Posterior polymorphous dystrophy –
Posterior polymorphous dystrophy

It is a dominantly inherited dystrophy of endothelium and Descemet's membrane
The most typical finding is a cluster or linear arrangement of vesicles in the posterior cornea surrounded by a grey haze
The deep corneal stroma and Descemet's membrane may also have band- like thickenings, white patches, peau d ' orange appearance or excrescences that project into the anterior chamber.
There may be associated corneal edema, iris atrophy, mild corectopia and iridocorneal adhesions.
Most cases of this syndrome are non- progressive and the individuals affected maintain good vision throughout their lives.
A minority of patients develop corneal edema and glaucoma.
Most cases require no treatment. If the cornea becomes edematous, the patient should be treated with hypertonic saline, soft contact lenses and penetrating keratoplasty.

Note :

Corectopia is the displacement of the eye's pupil from its normal central position, i.e., abnormal location of the pupil of the eye.
Causes corectopia
Is inherited in an autosomal recessive fashion
Causes blindness in over 90% of sufferers
Can be treated with lamellar corneal grafts