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Ectopia lentis and its low vision management

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Ectopia lentis and its low vision management

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Ectopia lentis and its low vision management

  1. 1. ECTOPIA LENTIS AND ITS LOW VISION MANAGMENT Indra P Sharma Master of Clinical Optometry (Year I) Amity Medical School
  2. 2. Objective  To have a better understanding about ectopia lentis with regard to its pathophysiology, etiology, clinical manifestation and management.  To understand the management of ectopia lentis with low vision aids
  3. 3. Contents 1. An overview 2. Signs and symptoms 3. Etiology of ectopia lentis 4. Workup and evaluation 5. Treatment 6. Low Vision Management 7. Conculsion 8. Reference
  4. 4. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference An Overview
  5. 5. Introduction  Ectopia lentis is defined as displacement or malposition of the crystalline lens of the eye.  1749 - Berryat first reported case of lens dislocation  1856- Stellwag coined the term “ectopia lentis”  Ectopia lentis can be : 1. Subluxation 2. Luxation (dislocated) Sharma IP
  6. 6. Subluxation  Condition when the crystalline lens is partially displaced but contained within the lens space.  Few zonular attachment present Sharma IP
  7. 7. Dislocation  Also known as luxated  When lens lies completely outside the lens patellar fossa, in the anterior chamber, free-floating in the vitreous, or directly on the retina. Sharma IP
  8. 8. Anatomy of zonules  The lens is suspended in its anatomic position by ciliary zonules (zonules of Zinn or suspensory ligament of Zinn)  Zonules fibers which run from ciliary body and insert into the outer layer of the lens capsule around the equator (1.5 mm anteriorly and 1mm posterior)  Stronger zonules in anterior capsule  Each zonule measures 5 to 30μm in diameter and is composed of bundles of microfibrils Sharma IP
  9. 9. Pathophysiology  Underlying pathophysiology - Disruption or dysfunction of the zonular fibers of the lens, regardless of cause (trauma or heritable condition)  The degree of zonular impairment determines the degree of lens Sharma IP
  10. 10. Epidemiology Frequency  A rare condition.  Incidence in the general population is unknown. Mortality/Morbidity  Can cause marked visual disturbance, depending the degree of lens displacement and the underlying etiology Sex  More common in males. Age  At any age (At birth or it may manifest late in life) Sharma IP
  11. 11. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Signs and Symptoms Sharma IP
  12. 12. Symptoms  Distance visual acuity (moderate to severe impairment) depending on position of lens 1. Fluctuating vision dramatically as the vision may alternate between phakia and aphakic 2.Progressive movement of the lens- Extreme hyperopic shift or myopic astigmatism  Poor near vision Due to loss of accommodative power due to weakened, stretched or broken zonules Sharma IP
  13. 13. Contd...  Monocular diplopia  Visual field defects (Due to lens induced secondary glaucoma) A 1963 histological study found that crystalline lenses that were dislocated anteriorly were associated with glaucoma 77.2% of the time and that crystalline lenses that were subluxed or dislocated Source: Sihota R Sood NN, Argarwal He. 1995. Traumatic glaucoma.Acta Ophthalmol Scand posteriorly were associated with glaucoma 87.5% of the time. 73:252-254. Sharma IP
  14. 14. Contd....  Painful red eye (secondary to trauma)  Glare and photophobia Sharma IP
  15. 15. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Etiology of ectopia lentis Sharma IP
  16. 16. Traumatic dislocation  Traumatic dislocation is most common cause of ectopia lentis. Sharma IP
  17. 17. Hereditary ectopia lentis without systemic manifestations Single (isolated) ectopia lentis  Autosomal dominant inheritance  Genetic defect located on chromosome 15, causing a dysfunctional zonular apparatus.  Microspherophakia is common.  Present at birth, can even onset late  Typically, supertemporal displacement Sharma IP
