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Ectopia lentis edit

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Ectopia Lentis edit

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Ectopia lentis edit

  1. 1. ECTOPIA LENTIS Presenter: Dr. Rujuta Gore Moderator: Dr. Suhas Haldipurkar Dr. Prakash Chipade
  2. 2. Introduction  Ectopia lentis is defined as displacement or malposition of the crystalline lens of the eye  Berryat described the first reported case of lens dislocation in 1749, and Stellwag subsequently coined the term “ectopia lentis” in 1856
  3. 3. Topographic Classification Subluxated lens Dislocated lens • Incarcerated in the pupil • In the anterior chamber • In the vitreous- lens nutans • Lens fixata • In the subretinal space • Wandering lens • Extrusion out of the globe • In subconjunctival space
  4. 4. Etiologic Classification Etiology Congenital Metabolic Disorder Traumatic Consecutive or Spontaneous
  5. 5. Congenital Ectopia Lentis  Usually bilateral and symmetrical Simple Ectopia Lentis Ectopia Lentis et Pupillae
  6. 6. Congenital Ectopia Lentis  SIMPLE ECTOPIA LENTIS  Autosomal dominant inheritance  Ocular anomaly: Bilateral, symmetric, upward and temporal displacement of the lens.  Herniation of the vitreous associated with zonular degeneration may occur through the zonular defect into the anterior chamber  Associated with cataract and retinal detachment
  7. 7. Congenital Ectopia Lentis  ECTOPA LENTIS ET PUPILLAE  Autosomal recessive inheritance  Pupils are oval or slit shaped and ectopic, and they frequently dilate poorly  Bilateral, with the lenses and pupils displaced in the opposite direction from each other.  Associated ocular anomalies- Megalocornea, polycoria, cataract, glaucoma, retinal detachment, optic nerve hypoplasia, colobomata
  8. 8.  Pathogenesis of ectopia lentis et pupillae:  Mesodermal- persistent remnants of tunica vasculosa lentis mechanically interfere with the development of zonules  Neuroectodermal- Maldevelopment of the pigmentary epithelium of the iris, hypoplasia of the dilator muscle
  9. 9. Metabolic Disorders  MARFAN SYNDROME  Prevalence of approximately 5 per 100,000  Autosomal dominant condition characterized by skeletal, cardiovascular, and ocular anomalies.  Several point mutations involving the fibrillin gene on chromosomes 15 and 21 - relate to incompetent zonular fibers
  10. 10. MARFAN SYNDROME  Salient features:  Skeletal Manifestations  Tall stature  Increased arm span in relation to body height and elongated lower segment  Arachnodactyly  Joint laxity, scoliosis
  11. 11. MARFAN SYNDROME  Cardiovascular Manifestations  Mitral valve prolapse, aortic dilatation
  12. 12. MARFAN SYNDROME  Ocular Manifestations  Axial myopia  Corneal diameter may be increased, giving the appearance of megalocornea  Increased incidence of retinal degeneration & detachment  Lens subluxation occurs in about 75% of patients; usually is bilateral, symmetrical, and superotemporal
  13. 13. HOMOCYSTINURIA  Inborn error of metabolism of sulfur containing amino acids  Near absence of cystathionine b-synthetase (the enzyme that converts homocysteine to cystathionine)  Salient features:  Fair skin with coarse hair  Osteoporosis  Mental retardation (nearly 50%)  Seizure disorder  Marfanoid habitus
  14. 14. HOMOCYSTINURIA  Salient features continued:  Poor circulation - Thromboembolic events constitute the major threat to survival, especially following general anesthesia  Lens luxation usually is bilateral, symmetrical, and inferonasal, and presents in nearly 90% of patients
  15. 15. WEIL-MARCHESANI SYNDROME  Salient features:  Short stature  Brachycephaly  Limited joint mobility  Ectopia lentis  Microspherophakia (most prominent feature of this syndrome)  Lenticular myopia  Lens subluxation occurs inferiorly, often progressing to complete dislocation  Pupillary block glaucoma is common
  16. 16. SULFITE OXIDASE DEFICIENCY  Defect in sulfur metabolism  Salient features:  Progressive CNS abnormalities that develop within the first year of life  Ectopia lentis.
  17. 17. HYPERLYSINEMIA  Autosomal recessive enzymatic defect of amino acid metabolism  Characterized by mental retardation and lens dislocation.
