Corneal opacity

Last updated
Corneal opacity
Other namesCorneal scarring
Corneal opacity right eye 2.jpg
Complete corneal opacity in right eye with normal left eye
Specialty Ophthalmology
Symptoms Loss of vision
Complications Blindness
Usual onsetCongenital or acquired
Treatment Corneal transplantation
Frequency4th main cause of blindness globally (5.1%). [1]

The human cornea is a transparent membrane which allows light to pass through it. The word corneal opacification literally means loss of normal transparency of cornea. The term corneal opacity is used particularly for the loss of transparency of cornea due to scarring. Transparency of the cornea is dependent on the uniform diameter and the regular spacing and arrangement of the collagen fibrils within the stroma. Alterations in the spacing of collagen fibrils in a variety of conditions including corneal edema, scars, and macular corneal dystrophy is clinically manifested as corneal opacity. [2] The term corneal blindness is commonly used to describe blindness due to corneal opacity.

Contents

Types

Corneal opacity with neovascularization Corneaneo.jpg
Corneal opacity with neovascularization

Depending on the density, corneal opacity is graded as nebular, macular and leucomatous.

Nebular corneal opacity

Nebular corneal opacity is a faint opacity which results due to superficial scars involving Bowman's layer and superficial stroma. A nebular corneal opacity allows the details of the iris to be seen through the opacity. A thin, diffuse nebula covering the pupillary area interferes more with vision than a strictly localized dense leucoma, so long as the latter does not block the whole pupillary area. This is because the leucoma stops all the light which falls upon it, whereas the nebula refracts it irregularly, allowing many of the rays to fall upon the retina where they blur the image formed by the regularly refracted rays. [3]

Macular corneal opacity

Macular corneal opacity is a semidense opacity produced when scarring involves about half the corneal stroma. [4]

Leucomatous corneal opacity (leucoma simplex)

Leucomatous corneal opacity is a dense white opacity which results due to scarring of more than half of the stroma. [4] A number of different presentations of leucomatous corneal opacity exist:

Presentation

Signs and symptoms include the following:

Complications

Congenital corneal opacity that affecting vision will cause amblyopia. That type of amblyopia is known as form-deprivation amblyopia (or amblyopia ex anopsia). [9]

Secondary changes in corneal opacity

Secondary changes may be seen in long-standing cases include: hyaline degeneration, calcareous degeneration, pigmentation and atheromatous ulceration. [4]

Causes

Corneal opacity or "clouding" also gradually appears after death, and can be used to estimate the postmortem interval (PMI). Postmortem changes of the eye.jpg
Corneal opacity or "clouding" also gradually appears after death, and can be used to estimate the postmortem interval (PMI).

Genetics

Pediatric corneal opacities may be congenital or acquired.

Congenital corneal opacities

Congenital reasons for this condition include:

Acquired pediatric corneal opacities

Acquired reasons for this condition include:

Treatment

Keratoplasty

Keratoplasty also known as corneal transplantation is the main treatment option for visual improvement in corneal opacity. In this, the opaque cornea is replaced with donor tissue. Depending on type and density of corneal opacity different types of keratoplasty may be used such as: [28]

Optical iridectomy

Optical iridectomy creates a clear entrance pupil, improving vision in patients with segmental corneal opacities. An area of clear peripheral cornea can produce retinal images compatible with good visual acuity. [30]

Phototherapeutic keratectomy (PTK)

Excimer laser phototherapeutic keratectomy (PTK) is useful in superficial (nebular) corneal opacities. [31]

Tattooing of scar

Keratopigmentation or corneal tattooing is a procedure used for centuries to improve the cosmetic appearance of corneal scars. Tattooing will not improve vision. For tattooing procedure Indian black ink, gold or platinum may be used. [4]

Techniques

  • Staining method: In this technique, tattoo ink is directly applied to anterior surface of cornea. Benefits of this procedure include fast procedure with uniform dye application. Risk of fading is a main drawback. [32]
  • Femtosecond laser-assisted corneal tattooing: Femtosecond laser-assisted corneal tattooing is a new corneal tattooing technique, with many benefits. [33]

Keratoprosthesis

Keratoprosthesis is a surgical procedure where damaged or opaque cornea is replaced with an artificial cornea. Artificial corneas currently in commercial use include Boston keratoprosthesis, Osteo-Odonto-Keratoprosthesis (OOKP), AlphaCor, KeraKlear Artificial Cornea etc. [34] [35]

Epidemiology

Corneal opacity is the 4th main cause of blindness globally (5.1%). [1] Using the World Health Organization's (WHO; Geneva, Switzerland) blindness definition,1 45 million people worldwide are bilaterally blind, of which 6 to 8 million are blind due to corneal disease. In some African areas, nearly 90% of the total blindness is due to corneal pathology. [36]

