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Leukocoria

Updated : May 1, 2023





Background

Leukocoria, also known as white pupil reflex, is a medical condition in which the pupil of the eye appears white or yellowish instead of the normal black color. This abnormal reflection of light from the eye is caused by various conditions that affect the retina, lens, or vitreous of the eye, including congenital cataracts, retinoblastoma, Coats’ disease, persistent hyperplastic primary vitreous (PHPV), and other eye disorders.

Leukocoria is often a symptom of an underlying eye disease or condition, and it is usually identified in children during routine eye examinations or by parents who notice a white reflection in their child’s eye in photographs or in certain lighting conditions. Early detection and treatment of the underlying condition is important for preventing vision loss and other complications.

If left untreated, some conditions that cause leukocoria can result in blindness, eye tumors, or other serious eye problems. Therefore, it is essential to seek medical attention as soon as possible if you or your child have leukocoria or any other unusual changes in the appearance or function of the eyes.

Epidemiology

Leukocoria, also known as a white pupillary reflex, is a condition characterized by a white reflection from the eye. It is often associated with serious eye disorders such as retinoblastoma, cataracts, and Coats disease. The epidemiology of leukocoria varies depending on the underlying cause. Retinoblastoma, the most common malignant tumor of the eye in children, is the most common cause of leukocoria in infants and young children.

It affects approximately 1 in 15,000 to 20,000 live births and is more common in males than females. Cataracts, which are the leading cause of blindness worldwide, can also cause leukocoria. The prevalence of cataracts increases with age, with an estimated 20 million people worldwide affected by age-related cataracts. Coats disease, a rare condition that affects the blood vessels in the retina, can also cause leukocoria.

It primarily affects males and typically presents in children and young adults. Other less common causes of leukocoria include uveitis, retinopathy of prematurity, ocular toxocariasis, and retinal detachment. In summary, the epidemiology of leukocoria is dependent on the underlying cause. Retinoblastoma is the most common cause of leukocoria in infants and young children, while cataracts are the most common cause in older adults.

Anatomy

Pathophysiology

Leukocoria, also known as white pupillary reflex, is a condition that is caused by the reflection of light off the retina of the eye. The retina normally contains cells called photoreceptors that respond to light and transmit visual information to the brain. When the retina is healthy, light is absorbed by the photoreceptors and does not reflect back out of the eye. However, in certain conditions, the retina may become abnormal and reflect light back out of the eye, causing a white or opaque appearance in the pupil.

This can occur due to a variety of factors, including abnormalities in the structure of the eye or damage to the retina. In cases of retinoblastoma, for example, the leukocoria may be caused by a tumor that grows on the retina and reflects light back out of the eye. Similarly, in cases of congenital cataracts or other structural abnormalities, leukocoria may be caused by an obstruction in the passage of light through the eye.

Inflammatory conditions and infections can also cause leukocoria by causing damage to the retina or the structures around it. In these cases, the leukocoria may be a sign of inflammation, swelling, or other eye changes that affect light reflection. In summary, the pathophysiology of leukocoria varies depending on the underlying condition causing it. However, in general, it is caused by abnormalities in the retina or structures surrounding it that cause light to reflect back out of the eye.

Etiology

Leukocoria, also known as white pupillary reflex, is a medical condition characterized by a white reflection from the retina of the eye. This condition can be caused by a variety of underlying conditions, including:

Retinoblastoma: This is a type of eye cancer that occurs in young children. It is the most common cause of leukocoria in children. In some cases, the leukocoria may be the only visible symptom of retinoblastoma.

Congenital cataract: A congenital cataract is a clouding of the lens of the eye that is present at birth. It can cause leukocoria if it is large enough to obstruct the passage of light through the eye.

Coats disease: This is a rare eye disorder that affects the blood vessels behind the retina. It can cause exudation of fluid into the retina, leading to leukocoria.

Retinal detachment: Retinal detachment occurs when the retina separates from the underlying tissue. This can cause leukocoria if the detached area reflects light.

Persistent hyperplastic primary vitreous (PHPV): PHPV is a rare congenital eye abnormality that occurs when the blood vessels that nourish the eye during fetal development do not regress as they should. This can cause leukocoria due to the presence of a fibrovascular membrane in the eye.

Inflammatory eye conditions: Certain inflammatory eye conditions, such as uveitis and retinitis, can cause leukocoria.

Infections: Infections of the eye, such as retinitis caused by cytomegalovirus, can cause leukocoria.

