What does the term ectopia lentis describe?

Dislocation or displacement of the crystalline lens Ectopia lentis is the dislocation or displacement of the natural crystalline lens from its normal position, whether from trauma or from an underlying ocular or systemic disease.

Opacification of the crystalline lens nucleus

Congenital absence of the crystalline lens

Abnormal curvature of the anterior lens surface

In ectopia lentis, when is a lens described as subluxed rather than luxated?

When it is partially displaced but stays within the lens space A subluxed lens is only partly displaced and remains within the lens space; a luxated lens has left the hyaloid fossa entirely — floating in vitreous, sitting on the retina, or in the anterior chamber.

When it lies free-floating within the vitreous cavity

When it rests directly on the surface of the retina

When it has migrated into the anterior chamber

Besides trauma, ectopia lentis is associated with which broad category of cause?

Ocular or systemic disease Ectopia lentis may follow trauma or accompany ocular or systemic disease, so the cause must be established before management is planned.

Long-standing refractive amblyopia

Untreated bacterial conjunctivitis

Why is establishing the etiology of ectopia lentis so important?

It may be the first sign of serious systemic disease A displaced lens can be the presenting sign of a more serious systemic disorder, so identifying the etiology drives appropriate management well beyond the eye.

It determines the intraocular lens power needed

It predicts the rate of corneal endothelial loss

It decides whether topical steroids are safe

Which form of glaucoma is listed as a major complication of ectopia lentis?

Pupillary block glaucoma Major complications of ectopia lentis include significant refractive shifts, pupillary block glaucoma, retinal damage and ultimately blindness.

In the reported ectopia lentis case, when did the lens displacement become apparent?

Only on examination under mydriasis Through the undilated pupil the cornea, iris, anterior chamber and lens all looked normal; the ectopia lentis was revealed only once the pupil was dilated.

On undilated slit lamp examination

On measurement of intraocular pressure

On indirect ophthalmoscopy of the periphery

How is a dislocated lens that has migrated into the anterior chamber initially managed?

Pharmacological mydriasis to let it move back Treatment begins pharmacologically: the pupil is dilated so the lens can migrate posteriorly behind the iris rather than remain against the corneal endothelium.

Immediate lensectomy with vitrectomy

Topical miotics to hold it anteriorly

How should pupillary block glaucoma complicating ectopia lentis be treated?

Laser peripheral iridotomy Pupillary block glaucoma is treated with laser peripheral iridotomy, while any accompanying rise in intraocular pressure is managed medically.

Trabeculectomy with an antimetabolite

Cycloplegic drops and observation

Why is intraocular lens implantation difficult in eyes with ectopia lentis?

Inherent defects in the lens capsule The lens capsule itself is defective in these eyes, so an intraocular lens is hard to implant; many patients instead have lensectomy with vitrectomy and are rehabilitated with a contact lens.

Corneal steepening prevents accurate biometry

The pupil dilates poorly during surgery

Raised intraocular pressure distorts the sclera

Which finding is a listed indication for lensectomy in ectopia lentis?

A lens touching the corneal endothelium Indications for lensectomy include a lens in the anterior chamber, especially where it touches the corneal endothelium, alongside lens-induced glaucoma or uveitis, mature cataract, imminent complete luxation and retinal detachment.

An intraocular pressure of 16 mmHg

Astigmatism correctable with spectacles

Mild anterior capsule pigment dusting

Case study and management of Ectopia Lentis

CASE REPORT of Ectopia Lentis

A 21-year-old female presented with decreased vision in both eyes (left more than right) noticed for the last year.

Her unaided visual acuity was 20/200 and 20/400 in the right and left eye, respectively, improving to 20/40 with –4.5 DS/–3.0 DC x10° correction in the right eye and 20/80 with –10.0 DS/–6.5 Dc x10° correction in the left eye.

Intraocular pressures were 14 mmHg and 16 mmHg in the right and left eyes, respectively.

On slit lamp examination, the cornea, iris, anterior chamber, and lens appeared normal in the undilated pupil in both eyes.

However, on examination under mydriasis, the right eye revealed ectopia lentis.

DISEASE “Ectopia Lentis”

Ectopia lentis is the dislocation or displacement of the natural crystalline lens. The lens is defined as luxated (dislocated) when it lies completely outside of the hyaloid fossa, is free-floating in the vitreous, is in the anterior chamber, or lies directly on the retina.

The lens is considered subluxed when it is partially displaced but remains within the lens space.

Ectopia lentis may occur after trauma or may be associated with ocular or systemic disease. As ectopia lentis may be the first sign of a more serious systemic disease.

it is important to determine the etiology for appropriate patient management.

Additionally,

ectopic lentis can cause major complications within the eye, including significant refractive shifts, pupillary block glaucoma, retinal damage, and blindness.

