Program Operations Manual System (POMS)
TN 31 (08-20)
DI 23022.370 Bilateral Retinoblastoma
|
COMPASSIONATE ALLOWANCES INFORMATION
|
|
BILATERAL RETINOBLASTOMA
|
|
ALTERNATE NAMES
|
Malignant Neoplasm, Retina; Glioma Retinal; Rb; RB; Glioma, retinal
|
|
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:
-
•
Examination of the eyes with dilation of the pupils;
-
•
CT scan or MRI scan of the head to evaluate tumors and possible spread to the brain;
and
-
•
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 PROGRAMMATIC ASSESSMENT*
|
|
Suggested MER for Evaluation:
-
•
Clinical and ophthalmology examination that describes the diagnostic features of the
impairment;
-
-
-
-
|
|
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.
|
|
Equals
|
|
|
| * Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
|