BILATERAL RETINOBLASTOMA

ALTERNATE NAMES

Malignant Neoplasm, Retina

DESCRIPTION

Bilateral Retinoblastoma is a hereditary disease that results in malignant tumors that form in the tissues of the retinas of both eyes in children under age 6. The tumors can spread to the eye socket through the optic nerve and may spread to the brain, lungs, and bones. It is most commonly diagnosed in children 1-2 years of age.

The most typical finding is a whitening of the pupils, the so-called “cat’s eye reflex” (leukokoria). 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.

DIAGNOSTIC

TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

An examination of the eyes with dilation of the pupils, a CT scan or MRI of the head , to evaluate tumors and possible spread to the brain, and an ultrasound of the eyes (head and eye echoencephalogram ).

ICD-9: 190.5 Malignant Neoplasm, Retina

ONSET AND 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.

TREATMENT

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

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Clinical and ophthalmology examination, operative reports, pathology report, biopsy report, and imaging studies.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

113.12 C

Bilateral retinoblastoma meets 113.12C. The treatment for retinoblastoma usually results in a visual impairment. Evaluate any resulting visual impairment using the visual listings.

Medical Equals