Paediatric Ophthalmology: Case twelve

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This 3 year-old boy with a left strabismus was found to have this fundal appearance.

a. What is the most likely diagnosis?

Posterior persistent hyperplastic primary vitreous.
The picture shows a fibrous tissue extending from the optic disc causing distortion of the underlying retinal vessels.

b. What is responsible for this condition?

It is a congenital condition caused by the failure of the primary vitreous and hyaloid artery to regress.

c. What complications can occur with this condition?

The vision is usually poor in this condition and the following complications can occur:

• microphthalmos
• tractional retinal detachment with retinal tear
• vitreous haemorrhage from abnormal blood vessels.

The poor vision results in strabismus and/or nystagmus.