Europe

The prevalence of congenital corneal opacities (CCO) is estimated to be 3 in 100,000 newborns. This number increases to 6 in 100,000 if congenital glaucoma patients are included. A study of live births in Spain reported that corneal opacities accounted for 3.11% of congenital eye malformations (Bermejo et al, 1998). About 4% of keratoplasties done in the pediatric population in Denmark are due to congenital anomalies (Hovlykke et al, 2014). [37]

India

In NPCB survey (2001-2002) Corneal opacity was the 6th major cause of blindness in India, which accounts for 0.9% of total blind population. In the RAAB (Rapid Assessment of Avoidable Blindness) survey (2006-2007) Corneal opacity including Trachoma was mentioned as the second major cause of blindness, which accounts for 6.5% of total blindness. [38]

See also

Related Research Articles

<span class="mw-page-title-main">Cornea</span> Transparent front layer of the eye

The cornea is the transparent front part of the eye that covers the iris, pupil, and anterior chamber. Along with the anterior chamber and lens, the cornea refracts light, accounting for approximately two-thirds of the eye's total optical power. In humans, the refractive power of the cornea is approximately 43 dioptres. The cornea can be reshaped by surgical procedures such as LASIK.

<span class="mw-page-title-main">Corneal endothelium</span>

The corneal endothelium is a single layer of endothelial cells on the inner surface of the cornea. It faces the chamber formed between the cornea and the iris.

<span class="mw-page-title-main">Corneal transplantation</span> Surgical procedure of repairing corneal tissue to treat corneal blindness

Corneal transplantation, also known as corneal grafting, is a surgical procedure where a damaged or diseased cornea is replaced by donated corneal tissue. When the entire cornea is replaced it is known as penetrating keratoplasty and when only part of the cornea is replaced it is known as lamellar keratoplasty. Keratoplasty simply means surgery to the cornea. The graft is taken from a recently deceased individual with no known diseases or other factors that may affect the chance of survival of the donated tissue or the health of the recipient.

<span class="mw-page-title-main">Fuchs' dystrophy</span> Medical condition

Fuchs dystrophy, also referred to as Fuchs endothelial corneal dystrophy (FECD) and Fuchs endothelial dystrophy (FED), is a slowly progressing corneal dystrophy that usually affects both eyes and is slightly more common in women than in men. Although early signs of Fuchs dystrophy are sometimes seen in people in their 30s and 40s, the disease rarely affects vision until people reach their 50s and 60s.

<span class="mw-page-title-main">Descemet's membrane</span> Membrane in the cornea of the eye

Descemet's membrane is the basement membrane that lies between the corneal proper substance, also called stroma, and the endothelial layer of the cornea. It is composed of different kinds of collagen than the stroma. The endothelial layer is located at the posterior of the cornea. Descemet's membrane, as the basement membrane for the endothelial layer, is secreted by the single layer of squamous epithelial cells that compose the endothelial layer of the cornea.

<span class="mw-page-title-main">Corneal dystrophy</span> Medical condition

Corneal dystrophy is a group of rare hereditary disorders characterised by bilateral abnormal deposition of substances in the transparent front part of the eye called the cornea.

Iridocorneal endothelial (ICE) syndromes are a spectrum of diseases characterized by slowly progressive abnormalities of the corneal endothelium and features including corneal edema, iris distortion, and secondary angle-closure glaucoma. ICE syndromes are predominantly unilateral and nonhereditary. The condition occurs in predominantly middle-aged women.

<span class="mw-page-title-main">Corneal neovascularization</span> Medical condition

Corneal neovascularization (CNV) is the in-growth of new blood vessels from the pericorneal plexus into avascular corneal tissue as a result of oxygen deprivation. Maintaining avascularity of the corneal stroma is an important aspect of corneal pathophysiology as it is required for corneal transparency and optimal vision. A decrease in corneal transparency causes visual acuity deterioration. Corneal tissue is avascular in nature and the presence of vascularization, which can be deep or superficial, is always pathologically related.

<span class="mw-page-title-main">Bullous keratopathy</span> Medical condition

Bullous keratopathy, also known as pseudophakic bullous keratopathy (PBK), is a pathological condition in which small vesicles, or bullae, are formed in the cornea due to endothelial dysfunction.

<span class="mw-page-title-main">Meesmann corneal dystrophy</span> Medical condition

Meesmann corneal dystrophy (MECD) is a rare hereditary autosomal dominant disease that is characterized as a type of corneal dystrophy and a keratin disease. MECD is characterized by the formation of microcysts in the outermost layer of the cornea, known as the anterior corneal epithelium. The anterior corneal epithelium also becomes fragile. This usually affects both eyes rather than a single eye and worsens over time. There are two phenotypes, Meesmann corneal dystrophy 1 (MECD1) and Meesmann corneal dystrophy 2 (MECD2), which affect the genes KRT3 and KRT12, respectively. A heterozygous mutation in either of these genes will lead to a single phenotype. Many with Meesmann corneal dystrophy are asymptomatic or experience mild symptoms.

