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.

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

HOW TO TAKE SLIT-LAMP EXAM IMAGES WITH A SMARTPHONE?

Smartphone slit-lamp photography is the new advancement in the field of science and technology in which photographs of the desired slit-lamp finding can be taken with smartphones by using the slit-lamp adaptors.

Slit-lamp Smartphone photography

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.