Retina Review - Part 3 + 4

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Retina Review - Part 3 + 4

  1. 1. Retina Review <ul><li>Part 3 </li></ul>
  2. 2. 50 yo lifelong difficulty with night vision
  3. 3. CSNB <ul><li>What is the primary signal transduction defect in congenital stationary night blindness? </li></ul><ul><ul><li>Failure of communication between photoreceptor and bipolar cell </li></ul></ul><ul><ul><li>Loss of retinal ‘on-responses’ </li></ul></ul><ul><li>What is the most common ERG pattern? </li></ul><ul><ul><li>Negative ERG </li></ul></ul><ul><ul><li>Large a-wave on scotopic response </li></ul></ul><ul><ul><li>Absent or reduced b-wave </li></ul></ul>
  4. 5. Fundus Albipunctatus <ul><li>What is the main symptom of fundus albipunctatus? </li></ul><ul><ul><li>Nyctalopia </li></ul></ul><ul><li>Describe the pathophysiologic defect and findings on testing? </li></ul><ul><ul><li>Disorder of visual pigment regeneration </li></ul></ul><ul><ul><li>Delayed recovery of rhodopsin levels after light exposure </li></ul></ul><ul><ul><li>Prolonged dark adaptation </li></ul></ul><ul><ul><li>Normal ERG after several hours in dark </li></ul></ul>
  5. 6. Night Blinding Disorders <ul><li>What is a variant of RP that has a similar appearance to fundus albipunctatus? </li></ul><ul><ul><li>Retinitis punctata albescens </li></ul></ul><ul><li>How is this entity distinguished from fundus albipunctatus? </li></ul><ul><ul><li>Attenuated vessels </li></ul></ul><ul><ul><li>Severely depressed ERG </li></ul></ul><ul><ul><li>No recovery with dark adaptation </li></ul></ul><ul><ul><li>Progressive (not stationary) </li></ul></ul>
  6. 8. Retinitis Pigmentosa <ul><li>Describe the characteristic fundus appearance of RP </li></ul><ul><ul><li>‘ Waxy pallor’ of the disc </li></ul></ul><ul><ul><li>Attenuated retinal vasculature </li></ul></ul><ul><ul><li>Bone-spicule pigmentation </li></ul></ul><ul><li>What else is seen clinically? </li></ul><ul><ul><li>PSC cataract </li></ul></ul><ul><ul><li>Cystoid macular edema </li></ul></ul><ul><li>What is commonly seen on GVF and ERG? </li></ul><ul><ul><li>ring scotoma </li></ul></ul><ul><ul><li>marked reduction of rod and cone function </li></ul></ul>
  7. 9. Retinitis Pigmentosa <ul><li>Inheritance </li></ul><ul><ul><li>70% recessive </li></ul></ul><ul><ul><li>20% dominant </li></ul></ul><ul><ul><li>10% X-linked </li></ul></ul><ul><li>Variants </li></ul><ul><ul><li>RP sine pigmento </li></ul></ul><ul><ul><li>Retinitis punctata albescens </li></ul></ul><ul><ul><li>LCA </li></ul></ul><ul><ul><li>Ushers </li></ul></ul><ul><li>Rx </li></ul><ul><ul><li>Vitamin A may slow progression </li></ul></ul>
  8. 10. 30 yo 20/200 OU, nystagmus, no medical problems or meds
  9. 12. Cone Dystrophy <ul><li>Describe the symptoms of cone dystrophy </li></ul><ul><ul><li>Progressive central visual loss </li></ul></ul><ul><ul><li>Dyschromatopsia </li></ul></ul><ul><ul><li>Photoaversion </li></ul></ul><ul><li>Describe the clinical findings </li></ul><ul><ul><li>Bull’s eye maculopathy </li></ul></ul><ul><ul><li>Selective loss of cone function on ERG </li></ul></ul>
  10. 13. 25 yo 20/50 OU