<span class="mw-page-title-main">Macular corneal dystrophy</span> Medical condition

Macular corneal dystrophy, also known as Fehr corneal dystrophy, is a rare pathological condition affecting the stroma of cornea first described by Arthur Groenouw in 1890. Signs are usually noticed in the first decade of life and progress afterwards, with opacities developing in the cornea and attacks of pain. This gradual opacification leads to visual impairment often requiring keratoplasty in the later decades of life.

<span class="mw-page-title-main">Corneal dystrophies in dogs</span>

Corneal dystrophies are a group of diseases that affect the cornea in dogs.

<span class="mw-page-title-main">Congenital stromal corneal dystrophy</span> Medical condition

Congenital stromal corneal dystrophy (CSCD) is an extremely rare, autosomal dominant form of corneal dystrophy. Only 4 families have been reported to have the disease by 2009. The main features of the disease are numerous opaque flaky or feathery areas of clouding in the stroma that multiply with age and eventually preclude visibility of the endothelium. Strabismus or primary open angle glaucoma was noted in some of the patients. Thickness of the cornea stays the same, Descemet's membrane and endothelium are relatively unaffected, but the fibrils of collagen that constitute stromal lamellae are reduced in diameter and lamellae themselves are packed significantly more tightly.

<span class="mw-page-title-main">Anterior segment mesenchymal dysgenesis</span> Medical condition

Anterior segment mesenchymal dysgenesis, or simply anterior segment dysgenesis (ASD), is a failure of the normal development of the tissues of the anterior segment of the eye. It leads to anomalies in the structure of the mature anterior segment, associated with an increased risk of glaucoma and corneal opacity.

<span class="mw-page-title-main">Congenital hereditary endothelial dystrophy</span> Medical condition

Congenital hereditary corneal dystrophy (CHED) is a form of corneal endothelial dystrophy that presents at birth.

Dua's layer, according to a 2013 paper by Harminder Singh Dua's group at the University of Nottingham, is a layer of the cornea that had not been detected previously. It is hypothetically 15 micrometres thick, the fourth caudal layer, and located between the corneal stroma and Descemet's membrane. Despite its thinness, the layer is very strong and impervious to air. It is strong enough to withstand up to 2 bars of pressure. While some scientists welcomed the announcement, other scientists cautioned that time was needed for other researchers to confirm the discovery and its significance. Others have met the claim "with incredulity".

<span class="mw-page-title-main">Primary juvenile glaucoma</span> Medical condition

Primary juvenile glaucoma is glaucoma that develops due to ocular hypertension and is evident either at birth or within the first few years of life. It is caused due to abnormalities in the anterior chamber angle development that obstruct aqueous outflow in the absence of systemic anomalies or other ocular malformation.

<span class="mw-page-title-main">Corneal hydrops</span> Medical condition

Corneal hydrops is an uncommon complication seen in people with advanced keratoconus or other corneal ectatic disorders, and is characterized by stromal edema due to leakage of aqueous humor through a tear in Descemet's membrane. Although a hydrops usually causes increased scarring of the cornea, occasionally it will benefit a patient by creating a flatter cone, aiding the fitting of contact lenses. Corneal transplantation is not usually indicated during corneal hydrops.

Pre Descemet's endothelial keratoplasty (PDEK) is a kind of endothelial keratoplasty, where the pre descemet's layer (PDL) along with descemet's membrane (DM) and endothelium is transplanted. Conventionally in a corneal transplantation, doctors use a whole cornea or parts of the five layers of the cornea to perform correction surgeries. In May 2013, Dr Harminder Dua discovered a sixth layer between the stroma and the descemet membrane which was named after him as the Dua's layer. In the PDEK technique, doctors take the innermost two layers of the cornea, along with the Dua's layer and graft it in the patient's eye.

Descemet membrane endothelial keratoplasty (DMEK) is a method of corneal transplantation. The DMEK technique involves the removal of a very thin sheet of tissue from the posterior side of a person's cornea, replacing it with the two innermost layers of corneal tissue from a donor's eyeball. The two corneal layers which are exchanged are the Descemet's membrane and the corneal endothelium. The person's corneal tissue is gently excised and replaced with the donor tissue via small 'clear corneal incisions' (small corneal incisions just anterior to the corneal limbus. The donor tissue is tamponaded against the person's exposed posterior corneal stroma by injecting a small air bubble into the anterior chamber. To ensure the air tamponade is effective, it is necessary for people to strictly posture so that they are looking up at the ceiling during the recovery period and until the air bubble has fully resorbed.

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