Ectopia lentis edit

Ectopia Lentis edit

1. ECTOPIA LENTIS Presenter: Dr. Rujuta Gore Moderator: Dr. Suhas Haldipurkar Dr. Prakash Chipade

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. Topographic Classification

4. Etiologic Classification

5. Congenital Ectopia Lentis Usually bilateral and symmetrical

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. 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. 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. 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. 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. MARFAN SYNDROME Cardiovascular Manifestations Mitral valve prolapse, aortic dilatation

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. 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. 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. 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. SULFITE OXIDASE DEFICIENCY Defect in sulfur metabolism Salient features: Progressive CNS abnormalities that develop within the first year of life Ectopia lentis.

17. HYPERLYSINEMIA Autosomal recessive enzymatic defect of amino acid metabolism Characterized by mental retardation and lens dislocation.

18. Rare Systemic Associations Ehlers-Danlos syndrome Crouzon disease Refsum syndrome Kniest syndrome Mandibulofacial dysostosis Sturge-Weber syndrome

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. Consecutive/Spontaneous Ectopia Lentis Mechanical stretching Buphthalmos High myopia Staphyloma Intraocular Tumors Inflammatory Destruction of zonules Hypermature cataract Cyclitic adhesions Vitreous traction bands

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. 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. 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. 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. 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. 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. 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. 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. 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. Complications

31. Workup Cardiac evaluation for Marfan syndrome Serum and urine levels of homocysteine or methionine for homocystinuria Axial length measurement

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. Management Protocol

34. Management Protocol

35. Management Protocol

36. Management Protocol

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. 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. 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. 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. 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. Video: How to implant CTR

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. 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. Cionni Rings

46. Video: How to implant Cionni Ring

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. 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. Video: How to implant Ahmed Capsular Tension segment

50. Devices used in Surgery Iris Retractors Indications: Contracted pupils Floppy iris Bag fixation in phacodonesis / subluxated lens

51. Video: Use of iris hooks

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

53. Thankyou !