  18. 18. Rare Systemic Associations  Ehlers-Danlos syndrome  Crouzon disease  Refsum syndrome  Kniest syndrome  Mandibulofacial dysostosis  Sturge-Weber syndrome
  19. 19. Traumatic Ectopia Lentis  Mechanism-  Backward thrust and rebounding of the lens  Pressure wave of the aqueous forcing the root of iris backwards  Forcible recoil of the vitreous body which comes forward around the lens
  20. 20. Consecutive/Spontaneous Ectopia Lentis  Mechanical stretching  Buphthalmos  High myopia  Staphyloma  Intraocular Tumors  Inflammatory  Destruction of zonules  Hypermature cataract  Cyclitic adhesions  Vitreous traction bands
  21. 21. Presentation  Common presenting symptoms include the following:  Decreased distance visual acuity (secondary to astigmatism or lenticular myopia)  Poor near vision (loss of accommodative power)  Monocular diplopia  Red painful eye
  22. 22. Presentation  History:  Onset  Cardiovascular disease (Marfan syndrome)  Skeletal problems (Marfan syndrome, Weil-Marchesani syndrome, or homocystinuria)  Ocular trauma  Family history  Consanguinity  Mental retardation  Unexplained deaths at young age (eg, autosomal recessive conditions, including homocystinuria, hyperlysinemia, ectopia lentis et pupillae, or sulfite oxidase deficiency)
  23. 23. Ocular Examination  Visual Acuity  Ectopia lentis is potentially visually debilitating.  Visual acuity varies with the degree of malpositioning of the lens.  Amblyopia is a common cause of decreased vision in congenital ectopia lentis.
  24. 24. Ocular Examination  Retinoscopy and refraction  Edge of the dislocated lens may be identified in the pupillary space.  Careful retinoscopy and refraction is essential, often revealing myopia with astigmatism.  Keratometry may help ascertain degree of corneal astigmatism.
  25. 25. Ocular Examination  Signs  Megalocornea  Anterior chamber-  Irregular  Flat  Cells and flare- uveitis  Vitreous- present/ absent  Angle of AC  Recession seen in trauma  Presence or absence of synechiae
  26. 26. Ocular Examination  Iris- iridodonesis, transillumination  Appearance of the pupil  assess the adequacy of the pupillary space for a possible aphakic correction  Lens-  Position  Phacodonesis  Cataract  Zonules- stretched or broken
  27. 27. Ocular Examination  Complete dislocation:  Posterior dislocation  Aphakia  Lens seen in vitreous cavity  Anterior dislocation:  Clear lens- oil droplet appearance with golden lustre of rim  Cataractous lens- white disc in AC  Iridocyclitis  Endothelial damage  Secondary glaucoma
  28. 28. Ocular Examination  Raised intraocular pressure  Causes of glaucoma in ectopia lentis include the following:  (1) pupillary block  (2) phacoanaphylaxis or phacolytic  (3) posttraumatic angle recession  (4) poorly developed angle structures  Dilated fundus examination: Rule out retinal detachment
  29. 29. Ocular Associations  Persistent pupillary membrane  Aniridia  Rieger’s syndrome  Dominantly inherited blepharoptosis  High myopia  Congenital glaucoma/buphthalmos  Pseudoexfoliation  Retinitis pigmentosa  Hypermature cataract  Intraocular tumor
  30. 30. Complications Complications Glaucoma Uveitis Corneal Decompensation Amblyopia Retinal Detachment
  31. 31. Workup  Cardiac evaluation for Marfan syndrome  Serum and urine levels of homocysteine or methionine for homocystinuria  Axial length measurement
  32. 32. Indications for Surgery  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
  33. 33. Management Protocol Subluxated Clear Lens Minimal Spectacle correction through phakic portion Marked 1. Spectacle correction through aphakic portion 2. Optical iridectomy 3. Miotics/Mydriatics Surgical Removal Pars Plana 1. Vitrectomy probe 2. Phacofragmentome Limbal Route Phacoaspiration with help of iris hooks + PCIOL/ Iris clip/ SFIOL
  34. 34. Management Protocol Subluxated Cataractous Lens Minimal ECCE/ Phacoemulsificat ion + IOL Marked Pars Plana Route 1. Lensectomy 2. Phacofragmentation Limbal Route Phacoemulsification with help of iris hooks + PCIOL/ Iris clip/ SFIOL
  35. 35. Management Protocol Subluxated Lens- Surgical Management Subluxation <3 clock hours Slow Phaco + PCIOL Pars Plana Lensectomy + Pars Plana Vitrectomy + SFIOL/ Iris clip Subluxation 3-5 clock hours Slow Phaco with CTR/ Cionni ring + PCIOL Pars Plana Lensectomy + Pars Plana Vitrectomy + SFIOL/ Iris clip Subluxation 5-7 clock hours Slow Phaco + Cionni fixation of bag/ Ahmed segment + Capsular retractors + PCIOL Pars Plana Lensectomy + Pars Plana Vitrectomy + SFIOL/ Iris clip Subluxation >7 clock hours Pars Plana Lensectomy + Pars Plana Vitrectomy + SFIOL ECCE + Anterior vitrectomy + ACIOL/SFIOL/ Iris clip
  36. 36. Management Protocol Dislocated Lens Anterior Removal through limbal approach Posterior Mobile Complications: 1. Glaucoma 2. Inflammation 3. Obstruction in visual axis Surgical removal with complete vitrectomy Vitrectomy cutter Phacofragmentation PFCL floatation- limbal delivery Fixed
  37. 37. Management of Subluxated lens in Children  Treatment of aphakia in children is a challenge  Spectacle correction is not suitable for unilateral aphakia due to anisokenia  Contact lens - keratitis, corneal neovascularization, noncompliance, frequent lens change and cost  Surgical methods - implantation of ACIOL/SFIOL  ACIOL not preferred in the pediatric age group (corneal decompensation, glaucoma and retinal detachments)  Recently published studies reported late dislocation of IOL due to breakage of polypropylene sutures SFIOL, especially in young patients Vote BJ, Tranos P, Bunce C, Charteris DG, Da Cruz L. Long-term outcome of combined pars plana vitrectomy and scleral fixated sutured posterior chamber intraocular lens implantation. Am J Ophthalmol. 2006 Feb; 141(2):308-312. Assia EI, Nemet A, Sachs D. Bilateral spontaneous subluxation of scleral-fixated intraocular lenses. J Cataract Refract Surg. 2002 Dec; 28(12):2214-6.
