Reappearance of Kayser-Fleischer

 Q: 54 years old male with a history of liver transplantation seven years ago due to Wilson disease is admitted to ICU with sepsis. Kayser-Fleischer rings are noticed on physical exam. A review of chart shows that rings were noted to be disappeared on the last clinic visit about 2 years ago. The reappearance of Kayser-Fleischer rings signifies what? 

Answer: Probable noncompliance 

Kayser-Fleischer rings are brownish or gray-greenish rings in Descemet's membrane in the cornea, and they are close to the endothelial surface. They can be best seen on examination at the inferior and superior poles of the cornea. They are fine pigmented granular deposits of copper. Though they are not specific but highly suggestive of Wilson disease. Their presence also signifies neurological involvement. 

Kayser-Fleischer rings gradually disappear with medical treatment or after a liver transplant. The reappearance of Kayser-Fleischer rings raises high suspicion of non-compliance. In our patient, another red flag is absence from the follow-up clinic for 2 years.

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References: 

1. Song HS, Ku WC, Chen CL. Disappearance of Kayser-Fleischer rings following liver transplantation. Transplant Proc. 1992 Aug;24(4):1483-5. PMID: 1496628.

2. Suvarna JC. Kayser-Fleischer ring. J Postgrad Med. 2008 Jul-Sep;54(3):238-40. doi: 10.4103/0022-3859.41816. PMID: 18626182.