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DR ISHWAR MISTRY
INTRODUCTION
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
Simple
Aniridia Homocystinuria
Microspherophakia
Weil-Marchesani
syndrom
Rieger’s Anomaly
Hyperlysinemia
Sulfite Oxidase
deficiency
OTHER OCULAR CONDITIONS:
Megalocornea
Cornea plana
Iris coloboma
Congenital Glaucoma
Uveitis, endophthalmitis
Intraocular Tumors
Retinitis pigmentosa
Retinal detachment
SIMPLE ECTOPIA LENTIS
Congenital or Spontaneous
Congenital :
Autosomal dominant inheritance
Presents early in life
the lens.
Spontaneous:
Presents in adult life
Displaced often downwards
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:
Other:
Dural ectasia
Spontaneous pneumothorex
Striae atrophicae
Recurrant or incisional hernias
Salient features:
Fair skin with coarse hair
Osteoporosis
Mental retardation (nearly 50%)
Seizure disorder
Marfanoid habitus
Spastic gait, Muscular weakness
Ocular features:
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
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
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
Minimal Marked
Spectacle
correction through 1. Spectacle correction
phakic portion through aphakic portion
2. Optical iridectomy
3. Miotics/Mydriatics
Surgical
Removal
Minimal Marked
ICCE/
Phacoemulsificati Pars Plana Route Limbal Route
on + IOL
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
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
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