Intraocular Calcification as a Diagnostic Marker for Retinoblastoma

Abstract

 

Purpose

 

The aim of this study was to determine pediatric conditions other than retinoblastoma (Rb) that may be associated with posterior segment intraocular calcification.

 

Methods

 

We reviewed 405 pathology reports from enucleation specimens of children ages 0 to 5 years collected between January 1960 and May 2011 at one U.S. ocular pathology laboratory. An ophthalmic pathologist re-reviewed a minimum of 5 representative H&E stained slides for each of the 154 specimens that had no mention of intraocular calcification in their reports. Specimens were classified according to pathological diagnosis and the presence/absence of calcification within the anterior segment of the eye (cornea, ciliary body, lens) or posterior segment (vitreous, choroid, retina, sclera, optic nerve). Optic disc drusen were not studied. We then performed a PubMed literature search for “intraocular calcification,” “osseous metaplasia eye,” and “leukocoria calcification” to find English-language reports of posterior segment intraocular calcification among children ages 0 to 5 years without Rb. We also searched the reference lists of the resulting articles.

 

Results

 

Posterior segment intraocular calcification was present in 280 of 330 (84.8%) Rb specimens and 13 of 75 (17.3%) non-Rb specimens. The diagnoses of these 13 non-Rb specimens included phthisis (4; due to sympathetic ophthalmia; complications of intraocular surgery; congenital rubella; endophthalmitis), congenital microphthalmos with cyst (2), persistent fetal vasculature (1), Coats’ disease (1), astrocytic hamartoma (1), retinal dysplasia (1), panophthalmitis with retained intraocular foreign body (1), retinal detachment of unknown etiology (1), and congenital uveitis of unknown etiology leading to autolytic degeneration of the globe (1). The literature review identified the following pediatric conditions with posterior segment intraocular calcification: astrocytic hamartoma, ocular choristoma in linear nevus sebaceous syndrome, medulloepithelioma, and retinal detachment in incontinentia pigmenti.

 

Conclusions

 

Diagnoses other than Rb should be considered in a young child with intraocular calcification, including phthisis from myriad causes, congenital anomalies, persistent fetal vasculature, Coats’ disease, astrocytic hamartoma, retinal dysplasia, and endophthalmitis.

 

 

(H&E stain) Intraocular psammomatous calcification in child with postoperative phthisis secondary to complications of congenital rubella.

 

Keywords: 703 retinoblastoma • 439 calcium