Diffuse infiltrating retinoblastoma requires keen diagnosis

PHILADELPHIA — Diffuse infiltrating retinoblastoma may present as uveitis, unexplained hyphema or keratic precipitates, an expert said here.

Carol L. Shields

Diffuse infiltrating retinoblastoma occurs in 1% to 2% of retinoblastoma cases and often requires additional chemotherapy, Carol L. Shields, MD, said at the Wills Eye Institute Alumni Conference.

"It's really the most difficult to recognize clinically," she said.

A retrospective series included 34 eyes with diffuse infiltrating retinoblastoma. The mean patient age of 4 years was slightly higher than in most cases of retinoblastoma.

A diagnosis of diffuse infiltrating retinoblastoma was made in 76% of cases. Uveitis was diagnosed in 9% of cases, Coats' disease in 3%, unspecified trauma in 3% and unspecified retinal problem in 9%, Dr. Shields said.

Anterior segment findings included lid edema, conjunctival infection, tumor seeds on the corneal endothelium, corneal edema and hypopyon.

Fundus features included vitreous seeds, vitreous hemorrhage, subretinal fluid and subretinal seeds. Total retinal detachment, calcified vitreous seeds and infiltration of the entire retina were also identified.

All 34 eyes underwent enucleation. No cases of metastases were reported at 47 months' follow-up, Dr. Shields said.