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Ectopia Lentis MANAGEMENT

Medical:

Medical management involves refractive correction as well as co-management of any associated systemic disease with the patient’s internist or pediatrician. As many predisposing diseases are hereditary, genetic counseling may be indicated.

Treatment of ectopia lentis itself depends on where and how far the lens moves, as well as any resulting complications. In all cases, timely optical correction is important for the prevention of amblyopia.

If the dislocated lens migrates into the anterior chamber, treatment is initially pharmacological with mydriasis to permit posterior migration of the lens behind the iris.

Pupillary block glaucoma should be treated with laser peripheral iridotomy, and elevations in intraocular pressure should be treated medically.

Progressive lens dislocation without major complications has been traditionally managed conservatively.

However,

some studies suggest that surgical intervention could lead to improved visual acuity. The decision to operate depends on many factors, including visual acuity, lens location, progressive subluxation of the lens, imminent total dislocation, and others.

Surgical:

Surgical management of ectopia lentis remains a challenge, as inherent defects in the lens capsule make implantation of an intraocular lens difficult.

As a result, many patients are managed with lensectomy/vitrectomy with optical rehabilitation with a contact lens.

Indications for lensectomy include, but are not limited to:

Lens-induced glaucoma
Lens in the anterior chamber, especially if the lens is touching the corneal endothelium
Lens opacity that is mature or hypermature
Evidence of lens-induced uveitis
Inadequate visual acuity not correctable by refraction
Imminent complete luxation of the lens
Retinal detachment

REFERENCES

Clarke, Clement C. “Ectopia lentis: a pathologic and clinical study.” Archives of Ophthalmology 21.1 (1939): 124- 153.
Harley, Robison D., Leonard B. Nelson, and Scott E. Olitsky, eds. Harley’s Pediatric Ophthalmology. Lippincott Williams & Wilkins, 2005. p.225.
Jarrett, William H. “Dislocation of the lens: a study of 166 hospitalized cases.” Archives of ophthalmology 78.3 (1967): 289-296.
Chandra, Aman, et al. “A genotype-phenotype comparison of ADAMTSL4 and FBN1 in isolated ectopia lentis.” Investigative ophthalmology & visual science53.8 (2012): 4889-4896.
Sadiq, Mohammad Ali, and Deborah Vanderveen. “Genetics of ectopia lentis.” Seminars in ophthalmology. Vol. 28. No. 5-6. New York: Informa Healthcare USA, Inc., 2013.
Nelson, Leonard B., and Irene H. Maumenee. “Ectopia lentis.” Survey of ophthalmology 27.3 (1982): 143-160.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

What does the term ectopia lentis describe?
Ectopia lentis is the dislocation or displacement of the natural crystalline lens from its normal position, whether from trauma or from an underlying ocular or systemic disease.
In ectopia lentis, when is a lens described as subluxed rather than luxated?
A subluxed lens is only partly displaced and remains within the lens space; a luxated lens has left the hyaloid fossa entirely — floating in vitreous, sitting on the retina, or in the anterior chamber.
Besides trauma, ectopia lentis is associated with which broad category of cause?
Ectopia lentis may follow trauma or accompany ocular or systemic disease, so the cause must be established before management is planned.
Why is establishing the etiology of ectopia lentis so important?
A displaced lens can be the presenting sign of a more serious systemic disorder, so identifying the etiology drives appropriate management well beyond the eye.
Which form of glaucoma is listed as a major complication of ectopia lentis?
Major complications of ectopia lentis include significant refractive shifts, pupillary block glaucoma, retinal damage and ultimately blindness.
In the reported ectopia lentis case, when did the lens displacement become apparent?
Through the undilated pupil the cornea, iris, anterior chamber and lens all looked normal; the ectopia lentis was revealed only once the pupil was dilated.
How is a dislocated lens that has migrated into the anterior chamber initially managed?
Treatment begins pharmacologically: the pupil is dilated so the lens can migrate posteriorly behind the iris rather than remain against the corneal endothelium.
How should pupillary block glaucoma complicating ectopia lentis be treated?
Pupillary block glaucoma is treated with laser peripheral iridotomy, while any accompanying rise in intraocular pressure is managed medically.
Why is intraocular lens implantation difficult in eyes with ectopia lentis?
The lens capsule itself is defective in these eyes, so an intraocular lens is hard to implant; many patients instead have lensectomy with vitrectomy and are rehabilitated with a contact lens.
Which finding is a listed indication for lensectomy in ectopia lentis?
Indications for lensectomy include a lens in the anterior chamber, especially where it touches the corneal endothelium, alongside lens-induced glaucoma or uveitis, mature cataract, imminent complete luxation and retinal detachment.