Correctopia is usually towards the major iris strand and iris atrophy 180 from it. The zone of differentiation between the corneal and anterior chamber angle endothelium is abnormally anterior and is associated with excessive basement membrane deposition which results in the prominent, anteriorly displaced Schwalbe's line. The reasons for this are not fully elucidated but may be due to the loss of normal contact inhibition between the two endothelial surfaces. The developmental arrest prevents the complete migration of the anterior uvea posteriorly, resulting in an anteriorly inserted iris root and therefore compression of the trabecular meshwork. The neural crest cells give rise to most of the mesenchyme related to the forebrain, pituitary gland, bones and cartilage of the face and teeth which would explain the associated extra-ocular findings.
Some patients have keratolenticular adherence, while others have anterior polar cataracts.
Pilocarpine is not effective. This interesting group of disorders has a characteristic abnormality of the corneal endothelium as the common denominator. Predilection for women, early to middle adult life. ICE cells distributed amongst mosaic of enlarged endothelial cells. Subtotal - patchy distribution.