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

• Eye Hemangioma
• Hemangioma of the Eye Region
• Intraocular Hemangioma

What is Hemangioma of the Eye? (Definition/Background Information)

• Hemangiomas are benign malformation of blood vessels that can occur in a number of tissues such as the kidney, liver, lung, skin, and brain. Hemangioma of the Eye affects the eye and surrounding structures including the eyelid and orbit
• Most of the Eye Hemangiomas are observed in infants and young children, and the most common type among these malformations are capillary hemangiomas
• Even though some of the Eye Hemangiomas are known to resolve over time, untreated and large-sized tumors can cause several complications including amblyopia and loss of vision

Who gets Hemangioma of the Eye? (Age and Sex Distribution)

• It is estimated that 1 in 10 children will be affected by hemangiomas somewhere in the body
• 30% of Hemangiomas of the Eye are observed at the time of birth, while a vast majority of the remaining tumors (almost 70%) are diagnosed by or before age 6 months
• In some rare cases, the benign malformation may also be present in adults
• Both males and females are affected; in capillary hemangiomas of infancy, the female-male ratio is 3:1
• 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 of the Eye? (Predisposing Factors)

The risk factors for Hemangioma of Eye may include:

• Premature birth and low birth weight infants
• Chorionic villous sampling (CVS) - there is a ten-fold increase in risk for infants born to women who have undergone CVS (a prenatal diagnostic test) during pregnancy
• Family history of the tumor
• The hemangioma is associated with the following syndromes:
  • PHACES syndrome which consists of posterior fossa malformation of the skull bone, heart defects, arterial abnormalities, coarctation of aorta, eye abnormalities, and sternal clefting
  • 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 of the Eye? (Etiology)

The exact cause of formation of Hemangioma of the Eye is unknown.

• Current research indicates that this tumor is caused due to a somatic mutation
• Many of the tumors occur in isolation (sporadic Eye Hemangioma), while others may be associated with syndromes (syndromic Eye Hemangioma)

What are the Signs and Symptoms of Hemangioma of the Eye?

The signs and symptoms of Hemangioma of the Eye may vary from one child to another and may be mild or severe. These may include:

• Red lesion on eye that may grow in size
• The lesion becomes larger when the infant cries
• Large hemangiomas can cause superficial ulceration resulting in significant bleeding
• Vision problems depending on what part of the eye is involved

In one study, 60% of Hemangiomas of the Eye were found in the superior orbital region, 16% in the inferior orbital region, and 2% involved both the upper and lower eyelids.

• Capillary hemangiomas can either be solitary (solitary Eye Hemangioma) or multiple (multiple Eye Hemangioma)
• In individuals with multiple capillary hemangiomas, in addition to the involvement of the eye region, the following regions may also be involved:
  • Nasal region (nasal capillary hemangioma)
  • Oral region (oral capillary hemangioma)
  • Paratracheal region (paratracheal capillary hemangioma, causing compression of the trachea/larynx resulting in breathing difficulties)
  • Subglottic region (subglottic capillary hemangioma)

How is Hemangioma of the Eye Diagnosed?

The diagnosis of Hemangioma of the Eye may involve the following exams and procedures: 

• A thorough physical examination and evaluation of complete medical history
• Assessment of signs and symptoms
• General eye exam
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Angiography
• Slit-lamp examination
• Visual evoked potential (VEP) test
• Fundus fluorescein angiography
• Optical coherence tomography (OCT) of eye
• Imaging studies including CT and MRI scans of the head and neck region, if needed
• Tumor biopsy: It is usually not required, but may be performed if any malignancy is suspected

The healthcare provider may perform additional tests to rule out any associated conditions.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hemangioma of the Eye? 

The possible complications of Hemangioma of the Eye may include:

• Severe emotional distress for the parents
• Dropping of eyelid
• Cosmetic issues and disfigurement
• Exposure of the cornea
• Optic nerve compression
• Visual loss secondary to amblyopia - it is among the most common complication of Eye Hemangioma. Anisometropic amblyopia occurs due to indentation of the sclera and cornea of eye resulting in significant refractive errors
• 20-50% of the eyes affected by hemangioma are reported to have astigmatism
• Infiltration of the tumor into extraocular muscles, or mechanical compression due to the tumor can result in strabismus
• Permanent vision problems
• Microangiopathic hemolytic anemia (MAHA) may occur with large-sized hemangioma
• Ulceration of the tumor causing secondary infections and sepsis

How is Hemangioma of the Eye Treated?

Some cases of Hemangioma of the Eye are known to resolve on their own. However, even though it is a benign condition, the tumor can cause a variety of complications, and it is extremely important to treat these eye lesions.

The following treatment measures may be considered by the healthcare provider:

• Wait and watch approach
• Radiotherapy may be needed in some individuals
• Laser therapy
• Intralesional therapy
• Systemic corticosteroid therapy
• Ulcerated hemangiomas may be treated with topical creams, topical antibiotics, local steroid therapy, and aggressive wound care, if needed

New treatment measures, such as recombinant interferon alpha and medications that slow the growth of vascular tumors (angiogenesis inhibiting agents) may be considered in individuals with severe signs and symptoms who have failed steroidal therapy.

How can Hemangioma of the Eye be Prevented?

Currently, there are no known methods to prevent Hemangioma of the Eye. However, the following factors may help reduce one’s risk for developing the same:

• If there is a family history of a medical condition, then genetic counseling will help assess risks, before planning for a child
• Have regular prenatal visits to monitor one’s health during pregnancy

What is the Prognosis of Hemangioma of the Eye? (Outcomes/Resolutions)

The prognosis of Hemangioma of the Eye is dependent on the severity of the signs and symptoms, complications noted, early intervention, and one’s response to treatment.

Additional and Relevant Useful Information for Hemangioma of the Eye:

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

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