Mohammad Ghoreishi MD Isfahan University of medical sciences

Mohammad Ghoreishi, MD Isfahan University of medical sciences info@persianeyeclinic. com

Case history • 20 y/o male • Referred for refractive surgery due to high myopia • VA – OD: CF – OS: CF 4/10 5/10 • Refraction – OD: -19. 5 - 0. 5 x 180 – OS: -20. 0 -1. 00 x 180 • IOP – OD: 39 mm Hg – OS: 39 mm Hg He was diagnosed as ectopia lentis and microspherophakia with secondary NA glaucom

Definition • Displacement of the crystalline lens from its original position • Subluxation: when displacement is partial with part of the lens still in normal position • Luxation or dislocation: when lens is completely displaced, in AC or Vitreous

Causes • Traumatic (most common cause) • Hereditary ectopia lentis without systemic manifestations – Single (isolated) ectopia lentis – Ectopia lentis et pupillae

Ectopia lentis with systemic disease – Marfan syndrome • Autosomal dominant • Tall stature • Arachnodactyly, joint laxity, mitral valve prolapse, aortic dilatation • Axial myopia, and increased incidence of retinal detachment • Lens dislocation (about 75% of patients ) , usually bilateral, symmetrical, and supartemporal

Ectopia lentis with systemic disease – Homocystinuria • Absence of cystathionine b-synthetase (the enzyme that converts homocysteine to cystathionine). • Fair skin with coarse hair, osteoporosis, mental retardation, seizure disorder, marfanoid habitus, and 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

Ectopia lentis with systemic disease – Weil-Marchesani • Short stature, brachydactyly, limited joint mobility • Microspherophakia , ectopia lentis, lenticular myopia • Pupillary block glaucoma – Sulfite oxidase deficiency • Defect in sulfur metabolism • Progressive CNS abnormalities • Ectopia lentis – Hyperlysinemia • Enzymatic defect of amino acid metabolism • Mental retardation and lens dislocation. • Increased plasma levels of lysine

Primary ocular disorders associated with ectopia lentis – Congenital glaucoma/buphthalmos – Pseudoexfoliation syndrome – Syphilis/chronic uveitis – Retinitis pigmentosa – Megalocornea – Aniridia – Hypermature cataract – Intraocular tumor – High myopia

Indications for surgery • Impaired vision due to cataract or refractive error not improved by other corrective methods • Induced or thread for glaucoma

Preop evaluation • Refraction and vision • Systemic evaluation …Homocystinuria…. …Marfan…. • Glaucoma, angle • Retinal detachment • Corneal endothelium

Loose zonules , no subluxation • • Diagnosing the condition is important Preventive measures Pupil dilation Capsular staining, large rhexis Suporting the capsule with iris retractors Smooth phaco parameters Supracapsular technique CTR without or with fixation if necessary

• Standard • Cioni • Capsular tension The Cionni-modified CTR can segments (CTS) be sutured to the sclera. • Inserted before or after phacoemulsification The CTS, with its 90º arc, can be inserted at a specific site of zonular weakness

Surgical technique Lensectomy, Artisan aphakia

Surgical technique ECCE, Artisan Aphakia

Surgical technique Zonulolysis, CTR

Pre and postop cases

Thank you for your attentionn