ALTERNATE NAMES

Glioma Retinal; Glioma, Retinal; Malignant Neoplasm, Retina; Rb; RB

DESCRIPTION

Bilateral Retinoblastoma is a disease that results in malignant tumors that form in the tissues of the retinas of both eyes in children under age 6, with most children with this disease diagnosed between the ages of 1-2 years. Retinoblastoma is the most common intraocular malignancy of infancy and childhood; with an incidence of 1/15,000-20,000 live births. Retinoblastoma is bilateral in about 40% of cases. Bilateral and multifocal unilateral forms are usually hereditary in nature and are caused by mutations in the RB1 gene. The tumors can spread to the eye socket through the optic nerve and may spread to the brain, lungs, and bones.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: The diagnosis of bilateral retinoblastoma is made by:

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  Examination of the eyes with dilation of the pupils;

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  Computerized tomography (CT) scan or magnetic resonance imaging (MRI) scan of the head to evaluate tumors and possible spread to the brain; and

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  Ultrasound of the eyes (head and eye echoencephalogram).

Physical findings: The most typical finding is a whitening of the pupils, the so-called "cat's eye reflex" (leukocoria). In addition, the eyes may be misaligned so that they appear crossed (strabismus). The eyes may become red and painful and glaucoma may occur because the fluid from the eyes cannot drain properly. Other signs and symptoms may include soreness or swelling of the eyelids; and blindness or poor vision in both eyes.

ICD-9: 190.5

ICD-10: C69.2

TREATMENT

Treatment options depend upon the size and location of the tumors. Small tumors are treated by laser surgery. Radiation and chemotherapy may be needed if the tumors have spread beyond the eyes.

PROGRESSION

The likelihood of a cure for bilateral retinoblastoma is lower than the unilateral type (which may be cured by chemotherapy and radiation or removal of the eye) and depends on how the tumors have spread. If the tumors spread to the brain, the prognosis is almost uniformly fatal.

Suggested MER for Evaluation:

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  Clinical and ophthalmology examination that describes the diagnostic features of the impairment;

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  Operative reports;

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  Pathology report;

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  Biopsy reports; and

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  Imaging studies.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

113.12

The treatment for retinoblastoma usually results in a visual impairment. Evaluate any resulting visual impairment using the visual listings.

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