Diffuse Choroidal Hemangioma
Diffuse choroidal hemangioma is a benign overgrowth of blood vessels that occurs within the posterior portion of the eye. It is believed to be present at birth and gradually enlarges over time. This overgrowth is classified as a tumor but it does not become cancerous. It can extend over a broad area of the inner wall of the posterior portion of the eye and gradually thickens over time. It is frequently located ipsilateral (same side) to a facial hemangioma (nevus flammeus) and is often a component of the Sturge-Weber syndrome. This condition is suspected in any child with Sturge Weber syndrome or those with Klippel-Trenaunay-Weber syndrome. Occasionally diffuse choroidal hemangioma can occur in both eyes, particularly when the nevus flammeus involves both sides of the face.

 

Can diffuse choroidal hemangioma affect vision?
Diffuse choroidal hemangioma is usually diagnosed when the affected patient is young (median age 8 years), either because the facial nevus flammeus prompts a dilated eye exami­nation or because of vision loss. Young children typically do not complain of loss of vision. They tolerate gradually reducing vision because they do not realize that it is abnormal. Poor vision can be suspected in young children if the eye is drifting (strabismus), crossed (strabismus), or shaking (nystagmus), or if the child appears to have obvious difficulty with visual perception such as not looking directly at faces or objects. Older children might detect and report visual loss to their parents but not younger children.


The vision loss is from one of many reasons including:
1. Refractive (need for glasses) As the tumor elevates and thickens in the back of the eye, it induces blurred vision. In some cases, glasses can improve the vision.

2. Amblyopia (lazy eye) As the tumor blurs the vision, the brain gradually ignores the blurred vision from that eye and the eye drifts or crosses (strabismus). If amblyopia is detected, it must be corrected with glasses or patching as well as treatment of the tumor.

3. Retinal detachment (fluid leakage in the back of the eye) As the hemangioma thickens, it starts to leak clear fluid into the eye under the retina (important layer in the back of the eye that provides vision). When fluid leaks, it blisters the retina up and vision becomes blurred. Some patients perceive flashing lights as the retina is blistered. Glasses do not correct this problem. The hemangioma needs to be treated to resolve the blistering. If the retinal detachment is repaired early, then the vision might return partially or completely. If the retinal detachment becomes chronic, then permanent irreparable damage occurs and the vision does not improve.

4. Glaucoma (high pressure in the eye) As the retina blisters up with fluid, the pressure in the eye can elevate and this further reduces vision. The treatment of the tumor can resolve this problem.
 

Why is diffuse choroidal hemangioma important?
Diffuse choroidal hemangioma is a benign, noncancerous tumor. This tumor does not threaten the child’s life, but it can affect the quality of life and vision. This tumor can lead to blindness. In rare cases, it can grow out of proportion and lead to unrelenting ocular pain necessitating enucleation (eye removal). With proper monitoring, substantial vision loss can generally be avoided and enucleation is rarely necessary.  


How is choroidal hemangioma detected?
The best way to detect this tumor is to dilate the pupils and look into the back of the eye with the indirect ophthalmoscope (headlight equipment used by eye doctors). This might require the young child to be held in place while the doctor looks. Sometimes this tumor is subtle and barely visible. This tumor is generally not visible to the pediatrician or general medical doctor. It requires the eye doctor and his/her special equipment for detection. It can be difficult for some general ophthalmologists to see this tumor so a specialist familiar with this disease becomes necessary.

The child should be screened twice yearly for this condition beginning at the first year of life. It is really important to have the screening started at a young age as the child will not complain of vision loss and if the parents wait for vision problems or a lazy eye to occur, it could be too late for treatment. So early screening and continued screening twice yearly by experienced eye doctors is important. If diffuse choroidal hemangioma is not present by age 15 to 20 years, then it is not likely to occur. With proper screening, often it is visualized at a young age, when the child is about under 10 years old.
 

