitreous degeneration Peripheral retinal degenerations & RRD Myopic foveoschisis & Macular hole CNV in PM Lacquer cracks Post. Staphyloma
  26. 26. Vitreous degeneration  Syneresis  Vitreous liquefaction, fibril aggregation & condensation  Associated with floaters  Caused by myopia, senescence, trauma, inflammations, hereditary causes  PVD
  27. 27. Liquefaction of the vitreous gel Hole in the posterior hyaloid membrane Fluid tru defect into retrohyaloid space Vitreous gel collapses synchytic fluid in space Detachment of posterior vitreous from ILM Acute PVD
  28. 28. •PVD with gel collapse Without vitreous hage, 4% develop retinal breaks With vitreous hage, 20% develop breaks PVD without gel collapse Associated with future retinal hole or vitreous hage Scaffold for proliferative new vessels
  29. 29. Symptomatic PVD Approx 10-15 % Retinal breaks at first assessment Approx 90 % uncomlicated at first assessment High risk break Low risk break Low risk of detachment Approx 98 % uncomplicated At 4-6 weeks 1.5-3.4% Retinal breaks At 4-6 weeks Detachment In 33-46% Within 6 weeks Flow chart illustrating the natural history of an acute PVD
  30. 30. Ultrasound picture showing PVD. Note that the vitreous is still attached at the optic disc and the ora serrata.
  31. 31. Vitreous changes in PM  Vitreous liquefaction  Early PVD Presence of CPVD Years PM control 20- 39 27.8% 40-59 43% 8% 60 - 79 91% 60%  Larger posterior precortical vitreous pocket  Residual posterior cortex in CPVD
  32. 32. Myopic Foveoschisis  Prevalence – 9% to 34%  Pathogenesis : 1. Attachment of Contracted vitreous cortex to retinal surface 2. ERM 3. Retinal vascular traction 4. Rigidity of ILM 5. Progression of posterior staphyloma
  33. 33.  Natural history: Varied course with diverse visual outcomes- stable to development of macular holes Eyes with anterior traction had worst prognosis Progressive disease with poor outcomes  Treatment:  PPV+ILM peeling(traditional/foveal sparing) +/- tamponade – useful to relieve internal surface anterior traction  Scleral buckling – Addresses disparity between retina and elongated sclera  Suprachoroidal buckling – hyaluronic acid injected through a catheter into suprachoroidal space in the area of staphyloma to indent choroid  Complications: Choroidal hemorrhage and hyperpigmentation around area of indentation.
  34. 34. Macular hole Myopic macular hole may occur, but the exact mechanism is unknown. Whether attenuation of the neural retina and its supportive pigment epithelium and choroid are responsible is speculative.
  35. 35. Various surgical procedures have been performed for macular hole with or without RD and they include :  PPV with gas or silicone oil tamponade  Macular buckling  Scleral shortening surgeries.
  36. 36. Myopic macular chorioretinopathy  DEF: is a rare, genetic eye disorder that causes vision loss.  Grading(shih et al) MO - Normal post pole  M1 - Tesselation & choroidal pallor  M2 - M1+post staphyloma  M3 - M2+lacker cracks  M4 - M3+ focal deep choroidal atrophy  M5 - M4+geographic atrophy, CNV  M3>- myopic maculopathy
  37. 37. Peripheral retinal degenerations & RRD  “Lattice degeneration is a common retinal degeneration.”  1. Epidemiology  8-10% of general population (but 20-40% of RD)  More commonly in moderate myopes and is the most important degeneration directly related to RD  Location: Commonly -temporal superiorly fundus Between equator and ora serrata  2. Pathology  Discontinuity of internal limiting membrane  Atrophy of inner layers of retina  Overlying pocket of liquefied vitreous  Adherence of vitreous to edge of lattice (posterior edge)  Sclerosis of retinal vessels
  38. 38. Lattice degeneration - predispose to RRD Retinal tears - posterior and lateral margins of the lattice degeneration Role of prophylactic Laser photocoagulation: History of RD in the fellow eye Family history of RD Prior to ocular surgeries Symptomatic pt
  39. 39. In eyes with RD, laser photocoagulation alone is insufficient to treat the condition and V-R surgery is required. Surgical modalities for RRD - pneumatic retinopexy, SB surgery with cryopexy, and PPV+BB+EL+ C3F8/ SIO. CLINICAL PEARLS Lattice degeneration both with and without atrophic holes is generally benign and does not require prophylactic treatment, as the complications of treatment are more severe than the natural history of the untreated condition.
  40. 40. Myopic RD • Incidence of RD in general population range between 0.005 and 0.01 % . • RD occurs far more frequently in patients with myopia. • Disease Case-control study Group found that subjects with sepherical equivalent refractive error of -1 to -3 diopters had a fourfold greater risk of RD then a nonmyopic individual. • For refractive errors greater than -3 diopters the risk was tenfold greater  More than half of nontraumatic RRD occurs in myopic eyes.
