ECTOPIA LENTIS

DR ISHWAR MISTRY
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

Dislocated 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
ETIOLOGY

Etiology

Secondary to
Inheritable
Ocular Traumatic
Forms
Conditions
 Inheritable Forms

With Associated With Associated

Isolated Forms
Ocular Anomalies Systemic Anomalies

Simple Ectopia Ectopia Lentis et

Marfan’s syndrome
Lentis pupillae

Simple
Aniridia Homocystinuria
Microspherophakia

Weil-Marchesani
syndrom

Rieger’s Anomaly

Hyperlysinemia

Sulfite Oxidase
deficiency
OTHER OCULAR CONDITIONS:

 Mature or hypermature cataract

 High myopia

 Megalocornea

 Cornea plana

 Iris coloboma

 Congenital Glaucoma

 Uveitis, endophthalmitis

 Intraocular Tumors

 Persistant hyperplastic primary vitreous

 Retinitis pigmentosa

 Retinal detachment
 SIMPLE ECTOPIA LENTIS
 Congenital or Spontaneous
 Congenital :
 Autosomal dominant inheritance
 Presents early in life

 Bilateral, symmetric, upward and temporal displacement of

the lens.

 Spontaneous:
 Presents in adult life
 Displaced often downwards

 Herniation of the vitreous associated with zonular

degeneration may occur through the zonular defect into the
anterior chamber
 Associated with cataract and retinal detachment
 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
Simple Ectopia Lentis Ectopia Lentis et
Pupillae
 MARFAN’S SYNDROM
 First described completely by Marfan in 1896.
 Transmitted by AD inheritence.

 Features:
 Ocular Features:
 Ectopia lentis – superotemporal – in 50% of cases
 Angle anomalies – prominent iris processes
 Glaucoma
 Hypoplasia of dilator pupillae
 Cornea plana
 Axial Myopia
 Retinal Detachment
 Cataract Formation
 Accommodation is not affected if crystalline lens is clear.
 Skeletal System:

 Tall and Thin – Dolichostenomelia

 Reduced upper to lower body segment ratio (0.85)
 Arm span to height ratio greater than 1.05
 Arachinodactyly – Walker (wrist) and Steinberg (thumb)
signs
 Pectus excavatum, pectus carinatum
 Pes planus
 Protusio acetabuli
 Scoliosis
 Joint Hypermobility
 High Arched palate
 Dental Crowding
 Typical Facies – Malar hypoplasia, retrognathia,
dolichocephaly, enophthalmos, down slanting palpabral
fissures.
 Cardiovascular System :
 Aortic root dilatation ( 70%-80% cases) involving sinuses of
Valsalva
 Aortic dissection of ascending aorta
 Mitral valve prolapse
 Calcification of mitral annulus
 Dilatation of thoracic, abdominal aorta, main pulmonary
artery

 Other:
 Dural ectasia
 Spontaneous pneumothorex
 Striae atrophicae
 Recurrant or incisional hernias

 Modified Ghent Criteria for diagnosis .

 HOMOCYSINURIA

 Inborn error of metabolism of sulfur containing amino

acids
 Near absence of cystathionine b-synthetase (the
enzyme that converts homocysteine to cystathionine)
 AR inheritance

 Salient features:
 Fair skin with coarse hair
 Osteoporosis
 Mental retardation (nearly 50%)
 Seizure disorder
 Marfanoid habitus
 Spastic gait, Muscular weakness
 Ocular features:

 Lens luxation usually is bilateral,

symmetrical, and inferonasal, and
presents in nearly 90% of patients
 Disintegration of zonules – loss of
accommodative power.
 Glaucoma in one fourth cases.

 Thromboembolic events
constitute the major threat to
survival, especially following
general anesthesia
WEIL-MARCHESANI SYNDROME
 Salient features:
 Short stature
 Brachycephaly, MR
 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
SULFITE OXIDASE DEFICIENCY
 Defect in sulfur metabolism
 Salient 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.
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
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
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)
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.
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 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
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
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
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
OCULAR ASSOCIATIONS
 Persistent pupillary  Retinitis pigmentosa
membrane  Hypermature cataract
 Aniridia  Intraocular tumor
 Rieger’s syndrome

 Dominantly inherited
blepharoptosis
 High myopia

 Congenital
glaucoma/buphthalmos
 Pseudoexfoliation
COMPLICATIONS

Complications

Corneal Retinal
Glaucoma Uveitis Amblyopia
Decompensation Detachment
OTHER….
 Cardiac and Skeletal system evaluation for
Marfan syndrome
 Serum and urine levels of homocysteine or
methionine for homocystinuria
 Axial length measurement
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
MANAGEMENT PROTOCOL
Subluxated Clear Lens

Minimal Marked

Spectacle
correction through 1. Spectacle correction
phakic portion through aphakic portion
2. Optical iridectomy
3. Miotics/Mydriatics

Surgical
Removal

Pars Plana Limbal Route

1. Vitrectomy probe Phacoaspiration with
2. Phacofragmentome help of iris hooks +
PCIOL/ Iris clip/ SFIOL
 MANAGEMENT PROTOCOL

Subluxated Cataractous Lens

Minimal Marked

ICCE/
Phacoemulsificati Pars Plana Route Limbal Route
on + IOL

1. Lensectomy Phacoemulsification with help of iris

2. Phacofragmentation hooks + PCIOL/ Iris clip/ SFIOL
 MANAGEMENT PROTOCOL

Subluxated Lens- Surgical

Management

Subluxation <3 Subluxation 3-5 Subluxation 5-7 Subluxation >7 clock

clock hours clock hours clock hours hours

Slow Phaco
+ PCIOL Slow Phaco with CTR/ Pars Plana Lensectomy +
Slow Phaco + Cionni fixation Pars Plana Vitrectomy +
Cionni ring + PCIOL of bag/ Ahmed segment + SFIOL
Capsular retractors + PCIOL

Pars Plana
Lensectomy +
Pars Plana
Vitrectomy + Pars Plana ICCE + Anterior
SFIOL/ Iris clip Lensectomy + Pars Pars Plana vitrectomy +
Plana Vitrectomy + Lensectomy + Pars ACIOL/SFIOL/
SFIOL/ Iris clip Plana Vitrectomy + Iris clip
SFIOL/ Iris clip
MANAGEMENT PROTOCOL
Dislocated Lens

Anterior Posterior

Mobile Fixed
Removal through
limbal approach

Complications:
1. Glaucoma
2. Inflammation
3. Obstruction in visual axis

Surgical removal with

complete vitrectomy

Vitrectomy cutter Phacofragmentation PFCL floatation- limbal

delivery
MANAGEMENT OF SUBLUXATED LENS IN
CHILDREN
 Treatment of aphakia in children is a challenge
 Spectacle correction is not suitable for unilateral aphakia
due to anisokonia
 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.
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.
OTHER….
 Co-management with the patient's pediatrician
or internist is essential
 Appropriate genetic counselling

 All relatives with potential risk should be

examined