It is important to note that leukocoria can also be a symptom of other underlying conditions, such as retinal tumors, optic nerve anomalies, and certain genetic disorders. It is essential to seek medical attention if you or your child experiences leukocoria to determine the underlying cause and receive appropriate treatment.

Genetics

Prognostic Factors

The prognosis of leukocoria (white pupillary reflex) depends on the underlying cause of the condition. In some cases, leukocoria can be a sign of a serious eye condition such as retinoblastoma, cataract, or retinal detachment. Early diagnosis and prompt treatment of these conditions are crucial for a favorable prognosis.

In cases of retinoblastoma, a cancer of the retina, early detection and treatment offer the best chance for a cure. The survival rate for retinoblastoma is high if the cancer is detected and treated before it spreads beyond the eye. Cataracts, a clouding of the eye’s lens, can be successfully treated with surgery, with a high success rate in restoring vision.

Retinal detachment, which occurs when the retina becomes separated from the underlying tissue, is a medical emergency that requires prompt treatment to prevent permanent vision loss. Other causes of leukocoria, such as Coats’ disease or toxocariasis, may require prompt treatment to prevent further vision loss or complications.

The prognosis for leukocoria also depends on the age of the patient and the severity of the underlying condition. In general, children who are diagnosed and treated early have a better chance of preserving their vision and achieving a good visual outcome. Overall, the prognosis of leukocoria varies depending on the underlying cause and the promptness of diagnosis and treatment. It is important to seek prompt evaluation by an ophthalmologist if you or your child experiences leukocoria.

Clinical History

Clinical history

The clinical history of leukocoria, or the appearance of a white reflex in the pupil of the eye, can provide important clues as to the underlying cause of the condition. A thorough clinical history should include the following information:

Age of onset: Leukocoria may be present from birth (congenital) or develop later in life. The age of onset can help narrow down potential causes.

Duration of symptoms: The duration of leukocoria can provide information about the progression of the underlying condition.

Presence of other symptoms: Leukocoria may be accompanied by other symptoms, such as pain, redness, or inflammation. These symptoms can provide important diagnostic clues.

Family history: Some causes of leukocoria, such as retinoblastoma, can be hereditary. A family history of similar symptoms or conditions should be noted.

Medical history: Certain medical conditions or medications may increase the risk of developing leukocoria or may be associated with specific causes of the condition.

Visual acuity: Changes in visual acuity, or the ability to see clearly, should be noted. Leukocoria may be accompanied by changes in visual acuity, such as blurry or distorted vision.

Previous eye examinations: Previous eye examinations or surgeries should be noted, as they can affect the current presentation of leukocoria.

A comprehensive clinical history, in conjunction with a physical exam and diagnostic testing, can help narrow down the potential underlying causes of leukocoria and guide appropriate treatment. It is important to seek medical attention if leukocoria is present, as early diagnosis and treatment can improve outcomes.

Physical Examination

Physical examination

Leukocoria is a medical condition characterized by a white reflex in the pupil of the eye. It is most commonly seen in children and can be an indicator of various eye diseases, including retinoblastoma, cataracts, and other vision-threatening conditions.

A physical examination of leukocoria may involve the following steps:

Visual acuity test: This test measures the clarity of vision in each eye. It can be done using an eye chart or other specialized tools.

Dilated fundus exam: During this exam, the pupil is dilated with eye drops, and the doctor uses a specialized lens to examine the retina and other structures at the back of the eye.

External eye exam: The doctor examines the external structures of the eye, including the eyelids, cornea, and conjunctiva.

Intraocular pressure test: This test measures the pressure inside the eye and can help diagnose conditions such as glaucoma.

Additional tests: Depending on the suspected underlying cause of leukocoria, additional tests such as an ultrasound or MRI may be ordered.

It is important to note that the specific details of a physical examination for leukocoria may vary depending on the individual case and the underlying condition suspected. It is important to consult a qualified healthcare provider for proper diagnosis and treatment.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

Leukocoria, or the appearance of a white reflex in the pupil of the eye, can be a symptom of a wide range of underlying conditions. Therefore, it is important to conduct a thorough differential diagnosis to determine the cause of the leukocoria. Some of the conditions that may need to be considered in the differential diagnosis of leukocoria include:

Retinoblastoma: This type of eye cancer occurs in young children and is the most common cause of leukocoria in children.

Congenital cataracts: A clouding of the eye’s lens that is present at birth and can cause leukocoria if the obstruction of the lens is large enough.

Coats disease: A rare eye disorder that affects the blood vessels behind the retina and can cause fluid to leak into the retina, leading to leukocoria.