  11. 15. Stargardt Disease <ul><li>What is the typical inheritance pattern of Stargardt disease? </li></ul><ul><ul><li>Autosomal recessive </li></ul></ul><ul><li>What is the condition called if there are peripheral flecks with little macular involvement? </li></ul><ul><ul><li>Fundus flavimaculatus </li></ul></ul><ul><li>What is a characteristic pattern on fluorescein angiography? </li></ul><ul><ul><li>Dark choroid </li></ul></ul><ul><li>What is the typical range of visual acuity? </li></ul><ul><ul><li>20/50 to 20/200 </li></ul></ul><ul><ul><li>Most retain 20/70 to 20/100 in at least 1 eye </li></ul></ul>
  12. 16. 40 yo 20/60 OD, 20/40 OS
  13. 19. Best Disease <ul><li>What is the inheritance pattern of Best disease and what chromosome is involved? </li></ul><ul><ul><li>Autosomal dominant </li></ul></ul><ul><ul><li>11 </li></ul></ul><ul><li>What are the fluorescein angiographic findings? </li></ul><ul><ul><li>Blockage by vitelliform material </li></ul></ul><ul><ul><li>Hyperfluorescence in areas of RPE atrophy </li></ul></ul><ul><li>Is the ERG typically abnormal? </li></ul><ul><ul><li>No </li></ul></ul><ul><li>What is the typical level of visual acuity? </li></ul><ul><ul><li>20/40 or better </li></ul></ul>
  14. 20. Best Disease <ul><li>What is the critical diagnostic test and what findings would one expect? </li></ul><ul><ul><li>EOG </li></ul></ul><ul><ul><li>Decreased Arden ratio (<1.5; often 1.1) </li></ul></ul>
  15. 21. 50 yo 20/200 OU
  16. 22. Gyrate Atrophy <ul><li>What is the mode of inheritance of gyrate atrophy? </li></ul><ul><ul><li>Autosomal recessive </li></ul></ul><ul><li>What is the enzymatic defect? </li></ul><ul><ul><li>Ornithine aminotransferase </li></ul></ul><ul><li>What is the typical visual acuity in most patients by age 40? </li></ul><ul><ul><li>20/200 or worse </li></ul></ul><ul><li>What is the pathognomonic serum finding and how can this be addressed? </li></ul><ul><ul><li>Elevated ornithine </li></ul></ul><ul><ul><li>Arginine restriction </li></ul></ul>
  17. 23. 30 yo male 20/200 OU
  18. 24. Choroideremia <ul><li>What is the mode of inheritance of choroideremia? </li></ul><ul><ul><li>X-linked recessive </li></ul></ul><ul><li>What is the typical visual acuity by age 50? </li></ul><ul><ul><li>20/200 or worse </li></ul></ul><ul><li>What are the ERG findings of affected males and female carriers? </li></ul><ul><ul><li>Males: subnormal early; extinguished late </li></ul></ul><ul><ul><li>Females: normal </li></ul></ul><ul><li>For what enzyme does the abnormal gene encode? </li></ul><ul><ul><li>Rab geranylgeranyl transferase </li></ul></ul>
  19. 25. 20 yo male 20/40 OU
  20. 26. Juvenile Retinoschisis <ul><li>What is the mode of inheritance? </li></ul><ul><ul><li>X-linked recessive </li></ul></ul><ul><li>What layer is schisis supposed to occur? </li></ul><ul><ul><li>Nerve fiber layer </li></ul></ul><ul><li>FA leakage? </li></ul><ul><ul><li>No </li></ul></ul><ul><li>ERG? </li></ul><ul><ul><li>Loss of b wave (negative) </li></ul></ul>
  21. 30. Bardet-Biedl Complex <ul><li>Describe the clinical findings in Bardet-Biedl syndrome </li></ul><ul><ul><li>Obesity </li></ul></ul><ul><ul><li>Polydactyly </li></ul></ul><ul><ul><li>Hypogonadism </li></ul></ul><ul><ul><li>Mental retardation </li></ul></ul><ul><ul><li>Pigmentary retinopathy </li></ul></ul><ul><li>What is the mode of inheritance </li></ul><ul><ul><li>Autosomal recessive </li></ul></ul><ul><li>How is the ERG affected? </li></ul><ul><ul><li>Undetectable or severely impaired </li></ul></ul>
  22. 31. 1 mo old baby
  23. 32. Tay-Sachs Disease <ul><li>What is the enzyme deficiency in Tay-Sachs disease? </li></ul><ul><ul><li>Hexosaminidase A </li></ul></ul><ul><li>Explain the pathophysiology </li></ul><ul><ul><li>Glycolipid accumulation in the brain and retina leads to mental retardation and blindness </li></ul></ul><ul><ul><li>Death occurs between 2 and 5 years </li></ul></ul>
