| Ectopia lentis | |
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| Ectopia Lentis in Marfan syndrome. Zonular fibers are being seen. | |
| Specialty | Medical genetics  |
Ectopia lentis is a displacement or malposition of the eye's crystalline lens from its normal location. A partial dislocation of a lens is termed lens subluxation or subluxated lens; a complete dislocation of a lens is termed lens luxation or luxated lens.
Although observed in humans and cats, ectopia lentis is most commonly seen in dogs. Ciliary zonules normally hold the lens in place. Abnormal development of these zonules can lead to primary ectopia lentis, usually a bilateral condition. Luxation can also be a secondary condition, caused by trauma, cataract formation (decrease in lens diameter may stretch and break the zonules), or glaucoma (enlargement of the globe stretches the zonules). Steroid administration weakens the zonules and can lead to luxation, as well. Lens luxation in cats can occur secondary to anterior uveitis (inflammation of the inside of the eye).
With anterior lens luxation, the lens pushes into the iris or actually enters the anterior chamber of the eye. This can cause glaucoma, uveitis, or damage to the cornea. Uveitis (inflammation of the eye) causes the pupil to constrict (miosis) and trap the lens in the anterior chamber, leading to an obstruction of outflow of aqueous humour and subsequent increase in ocular pressure (glaucoma). [1] Better prognosis is valued in lens replacement surgery (retained vision and normal intraocular pressure) when it is performed before the onset of secondary glaucoma. [2] Glaucoma secondary to anterior lens luxation is less common in cats than dogs due to their naturally deeper anterior chamber and the liquification of the vitreous humour secondary to chronic inflammation. [3] Anterior lens luxation is considered to be an ophthalmological emergency.
With posterior lens luxation, the lens falls back into the vitreous humour and lies on the floor of the eye. This type causes fewer problems than anterior lens luxation, although glaucoma or ocular inflammation may occur. Surgery is used to treat dogs with significant symptoms. Removal of the lens before it moves to the anterior chamber may prevent secondary glaucoma. [2]
Lens subluxation is also seen in dogs and is characterized by a partial displacement of the lens. It can be recognized by trembling of the iris (iridodonesis) or lens (phacodonesis) and the presence of an aphakic crescent (an area of the pupil where the lens is absent). [4] Other signs of lens subluxation include mild conjunctival redness, vitreous humour degeneration, prolapse of the vitreous into the anterior chamber, and an increase or decrease of anterior chamber depth. [5] Removal of the lens before it completely luxates into the anterior chamber may prevent secondary glaucoma. [2] Extreme degree of luxation of lens is called "lenticele" in which lens comes out of the eyeball and becomes trapped under the Tenon's capsule or conjunctiva. [6] A nonsurgical alternative treatment involves the use of a miotic to constrict the pupil and prevent the lens from luxating into the anterior chamber. [7]
Terrier breeds are predisposed to lens luxation, and it is probably inherited in the Sealyham Terrier, Jack Russell Terrier, Wirehaired Fox Terrier, Rat Terrier, Teddy Roosevelt Terrier, Tibetan Terrier, [8] Miniature Bull Terrier, Shar Pei, and Border Collie. [9] The mode of inheritance in the Tibetan Terrier [5] and Shar Pei [10] is likely autosomal recessive. Labrador Retrievers and Australian Cattle Dogs are also predisposed. [11]
In humans, a number of systemic conditions are associated with ectopia lentis: [12]
More common:
Less common:
A subluxation is an incomplete or partial dislocation of a joint or organ. According to the World Health Organization, a subluxation is a "significant structural displacement" and is therefore visible on static imaging studies, such as X-rays. Unlike real subluxations, the pseudoscientific concept of a chiropractic "vertebral subluxation" may or may not be visible on x-rays.
The aqueous humour is a transparent water-like fluid similar to blood plasma, but containing low protein concentrations. It is secreted from the ciliary body, a structure supporting the lens of the eyeball. It fills both the anterior and the posterior chambers of the eye, and is not to be confused with the vitreous humour, which is located in the space between the lens and the retina, also known as the posterior cavity or vitreous chamber. Blood cannot normally enter the eyeball.
