Neuroradiology Case of the Week

Case 36

Francisco Garcia-Morales, MD and P-L Westesson, MD, PhD, DDS

Clinical Presentation: Patient has been blind since age 18.

Radiological Findings: Axial CT scans demonstrate atrophic, small ocular globes bilaterally (microphthalmia) with very dense calcifications (phthisis bulbi).

Figure 1: Axial CT image shows small ocular globes with dense calcifications (arrow).

Diagnosis: Microphthalmia and phthisis bulbi.

Discussion:
   Microphthalmia refers to a congenital underdevelopment of or acquired diminution in the size of the globe [1]. Microphthalmia is referred as an eye that has an axial length less than 21 mm in adults or less than 19 mm in a 1-year-old child. Microphthalmia in the child can occur as an isolated finding or associated to other ocular, craniofacial abnormalities, and entities such as congenital rubella, persistent hyperplastic primary vitreous and retinopathy of prematurity. In older patients, though, microphthalmia can occur as result of trauma, surgery, inflammation, radiation or other processes which result in disorganization of the eye (phthisis bulbi) usually with extensive intraocular calcifications or ossification or intraocular osseous metaplasia that had been found associated to retinal detachment, gliosis, retinal pigment epithelial hyperplasia, drusen, fibrovascular proliferation and inflammation [2].

References:

1. Som PM, Curtin HD. Head and Neck Imaging, 4th Edition, St, Louis, Missouri: Mosby, 2003; 461-463.
2. Vemuganti GK, Honovar SG, Jalali S, Intraocular metaplasia. A clinico-pathological study. Indian J Ophthalmo. 2002 Sep; 50(3):183-8.