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What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023
| Contributor Information
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Image of an anterior lenticonus courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Lenticonus
An anterior lenticonus with a forward-protruding lens (shown) may occur in association with Alport syndrome, spina bifida, or Waardenburg syndrome.[1] Lenticonus is a rare congenital developmental abnormality in which the anterior or (more commonly) posterior lens surface projects spherically or conically due to thinning of the respective central lens surface.[2-4] This type of lens shape abnormality results in astigmatism[2,4]; later, it may progress to cataract formation,[3,5] or spontaneous rupture of the lens capsule may occur.[4] Posterior lenticonus, a more frequent disorder than anterior lenticonus, sometimes occurs in Lowe syndrome.
Image of a lenticulocorneal adhesion courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Lenticulocorneal Adhesion
The image shows an adhesion between the lens and cornea. This ocular abnormality may result from a congenital defect within the posterior cornea, which includes the corneal posterior stroma, Descemet membrane, and corneal endothelium. Embryologically, during the development of the eye, the elements of the anterior segment form separate structures, including the cornea, iris, and lens. However, in anterior chamber cleavage syndromes, such as Peters anomaly, there is incomplete separation of the cornea from the iris and/or the lens.[5] Lenticulocorneal adherence may also occur with a corneal ulcer due to perforation, trauma, or Rieger anomaly. To restore vision, a corneal transplant procedure may be used to remove the corneal opacity together with lens removal to extract the lenticular opacity.
Image of a congenital cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Congenital Cataract
When the lens forms in the embryo before birth, the lens fibers have a meeting point that forms the central Y sutures (shown), an important landmark that identifies the extent of the central portion of the lens, called the fetal nucleus.[6] Lens material that is peripheral to the Y sutures is called the lens cortex, whereas lens material within and including the Y sutures is termed the lens nucleus. In the anterior lens, the Y suture is upright; in the posterior lens, the Y suture is inverted. The anterior Y suture, as well as the outline of the lens nucleus, is shown in the above image. This meeting point can be a focus for cataract formation in the embryo if maternal infections such as rubella occur in the first trimester of the mother's pregnancy.
Image of an embryonic nuclear cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Embryonic Nuclear Cataract
The image shows an embryonic nuclear cataract (cataracta centralis pulverulenta) that involves the entire embryonal nucleus and is caused by the early inhibition of lens development. Embryonic nuclear cataracts have a dominant inheritance pattern[5] and are characterized as small rounded opacities in the center of the lens that is usually bilateral.[2,5] These cataracts may be associated with microphthalmos and microcornea.[2,5]
Image of an anterior polar cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Anterior Polar Cataract
The image shows a small, very dense anterior polar cataract (a central, pyramidal opacity at the front of the lens) on slit-lamp examination. An anterior polar cataract is an uncommon congenital lens abnormality that may occur as a sole sporadic finding, in association with other ocular abnormalities (eg, anterior lenticonus), or as an inherited autosomal dominant condition.[6-9] Most anterior polar cataracts occur as a result of the abnormal separation of the lens vesicle during embryonic lens development.[3,5] These cataracts are often bilateral, generally do not cause visual impairment, and usually do not require surgery.[1,3,5] However, when anterior polar cataracts progress, strabismus, anisometropia, or amblyopia may cause vision loss.[3,5]
Image of a lens dislocation courtesy of the University of Michigan Kellogg Eye Center.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Lens Dislocation
A young man with Marfan syndrome presented with a superiorly and medially dislocated right lens (shown). His left eye had a similar appearance. The edge of the fairly clear lens can be seen posterior to the iris through the dilated pupil. Bilateral lens dislocation is common in patients with Marfan syndrome, Weill-Marchesani syndrome, and homocystinuria, but it can also be seen unilaterally in patients with ocular trauma.[2,3,5]
Image of a senile nuclear cataract courtesy of Manolette R. Roque, MD, MBA.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Senile Nuclear Cataract
Age-related (senile) nuclear cataract is the most common type of cataract and involves slowly progressive yellowing and clouding of the lens nucleus.[10] The yellowing may progress to a brown color, (ie, a brunescent cataract). Nuclear sclerosis often changes the refractive index of the lens, causing increasing myopia (or decreasing hyperopia). Patients who are accustomed to wearing only reading glasses may experience "second sight" with nuclear sclerosis, being able to shed their reading glasses due to the increase in myopia. Surgical removal of the lens (cataract extraction) is indicated when the level of visual acuity decline interferes with the patient's quality of life.
Image of a mature cataract courtesy of Manolette Roque, MD, MBA.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Mature Cataract
A mature cataract (shown) is entirely dense, with a completely opaque lens and a corresponding severe loss of visual acuity.[2,11] These cataracts are not specific to any single disorder, but they can occur in association with various conditions (eg, retinal detachment, tumor, trauma, uveitis, diabetes).[12] Through different mechanisms, a mature cataract may result in increased intraocular pressure.[11] In those cases, surgical removal of the cataract usually reduces the pressure to normal levels. An immature cataract, by definition, involves only partial opacification of the lens.
Image of a senile cortical cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Senile Cortical Cataract
A senile cortical cataract, a cataract related to aging, may feature radial-like opacities within the lens cortex (shown); this type of lens abnormality impairs vision. One of the leading causes of blindness in the world, senile cataracts are characterized by gradual, progressive thickening and clouding of the lens, although the visual morbidity is reversible with surgical treatment.[13] The pathogenesis of age-related cataracts has not yet been elucidated, but it is believed to be multifactorial and involve complex physiologic processes. The three main types of senile cataracts are nuclear, cortical, and posterior subcapsular.[13] A surgical extraction is used to manage these lens abnormalities.
