The clinical spectrum between posterior polymorphous dystrophy and iridocorneal endothelial syndromes

There are many conditions affecting the corneal endothelium with similar clinical appearances, though with different prognoses, management approaches, and pathophysiologic development.

A 39-year-old black woman with a previous diagnosis of asymmetric corneal posterior polymorphous dystrophy (PPMD) presented complaining of irritation in the left eye, worsening over the last week. On examination, her left eye had profuse edema and bullous keratopathy overlying a large placoid gray lesion at the level of the endothelium. Gonioscopy of the left eye found evidence of a membranous development extending from the endothelium to the trabecular meshwork. Examination of her right eye found a perfectly clear cornea and a normal angle. The patient subsequently had Chandler's syndrome diagnosed in the left eye, an iridocorneal endothelial (ICE) syndrome, rather than PPMD.

PPMD and ICE syndromes present with many similarities; these clinical entities are in fact thought to be variants of the same disease. Unlike PPMD, Chandler's syndrome is unilateral, acquired, and typically symptomatic and progressive. It also characteristically affects middle-age women.

Understanding the clinical features of PPMD and ICE syndromes is important in proper diagnosis and management.