Fig. 7.

3-year-old girl with corneal dermoid. The mass had been present since birth and grew in proportion with the child. a Diagram showing normal fetal eye development from earliest (left) to most mature (right). During early normal eye development, the surface ectoderm thickens to form a lens placode and invaginates with the underlying neuroepithelium of the optic vesicle (image 2) to form a cup-shaped structure (image 3). The bilayered optic cup will give rise to the neural retina (inner layer) and retinal pigmented epithelium (outer layer, image 4) (adapted from [33]). b Clinical photograph of typical corneal dermoid, a round, yellowish mass arising from the ocular surface c Axial T1W and d CISS MR images show septated, cystic dermoid arising from corneal surface (arrows) and absence of lens. e Transverse US image of corneal dermoid showing anterior chamber cysts (arrow) and absent lens. In this example, vitreous debris is evident on T2W MRI and US images (curved arrows). f Whole eye H&E showing keratinized epithelium in the place of cornea (arrows). The lens is absent suggesting failure to of the embryonic lens placode to invaginate. The neurosensory retina, choroid, sclera and optic nerve appear normal. g Higher power image showing associated dermal elements (straight arrow) and cystic inclusions (curved arrow)