Childhood cataract

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Childhood cataract is cataract that occurs at birth or in childhood. [1] It may be congenital or acquired.

Contents

Congenital cataracts are defined as the presence of lens opacification during childhood. [2] About 1.14 million children in the world are blind. [3] Cataracts are the leading cause of blindness in children. [4]

About

Prevalence

Childhood cataracts are the primary cause of childhood blindness. [4] Childhood cataracts make up 7.4% to 15.3% of blindness in kids. [4] The prevalence of childhood cataracts ranges from 0.63/10,000 to 9.74/10,000 children, with a median of 1.71. [5] This is dependent on factors like economic status but not gender or laterality. [5] The incidence of childhood cataracts ranges from 1.8/10,000 to 3.6/10,000 children per year. [5]

Genetics

Childhood cataracts are hereditary in 8.3% to 25% of cases. [4] Of which, 75% of cases are inherited in an autosomal dominant fashion. [4] The diagnosis of childhood cataracts are associated with other eye abnormalities in 27% of cases and associated with systemic abnormalities/findings in 22% of cases. [4]

Disease

Causes

There are several causes of childhood cataracts. [2] [4] A few of these causes are detailed below.

Idiopathic

Most cases of childhood cataracts, both unilateral and bilateral, are idiopathic in nature. [2] [4]

Hereditary

Congenital cataracts are typically inherited in an autosomal dominant pattern with incomplete inheritance. [4] There are multiple different genetic disorders associated with congenital cataracts: Norrie disease (X-linked recessive inheritance), Nance-Horan syndrome (X-linked recessive inheritance), Down's syndrome (also associated with other eye abnormalities like nystagmus and strabismus), and Lowe syndrome (X-linked inheritance). [4]

Metabolic

One specific metabolic disorder associated with congenital cataracts is Galactosemia. [4]

Congenital Infections

Congenital infections include Toxoplasma, Rubella, Cytomegalovirus (CMV), Herpes, and Syphilis. [4] The most common congenital infection to cause congenital cataracts is Rubella. [4] Rubella is especially common, with a higher incidence in India. [4] Rubella is characterized by a triad of features: congenital cataracts, sensorineural hearing loss, and patent ductus arteriosus (a congenital heart condition). [4]

Trauma

Trauma can cause cataracts in childhood. [2] [4] There are different types of traumatic injury that cause cataracts in different parts of the world. Child abuse may cause traumatic injury to the eye and cataracts. [2] Open globe injuries are more often associated with cataracts than closed globe injuries. [4] The leading cause of these open globe injuries are bow and arrow injuries. [4]

Diagnosis

Cataracts in childhood are often detected via routine screening. [4]

Signs & Symptoms

The first sign is usually leukocoria, meaning white pupil upon fundoscopic exam. [4] Another sign is if the child has been struggling to follow objects with their eyes or has been making decreased eye contact with family members. [4] Other general complaints associated with childhood cataracts include kids squeezing their eyes shut in response to bright lights, squinting of eyes, history of small set eyes or large set eyes, and nystagmus. [4] Older kids are more likely to complain of the following: difficulty viewing objects at a distance, unable to view whiteboard in class, parent may notice children are bringing objects closer to their face. [4]

History

Routine screening is the method in which cataracts are typically diagnosed in childhood. [4] A thorough history is obtained to assist in making this diagnosis. [4] The following are questions to consider addressing in a visit: "at what age did the symptoms begin?", "how long have the symptoms been present?", "is there a family history of childhood cataracts?", "is there a history of pregnancy complications or symptoms?", "any drug use during pregnancy?", "is there history of trauma during pregnancy?", "does the child have any other accompanying symptoms like vomiting?". [4] A specific question regarding vomiting is asked as it may lead to increased suspicion for Galactosemia. [4]

Physical Examination

A complete physical examination is done to identify if a child has any associated systemic symptoms which may aid in a diagnosis of childhood cataracts. [4] In addition, a complete eye examination is done. [2] [4] This eye exam includes fixation, pupillary reflex test, and test for visual acuity. [4] Visual acuity is tested differently based on patient's age. For infants, visual acuity can be tested by visual evoked response, Catford drum, optokinetic nystagmus, and Teller's acuity cards tests. [4] For children aged 1–2 years old, visual acuity can be tested by Worth's ivory ball test, Boeck's candy test, the "Screening Test for Young Children and Retards", and Cardiff's acuity test. [4] For children aged 2–3 years old, visual acuity can be tested by miniature toy test, coin test, and LEA symbols tests. [4] For children aged 3–5 years old, visual acuity can be tested by Allen's picture card, Lippman's HOTV test, and letter test. [4] For children greater than age 5, visual acuity can be tested by Tumbling E, Landolt's broken ring, Snellen's chart, and LogMAR chart tests. [4]

Treatment

Early detection is crucial in treatment of congenital cataracts. [2] [6] Prognosis is good if cataracts are detected early and surgery occurs aged 6 weeks for unilateral cataracts and at 8 weeks for bilateral cataracts. [6] Favorable outcomes are associated with timely surgery, appropriate postoperative care, and visual rehabilitation postoperatively. [4] Postoperative care requires effective communication between an interdisciplinary team including parents, pediatricians, surgeons, anesthesiologists, and optometrists. [4]

