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Ectopia LentisMohammad Ghoreishi, MDIsfahan University of medical sciencesinfo@persianeyeclinic.com

Case history 20 y/o maleReferred for refractive surgery due to high myopiaVAOD: CF 4/10OS: CF 5/10RefractionOD: -19.5- 0.5 x 180OS: -20.0-1.00 x 180IOPOD: 39 mm HgOS: 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 positionLuxation or dislocation: when lens is completely displaced, in AC or Vitreous

CausesTraumatic (most common cause) Hereditary ectopia lentis without systemic manifestations Single (isolated) ectopia lentisEctopia lentis et pupillae

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

Ectopia lentis with systemic diseaseHomocystinuriaAbsence 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 circulationThromboembolic 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 diseaseWeil-Marchesani Short stature, brachydactyly, limited joint mobilityMicrospherophakia , ectopia lentis, lenticular myopiaPupillary block glaucoma Sulfite oxidase deficiency Defect in sulfur metabolismProgressive CNS abnormalities Ectopia lentisHyperlysinemia 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 surgeryImpaired vision due to cataract or refractive error not improved by other corrective methodsInduced or thread for glaucoma

9Preop evaluationRefraction and visionSystemic evaluationHomocystinuria.Marfan.Glaucoma, angleRetinal detachmentCorneal endothelium

10Loose zonules , no subluxationDiagnosing the condition is importantPreventive measuresPupil dilationCapsular staining, large rhexisSuporting the capsule with iris retractorsSmooth phaco parametersSupracapsular techniqueCTR without or with fixation if necessary

11Capsular tension rings (CTR)StandardCioniCapsular tension segments (CTS)Inserted before or after phacoemulsification

The Cionni-modified CTR can be sutured to the sclera.

The CTS, with its 90 arc, can be inserted at a specific site ofzonular weakness

12Surgical technique

Lensectomy, Artisan aphakia

Surgical technique

ECCE, Artisan Aphakia

Surgical technique

Zonulolysis, CTR

Pre and postop cases

Posteriorly fixed Artisan IOL

Thank you for your attentionn