  24. 36. Bietti’s crystalline retinopathy <ul><li>What is mode of inheritance? </li></ul><ul><ul><li>Autosomal recessive </li></ul></ul><ul><li>Clinical findings? </li></ul><ul><ul><li>Pigmentary and crystalline retinopathy </li></ul></ul><ul><ul><li>Corneal crystals </li></ul></ul><ul><ul><li>Progressive decrease in vision starting in 40s </li></ul></ul><ul><li>Treatment? </li></ul><ul><ul><li>No effective treatment </li></ul></ul>
  25. 40. Medications <ul><li>Crystals? </li></ul><ul><ul><li>Tamoxifen </li></ul></ul><ul><ul><li>Canthoxanthine </li></ul></ul><ul><ul><li>Talc </li></ul></ul><ul><li>Pigmentary retinopathy? </li></ul><ul><ul><li>Thorazine </li></ul></ul><ul><ul><li>Mellaril </li></ul></ul><ul><li>Bullseye? </li></ul><ul><ul><li>Plaquenil (6.5 mg/kg/d) </li></ul></ul><ul><ul><li>Chloroquine (250 mg/d) </li></ul></ul>
  26. 41. 20 yo hit in OS
  27. 42. Day One Post-injury One Month Post-Injury
  28. 43. Commotio Retinae <ul><li>Describe the pathophysiology </li></ul><ul><ul><li>Damage to outer retinal layers caused by shock waves </li></ul></ul><ul><ul><li>Retinal whitening within hours of injury </li></ul></ul><ul><ul><li>Proposed mechanisms: </li></ul></ul><ul><ul><ul><li>Extracellular edema </li></ul></ul></ul><ul><ul><ul><li>Glial swelling </li></ul></ul></ul><ul><ul><ul><li>Photoreceptor damage/disorganization of outer segments </li></ul></ul></ul><ul><li>What is the typical time for resolution? </li></ul><ul><ul><li>3 to 4 weeks </li></ul></ul><ul><li>What are 2 causes for limited visual recovery? </li></ul><ul><ul><li>Macular pigment epitheliopathy </li></ul></ul><ul><ul><li>Macular hole formation </li></ul></ul>
  29. 45. 18 yo s/p trauma to right eye
  30. 48. Choroidal Rupture <ul><li>Describe the mechanism </li></ul><ul><ul><li>Anterior-posterior compression </li></ul></ul><ul><ul><li>Stretching in horizontal axis </li></ul></ul><ul><ul><li>Tearing of Bruchs membrane along with RPE and choriocapillaris </li></ul></ul><ul><li>What is the most visually threatening complication? </li></ul><ul><ul><li>Choroidal neovascularization </li></ul></ul>
  31. 49. 16 yo shot with BB
  32. 50. Intraocular Foreign Body <ul><li>What is caused by retained copper foreign bodies? </li></ul><ul><ul><li>Chalcosis </li></ul></ul><ul><li>What are the findings of chalcosis? </li></ul><ul><ul><li>Deposits in Descemets membrane </li></ul></ul><ul><ul><li>Greenish aqueous particles </li></ul></ul><ul><ul><li>Green iris discoloration </li></ul></ul><ul><ul><li>Sunflower cataract </li></ul></ul><ul><ul><li>Vitreous opacities </li></ul></ul><ul><ul><li>Metallic retinal flecks </li></ul></ul>
  33. 51. Intraocular Foreign Body <ul><li>What condition is caused by retained iron foreign bodies? </li></ul><ul><ul><li>Siderosis bulbi </li></ul></ul><ul><li>Describe the findings? </li></ul><ul><ul><li>Corneal stromal staining </li></ul></ul><ul><ul><li>Iris heterochromia </li></ul></ul><ul><ul><li>Pupillary mydriasis & poor reactivity </li></ul></ul><ul><ul><li>Anterior lens deposits and cataract </li></ul></ul><ul><ul><li>Peripheral and diffuse retinal pigmentation </li></ul></ul><ul><ul><li>Narrowed vessels </li></ul></ul><ul><ul><li>Optic atrophy </li></ul></ul><ul><li>What are ERG findings? </li></ul><ul><ul><li>Increased a-wave and normal b-wave early </li></ul></ul><ul><ul><li>Progressive diminution of b-wave amplitude </li></ul></ul>