Retinal detachment: Separation of the retina from the underlying tissue that can cause leukocoria if the detached area reflects light.

Persistent hyperplastic primary vitreous (PHPV): A rare congenital eye abnormality that occurs when the blood vessels that nourish the eye during fetal development do not regress as they should. This can cause leukocoria due to the presence of a fibrovascular membrane in the eye.

Inflammatory eye conditions: Certain inflammatory eye conditions, such as uveitis and retinitis, can cause leukocoria.

Infections: Infections of the eye, such as retinitis caused by cytomegalovirus, can cause leukocoria.

Optic nerve abnormalities: Certain optic nerve abnormalities, such as optic nerve glioma, can cause leukocoria.

Other eye tumors: Other eye tumors, such as choroidal melanoma or hemangioma, can cause leukocoria.

Genetic disorders: Certain genetic disorders, such as Norrie and Coat’s disease, can cause leukocoria.

The differential diagnosis of leukocoria will depend on the patient’s age, medical history, and other symptoms. It is essential to seek medical attention if leukocoria is present to determine the underlying cause and receive appropriate treatment.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The treatment of leukocoria depends on the underlying condition causing it. Leukocoria is not a disease in itself but rather a symptom of an underlying condition. Therefore, treatment is focused on addressing the underlying condition. Some potential treatment options include:

Retinoblastoma: Retinoblastoma is a type of cancer that can cause leukocoria. Treatment may involve surgery, chemotherapy, radiation therapy, or a combination of these approaches. The specific treatment plan will depend on the size and location of the tumor.

Cataracts: Cataracts can cause leukocoria, especially in older adults. Treatment typically involves surgery to remove the clouded lens and replace it with an artificial lens.

Other eye diseases: Other eye diseases that can cause leukocoria, such as Coats disease or retinal detachment, may require surgical intervention or other specialized treatment approaches.

Monitoring: In some cases, such as small retinoblastoma tumors, the doctor may recommend regular monitoring to ensure the condition does not worsen.

It is important to note that early detection and treatment of the underlying condition causing leukocoria is critical for preserving vision and preventing more serious complications. Therefore, it is important to seek prompt medical attention if you or your child experiences leukocoria or any other unusual eye symptoms.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK560794/

Leukocoria

Updated : May 1, 2023




Leukocoria, also known as white pupil reflex, is a medical condition in which the pupil of the eye appears white or yellowish instead of the normal black color. This abnormal reflection of light from the eye is caused by various conditions that affect the retina, lens, or vitreous of the eye, including congenital cataracts, retinoblastoma, Coats’ disease, persistent hyperplastic primary vitreous (PHPV), and other eye disorders.

Leukocoria is often a symptom of an underlying eye disease or condition, and it is usually identified in children during routine eye examinations or by parents who notice a white reflection in their child’s eye in photographs or in certain lighting conditions. Early detection and treatment of the underlying condition is important for preventing vision loss and other complications.

If left untreated, some conditions that cause leukocoria can result in blindness, eye tumors, or other serious eye problems. Therefore, it is essential to seek medical attention as soon as possible if you or your child have leukocoria or any other unusual changes in the appearance or function of the eyes.

Leukocoria, also known as a white pupillary reflex, is a condition characterized by a white reflection from the eye. It is often associated with serious eye disorders such as retinoblastoma, cataracts, and Coats disease. The epidemiology of leukocoria varies depending on the underlying cause. Retinoblastoma, the most common malignant tumor of the eye in children, is the most common cause of leukocoria in infants and young children.

It affects approximately 1 in 15,000 to 20,000 live births and is more common in males than females. Cataracts, which are the leading cause of blindness worldwide, can also cause leukocoria. The prevalence of cataracts increases with age, with an estimated 20 million people worldwide affected by age-related cataracts. Coats disease, a rare condition that affects the blood vessels in the retina, can also cause leukocoria.

It primarily affects males and typically presents in children and young adults. Other less common causes of leukocoria include uveitis, retinopathy of prematurity, ocular toxocariasis, and retinal detachment. In summary, the epidemiology of leukocoria is dependent on the underlying cause. Retinoblastoma is the most common cause of leukocoria in infants and young children, while cataracts are the most common cause in older adults.

Leukocoria, also known as white pupillary reflex, is a condition that is caused by the reflection of light off the retina of the eye. The retina normally contains cells called photoreceptors that respond to light and transmit visual information to the brain. When the retina is healthy, light is absorbed by the photoreceptors and does not reflect back out of the eye. However, in certain conditions, the retina may become abnormal and reflect light back out of the eye, causing a white or opaque appearance in the pupil.

