Kayser–Fleischer rings (KF rings) are dark rings that appear to encircle the

cornea The cornea is the transparency (optics), transparent front part of the eyeball which covers the Iris (anatomy), iris, pupil, and Anterior chamber of eyeball, anterior chamber. Along with the anterior chamber and Lens (anatomy), lens, the cornea ...

of the

eye An eye is a sensory organ that allows an organism to perceive visual information. It detects light and converts it into electro-chemical impulses in neurons (neurones). It is part of an organism's visual system. In higher organisms, the ey ...

. They are due to

copper Copper is a chemical element; it has symbol Cu (from Latin ) and atomic number 29. It is a soft, malleable, and ductile metal with very high thermal and electrical conductivity. A freshly exposed surface of pure copper has a pinkish-orang ...

deposition in the

Descemet's membrane Descemet's membrane ( or the Descemet membrane) is the basement membrane that lies between the corneal proper substance, also called stroma, and the endothelial layer of the cornea. It is composed of different kinds of collagen (Type IV and VIII ...

as a result of particular liver diseases. They are named after

German German(s) may refer to: * Germany, the country of the Germans and German things **Germania (Roman era) * Germans, citizens of Germany, people of German ancestry, or native speakers of the German language ** For citizenship in Germany, see also Ge ...

ophthalmologist Ophthalmology (, ) is the branch of medicine that deals with the diagnosis, treatment, and surgery of eye diseases and disorders. An ophthalmologist is a physician who undergoes subspecialty training in medical and surgical eye care. Following a ...

s Bernhard Kayser and

Bruno Fleischer Bruno Otto Fleischer (2 May 1874 – 26 March 1965) was a German ophthalmologist. Kayser–Fleischer rings and Fleischer ring Fleischer rings are pigmented rings in the peripheral cornea, resulting from iron deposition in basal epithelial cells, ...

who first described them in 1902 and 1903. Initially thought to be due to the accumulation of silver, they were first demonstrated to contain copper in 1934.

Presentation

The rings, which consist of

deposits where the

meets the

sclera The sclera, also known as the white of the eye or, in older literature, as the tunica albuginea oculi, is the opaque, fibrous, protective outer layer of the eye containing mainly collagen and some crucial elastic fiber. In the development of t ...

, in

, first appear as a crescent at the top of the cornea. Eventually, a second crescent forms below, at the "six o'clock position", and ultimately completely encircles the cornea.

Associations

Kayser–Fleischer rings are a sign of

Wilson's disease Wilson's disease (also called hepatolenticular degeneration) is a genetic disorder characterized by the excess build-up of copper in the body. Symptoms are typically related to the brain and liver. Liver-related symptoms include vomiting, wea ...

, which involves abnormal copper handling by the liver resulting in copper accumulation in the body and is characterised by abnormalities of the

basal ganglia The basal ganglia (BG) or basal nuclei are a group of subcortical Nucleus (neuroanatomy), nuclei found in the brains of vertebrates. In humans and other primates, differences exist, primarily in the division of the globus pallidus into externa ...

of the

brain The brain is an organ (biology), organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It consists of nervous tissue and is typically located in the head (cephalization), usually near organs for ...

liver cirrhosis Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, chronic liver failure or chronic hepatic failure and end-stage liver disease, is a chronic condition of the liver in which the normal functioning tissue, or parenchyma, is replaced ...

splenomegaly Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of ''hypersplenism'' which include: some reduction in number of circulat ...

, involuntary movements,

muscle rigidity Hypertonia is a term sometimes used synonymously with ''spasticity'' and ''rigidity'' in the literature surrounding damage to the central nervous system, namely upper motor neuron lesions. Impaired ability of damaged motor neurons to regulate ...

, psychiatric disturbances,

dystonia Dystonia is a neurology, neurological Hyperkinesia, hyperkinetic Movement disorders, movement disorder in which sustained or repetitive muscle contractions occur involuntarily, resulting in twisting and repetitive movements or abnormal fixed po ...

and

dysphagia Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or l ...

. The combination of neurological symptoms, a low blood

ceruloplasmin Ceruloplasmin (or caeruloplasmin) is a ferroxidase enzyme that in humans is encoded by the ''CP'' gene. Ceruloplasmin is the major copper-carrying protein in the blood, and in addition plays a role in iron metabolism. It was first described in ...

level and KF rings is diagnostic of Wilson's disease. Other causes of KF rings are

cholestasis Cholestasis is a condition where the flow of bile from the liver to the duodenum is impaired. The two basic distinctions are: * obstructive type of cholestasis, where there is a mechanical blockage in the duct system that can occur from a gallston ...

(obstruction of the

bile duct A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates. The bile duct is separated into three main parts: the fundus (superior), the body (middle), and the neck (inferior). Bile is requ ...

s),

primary biliary cirrhosis Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to buil ...

and "cryptogenic" cirrhosis (cirrhosis in which no cause can be identified).

Diagnosis

As Kayser–Fleischer rings do not cause any symptoms, it is common for them to be identified during investigations for other medical conditions. In certain situations, they are actively sought; in that case, the early stages may be detected by

slit lamp In ophthalmology and optometry, a slit lamp is an instrument consisting of a high-intensity light source that can be focused to shine a thin sheet of light into the eye. It is used in conjunction with a biomicroscope. The lamp facilitates an ...

examination before they become visible to the naked eye.

References

External links

{{DEFAULTSORT:Kayser-Fleischer ring Disorders of sclera and cornea Eye color

Presentation

Associations

Diagnosis

See also

References

External links