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Writer's pictureMadhuvanthi Mohan

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:


Characterized by 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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