What are the tests that help for detection?
There are several tests for detection of choroidal hemangioma. These include visual acuity, fundus photography, ultrasonography, optical coherence tomography, fluorescein angiography, indocyanine green angiography, and magnetic resonance imaging.   These tests are complimentary and each provides slightly different information so that the entire aspect and risks of the choroidal hemangioma is understood. Not all tests are done at each visit. They are only ordered as needed.
 

Visual Acuity
Visual acuity is the measurement of vision. In children older than age 4 or 5 years, a reasonable vision can be obtained and if reduced, this suggests a problem in refraction (needs glasses), lazy eye (amblyopia), or retinal detachment (fluid in the eye). Children under age 4 do not reliably read the vision chart.
 

Fundus Photography
This test involves taking a photographic image of the back of each eye. By comparison, the eye with choroidal hemangioma will appear redder in color than the normal eye. Retinal detachment can sometimes be detected.

Ultrasonography
This test uses sound waves to detect abnormalities in the eye. This is an excellent test for detecting choroidal hemangioma. It is painless and can be performed with the eyes closed so the child is comfortable and held in the arms of the parents. This test can also detect retinal detachment.

Optical Coherence Tomography
This is the most sensitive test available for detection of retinal detachment or any leakage from choroidal hemangioma. This test uses light waves and is completely painless. It takes only a few minutes to perform with an invisible light aimed at the eye from a source and the retina reading is made. This test indicates the status of the retina, if it is healthy, or if there is fluid under it or within it.

Fluorescein Angiography
This is a dye test to image the retinal blood vessels of the eye. An intravenous injection of a safe dye, called fluorescein, is given and then the flow to the eye is measured with a camera. This can indicate sites and amount of retinal fluid leakage.

Indocyanine Green Angiography
This is another dye test, using a dye called indocyanine, to image blood vessels under the retina in the choroid. An intravenous injection then special camera imaging of the vessels helps to confirm the extent of the hemangioma.

Magnetic Resonance Imaging
This is a non-radiation test whereby the patient must lay on a table and a magnetic field is created around the patient to provide an image of the brain and eyes or other parts of the body. This is useful to image the entire eye in cross section and helps to delineate the size of the hemangioma.

How is choroidal hemangioma treated?
There are several ways to manage choroidal hemangioma. Fortunately, these methods are quite successful in controlling the hemangioma and its leakiness.

Observation
If there is no leakage, then the tumor is not treated but the child is carefully watched and glasses might be given for lazy eye.

Photodynamic Therapy
This is used if the hemangioma is only partially leaking. This is a special laser treatment that uses a light sensitive intravenous dye called verteporfin. The treatment takes about 1 hour and the patient cannot go into sunlight for 5 days due to risk for sunburn. The laser closes off the leaking vessels and the fluid dries up.

Radiation
There are two types of radiation, focal and the whole eye radiation. If the hemangioma is only partly leaking, focal radiation with plaque radiotherapy can be given.   This takes 5 days and requires surgery. If the entire hemangioma is leaking then external beam radiotherapy is given over a period of 4 weeks with a small dose each day. This does not require surgery. Fortunately, both methods are successful. With the latter treatment, the child might temporarily lose the eyelashes and the eye will look red. Ointments will be given.

What should I do for my child with Sturge Weber?
It is advised that all children with Sturge Weber syndrome obtain routine eye care to evaluate for reduced vision, lazy eye, crossed eye, glaucoma, retinal detachment, and choroidal hemangioma. The screening should begin after birth when the child is an infant and continue throughout life twice a year.

 

Textbook references
Shields JA, Shields CL. Intraocular Tumors. An Atlas and Textbook. 2nd edition.   Philadelphia, Lippincott Williams and Wilkins, 2008.
Shields JA, Shields CL. Intraocular Tumors. A Text and Atlas. Philadelphia, WB Saunders, 1992

Diffuse Choroidal Hemangioma
Carol L. Shields, M.D.
From the Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA.
Support provided by the Eye Tumor Research Foundation, Philadelphia, PA.
Inquiries to Carol L. Shields, M.D., Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107