ECTOPIA LENTIS

Presenter: Dr. Rujuta Gore Moderator: Dr. Suhas

Haldipurkar Dr. Prakash

Chipade

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

Topographic Classification

Subluxated 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

Dislocated lens

Etiologic ClassificationEtiology

CongenitalMetabolic Disorder

Traumatic Consecutive or Spontaneous

Congenital Ectopia Lentis

Usually bilateral and symmetrical

Simple Ectopia Lentis

Ectopia Lentis et Pupillae

Congenital Ectopia Lentis

SIMPLE ECTOPIA LENTISAutosomal dominant inheritanceOcular 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

Congenital Ectopia Lentis

ECTOPA LENTIS ET PUPILLAEAutosomal recessive inheritancePupils are oval or slit shaped and ectopic, and they frequently dilate poorlyBilateral, 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

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

Metabolic Disorders

MARFAN SYNDROMEPrevalence of approximately 5 per 100,000Autosomal 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

MARFAN SYNDROME

Salient features:Skeletal Manifestations

Tall statureIncreased arm span in

relation to body height and elongated lower segment

ArachnodactylyJoint laxity, scoliosis

MARFAN SYNDROME

Cardiovascular ManifestationsMitral valve prolapse,

aortic dilatation

MARFAN SYNDROME

Ocular ManifestationsAxial myopiaCorneal 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

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 hairOsteoporosisMental retardation (nearly 50%)Seizure disorderMarfanoid habitus

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

WEIL-MARCHESANI SYNDROMESalient features:

Short statureBrachycephalyLimited joint mobilityEctopia lentisMicrospherophakia (most

prominent feature of this syndrome)

Lenticular myopia Lens subluxation occurs

inferiorly, often progressing to complete dislocation

Pupillary block glaucoma is common

SULFITE OXIDASE DEFICIENCY

Defect in sulfur metabolismSalient features:

Progressive CNS abnormalities that develop within the first year of life

Ectopia lentis.

HYPERLYSINEMIA

Autosomal recessive enzymatic defect of amino acid metabolism

Characterized by mental retardation and lens dislocation.

Rare Systemic Associations

Ehlers-Danlos syndromeCrouzon diseaseRefsum syndromeKniest syndromeMandibulofacial dysostosisSturge-Weber syndrome

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

Consecutive/SpontaneousEctopia Lentis

Mechanical stretchingBuphthalmosHigh myopiaStaphylomaIntraocular Tumors

InflammatoryDestruction of zonulesHypermature cataractCyclitic adhesionsVitreous traction bands

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 diplopiaRed painful eye

Presentation

History: Onset Cardiovascular disease (Marfan syndrome)Skeletal problems (Marfan syndrome, Weil-

Marchesani syndrome, or homocystinuria)Ocular traumaFamily history

Consanguinity Mental retardation Unexplained deaths at young age (eg, autosomal

recessive conditions, including homocystinuria, hyperlysinemia, ectopia lentis et pupillae, or sulfite oxidase deficiency)

Ocular Examination

Visual AcuityEctopia 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.

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.

Ocular ExaminationSignsMegalocorneaAnterior chamber-

IrregularFlatCells and flare- uveitisVitreous- present/ absent

Angle of ACRecession seen in traumaPresence or absence of synechiae

Ocular Examination

Iris- iridodonesis, transilluminationAppearance of the pupil

assess the adequacy of the pupillary space for a possible aphakic correction

Lens-PositionPhacodonesisCataractZonules- stretched or broken

Ocular Examination

Complete dislocation:Posterior dislocation

AphakiaLens seen in vitreous cavity

Anterior dislocation:Clear lens- oil droplet

appearance with golden lustre of rim

Cataractous lens- white disc in AC

IridocyclitisEndothelial damageSecondary glaucoma

Ocular Examination

Raised intraocular pressureCauses 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