  18. 18. Ectopia lentis et pupillae  Usually is bilateral and typically autosomal recessive.  characterized by asymmetric eccentric pupils that are displaced in the opposite direction of the lens dislocation (toward the most dysfunctional zonular fibers).  The irides often appear atrophic with transillumination defects  Cataracts (common) Sharma IP
  19. 19. Pupil may be displaced in opposite Single (isolated) ectopia lentis direction (ectopia lentis et pupillae) Sharma IP
  20. 20. Systemic conditions associated with ectopia lentis  Marfan syndrome (most frequent cause of hereditary ectopia lentis)  Homocystinuria (second most common cause of hereditary ectopia lentis)  Weil-Marchesani syndrome  Sulfite oxidase  Hyperlysinemia Sharma IP
  21. 21. Marfan syndrome  Transmitted as an autosomal dominant trait  Prevalence: approx. 5 per 100,000.  Mutations involving the fibrillin gene on chromosomes 15 and 21 and may relate to incompetent zonular fibers. Sharma IP
  22. 22. Systemic association of Marfan • Limb-trunk • disproportion • Arachnodactyly • Pectus excavatum • High-arched palate • Aortic dilatation, dissection and regurgitation • Mitral valve prolapse Sharma IP
  23. 23. Ocular manifestation  Superio-temporal dislocation of a lens in 80% of cases  Zonules usually intact  Blue sclera Sharma IP
  24. 24. Contd..  Axial myopia  Lattice degeneration  Cornea plana  Angle anomaly and glaucoma Sharma IP
  25. 25. Homocystinuria  It is an inborn error of metabolism.  Most often caused by a deficiency of cystathionine b-synthetase (the enzyme that converts homocysteine to cystathionine). Sharma IP
  26. 26. Systemic and ocular manifestation  Malar flush and fine fair hair  Marfanoid habaitus  Increased platelet stickiness  Mental retardation-50%  Inferonasal lens subluxation -90%  Disintegretation of zonules Sharma IP
  27. 27. Weil-Marchesani Syndrome  Rare syndrome characterized by skeletal malformations  The inheritance pattern is not well understood.  Pupillary block glaucoma is common; therefore, prophylactic laser peripheral iridotomies are recommended. Sharma IP
  28. 28. Systemic and ocular manifestation  Short stature  Short stubby fingers (brachydactyly)  Mental handicap  Microspherophakia (most common)  Anterior lens subluxation (usually) Sharma IP
  29. 29. Sulfite oxidase deficiency  Extremely rare disorder caused by a defect in sulfur metabolism. Salient features are:  Progressive CNS abnormalities that develop within the first year of life  Ectopia lentis. Sharma IP
  30. 30. Hyperlysinemia  Extremely rare autosomal recessive enzymatic defect of amino acid metabolism Salient features:  Mental retardation and lens dislocation.  Diagnosis is made by demonstration of increased plasma levels of lysine. Sharma IP
  31. 31. Primary ocular disorders associated  Congenital glaucoma/buphthalmos  Pseudoexfoliation syndrome  Syphilis/chronic uveitis  Retinitis pigmentosa  Hypermature cataract  Intraocular tumor  High myopia Sharma IP
  32. 32. Systemic diseases rarely associated  Ehlers-Danlos syndrome  Crouzon disease  Refsum syndrome  Kniest syndrome  Mandibulofacial dysostosis  Sturge-Weber syndrome  Conradi syndrome  Pfaundler syndrome  Pierre Robin syndrome  Wildervanck syndrome  Sprengel deformity Sharma IP
  33. 33. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Workup and evaluation Sharma IP
  34. 34. Detailed history History of ocular trauma. History investigating possible systemic disease associations.  Cardiovascular disease in Marfan syndrome  Skeletal problems in Marfan syndrome,Weil-Marchesani syndrome or homocystinuria Pertinent family history  Consanguinity  Mental retardation  Unexplained deaths at young age Sharma IP
  35. 35. Ophthalmic evaluation Visual acuity  Amblyopia is a common cause of decreased vision in congenital ectopia lentis. External ocular examination  Orbital anatomy for hereditary malformations (eg, enophthalmos with facial myopathic appearance seen in Marfan syndrome).  Measure corneal diameter (megalocornea in Marfan syndrome).  Strabismus is common (secondary to amblyopia). Sharma IP
