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Early-onset posterior polymorphous dystrophy

S G Levy¹, J Moss, B A Noble

¹Department of Histopathology, Charing Cross Hospital Medical School, England.

Archives of Ophthalmology (Chicago, Ill. : 1960)

|October 1, 1996

Summary

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This study details a rare infantile posterior polymorphous dystrophy case with early corneal failure. Abnormal endothelial cells and Descemet membrane changes were observed from birth, offering new insights into the disease's early pathology.

Area of Science:

Ophthalmology
Histopathology
Genetics

Background:

Posterior polymorphous dystrophy (PPMD) is a rare inherited eye disorder.
Typically, symptoms manifest later in childhood or adulthood.
Early-onset cases with rapid corneal failure are exceptionally uncommon.

Observation:

A neonate presented with corneal failure within weeks of birth, diagnosed as posterior polymorphous dystrophy.
Histopathology revealed abnormal corneal endothelial cells with surface microvilli.
Descemet membrane was significantly thinned, with an unusual posterior collagenous layer.

Findings:

The posterior collagenous layer contained tenascin, fibronectin, and collagen type I.
Abnormal microvilli-covered endothelial cells were present from the disease onset.

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This contrasts with previous reports of these cellular changes only in long-standing cases.

Implications:

This case provides crucial histopathologic data on infantile PPMD.
Early presence of cellular abnormalities suggests a congenital origin.
Descemet membrane composition and alterations may drive disease progression in PPMD.