Corneal keloid in Lowe's syndrome.
Bilateral corneal keloids in a boy wih Lowe's syndrome were examined by conventional light and electron microscopy. There were no signs of perforating corneal trauma or iridocorneal incarceration in either eye. The corneal keloids consisted of haphazardly arranged bundles of collagen fibers, fibroblasts, and fenestrated blood vessels. The anterior region of the keloids showed signs of active progression and epithelial epidermalization. The etiology of keloids in Lowe's syndrome remains obscure. Considerations include excessive local delivery of amino acids and unknown noxious substances through the leak corneal vessels, seepage of similar substances across the defective blood-aqueous barrier and the decompensated endothelium, repeated external trauma with associated inflammation, phenytoin (Dilantin) therapy, and congenital predisposition. No data are available on the management of the progressive course of corneal keloids. Possible empirical regimens include local excision, pressure therapy, topical corticosteroids, and cromolyn sodium.
D002648:Child; D003315:Cornea / Q000473:pathology; D003316:Corneal Diseases / Q000150:complications* / Q000473:pathology; D006801:Humans; D007627:Keloid / Q000150:complications* / Q000473:pathology; D008297:Male; D009800:Oculocerebrorenal Syndrome / Q000150:complications* / Q000235:genetics; D015499:Renal Tubular Transport, Inborn Errors / Q000150:complications*