Clinical Manifestations:Only about 10% of retinoblastomas are detected by routine ophthalmologic screening in the context of a positive family history. Retinoblastoma classically presents with leukocoria, a white pupillary reflex , which often is first noticed when a red reflex is not present at routine newborn or well-child examination or in a flash photograph of the child. Strabismus often is the initial presenting complaint. Orbital inflammation, hyphema, or pupil irregularity occurs with advancing disease.
Pain usually is a feature if secondary glaucoma is present.
Diagnosis:
The diagnosis is established by the characteristic ophthalmologic findings. Biopsy is contraindicated. Evaluation usually requires an examination under general anesthesia by an ophthalmologist to obtain complete visualization of both eyes, which also facilitates photographing and mapping of the tumors. Retinal detachment or vitreous hemorrhage can complicate the evaluation.
Orbital ultrasonography and CT or MRI are used to evaluate the extent of intraocular disease and extraocular spread. Occasionally, a pineal area tumor is detected, a phenomenon known as trilateral retinoblastoma.MRI allows for better evaluation of optic nerve involvement. Bone scan, cerebrospinal fluid evaluation, and bone marrow evaluation are required only if indicated by other clinical, laboratory, or imaging findings.
The differential diagnosis includes
- hyperplastic primary vitreous,
- Coats disease,
- cataract,
- visceral larva migrans,
- choroidal coloboma, and
- retinopathy of prematurity.
No comments:
Post a Comment