  36. 36. Contd.. Retinoscopy and and keratometry  Careful retinoscopy and refraction is essential, often revealing myopia with astigmatism.  Keratometry may help ascertain degree of corneal astigmatism. Slit lamp examination  Evaluate lens position, and identify phacodonesis or cataract.  Measure intraocular pressure. Dilated fundus examination  To rule out retinal detachment Sharma IP
  37. 37. Laboratory studies  Perform appropriate diagnostic and laboratory evaluation, if a hereditary condition is suspected  Cardiac evaluation for Marfan syndrome  Check serum and urine levels of homocysteine or methionine for homocystinuria. Sharma IP
  38. 38. Imaging studies A scan  Axial length measurement may be of benefit (patients with Marfan syndrome have large globes) B scan  For any other associated vitrous and retinal complications Sharma IP
  39. 39. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Treatment Sharma IP
  40. 40. Refractive management The key to managing lens subluxation optically is to perform two refractions:  1.For that portion of the pupil covered by the crystalline lens (myopic astigmatism zone) and  2. For that portion of the pupil not covered by the crystalline lens (highly hyperopic zone). Sharma IP
  41. 41. Contd...  For moderate to severe subluxation Option: An aphakic prescription with bifocals and a pharmacologic dilation  For lens is subluxed out of the visual axis (bilateral) Best option: An aphakic prescription  Both spectacle and contact lens may be helpful. Sharma IP
  42. 42. Medical management  If no, history of trauma, patients may possess a systemic disease with potentially deleterious effects; therefore, comanagement with pediatrician or internist is essential.  Dietary restriction: partially effective in homocystinuria. Sharma IP
  43. 43. Contd...  Treatment of glaucoma is dependent on the etiologic mechanism.  Pupillary block requires laser peripheral iridotomy or iridectomy and raised IOP treated medically.  Prophylactic laser iridotomy in microspherophakia.  Dislodged lens into the AC is initially pharmacological (mydriasis/cycloplegia) in conjunction with ocular massage through a closed lid.  Treatment of a dislocated lens in the vitreous is Sharma IP surgical.
  44. 44. Sugical treatment 1.Nd:YAG laser zonulysis  to displace lens out of visual axis 2.Lesectomy Lensectomy is technically challenging and are indication in the following:  Lens in the anterior chamber  Lens-induced uveitis  Lens-induced glaucoma  Lenticular opacity with poor visual function  Anisometropia or refractive error not amenable to optical correction (eg, in a child to prevent amblyopia)  Impending dislocation of the lens Sharma IP
  45. 45. Prevention and prognsis Deterrence/Prevention  Early diagnosis of ectopia lentis with appropriate optical correction can prevent amblyopia. Complications  The most common ocular complications include amblyopia, uveitis, glaucoma, and retinal detachment Prognosis  Depending on the degree of lens dislocation, the age of onset, and its associated secondary complications, most patients do well.  Trauma-associated ectopia lentis – poor visual prognosis Sharma IP
  46. 46. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Low vision management Sharma IP
  47. 47. Rule of thumb  Provide LVA to help releive the main symptoms first Sharma IP
  48. 48. Distance viewing devices Why do we need it?  To compensate for high hypermetropia or myopic astigmatism Sharma IP
  49. 49. High power lenses  High plus spectacle (aspheric lenses)  Aphakic contact lens (Silsoft/silsoft super plus) Sharma IP
  50. 50. Roving Ring Scotoma  Circular restriction in the peripheral VF due to prismatic effect of high plus lenses.  Aspheric lenses reduces this scotoma Sharma IP
  51. 51. Telescopes Sharma IP
  52. 52. Sharma IP
  53. 53. Near optical devices Why is it required?  To compensate for high hyperopia and loss of accommodation  Preferable near viewing devices 1. Spectacle-mounted Reading Lenses 2. Telemicroscopes 3. Magnifiers 4. Electronic Devices- CCTV Sharma IP
  54. 54. Spetacle mounted reading glasses Sharma IP