  38. 38. Management of Subluxated lens in Children  Phacoaspiraton with the help of CTR is a safe and predictable procedure in subluxated lens in children  Elimination of PCO still remains a challenge  Long-term follow-up to observe any change in the stability of capsular bag-zonular complex is necessary Pranab Das, Jagat Ram, Gagandeep Singh Brar, and Mangat R Dogra. Results of intraocular lens implantation with capsular tension ring in subluxated crystalline or cataractous lenses in children. Indian J Ophthalmol. 2009 Nov- Dec; 57(6): 431–436.
  39. 39. Management of Subluxated lens in Children  Key points to successful CTR implantation  To use high-viscosity visco-elastic material  Making the incision at a meridian with intact zonules  To avoid damaging zonular fibers with the movement of the phacotip  Perform slow-motion phacoemulsification  Low flow rate, low vacuum, and low bottle height
  40. 40. Devices used in Surgery  Capsular Tension Ring  Indications:  Missing or damaged zonules  Lens subluxation  Pseudoexfoliation  High myopia  Marfan Syndrome  Mechanism:  Circular expansion of capsular bag  Stable conditions during surgery  Improves IOL centration  Reduced risk of capsular fibrosis  Resists capsular Shrinkage
  41. 41. Capsular Tension Rings Axial length <24mm for normal eyes Axial length >28mm for highly myopic eyes Axial length 24-28mm for normal or myopic eyes
  42. 42. Video: How to implant CTR
  43. 43. Devices used in Surgery  Cionni Ring  Indications:  Missing or damaged zonules larger than 4 clock hours  Lens subluxation  Pseudoexfoliation  High myopia  Marfan Syndrome  Mechanism:  Similar to CTR  Additionally corrects capsular bag decentration
  44. 44. Devices used in Surgery  Cionni Ring  Designed for scleral fixation with suture  One or two hooks extending from the ring, an eyelet located on the hook located behind the iris just in front of anterior capsule  The sutured eyelet anchors the ring to sclera in the area of missing zonules  Most frequent complication - posterior capsule opacification (PCO) -20% Vasavada AR, Praveen MR, Vasavada VA, Yeh RY, Srivastava S, Koul A, Trivedi RH. Cionni ring and in-the-bag intraocular lens implantation for subluxated lenses: a prospective case series. Am J Ophthalmol. 2012 Jun;153(6):1144-53. Irit Bahar, Igor Kaiserman, David Rootman. Cionni endocapsular ring implantation in Marfan's Syndrome. Br J Ophthalmol. 2007 November; 91(11): 1477–1480.
  45. 45. Cionni Rings
  46. 46. Video: How to implant Cionni Ring
  47. 47.  Ahmed Capsular Tension Segment  Partial ring of PMMA covering approximately one quadrant  Hole for temporary or permanent fixation  One or more segments may be used to support the areas of weak capsule Devices used in Surgery
  48. 48. Devices used in Surgery  Ahmed Capsular Tension Segment  Advantages:  May be used intraoperatively, secured by an iris hook  May be fixated to the sclera for permanent support  Stabilizes conditions during cataract surgery  Stripping cortex out from under and around the CTS is easy  Improves IOL centration
  49. 49. Video: How to implant Ahmed Capsular Tension segment
  50. 50. Devices used in Surgery  Iris Retractors  Indications:  Contracted pupils  Floppy iris  Bag fixation in phacodonesis/ subluxated lens
  51. 51. Video: Use of iris hooks
  52. 52. Associated Management  Co-management with the patient's pediatrician or internist is essential  Appropriate genetic counselling  All relatives with potential risk should be examined

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