This can occur due to a variety of factors, including abnormalities in the structure of the eye or damage to the retina. In cases of retinoblastoma, for example, the leukocoria may be caused by a tumor that grows on the retina and reflects light back out of the eye. Similarly, in cases of congenital cataracts or other structural abnormalities, leukocoria may be caused by an obstruction in the passage of light through the eye.

Inflammatory conditions and infections can also cause leukocoria by causing damage to the retina or the structures around it. In these cases, the leukocoria may be a sign of inflammation, swelling, or other eye changes that affect light reflection. In summary, the pathophysiology of leukocoria varies depending on the underlying condition causing it. However, in general, it is caused by abnormalities in the retina or structures surrounding it that cause light to reflect back out of the eye.

Leukocoria, also known as white pupillary reflex, is a medical condition characterized by a white reflection from the retina of the eye. This condition can be caused by a variety of underlying conditions, including:

Retinoblastoma: This is a type of eye cancer that occurs in young children. It is the most common cause of leukocoria in children. In some cases, the leukocoria may be the only visible symptom of retinoblastoma.

Congenital cataract: A congenital cataract is a clouding of the lens of the eye that is present at birth. It can cause leukocoria if it is large enough to obstruct the passage of light through the eye.

Coats disease: This is a rare eye disorder that affects the blood vessels behind the retina. It can cause exudation of fluid into the retina, leading to leukocoria.

Retinal detachment: Retinal detachment occurs when the retina separates from the underlying tissue. This can cause leukocoria if the detached area reflects light.

Persistent hyperplastic primary vitreous (PHPV): PHPV is a rare congenital eye abnormality that occurs when the blood vessels that nourish the eye during fetal development do not regress as they should. This can cause leukocoria due to the presence of a fibrovascular membrane in the eye.

Inflammatory eye conditions: Certain inflammatory eye conditions, such as uveitis and retinitis, can cause leukocoria.

Infections: Infections of the eye, such as retinitis caused by cytomegalovirus, can cause leukocoria.

It is important to note that leukocoria can also be a symptom of other underlying conditions, such as retinal tumors, optic nerve anomalies, and certain genetic disorders. It is essential to seek medical attention if you or your child experiences leukocoria to determine the underlying cause and receive appropriate treatment.

The prognosis of leukocoria (white pupillary reflex) depends on the underlying cause of the condition. In some cases, leukocoria can be a sign of a serious eye condition such as retinoblastoma, cataract, or retinal detachment. Early diagnosis and prompt treatment of these conditions are crucial for a favorable prognosis.

In cases of retinoblastoma, a cancer of the retina, early detection and treatment offer the best chance for a cure. The survival rate for retinoblastoma is high if the cancer is detected and treated before it spreads beyond the eye. Cataracts, a clouding of the eye’s lens, can be successfully treated with surgery, with a high success rate in restoring vision.

Retinal detachment, which occurs when the retina becomes separated from the underlying tissue, is a medical emergency that requires prompt treatment to prevent permanent vision loss. Other causes of leukocoria, such as Coats’ disease or toxocariasis, may require prompt treatment to prevent further vision loss or complications.

The prognosis for leukocoria also depends on the age of the patient and the severity of the underlying condition. In general, children who are diagnosed and treated early have a better chance of preserving their vision and achieving a good visual outcome. Overall, the prognosis of leukocoria varies depending on the underlying cause and the promptness of diagnosis and treatment. It is important to seek prompt evaluation by an ophthalmologist if you or your child experiences leukocoria.

Clinical history

The clinical history of leukocoria, or the appearance of a white reflex in the pupil of the eye, can provide important clues as to the underlying cause of the condition. A thorough clinical history should include the following information:

Age of onset: Leukocoria may be present from birth (congenital) or develop later in life. The age of onset can help narrow down potential causes.

Duration of symptoms: The duration of leukocoria can provide information about the progression of the underlying condition.

Presence of other symptoms: Leukocoria may be accompanied by other symptoms, such as pain, redness, or inflammation. These symptoms can provide important diagnostic clues.

Family history: Some causes of leukocoria, such as retinoblastoma, can be hereditary. A family history of similar symptoms or conditions should be noted.

Medical history: Certain medical conditions or medications may increase the risk of developing leukocoria or may be associated with specific causes of the condition.

