Retinoblastoma with unusual association of postaxial polydactyly.

Background: Retinoblastoma is the most common primary intraocular malignancy of childhood, arising from retinal progenitor cells. The most common presenting feature is leucocoria, followed by strabismus, defective vision, and rarely nystagmus. The unusual associations reported with retinoblastoma are well-differentiated liposarcoma and lipomatous tissues, chromosome breakage syndromes, and the myriad findings of rare 13q deletion syndrome.


Methods: Case report.


Results: An 8-year-old boy presented with features of retinoblastoma, having leucocoria in the left eye and an unusual association of postaxial polydactyly in the left hand.


Conclusions: Postaxial polydactyly should be considered as an association of retinoblastoma.