Kayer-Fleicher ring a the preenting ign of Wilon dieae

PURPOE: To decribe a cae in which the recognition by the ophthalmologit of Kayer-Fleicher ring played a crucial role in the diagnoi of Wilon dieae (hepatolenticular degeneration). DEIGN: Interventional cae report. METHOD: An 18-year-old woman wa found to have bilateral peripheral golden brown pigment depoit at the level of the Decemet membrane conitent with Kayer-Fleicher ring. he initially denied ytemic ymptom. REULT: The erum cerumoplamin level of the patient wa normal. Liver function tet were alo normal. Further evaluation with 24-hour urine copper aay and liver biopy were poitive for Wilon dieae. he underwent anticopper therapy and, 6 month later, reported improved concentration and balance. No appreciable change occurred in the Kayer-Fleicher ring. CONCLUION: Wilon dieae occur when a defective copper-tranporting enzyme in the liver reult in toxic copper accumulation in multiple organ. Becaue of the inidiou nature of the dieae, patient may not eek medical attention until evere irreverible damage ha occurred. By diagnoi of Kayer-Fleicher ring, ophthalmologit may play a critical role in the early recognition and proper evaluation of uch patient