Cherry eye is a disorder of the nictitating membrane (NM), also called the third eyelid, present in the eyes of dogs and cats. Cherry eye is most often seen in young dogs under the age of two. Common misnomers include adenitis, hyperplasia, adenoma of the gland of the third eyelid; however, cherry eye is not caused by hyperplasia, neoplasia, or primary inflammation. In many species, the third eyelid plays an essential role in vision by supplying oxygen and nutrients to the eye via tear production. Normally, the gland can turn inside-out without detachment. Cherry eye results from a defect in the retinaculum which is responsible for anchoring the gland to the periorbita. This defect causes the gland to prolapse and protrude from the eye as a red fleshy mass. Problems arise as sensitive tissue dries out and is subjected to external trauma Exposure of the tissue often results in secondary inflammation, swelling, or infection. If left untreated, this condition can lead to dry eye syndrome and other complications.
Uveitis is inflammation of the uvea, the pigmented layer of the eye between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is described anatomically, by the part of the eye affected, as anterior, intermediate or posterior, or panuveitic if all parts are involved. Anterior uveitis (iridocyclitis) is the most common, with the incidence of uveitis overall affecting approximately 1:4500, most commonly those between the ages of 20-60. Symptoms include eye pain, eye redness, floaters and blurred vision, and ophthalmic examination may show dilated ciliary blood vessels and the presence of cells in the anterior chamber. Uveitis may arise spontaneously, have a genetic component, or be associated with an autoimmune disease or infection. While the eye is a relatively protected environment, its immune mechanisms may be overcome resulting in inflammation and tissue destruction associated with T-cell activation.
Aphakia is the absence of the lens of the eye, due to surgical removal, such as in cataract surgery, a perforating wound or ulcer, or congenital anomaly. It causes a loss of ability to maintain focus (accommodation), high degree of farsightedness (hyperopia), and a deep anterior chamber. Complications include detachment of the vitreous or retina, and glaucoma.
The zonule of Zinn is a ring of fibrous strands forming a zonule that connects the ciliary body with the crystalline lens of the eye. These fibers are sometimes collectively referred to as the suspensory ligaments of the lens, as they act like suspensory ligaments.
A corneal ulcer, or ulcerative keratitis, is an inflammatory condition of the cornea involving loss of its outer layer. It is very common in dogs and is sometimes seen in cats. In veterinary medicine, the term corneal ulcer is a generic name for any condition involving the loss of the outer layer of the cornea, and as such is used to describe conditions with both inflammatory and traumatic causes.
Pigment dispersion syndrome (PDS) is an eye disorder that can lead to a form of glaucoma known as pigmentary glaucoma. It takes place when pigment cells slough off from the back of the iris and float around in the aqueous humor. Over time, these pigment cells can accumulate in the anterior chamber in such a way that they begin to clog the trabecular meshwork, which can in turn prevent the aqueous humour from draining and therefore increases the pressure inside the eye. A common finding in PDS are central, vertical corneal endothelial pigment deposits, known as Krukenberg spindle. With PDS, the intraocular pressure tends to spike at times and then can return to normal. Exercise has been shown to contribute to spikes in pressure as well. When the pressure is great enough to cause damage to the optic nerve, this is called pigmentary glaucoma. As with all types of glaucoma, when damage happens to the optic nerve fibers, the vision loss that occurs is irreversible and painless.
Hypopyon is a medical condition involving inflammatory cells in the anterior chamber of the eye.