Image of a posterior subcapsular cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Posterior Subcapsular Cataract
The long-term use of corticosteroids frequently causes a posterior subcapsular cataract (shown). Three distinct characteristics have been described for steroid-induced posterior subcapsular cataracts: (1) association only with steroids with glucocorticoid activity, (2) involvement of aberrant migrating lens epithelial cells, and (3) a central posterior location.[14] Proposed mechanisms include glucocorticoid receptor activation, altered gene transcription in lens epithelial cells, alterations of growth factors, osmotic imbalance, and oxidative damage, among others.[13-15] Posterior subcapsular cataracts are very common; they are often present in people in their 50s with symptoms of light glare during night driving.
Image of a traumatic cataract from a penetrating injury courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Traumatic Cataract
This image shows a traumatic cataract from a penetrating injury. Penetrating trauma with disruption of the lens capsule can result in early focal cortical changes, or it may rapidly progress to total cortical opacification.[2,13] The lens shown is opaque, shrunken, and subluxated downward. In addition, there are changes in the shape and color of the iris. By contrast, blunt trauma causes cataracts to have the classic appearance of stellate or rosette-shaped posterior axial opacities.[2]
Image of a shield cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Shield Cataract
A patient with a longstanding history of atopic dermatitis presented with a shield cataract (shown) (note the dense anterior subcapsular plaque). The patient had a similar cataract in the other eye. This type of acquired lens abnormality typically has a shieldlike or starlike appearance, with a wrinkled anterior capsule.[2] It is often related to atopy, although it may also result from uveitis or trauma.[15] Anterior subcapsular cataracts and cortical shield cataracts may result from the proliferation and subsequent degeneration of lens epithelial cells.[15]
Image of a subcapsular cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Subcapsular Cataract
A subcapsular cataract (shown in a patient with diabetes) is located immediately beneath either the anterior or posterior capsule, leaving the rest of the lens unaffected. Subcapsular cataracts generally indicate the presence of an acquired lens abnormality that developed at some point after birth.[3,5] Opacities in the posterior subcapsular area are typically idiopathic, but they may also occur under a variety of clinical conditions, including diabetes, chronic systemic steroid usage, use of nasal steroids, Down syndrome, and trauma.[3,5]
Image of a sunflower cataract courtesy of Daljit Singh, MBBS, MS, DSc.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Sunflower Cataract
A sunflower cataract (shown), or chalcosis of the lens, can occur with Wilson disease.[1,2,3,5] This uncommon lens abnormality usually has a brown, yellow, or green, petal-like appearance of the anterior and posterior lens capsule due to copper deposition.[2,3,5] Copper deposition in the lens may result from a copper-containing intraocular foreign body, the use of eye drops containing copper sulfate, or Wilson disease.[17] Chalcosis lentis has also been associated with lung carcinoma and multiple myeloma.[18] Kayser-Fleischer rings (golden-brown or green rings at the periphery of the cornea) are also present in the above image of a patient with Wilson disease.
Image courtesy of Manolette R. Roque, MD, MBA.
What the Eyes Tell You: 15 Abnormalities of the Lens
Manolette R. Roque, MD, MBA | January 13, 2023 | Contributor Information
Phacolytic Glaucoma
The image above shows a white hypermature cataract, mid-dilated pupil (non-reactive) with corneal haze (bedewing), and lack of keratic precipitates (as differentiated from phacoantigenic glaucoma). The usual presentation consists of a sudden onset of eye pain and redness in an eye with a known white cataract and poor vision. The pain occurs from increased intraocular pressure resulting from leaked lens proteins and engorged macrophages clogging the trabecular meshwork.[19]
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Contributor Information
Manolette R. Roque, MD, MBA
Chief of Section on Cataract and Refractive Surgery
Department of Ophthalmology
Asian Hospital and Medical Center
Muntinlupa, Philippines;
Section Chief, Ocular Immunology and Uveitis
International Eye Institute
St Luke's Medical Center Global City
Taguig, Philippines
Disclosure: Manolette R. Roque, MD, MBA, has disclosed no relevant financial relationships.
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References
Roy FH. Lens. Ocular Differential Diagnosis. 9th ed. New Delhi, India: Jaypee Brothers Medical Publishers (P) Ltd; 2012: chapter 13.
Nema HV, Nema N. Diseases of the lens. Textbook of Ophthalmology. 6th ed. New Delhi, India: Jaypee Brothers Medical Publishers (P) Ltd; 2012: chapter 16.
Vanderveen DK. Pediatric cataracts and other lens abnormalities. In: Wright KW, Strube YNJ, eds. Pediatric Ophthalmology and Strabismus. 3rd ed. New York, NY: Oxford University Press; 2012: chapter 41.
Chen P, Hao X, Li W, Zhao X, Huang Y. Mutations in the TMCO3 gene are associated with cornea guttata and anterior polar cataract. Sci Rep. 2016 Aug 3;6:31021. PMID: 27484837 [Full text].
Na KS, Park YG, Han K, Mok JW, Joo CK. Prevalence of and risk factors for age-related and anterior polar cataracts in a Korean population. PLoS One. 2014 Jun 17;9(6):e96461. PMID: 24936893 [Full text].
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