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Small angle ET	Whitish band of tissue connecting posterior lens and optic disc

This 8-year-old girl presented with a small angle right esotropia and hand motion vision in the right eye. The left eye appeared normal and was normal on examination. When attempting retinoscopy in the right eye after cycloplegia, no retinal reflex could be seen. With the slit lamp there was a band of whitish tissue that extended from the back of the lens to the optic nerve.

1.	This condition is called:
	a -- persistent hyperplastic primary vitreous (PHPV) This is an example of persistent hyperplastic primary vitreous. It is caused by the failure of the hyaloid vascular to undergo regression.  Lesser degrees of this condition can occur as a Mittendorf dot at that posterior lens capsule and a Burgmeister papilla arising from the disc. Both represent lesser expressions of a persistent fetal vascular system.  FEVR is an autosomal dominant condition with vitreous traction and posterior vitreous detachment that results in retinal traction and detachment. It must be differentiated from retinopathy of prematurity and Coats disease.  Posterior lentiglobus involves only the posterior lens.  Coats disease is exudative retinal detachment from retinal telangectasia or grape-like clusters.
2.	The globe size in cases like this is usually:
	c -- smaller In PHPV the eye is smaller.
3.	Visual acuity in these cases:
	b -- depends on the amount of microphthalmos and the involvement of the posterior pole Visual acuity in PHPV depends on the amount of change in the posterior pole.  In many cases the membrane at the posterior lens creates traction on the ciliary processes, pushing the lens forward, resulting in glaucoma.