PURPOSE
To evaluate the enucleation rate for children with unilateral sporadic retinoblastoma based on initial external findings detected by the family and the pediatrician.
PATIENTS AND METHODS
A retrospective, nonrandomized review was performed on 257 consecutive patients with unilateral sporadic retinoblastoma treated at a major ocular oncology center. Data were gathered regarding the initial external clinical finding noted by the family members, pediatrician, and ocular oncologist and then correlated with the final ocular outcome of enucleation versus nonenucleation measures.
RESULTS
Of the 257 cases of unilateral sporadic retinoblastoma, enucleation was necessary in 80%. Of the 223 patients in whom the family detected an eye problem, enucleation was necessary in 92%, whereas of the 34 patients in whom there were no findings detected by the family, enucleation was necessary in 53%. Specifically, enucleation was necessary in 86% of patients with family-detected leukocoria, 68% of those with family-detected strabismus, and 100% of those with family-detected red eye, heterochromia, eye pain, decreased vision, or blepharoptosis. Eye problems detected by a pediatrician resulted in enucleation in 77%. Specifically, enucleation was necessary in 75% of patients with pediatrician- detected leukocoria, 46% of those with pediatrician- detected strabismus, and 86% of those with pediatrician-detected red eye, heterochromia, decreased visual acuity, or an unspecified eye problem. Enucleation was necessary in 81% of those patients in whom an ocular oncologist detected any external finding such as leukocoria, strabismus, red eye, heterochromia, or buphthalmos and in only 33% of those without external findings.
CONCLUSIONS
Children with retinoblastoma who present with obvious external findings of leukocoria, strabismus, or red eye detectable by their family or pediatrician most often require enucleation. Children who manifest no obvious external findings can often avoid enucleation.
J Pediatr Ophthalmol Strabismus 2004;41:143-149.
AUTHORS
The authors are from the Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania.
Originally submitted June 8, 2003.
Accepted for publication February 10, 2004.
Address reprint requests to Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 840 Walnut Street, Philadelphia, PA 19107.
Supported by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania (CLS); the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (JAS); and the Rosenthal Award of the Macula Society, Barcelona, Spain (CLS).
The authors have no industry relationships to disclose.
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