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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
Congenital Metabolic 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 Uveitis
Corneal 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
MobileComplicatio
ns: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!