In order to decrease presentations of avoidable blindness in childhood, it is important to focus on maternal and neonatal health prior to symptomatic presentation. [3]

Surgical Indications

There are multiple indications for cataract surgery. [2] An absolute indication signifies need for surgical correction. [2] Surgical correction of the cataract is indicated when lens opacification blocks the ref reflex and dilated pupils are detected, strabismus with unilateral cataract, or nystagmus with bilateral cataracts. [2]

Postoperative Care

After surgery, postoperative care is necessary for recovery. [2] Topical corticosteroids are used to decrease postoperative optical inflammation. [2] Topical antibiotics are used to decrease risk of infection after surgery. [2] Topical anesthetics are used for postoperative pain. [2] Contact lens are used immediately after surgery to allow for visual rehabilitation. [2] Visual rehabilitation decreases risk of strabismus, amblyopia, and poor fusion after cataract surgery. [2]

Complications

Untreated childhood cataracts can cause emotional, financial, and societal burdens. [4] Even when treated, childhood cataracts may be associated with some complications. [2] About 10% to 25% of children have been diagnosed with glaucoma after cataract surgery. [2] [4] The most common complication after cataract surgery in childhood is visual axis opacification. [2] [4] Cataract surgery may be associated with decreased accommodation. [2]

See also

Related Research Articles

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Glaucoma is a group of eye diseases that result in damage to the optic nerve and cause vision loss. The most common type is open-angle glaucoma, in which the drainage angle for fluid within the eye remains open, with less common types including closed-angle glaucoma and normal-tension glaucoma. Open-angle glaucoma develops slowly over time without pain. Peripheral vision may begin to decrease, followed by central vision, resulting in blindness if not treated. Closed-angle glaucoma can present gradually or suddenly. The sudden presentation may involve severe eye pain, blurred vision, mid-dilated pupil, redness of the eye, and nausea. Vision loss from glaucoma, once it has occurred, is permanent. Eyes affected by glaucoma are referred to as being glaucomatous.

<span class="mw-page-title-main">Cataract</span> Clouding of the lens inside the eye causing poor vision

A cataract is a cloudy area in the lens of the eye that leads to a decrease in vision. Cataracts often develop slowly and can affect one or both eyes. Symptoms may include faded colours, blurry or double vision, halos around light, trouble with bright lights, and difficulty seeing at night. This may result in trouble driving, reading, or recognizing faces. Poor vision caused by cataracts may also result in an increased risk of falling and depression. Cataracts cause 51% of all cases of blindness and 33% of visual impairment worldwide.

<span class="mw-page-title-main">Esotropia</span> Form of strabismus

Esotropia is a form of strabismus in which one or both eyes turns inward. The condition can be constantly present, or occur intermittently, and can give the affected individual a "cross-eyed" appearance. It is the opposite of exotropia and usually involves more severe axis deviation than esophoria. Esotropia is sometimes erroneously called "lazy eye", which describes the condition of amblyopia; a reduction in vision of one or both eyes that is not the result of any pathology of the eye and cannot be resolved by the use of corrective lenses. Amblyopia can, however, arise as a result of esotropia occurring in childhood: In order to relieve symptoms of diplopia or double vision, the child's brain will ignore or "suppress" the image from the esotropic eye, which when allowed to continue untreated will lead to the development of amblyopia. Treatment options for esotropia include glasses to correct refractive errors, the use of prisms, orthoptic exercises, or eye muscle surgery. The term is from Greek eso meaning "inward" and trope meaning "a turning".

<span class="mw-page-title-main">Strabismus</span> Eyes not aligning when looking at something

Strabismus is a vision disorder in which the eyes do not properly align with each other when looking at an object. The eye that is pointed at an object can alternate. The condition may be present occasionally or constantly. If present during a large part of childhood, it may result in amblyopia, or lazy eyes, and loss of depth perception. If onset is during adulthood, it is more likely to result in double vision.

<span class="mw-page-title-main">Amblyopia</span> Failure of the brain to process input from one eye

Amblyopia, also called lazy eye, is a disorder of sight in which the brain fails to fully process input from one eye and over time favors the other eye. It results in decreased vision in an eye that typically appears normal in other aspects. Amblyopia is the most common cause of decreased vision in a single eye among children and younger adults.

<span class="mw-page-title-main">Aniridia</span> Absence of the iris, usually involving both eyes

Aniridia is the absence of the iris, a muscular structure that opens and closes the pupil to allow light into the eye. It is also responsible for eye color. Without it, the central eye appears all black. It can be congenital, in which both eyes are usually involved, or caused by a penetrant injury. Isolated aniridia is a congenital disorder which is not limited to a defect in iris development, but is a panocular condition with macular and optic nerve hypoplasia, cataract, and corneal changes. Vision may be severely compromised and the disorder is frequently associated with a number of ocular complications: nystagmus, amblyopia, buphthalmos, and cataract. Aniridia in some individuals occurs as part of a syndrome, such as WAGR syndrome, or Gillespie syndrome.

