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Kayser-Fleischer Ring

Original Editor - Angeliki Chorti

Top Contributors - Angeliki Chorti  

Introduction

Kayser-Fleischer ring
Wilson's disease Kayser-Fleischer ring.

A Kayser-Fleischer (K-F) ring is an ophthalmological finding that usually refers to a dark brown or grayish-green ring in the periphery of the cornea, above the iris of the eye. [1] This is the result of copper deposition in the Descemet membrane of the cornea because of an overly increasing accumulation of copper from cholestasis, [2] liver disease, [3] or errors in copper metabolism.[4]

Kayser-Fleischer rings are reported not to affect vision nor there are long-term complications. [2] These rings can be resolved with treatment and serve as an indicator of disease progression or response to therapy. [1] However, without proper treatment of the disease causing a Kayser-Fleischer ring, complications such as cirrhosis and liver failure may occur, ultimately resulting in death. [1] Timely recognition of Kayser-Fleischer rings is essential for exposing the underlying cause and providing appropriate treatment for affected patients.

Historical Perspective

Bernhard Kayser and Bruno Freischer, who were German ophthalmologists, were the first who described Kayser-Fleischer rings in the 1900s. [5]

Epidemiology

Kayser-Fleischer ring with sunflower cataract.

Kayser-Fleischer rings are usually found in late childhood or early adolescence of patients with Wilson's disease (ATP7B mutation), and occasionally in other conditions, such as primary biliary cholangitis, neonatal cholestasis, and liver disease. [1] A Kayser-Fleischer ring is rare in children but its absence does not exclude diagnosis of Wilson's disease.[6] They are reported in 95% of patients with Wilson's disease, and 50% of patients without neurological symptoms. [7] [8] They may present with other signs and symptoms that cause significant morbidity and mortality such as mood and other psychiatric changes, [9] neurological symptoms e.g. tremor, bradykinesia, dystonia, difficulty speaking, [10] cardiac manifestations, [11] infertility, [12] and hemolytic anemia.[13] In some cases of Wilson's disease, sunflower cataracts are also evident. [14]

Diagnosis

Slit Lamp

Diagnosis is traditionally made by using a slit-lamp (SL) biomicroscopy examination.[15] This examination is particularly useful in the early stages where the rings are not easily visible to the naked eye. However, other alternatives have also been proposed for the evaluation of Kayser-Fleischer rings.

Gonioscopy uses a specialised lens to examine the eye's drainage angle (i.e. the area between the iris and cornea where fluid may exit). Although it is a key test for diagnosing angle-closure glaucoma, it may also detect early Kayser-Fleischer rings.

Anterior segment optical coherence tomography (AS-OCT) can also idetify a KF ring as an intense hyperreflective band at the level of Descemet membrane. [15] This band is usually shown in AS-OCT as greenish/greenish yellow/orange yellow/yellowish/red. [15]

ICD-10 Classification

Kayser-Fleischer rings are generally classified under the H18.04 code (general code, not used for reinbursement purposes). [16]

Differential Diagnosis

Although Kayser-Fleischer rings are usually present in Wilson's disease, they can also be rarely found in non-Wilsonian liver disease. [17] [18]

These rings are different from the yellowish to dark-brown Fleicher rings, a common presentation in keratoconus, another ophthalmological condition caused by build-up of iron in the corneal limbus. [19] However, there is a case study reporting the co-existense of both rings in a young adult. [20]

Treatment

Treatment of Kayser-Fleischer rings involves the treatment of the underlying cause.

Medication

Treatment with chelators is about reversing the positive copper balance and are considered the treatment of choice in Wilson's disease. [21] Early approaches involved medications such as penicillamine (Cupramine, Depen) and trientine (Syprine) that bind to copper and prevent its accumulation in the body by to getting rid of it in the urine; more recent approaches used zinc, which serves as a blocker to the absorption of copper and promoter of copper intestinal excretion. [21]

For the initial treatment of the patient presenting with mild liver failure, some authors use a combination of trientine and zinc . [22] Another drug, tetrathiomolybdate forms a tripartite complex with copper and protein, preventing copper intestinal absorption, or render blood copper non-toxic. [22] In cases of clinical neurological presentations, tetrathiomolybdate, may provide more rapid, safe control of copper compared to penicillamine and zinc. [22]

Those with acute liver failure or severe neurological symptoms that do not respond to pharmacological treatment are more suitable for liver transplant. [23]

Dietary Modifications

Although diet on its own is not adequate for restoring copper balance, patients are advised to avoid foods with high concentrations of copper such as shellfish, nuts, chocolate, mushrooms, and organ meats - especially in the first year of treatment. [1]

Other Therapies

Current guidelines [24] on Wilson's disease suggest that therapies with better efficacy, ease of administration and improved safety profiles are needed. Research efforts are focusing on new pharmacological and treatment options as well as gene therapies to restore the function of ATP7B. [25]

Case Studies

An interesting case study of a patient presenting with abdominal distention and melena where Kayser-Fleischer rings were also observed is found here.

For a case of Kayser-Fleischer rings with sunflower cataract you can click here.

The case report of both Kayser-Fleischer ring and keratoconus in the same person can be found here.

A case of a pseudo-Kayser-Fleischer ring in Sturge-Weber syndrome is available here.

A case presentation of Kayser-Fleischer like rings in a patient with cryptogenic cirrhosis is found here.

Resources

Wilson Disease Association page on Kayser-Fleischer Rings.