Ocular Associations

Persistent pupillary membrane

AniridiaRieger’s syndromeDominantly inherited

blepharoptosisHigh myopiaCongenital

glaucoma/buphthalmosPseudoexfoliation

Retinitis pigmentosaHypermature cataractIntraocular tumor

ComplicationsComplications

Glaucoma

UveitisCorneal Decompensation

Amblyopia

Retinal Detachm

ent

Workup

Cardiac evaluation for Marfan syndromeSerum and urine levels of homocysteine or

methionine for homocystinuriaAxial length measurement

Indications for Surgery

Lens in the anterior chamberLens-induced uveitisLens-induced glaucomaLenticular opacity with poor visual functionAnisometropia or refractive error not amenable

to optical correction (eg, in a child to prevent amblyopia)

Impending dislocation of the lens

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 RemovalPars Plana

1. Vitrecto

my probe

2. Phacofragmento

me

Limbal Route

Phacoaspiration

with help of

iris hooks + PCIOL/ Iris clip/ SFIOL

Management Protocol

Subluxated Cataractous Lens

Minimal

ECCE/ Phacoemulsification + IOL

Marked

Pars Plana Route

1. Lensectomy

2. Phacofragm

entation

Limbal Route

Phacoemulsification

with help of iris hooks + PCIOL/ Iris clip/ SFIOL

Management ProtocolSubluxated Lens- Surgical Management

Subluxation <3 clock

hours

Slow Phaco + PCIOLPars 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

hoursSlow

Phaco + Cionni fixation of bag/ Ahmed

segment +

Capsular retractor

s + PCIOL

Pars Plana

Lensectomy

+ Pars Plana Vitrectomy + SFIOL/

Iris clip

Subluxation >7 clock

hoursPars Plana

Lensectomy + Pars Plana

Vitrectomy +

SFIOL

ECCE +

Anterior

vitrectomy + ACIOL/SFIOL/ Iris clip

Management Protocol

Dislocated Lens

Anterior

Removal through limbal approach

Posterior

Mobile

Complications:1.

Glaucoma2.

Inflammation3.

Obstruction in visual

axis

Surgical removal

with complete vitrecto

my

Vitrectomy cutter

Phacofragmentation

PFCL floatation- limbal delivery

Fixed

Management of Subluxated lens in ChildrenTreatment 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/SFIOLACIOL 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.

Management of Subluxated lens in ChildrenPhacoaspiraton with the help of CTR is a safe and

predictable procedure in subluxated lens in children

Elimination of PCO still remains a challengeLong-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.

Management of Subluxated lens in ChildrenKey points to successful CTR implantation

To use high-viscosity visco-elastic materialMaking the incision at a meridian with intact

zonulesTo avoid damaging zonular fibers with the

movement of the phacotipPerform slow-motion phacoemulsificationLow flow rate, low vacuum, and low bottle height

Devices used in Surgery

Capsular Tension RingIndications:

Missing or damaged zonulesLens subluxationPseudoexfoliationHigh myopiaMarfan Syndrome

Mechanism:Circular expansion of capsular bagStable conditions during surgeryImproves IOL centrationReduced risk of capsular fibrosisResists capsular Shrinkage

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

Video: How to implant CTR

Devices used in Surgery

Cionni RingIndications:

Missing or damaged zonules larger than 4 clock hours

Lens subluxationPseudoexfoliationHigh myopiaMarfan Syndrome

Mechanism:Similar to CTRAdditionally corrects capsular bag

decentration

Devices used in Surgery

Cionni RingDesigned for scleral fixation with sutureOne 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.

Cionni Rings

Video: How to implant Cionni Ring

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

Devices used in Surgery

Ahmed Capsular Tension SegmentAdvantages:

May be used intraoperatively, secured by an iris hook

May be fixated to the sclera for permanent supportStabilizes conditions during cataract surgeryStripping cortex out from under and around the

CTS is easyImproves IOL centration

Video: How to implant Ahmed Capsular Tension segment

Devices used in Surgery

Iris RetractorsIndications:

Contracted pupilsFloppy iris Bag fixation in phacodonesis/ subluxated lens

Video: Use of iris hooks

Associated Management

Co-management with the patient's pediatrician or internist is essential

Appropriate genetic counsellingAll relatives with potential risk should be

examined

Thankyou!