Central Cloudy Dystrophy of Francois
CCDF is a rare corneal dystrophy with an unknown inheritance pattern
Risk factors - family history
Pathology:
These opacities are larger and more numerous in the posterior stroma immediately anterior to the Descemet membrane and fade peripherally
Sawtooth folds in deep stromal collagen, and extracellular vacuoles containing mucopolysaccharide and lipid-like materials correlate with the location of stromal opacities.
The irregular pattern of the stromal collagen and the deposition of lipid extracellular lipid vacuoles in CCDF both interrupt the normally dense, parallel arrangements of the collagen in the corneal leading to clouding and opacities in effected areas
Pathophysiology:
Opacification is due to the accumulation of mucopolysaccharides and lipids extracellularly and intracellular vacuoles in keratocytes
Symptoms:
Bilateral, symmetrical
Non progressive and asymptomatic
Signs:
Bilateral, symmetric, polygonal, cloudy gray stromal opacities separated by relatively clear zones in the central cornea involving the posterior stroma with relatively free periphery
Diagnosis:
Slit lamp
Family history
Confocal microscopy - small hyper-refractile granules in the anterior stromal layer and multiple dark striae among the extracellular matrix in the posterior stroma
Corneal transmission electron microscopy - many extracellular granules in the stroma, with a thickened basement membrane and degenerated keratocytes
Differentials:
Posterior crocodile shagreen
Fleck corneal dystrophy
Pre descemet corneal dystrophy
Congenital stromal corneal dystrophy
Management:
Observation - does not threaten vision or progress
Image from Rajan Eye Care Hospital
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