Optic nerve hypoplasia (ONH) is a medical condition arising from the underdevelopment of the optic nerve(s). This condition is the most common congenital optic nerve anomaly. The optic disc appears abnormally small, because not all the optic nerve axons have developed properly. It is often associated with endocrinopathies, developmental delay, and brain malformations. The optic nerve, which is responsible for transmitting visual signals from the retina to the brain, has approximately 1.2 million nerve fibers in the average person. In those diagnosed with ONH, however, there are noticeably fewer nerves.

<span class="mw-page-title-main">Nyctalopia</span> Condition making it difficult or impossible to see in relatively low light

Nyctalopia, also called night-blindness, is a condition making it difficult or impossible to see in relatively low light. It is a symptom of several eye diseases. Night blindness may exist from birth, or be caused by injury or malnutrition. It can be described as insufficient adaptation to darkness.

<span class="mw-page-title-main">Coloboma</span> Hole in one of the structures of the eye

A coloboma is a hole in one of the structures of the eye, such as the iris, retina, choroid, or optic disc. The hole is present from birth and can be caused when a gap called the choroid fissure, which is present during early stages of prenatal development, fails to close up completely before a child is born. Ocular coloboma is relatively uncommon, affecting less than one in every 10,000 births.

Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as corrective eyewear, assistive devices, and medical treatment– visual impairment may cause the individual difficulties with normal daily tasks including reading and walking. Low vision is a functional definition of visual impairment that is chronic, uncorrectable with treatment or conventional corrective lenses, and impacts daily living. As such low vision can be used as a disability metric and varies based on an individual's experience, environmental demands, accommodations, and access to services. The American Academy of Ophthalmology defines visual impairment as the best-corrected visual acuity of less than 20/40 in the better eye, and the World Health Organization defines it as a presenting acuity of less than 6/12 in the better eye. The term blindness is used for complete or nearly complete vision loss. In addition to the various permanent conditions, fleeting temporary vision impairment, amaurosis fugax, may occur, and may indicate serious medical problems. The abbreviation VIP is sometimes used for Visually Impaired Person, Persons or People.

Leber congenital amaurosis (LCA) is a rare inherited eye disease that appears at birth or in the first few months of life.

Polycoria is a pathological condition of the eye characterized by more than one pupillary opening in the iris. It may be congenital or result from a disease affecting the iris. It results in decreased function of the iris and pupil, affecting the physical eye and visualization.

<span class="mw-page-title-main">Emmetropia</span> State of vision

Emmetropia is the state of vision in which a faraway object at infinity is in sharp focus with the ciliary muscle in a relaxed state. That condition of the normal eye is achieved when the refractive power of the cornea and eye lens and the axial length of the eye balance out, which focuses rays exactly on the retina, resulting in perfectly sharp distance vision. A human eye in a state of emmetropia requires no corrective lenses for distance; the vision scores well on a visual acuity test.

Infantile esotropia is an ocular condition of early onset in which one or either eye turns inward. It is a specific sub-type of esotropia and has been a subject of much debate amongst ophthalmologists with regard to its naming, diagnostic features, and treatment.

<span class="mw-page-title-main">Nystagmus</span> Dysfunction of eye movement

Nystagmus is a condition of involuntary eye movement. People can be born with it but more commonly acquire it in infancy or later in life. In many cases it may result in reduced or limited vision.

<span class="mw-page-title-main">Persistent fetal vasculature</span> Medical condition

Persistent fetal vasculature(PFV), also known as persistent fetal vasculature syndrome (PFSV), 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.

<span class="mw-page-title-main">Congenital cataract</span> Medical condition

Congenital cataracts are a lens opacity that is present at birth. Congenital cataracts occur in a broad range of severity. Some lens opacities do not progress and are visually insignificant, others can produce profound visual impairment.

Cyclotropia is a form of strabismus in which, compared to the correct positioning of the eyes, there is a torsion of one eye about the eye's visual axis. Consequently, the visual fields of the two eyes appear tilted relative to each other. The corresponding latent condition – a condition in which torsion occurs only in the absence of appropriate visual stimuli – is called cyclophoria.

<span class="mw-page-title-main">Congenital blindness</span>

Congenital blindness refers to blindness present at birth. Congenital blindness is sometimes used interchangeably with "Childhood Blindness." However, current literature has various definitions of both terms. Childhood blindness encompasses multiple diseases and conditions present in ages up to 16 years old, which can result in permanent blindness or severe visual impairment over time. Congenital blindness is a hereditary disease and can be treated by gene therapy. Visual loss in children or infants can occur either at the prenatal stage or postnatal stage. There are multiple possible causes of congenital blindness. In general, 60% of congenital blindness cases are contributed from prenatal stage and 40% are contributed from inherited disease. However, most of the congenital blindness cases show that it can be avoidable or preventable with early treatment.

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

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. The term corneal blindness is commonly used to describe blindness due to corneal opacity.

References

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Further reading