What are the other Names for this Condition? (Also known as/Synonyms)

• Amblyopia due to Hemangioma

What is Hemangioma-Related Amblyopia? (Definition/Background Information)

• Hemangioma-Related Amblyopia is typically a form of deprivation amblyopia, which is caused by an obstruction of the visual axis. Amblyopia, also known as lazy eye, is a condition that results in decreased vision in an eye that typically appears normal
• Hemangiomas are benign malformation of blood vessels that can occur in a number of tissues such as the kidney, liver, and brain. Hemangiomas of the head and neck region, specifically orbital hemangiomas can block the visual axis or exert pressure on the globe of the eye, depending on the tumor location
• This obstructive or pressure effect of the tumor can potentially lead to a variety of visual conditions such as stimulus-deprived amblyopia, induced astigmatism, anisometropic amblyopia, or squint eye. Thus, prompt diagnosis and early medical attention is necessary to prevent long-term and irreversible vision loss from Hemangioma-Related Amblyopia

Who gets Hemangioma-Related Amblyopia? (Age and Sex Distribution)

• The incidence of amblyopia in children with hemangioma of the eye is estimated to be between 40-60%
• Hemangioma-Related Amblyopia is noted in infancy and early childhood. The condition is rare in adults
• Males and females are both affected, but the capillary form of hemangioma is mostly noted in girls (in 75% of the cases)
• Worldwide, the tumor is noted among all racial and ethnic groups. However, some reports indicate that Caucasian children have a higher risk for these tumors

What are the Risk Factors for Hemangioma-Related Amblyopia? (Predisposing Factors)

The primary risk factor for Hemangioma-Related Amblyopia is the presence of an orbital or periorbital hemangioma. The other risk factors may include:

• Family history of amblyopia
• Kasabach-Merritt syndrome (KMS) - a rare, life-threatening condition affecting the ability of blood to clot that is observed in newborns and infants

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hemangioma-Related Amblyopia? (Etiology)

Hemangioma-Related Amblyopia is a form of amblyopia that typically occurs from large-sized hemangiomas that involve the eye surface, eye socket, or eyelids.

• The presence of a rapidly-growing capillary hemangioma can result in complete closure of eye or inability to open the eye
• In general, hemangiomas of the eyelid and orbit, less than 1 cm in size, have less likelihood of causing visual problems
• A particular study has shown that over 50% of the individuals with hemangioma measuring greater than 1 cm have amblyopia in the affected eye

Capillary hemangiomas are most commonly periorbital/periocular tumors that typically occur in infancy.

What are the Signs and Symptoms of Hemangioma-Related Amblyopia?

The signs and symptoms of Hemangioma-Related Amblyopia may vary from one child to another and may be mild or severe. A red lesion may be visible on the eye or eyelid, and the vision concerns depend on the size and on what part/structure of the eye the hemangioma is noted.

Hemangiomas can grow rapidly during infancy within a matter of few weeks preventing normal development of vision. Hemangiomas that are most likely to interfere with vision are those involving the upper eyelid and occupying greater than one-third of the lid margin or those extending beyond the eyelid region, and the ones that do not resolve early.

In general, amblyopia may cause the following signs and symptoms: 

• Depth perception problems
• Crossed eyes
• Eyes turned out
• Blurry vision in one or both eyes
• Eye coordination difficulties
• Headaches
• Squinting
• Other vision signs and symptoms such as lack of binocular vision

Associated signs and symptoms of the underlying condition/disorder, if any present, may also be observed.

How is Hemangioma-Related Amblyopia Diagnosed?

A diagnosis of Hemangioma-Related Amblyopia may involve the following test and exams:

• Physical examination and medical history evaluation
• Assessment of signs and symptoms
• General eye exam
• Neuronal receptive fields
• Corneal light reflex test
• Contrast sensitivity
• Grating acuity
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Refraction studies using various instruments
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Slit-lamp examination
• Imaging studies including CT and MRI scans of the head and neck region, if necessary

Note:

• A tumor biopsy is usually not required but may be performed if any malignancy is suspected
• Tests and exams to determine an underlying cause, if present, may be necessary

Many other clinical conditions may cause vision loss in children. Your optometrist or pediatric ophthalmologist may perform additional tests to rule out other conditions.

What are the possible Complications of Hemangioma-Related Amblyopia?

The possible complications of Hemangioma-Related Amblyopia include:

• Cosmetic concerns
• Anisometropic amblyopia may occur due to indentation of the sclera and cornea of eye resulting in significant refractive errors
• Infiltration of the tumor into extraocular muscles, or mechanical compression due to the tumor can result in strabismus
• Permanent vision problems
• Decreased quality of life due to loss of stereoscopic vision
• Treatment related complications

How is Hemangioma-Related Amblyopia Treated?

Early intervention is important to prevent long-term permanent visual damage from Hemangioma-Related Amblyopia. The treatment measures are individualized weighing the risks of complications due to treatment with benefits of preventing long-term vision impairment.

• In some cases, a wait and watch approach may be considered since steroidal injections and surgery may be associated with significant risk for complications
• Steroidal therapy can be systemic therapy or intralesional injections
• Surgical treatment involves removal of the tumor

The treatment measures include treating the symptoms and managing the underlying condition/disorder.

How can Hemangioma-Related Amblyopia be Prevented?

Currently, there are no known prevention methods for Hemangioma-Related Amblyopia. However, a prompt diagnosis and appropriate treatment of hemangiomas involving the eye can avoid the development of amblyopia.

• Efforts can be taken to reduce one’s risk of developing amblyopia by having regular eye examinations, especially in infants and young children
• If there is a family history of a medical condition, then genetic counseling will help assess risks, before planning for a child
• Holding regular prenatal visits to monitor one’s health during pregnancy

What is the Prognosis of Hemangioma-Related Amblyopia? (Outcomes/Resolutions)

The prognosis of Hemangioma-Related Amblyopia depends on the severity, age of the child, early treatment, and one’s response to therapy.

• The younger the children with the condition are corrected, the better are the outcomes (typically before age 10)
• Some eye hemangiomas are known to resolve spontaneously on their own without any treatment 
• If diagnoses and treatments are delayed, the vision may be permanently affected

Additional and Relevant Useful Information for Hemangioma-Related Amblyopia:

Please visit our Eye & Vision Center for more physician-approved health information:

http://www.dovemed.com/health-topics/vision-center/