  41. 41. Syneresis of the central vitreous Traction caused by spontaneous or PVD RETINAL TEAR
  42. 42. CNV in Pathological Myopia Among various lesions associated with high myopia, macular CNV is one of the most vision threatening complications. It develops in around 5 to 10% of eyes with high myopia and is the commonest cause of CNV in young individuals and accounts for around 60% of CNV in young patients aged 50 years or younger. Macular hage ass. with CNV in high myopia
  43. 43. - Develops from laquer cracks. - Smaller, less exudation. - Type 1 (severe myopic degeneration)- Leakage does not extend beyond initial CNVM border- Quiescent scar. - Type2( Minimal degeneration)- Leakage beyond CNVM borders- Fibrovascular scarring.
  44. 44. The mechanism of CNV formation in myopic CNV is still unclear.  A possible explanation includes, certainly, the induced hypoxia in the outer retina, which is a large source of VEGF secretion. Chorioretinal stretching, lacquer crack formation, choroidal thinning, choroidal flow disturbance with reduced flow, choroidal filling delay, RPE and overlying retina atrophy, loss of photoreceptors, all of them can be involved in growth factor release and myopic CNV formation. The role of each of these features and the interconnections between them remain unclear
  45. 45. Treatment of myopic CNV More recently, the use of anti-VEGF agents The most commonly used currently is PDT with verteporfin. A combination therapy of PDT with anti-VEGF agents appears efficacious in the treatment of eyes with CNV secondary to pathological myopia, and may afford better visual outcomes as compared to PDT monotherapy •Laser photocoagulation of …. no longer performed. • Other treatment modalities - Submacular surgery - Macular translocation surgery
  46. 46. Features of choroid in PM  Stretched choroid without additional vasculature  Thinner choroid  Choriocapillaries and larger ch.vessel have decreased lumen  Choriocapillaries have loss of fenestrations  Increased number of vortex veins(>4)  Posterior vortex veins(ciliovaginal veins)  Reduction of choroidal thickness is proportional to age and refractive status  Per dioptermyopia caused 8μm reduction in choroidal thickness  Per decade causing 12-15μm reduction in choroidal thickness  Intrachoroidal cavitation – the expansion of distance between inner wall of sclera and posterior surface of bruch’s membrane  Attenuated choroid to absent choroid – myopic chorioretinal atrophy
  47. 47. Lacquer cracks Spontaneous ruptures in the Bruch's membrane . Small hages may develop within the lacquer cracks. Lacquer cracks predispose - macular CNV Small ingrowth of fibrovascular tissue may also give rise to small elevated pigmented circular lesions and are known as Fuchs‘ spots.
  48. 48. Post. Staphyloma
  49. 49. post. staphyloma (ectasia) Equatorial staphyloma with scleral dehiscence - STQ. Visual loss is most often due to macular involvement of a post. pole staphyloma.
  50. 50. Curtin classified the staphylomas into ten categories. The first five were simpler configurations, while the last five were either more intricate in their configuration
  51. 51. Tesselated Fundus  Hypoplasia of the RPE following axial elongation reduces the pigment, allowing the choroidal vessels to be seen.  Commonly seen in elderly or brunette patients.  May not be associated with any clinical significance
  52. 52. References
  53. 53.  Ohno-Matsui K, Yoshida T, Futagami S, Yasuzumi K, Shimada N, Kojima A, et al. Patchy atrophy and lacquer cracks predispose to the development of CNV in PM. Br J Ophthalmol 2003; 87: 570-573.  Cheung BT, Lai YY, Yuen CY, et al. Results of high-density silicone oil as a tamponade agent in macular hole RD in patients with high myopia. Br J Ophthalmol 2007;91:719-721.  Chinese Medical Journal 2013;126(8):1578-1583  Bhatt N S, Diamond J G, Jalali S, Das T. Choroidal neovascular membrane. Indian J Ophthalmol 1998;46:67-80  Hamelin N, Glacet-Bernard A, Brindeau C, et al. Surgical treatment of subfoveal neovascularization in myopia: macular translocation vs surgical removal. Am J Ophthalmol 2002;133:530-6.  Flower RW. Expanded hypothesis on the mechanism of photodynamic therapy action on CNV. Retina 1999;19:365-69.  Albert & Jakobiec,Principles and Practice of Ophthalmology, Volume 2, Chapter 154 PM P 2023-2027, 3rd ed 2008.  Pathological Myopia, Richard F. Spaide, Kyoko Ohno-Matsui, Lawrence A. Yannuzzi Editors  Kyoko Ohno – Matstui MD, Phd, Muka Moriyama MD, PhD Staphyloma II: Analyses of Morphological Features of Posterior Staphyloma in Pathologic Myopia Analyzed by a Combination of Wide-View Fundus Observation and 3D MRI Analyses Pathological Myopia 2014, pp 177-185
  54. 54.  Pukhrai Rishi, … et al …..Photodynamic monotherapy or combination treatment with intravitreal triamcinolone acetonide, bevacizumab or ranibizumab for choroidal neovascularization associated with pathological myopia.. 2011
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