Visual acuity: Changes in visual acuity, or the ability to see clearly, should be noted. Leukocoria may be accompanied by changes in visual acuity, such as blurry or distorted vision.

Previous eye examinations: Previous eye examinations or surgeries should be noted, as they can affect the current presentation of leukocoria.

A comprehensive clinical history, in conjunction with a physical exam and diagnostic testing, can help narrow down the potential underlying causes of leukocoria and guide appropriate treatment. It is important to seek medical attention if leukocoria is present, as early diagnosis and treatment can improve outcomes.

Physical examination

Leukocoria is a medical condition characterized by a white reflex in the pupil of the eye. It is most commonly seen in children and can be an indicator of various eye diseases, including retinoblastoma, cataracts, and other vision-threatening conditions.

A physical examination of leukocoria may involve the following steps:

Visual acuity test: This test measures the clarity of vision in each eye. It can be done using an eye chart or other specialized tools.

Dilated fundus exam: During this exam, the pupil is dilated with eye drops, and the doctor uses a specialized lens to examine the retina and other structures at the back of the eye.

External eye exam: The doctor examines the external structures of the eye, including the eyelids, cornea, and conjunctiva.

Intraocular pressure test: This test measures the pressure inside the eye and can help diagnose conditions such as glaucoma.

Additional tests: Depending on the suspected underlying cause of leukocoria, additional tests such as an ultrasound or MRI may be ordered.

It is important to note that the specific details of a physical examination for leukocoria may vary depending on the individual case and the underlying condition suspected. It is important to consult a qualified healthcare provider for proper diagnosis and treatment.

Differential diagnosis

Leukocoria, or the appearance of a white reflex in the pupil of the eye, can be a symptom of a wide range of underlying conditions. Therefore, it is important to conduct a thorough differential diagnosis to determine the cause of the leukocoria. Some of the conditions that may need to be considered in the differential diagnosis of leukocoria include:

Retinoblastoma: This type of eye cancer occurs in young children and is the most common cause of leukocoria in children.

Congenital cataracts: A clouding of the eye’s lens that is present at birth and can cause leukocoria if the obstruction of the lens is large enough.

Coats disease: A rare eye disorder that affects the blood vessels behind the retina and can cause fluid to leak into the retina, leading to leukocoria.

Retinal detachment: Separation of the retina from the underlying tissue that can cause leukocoria if the detached area reflects light.

Persistent hyperplastic primary vitreous (PHPV): A rare congenital eye abnormality that occurs when the blood vessels that nourish the eye during fetal development do not regress as they should. This can cause leukocoria due to the presence of a fibrovascular membrane in the eye.

Inflammatory eye conditions: Certain inflammatory eye conditions, such as uveitis and retinitis, can cause leukocoria.

Infections: Infections of the eye, such as retinitis caused by cytomegalovirus, can cause leukocoria.

Optic nerve abnormalities: Certain optic nerve abnormalities, such as optic nerve glioma, can cause leukocoria.

Other eye tumors: Other eye tumors, such as choroidal melanoma or hemangioma, can cause leukocoria.

Genetic disorders: Certain genetic disorders, such as Norrie and Coat’s disease, can cause leukocoria.

The differential diagnosis of leukocoria will depend on the patient’s age, medical history, and other symptoms. It is essential to seek medical attention if leukocoria is present to determine the underlying cause and receive appropriate treatment.

The treatment of leukocoria depends on the underlying condition causing it. Leukocoria is not a disease in itself but rather a symptom of an underlying condition. Therefore, treatment is focused on addressing the underlying condition. Some potential treatment options include:

Retinoblastoma: Retinoblastoma is a type of cancer that can cause leukocoria. Treatment may involve surgery, chemotherapy, radiation therapy, or a combination of these approaches. The specific treatment plan will depend on the size and location of the tumor.

Cataracts: Cataracts can cause leukocoria, especially in older adults. Treatment typically involves surgery to remove the clouded lens and replace it with an artificial lens.

Other eye diseases: Other eye diseases that can cause leukocoria, such as Coats disease or retinal detachment, may require surgical intervention or other specialized treatment approaches.

Monitoring: In some cases, such as small retinoblastoma tumors, the doctor may recommend regular monitoring to ensure the condition does not worsen.

It is important to note that early detection and treatment of the underlying condition causing leukocoria is critical for preserving vision and preventing more serious complications. Therefore, it is important to seek prompt medical attention if you or your child experiences leukocoria or any other unusual eye symptoms.

https://www.ncbi.nlm.nih.gov/books/NBK560794/