Presentation
Orbital abnormal shape.
Patient Data
Bilateral microphthalmia and dysplastic lens are associated with large orbital colobomatous cysts occupying both orbital cavities. Additionally, there are bilateral small optic nerves and extra-ocular muscles.
Corpus callosal dysgenesis is evident by the parallel orientation of the lateral ventricles.
Case Discussion
Microphthalmia is considered to be the most common congenital malformation of the eye after congenital cataracts. The association of microphthalmia with the intraorbital cyst is considered a rare developmental anomaly of the globe that might affect one or both eyes.
Common associations include the cleft lip, basal encephalocele, mid-brain deformity, microcephalus, agenesis of the corpus callosum, and saddle nose, mostly in bilateral cases.
Microphthalmia with cysts is similar to the congenital cystic eye. In congenital cystic globes, the cyst is located centrally or slightly upward in the orbit, and histologically, it lacks normal ocular structures and shows the presence of a cyst lined with neuroglial tissue. However, in microphthalmos with a cyst, there is bulging of the lower eyelid as the cyst is attached to the inferior portion of the globe, along with evidence of ocular development in conjunction with a small cornea, iris, ciliary body, lens, vitreous cavity, retina, and choroid.