  34. 52. 40 yo s/p ruptured globe OS; OD 20/40, AC cell
  35. 53. Sympathetic Ophthalmia <ul><li>What is the reported range of time interval from initial injury to development of SO? </li></ul><ul><ul><li>5 days to 66 years </li></ul></ul><ul><li>What uveitic entity most closely resembles SO and how can they be differentiated? </li></ul><ul><ul><li>VKH </li></ul></ul><ul><ul><li>History of ocular trauma or multiple surgical interventions </li></ul></ul><ul><ul><li>VKH involves choriocapillaris; SO spares it </li></ul></ul>
  36. 54. Sympathetic Ophthalmia <ul><li>What are possible extraocular manifestations shared with VKH? </li></ul><ul><ul><li>Vitiligo </li></ul></ul><ul><ul><li>Poliosis </li></ul></ul><ul><ul><li>Alopecia </li></ul></ul><ul><ul><li>Dysacusis </li></ul></ul><ul><ul><li>Meningeal irritation </li></ul></ul><ul><ul><li>CSF pleocytosis </li></ul></ul><ul><li>What are the fundus lesions called and how do they appear on fluorescein angiography? </li></ul><ul><ul><li>Dalen-Fuchs nodules </li></ul></ul><ul><ul><li>Early hypofluorescence with late hyperfluorescence </li></ul></ul>
  37. 55. Sympathetic Ophthalmia <ul><li>What is the treatment for SO? </li></ul><ul><ul><li>Corticosteroids </li></ul></ul><ul><ul><li>Cyclosporine A </li></ul></ul><ul><ul><li>Azathioprine/Methotrexate </li></ul></ul>
  38. 56. 3 mo old; consult from PICU
  39. 57. Shaken Baby Syndrome <ul><li>What are the ocular signs? </li></ul><ul><ul><li>Retinal hemorrhages </li></ul></ul><ul><ul><li>Cotton wool spots </li></ul></ul><ul><ul><li>Retinal folds </li></ul></ul><ul><ul><li>Schisis cavities </li></ul></ul><ul><li>What disorders can lead to hemorrhage in the vitreous cavity, sub-ILM space, subretinal space, and sub-RPE space? </li></ul><ul><ul><li>Shaken baby syndrome </li></ul></ul><ul><ul><li>Trauma </li></ul></ul><ul><ul><li>Exudative AMD </li></ul></ul><ul><ul><li>Retinal arterial macroaneurysm </li></ul></ul>
  40. 58. 20 yo s/p MVA; NLP OD
  41. 62. Valsalva Retinopathy <ul><li>Describe the pathophysiology </li></ul><ul><ul><li>Sudden rise in intra-abdominal pressure (cough, etc) </li></ul></ul><ul><ul><li>Corresponding rise in intraocular venous pressure </li></ul></ul><ul><ul><li>Rupture of macular capillaries </li></ul></ul><ul><ul><li>Hemorrhage </li></ul></ul><ul><li>What is the typical location of hemorrhage? </li></ul><ul><ul><li>Sub-ILM </li></ul></ul><ul><li>What is the typical visual prognosis? </li></ul><ul><ul><li>Excellent </li></ul></ul><ul><ul><li>Spontaneous resolution within months </li></ul></ul>
  42. 65. Purtschers Retinopathy <ul><li>What are the etiologies? </li></ul><ul><ul><li>Acute compression injury to thorax or head </li></ul></ul><ul><ul><li>Acute pancreatitis </li></ul></ul><ul><ul><li>Fractures </li></ul></ul><ul><li>Describe the pathophysiology </li></ul><ul><ul><li>Activation of complement with granulocyte aggregation and leukoembolization </li></ul></ul><ul><ul><li>Complement-mediated leukostasis and obstruction </li></ul></ul><ul><ul><li>Formation of cotton wool spots, hemorrhage, & edema </li></ul></ul><ul><ul><li>Fat embolization for fractures </li></ul></ul><ul><li>What is seen on fluorescein angiography? </li></ul><ul><ul><li>Arteriolar obstruction </li></ul></ul><ul><ul><li>Vascular leakage </li></ul></ul>

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