EASL-ERN Clinical Practice Guidelines on Wilson's Disease, 2025 update

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Pandey N, Blair K, John S. Kayser-Fleischer Ring. [Updated 2024 Jan 25]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459187/
  2. ↑ 2.0 2.1 Prasad D, Bhriguvansh A.Ocular manifestations of liver disease in children: Clinical aspects and implications. Ann Hepatol. 2020; 19(6): 608-13.
  3. ↑ Patel R, Nair S, Choudhry H, Jaffry M, Dastjerdi M. Ocular manifestations of liver disease: an important diagnostic aid. Int Ophthalmol. 2024 Apr 15;44(1):177.
  4. ↑ Kaler SG. Inborn errors of copper metabolism. Handb Clin Neurol. 2013;113:1745-54.
  5. ↑ Sridhar MS, Rangaraju A, Anbarasu K, Reddy SP, Daga S, Jayalakshmi S, Shaik B. Evaluation of Kayser-Fleischer ring in Wilson disease by anterior segment optical coherence tomography. Indian J Ophthalmol. 2017 May;65(5):354-357.
  6. ↑ Oracz G, Klimczak-Slaczka D, Sokołowska-Oracz A, Socha P, Grałek M, Szaflik J, Dadalski M. Ocena czestości wystepowania pierścienia Kaysera-Fleischera u pacjentów z choroba Wilsona [Prevalence of Kayser-Fleischer ring in patients with Wilson's disease]. Klin Oczna. 2005;107(1-3):54-6. Polish.
  7. ↑ Steindl P, Ferenci P, Dienes HP, Grimm G, Pabinger I, Madl C, Maier-Dobersberger T, Herneth A, Dragosics B, Meryn S, Knoflach P, Granditsch G, Gangl A. Wilson's disease in patients presenting with liver disease: a diagnostic challenge. Gastroenterology. 1997 Jul;113(1):212-8.
  8. ↑ Gow PJ, Smallwood RA, Angus PW, Smith AL, Wall AJ, Sewell RB. Diagnosis of Wilson's disease: an experience over three decades. Gut. 2000 Mar;46(3):415-9.
  9. ↑ Litwin T, Dusek P, Szafrański T, Dzieżyc K, Członkowska A, Rybakowski JK. Psychiatric manifestations in Wilson's disease: possibilities and difficulties for treatment. Ther Adv Psychopharmacol. 2018 Jul;8(7):199-211.
  10. ↑ Członkowska A, Litwin T, Chabik G. Wilson disease: neurologic features. Handb Clin Neurol. 2017;142:101-9.
  11. ↑ Kuan P. Cardiac Wilson's disease. Chest. 1987 Apr;91(4):579-83.
  12. ↑ Frikha R, Abdelmoula NB, Rebai T. Wilson disease, genotype and infertility: is there a correlation? Endocrine. 2013 Aug;44(1):266-7.
  13. ↑ McIntyre N, Clink HM, Levi AJ, Cumings JN, Sherlock S. Hemolytic anemia in Wilson's disease. N Engl J Med. 1967 Feb 23;276(8):439-44.
  14. ↑ Langwińska-Wośko E, Litwin T, Dzieżyc K, Członkowska A. The sunflower cataract in Wilson's disease: pathognomonic sign or rare finding? Acta Neurol Belg. 2016 Sep;116(3):325-8.
  15. ↑ 15.0 15.1 15.2 Sridhar MS, Rangaraju A, Anbarasu K, Reddy SP, Daga S, Jayalakshmi S, Shaik B. Evaluation of Kayser-Fleischer ring in Wilson disease by anterior segment optical coherence tomography. Indian J Ophthalmol. 2017 May;65(5):354-57.
  16. ↑ 2025 ICD-10-CM Diagnosis Code H18.04. Available from: https://www.icd10data.com/ICD10CM/Codes/H00-H59/H15-H22/H18-/H18.04 [accessed 15/7/2025]
  17. ↑ Frommer D, Morris J, Sherlock S, Abrams J, Newman S. Kayser-Fleischer-like rings in patients without Wilson's Disease. Gastroenterology. 1977; 72:1331-35.
  18. ↑ Tauber J, Steinert RF. Pseudo-Kayser-Fleischer ring of the cornea associated with non-Wilsonian liver disease. A case report and literature review. Cornea. 1993 Jan;12(1):74-7.
  19. ↑ Amalnath DS, Subrahmanyam DK. Ocular signs in Wilson disease. Ann Indian Acad Neurol. 2012 Jul;15(3):200-1.
  20. ↑ Hu P, Lin L, Wu Z, Jin X, Ni H. Kayser–Fleischer ring with keratoconus: a coincidence? A case report. BMC Ophthalmol 2020; 20:190.
  21. ↑ 21.0 21.1 Brewer GJ, Dick RD, Johnson VD, Brunberg JA, Kluin KJ, Fink JK. Treatment of Wilson's disease with zinc: XV long-term follow-up studies. J Lab Clin Med. 1998 Oct;132(4):264-78.
  22. ↑ 22.0 22.1 22.2 Brewer GJ. Practical recommendations and new therapies for Wilson's disease. Drugs. 1995 Aug;50(2):240-9.
  23. ↑ Litwin T, Bembenek J, Antos A, Przybyłkowski A, Skowrońska M, Kurkowska-Jastrzębska I, Członkowska A. Liver transplantation as a treatment for Wilson's disease with neurological presentation: a systematic literature review. Acta Neurol Belg. 2022 Apr;122(2):505-18.
  24. ↑ Socha P, et al. EASL-ERN Clinical Practice Guidelines on Wilson's Disease. J Hepatol 2025; 82(4):690-728
  25. ↑ Litwin T, Dzieżyc K, Członkowska A. Wilson disease-treatment perspectives. Ann Transl Med. 2019 Apr;7(Suppl 2):S68.
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