Persistent fetal vasculature(PFV), also known as persistent fetal vasculature syndrome (PFVS), and until 1997 known primarily as persistent hyperplastic primary vitreous (PHPV), is a rare congenital anomaly which occurs when blood vessels within the developing eye, known as the embryonic hyaloid vasculature network, fail to regress as they normally would in-utero after the eye is fully developed. Defects which arise from this lack of vascular regression are diverse; as a result, the presentation, symptoms, and prognosis of affected patients vary widely, ranging from clinical insignificance to irreversible blindness. The underlying structural causes of PFV are considered to be relatively common, and the vast majority of cases do not warrant additional intervention. When symptoms do manifest, however, they are often significant, causing detrimental and irreversible visual impairment. Persistent fetal vasculature heightens the lifelong risk of glaucoma, cataracts, intraocular hemorrhages, and Retinal detachments, accounting for the visual loss of nearly 5% of the blind community in the developed world. In diagnosed cases of PFV, approximately 90% of patients with a unilateral disease have associated poor vision in the affected eye.
Canine glaucoma refers to a group of diseases in dogs that affect the optic nerve and involve a loss of retinal ganglion cells in a characteristic pattern. An intraocular pressure greater than 22 mmHg (2.9 kPa) is a significant risk factor for the development of glaucoma. Untreated glaucoma in dogs leads to permanent damage of the optic nerve and resultant visual field loss, which can progress to blindness.
Secondary glaucoma is a collection of progressive optic nerve disorders associated with a rise in intraocular pressure (IOP) which results in the loss of vision. In clinical settings, it is defined as the occurrence of IOP above 21 mmHg requiring the prescription of IOP-managing drugs. It can be broadly divided into two subtypes: secondary open-angle glaucoma and secondary angle-closure glaucoma, depending on the closure of the angle between the cornea and the iris. Principal causes of secondary glaucoma include optic nerve trauma or damage, eye disease, surgery, neovascularization, tumours and use of steroid and sulfa drugs. Risk factors for secondary glaucoma include uveitis, cataract surgery and also intraocular tumours. Common treatments are designed according to the type and the underlying causative condition, in addition to the consequent rise in IOP. These include drug therapy, the use of miotics, surgery or laser therapy.
Schwartz–Matsuo syndrome is a human eye disease characterised by rhegmatogenous retinal detachment, elevated intraocular pressure (IOP) and open angle of anterior chamber.
Uveitis–glaucoma–hyphaema (UGH) syndrome, also known as Ellingson syndrome, is a complication of cataract surgery, caused by intraocular lens subluxation or dislocation. The chafing of mispositioned intraocular lens over iris, ciliary body or iridocorneal angle cause elevated intraocular pressure (IOP) anterior uveitis and hyphema. It is most commonly caused by anterior chamber IOLs and sulcus IOLs but, the condition can be seen with any type of IOL, including posterior chamber lenses and cosmetic iris implants.
Posner–Schlossman syndrome (PSS) also known as glaucomatocyclitic crisis (GCC) is a rare acute ocular condition with unilateral attacks of mild granulomatous anterior uveitis and elevated intraocular pressure. It is sometimes considered as a secondary inflammatory glaucoma.
Ghost cell glaucoma (GCG) is a type of secondary glaucoma occurs due to long standing vitreous hemorrhage. The rigid and less pliable degenerated red blood cells block the trabecular meshwork and increase the pressure inside eyes.
Uveitic glaucoma is most commonly a progression stage of noninfectious anterior uveitis or iritis.
Anterior chamber paracentesis (ACP) is a surgical procedure done to reduce intraocular pressure (IOP) of the eye. The procedure is used in management of glaucoma and uveitis. It is also used for clinical diagnosis of infectious uveitis.
The crystalline lens inside the human eye has been implicated as a causative factor in many forms of glaucoma. Lens induced glaucomas or Lens related glaucomas are either open-angle or closed-angle glaucomas that can occur due to a neglected advanced cataract or a dislocated lens. It is a type of secondary glaucoma. The angle-closure glaucoma can be caused by a swollen or dislocated lens. The open-angle glaucoma can be caused by leakage of lens proteins through the capsule of a mature or hyper mature cataract or by a hypersensitivity reaction to own lens protein following surgery or penetrating trauma.
Panuveitis also known as Diffuse uveitis or Total uveitis is an eye disease affecting the internal structures of the eye. In this inflammation occurs throughout the uveal tract, with no specific areas of predominant inflammation. In most cases, along with the uvea, the retina, vitreous humor, optic nerve or lens are also involved.