  55. 55. Telemicroscope  Eyeglass mounted microscopes can be made to focus at any working distance. Sharma IP
  56. 56. Magnifiers  Illuminated and non-illuminated  Hand held and stand magnifiers Sharma IP
  57. 57. Electronic magnification devices  Head mounted video display systems  CCTV Sharma IP
  58. 58. Visual field enhancing devices  Reverse telescope To compensate for VF defects due to secondary Glaucoma.  Prisms  Mirrors  Reverse Telescope system Sharma IP
  59. 59. Non-optical aids  Relative size and larger assistive devices  Glare, contrast, and lighting control devices  Handwriting and written communication devices  Medical management devices  Orientation and mobility management techniques and devices Sharma IP
  60. 60. Relative size and larger assistive devices  Large print  Books, magazines, newspaper, dictionaries, thesauri, atlas, cookbooks, encyclopedias, bibles  Photocopy machines ( e.g. A4 size to A3 size)  Computer with large prints font  Computer software program  Large print typewriters  Others  Telephone dial, bank checks, watches, clocks, calculators etc  Games like cross word puzzles, playing cards, chess checkers, bingo etc. Sharma IP
  61. 61.  Magic 8.0  Large print telephones Sharma IP
  62. 62.  Large print calculator  Playing cards Sharma IP
  63. 63. Glare, contrast, and lighting control devices Aphakia allows for increased transmission of UV radiation  Shades  Tennis shades, caps, hats, side-shields, visors  Filters  NoIR and CPF lenses Sharma IP
  64. 64.  Sunglasses/ photochromatic lenses to reduce illumination levels Sharma IP
  65. 65. Typoscopes – a reading guide cut off glare Glare cutter ( 390-410nm)- cuts 100% UVB, 99% UVA Sharma IP
  66. 66. Diplopia eliminating devices  Stenopeic slit  Aperture control contact lens  Pinhole lenses Sharma IP
  67. 67. Color and contrast adjustments Environmental modification Painted edges of Staircase Sharma IP
  68. 68.  Contrast in kitchen Sharma IP
  69. 69. Hand writing and written communication  Signature guide  Check guides  Reading guide  Large prints  Bold felt-tip pens  Bold line paper  Large print typewriters Sharma IP
  70. 70. Medical management devices  Monitoring blood glucose  Large print syringe  Syringe with magnifiers ( optical device)  Preset dosage  Pre-filled syringes- filled with clicking sounds  Monitoring blood pressure  Readout sphygmomanometers for hearing problems  Monitoring temperatures  Large readout thermometers and talking thermometer Sharma IP
  71. 71.  Talking colour detector IPS Sharma IP
  72. 72. Counselling  If a hereditary condition is discovered, appropriate genetic counseling recommended.  All relatives with potential risk should be examined.  Advised against playing contact sports or doing physically strenous activities. Sharma IP
  73. 73. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Conclusion Sharma IP
  74. 74. Take home message  Managing patients with ectopia lentis including refraction is usually difficult and needs expertise to manage them well.  As most patients with ectopia lentis are syndromic, co-management with other specialist is essential.  While managing patient with low vision we need to looks at various cause and other ocular association like glaucoma, retinal detachment and amblyopia.  An optometrist needs to understand the need of the patient and prescribe according. Sharma IP
  75. 75. An overview Signs and Symptoms Etiology of ectopia lentis Workup and evaluation Treatment Low Vision Management Conculsion Reference Reference Sharma IP
  76. 76. Reference Books  Jonathan A Jackson, Low Vision Mannual,2007, Butterworth Heineman Elsevier.  William J Benjamin,2006, Borish’s Clinical Refraction, Butterworth Heineman Elsevier. 20: 816-829  Lighthouse International, The Lighthouse Clinician’s Guide to Low Vision Practice  Brilliant Richard L, 1999, Essentials of Low vision Practice, Butterworth Heineman Elsevier. 6.8.9.10 Websites  www.emedicine.medscape.com  www.rootatlas.com  en.wikipedia.org  www.google.com/imghp Sharma IP
  77. 77. Tashi Delek Sharma IP

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