Purtscher's retinopathy
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Purtscher's retinopathy is a disease where part of the eye (
retina The retina (from la, rete "net") is the innermost, light-sensitive layer of tissue of the eye of most vertebrates and some molluscs. The optics of the eye create a focused two-dimensional image of the visual world on the retina, which then ...
) is damaged. Usually associated with severe head injuries, it may also occur with other types of trauma, such as long bone fractures, or with several non-traumatic systemic diseases. However, the exact cause of the disease is not well understood. There are no treatments specific for Purtscher's retinopathy, and the prognosis varies. The disease can threaten vision, sometimes causing temporary or permanent
blindness Visual impairment, also known as vision impairment, is a medical definition primarily measured based on an individual's better eye visual acuity; in the absence of treatment such as correctable eyewear, assistive devices, and medical treatment ...
. It is named for the
Austrian Austrian may refer to: * Austrians, someone from Austria or of Austrian descent ** Someone who is considered an Austrian citizen, see Austrian nationality law * Austrian German dialect * Something associated with the country Austria, for example: ...
ophthalmologist, Othmar Purtscher (1852–1927), who detected it in 1910 and described it fully in 1912.


Presentation


Associated diseases

* Severe head,
chest The thorax or chest is a part of the anatomy of humans, mammals, and other tetrapod animals located between the neck and the abdomen. In insects, crustaceans, and the extinct trilobites, the thorax is one of the three main divisions of the crea ...
, or long bone trauma *
Acute pancreatitis Acute pancreatitis (AP) is a sudden inflammation of the pancreas. Causes in order of frequency include: 1) a gallstone impacted in the common bile duct beyond the point where the pancreatic duct joins it; 2) heavy alcohol use; 3) systemic disea ...
*
Amniotic fluid embolism An amniotic fluid embolism (AFE) is a very uncommon childbirth ( obstetric) emergency in which amniotic fluid enters the blood stream of the mother, triggering a serious reaction, which results in cardiorespiratory (heart and lung) collapse and ...
*
Chronic kidney failure Chronic kidney disease (CKD) is a type of kidney disease in which a gradual loss of kidney function occurs over a period of months to years. Initially generally no symptoms are seen, but later symptoms may include leg swelling, feeling tired, vo ...
*
Dermatomyositis Dermatomyositis (DM) is a long-term inflammatory disorder which affects skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may inc ...
* Fat embolism syndrome *
Scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas ...
* Systemic lupus erythematosus (SLE) *
Thrombotic thrombocytopenic purpura Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, h ...
(TTP)


Pathophysiology

Purtscher's retinopathy likely involves complex pathophysiology, with several contributing factors, including
complement A complement is something that completes something else. Complement may refer specifically to: The arts * Complement (music), an interval that, when added to another, spans an octave ** Aggregate complementation, the separation of pitch-clas ...
-mediated aggregates, fat, air, fibrin clots and platelet clumps. The diseases leads to the formation of
cotton wool spots Cotton wool spots are opaque fluffy white patches on the retina of the eye that are considered an abnormal finding during a funduscopic exam (also called an ophthalmoscopic exam). Cotton wool spots are typically a sign of another disease state, mo ...
in the retina, a finding observed in several other diseases, and atrophy of the
optic nerve In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived fro ...
.


Diagnosis

Where trauma is involved, only a funduscopic examination of the back of the eye (retina) is necessary to make the diagnosis. Fluoroscein angiography may show a decrease in blood flow to the areas of whiteness in the retina.


Treatment

It may be treated with
triamcinolone Triamcinolone is a glucocorticoid used to treat certain skin diseases, allergies, and rheumatic disorders among others. It is also used to prevent worsening of asthma and COPD. It can be taken in various ways including by mouth, injection i ...
in some cases. However, in general, there are no treatments for Purtscher's retinopathy. If it is caused by a systemic disease or emboli, then those conditions should be treated.


Prognosis

Purtscher's retinopathy can lead to loss of vision, and recovery of vision may occur very little. However, vision recovery does occur in some cases, and reports have varied on the long-term prognosis.


History

Purtscher's retinopathy was first characterized in 1910 and 1912 as a
syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek language, Greek σύνδρομον, meaning "concurrence". When a sy ...
of sudden blindness after
head trauma A head injury is any injury that results in trauma to the skull or brain. The terms ''traumatic brain injury'' and ''head injury'' are often used interchangeably in the medical literature. Because head injuries cover such a broad scope of inju ...
, with patches of hemorrhage and whitening of the retina in both eyes. Later, it was discovered to occur after other types of
trauma Trauma most often refers to: * Major trauma, in physical medicine, severe physical injury caused by an external source * Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event *Traumatic i ...
, such as chest trauma, and is associated with several non-traumatic systemic diseases. Purtscher's retinopathy may also be associated with acute
pancreatitis Pancreatitis is a condition characterized by inflammation of the pancreas. The pancreas is a large organ behind the stomach that produces digestive enzymes and a number of hormones. There are two main types: acute pancreatitis, and chronic pancr ...
,
vasculitis Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis (inflammation of lymphatic vessels) is sometimes considered a type of vasculitis. Vasculitis is primarily caused ...
,
embolization Embolization refers to the passage and lodging of an embolus within the bloodstream. It may be of natural origin ( pathological), in which sense it is also called embolism, for example a pulmonary embolism; or it may be artificially indu ...
of such materials as fat and amniotic fluid, systemic lupus erythematosus, thrombotic thrombocytopenic purpura, and chronic kidney failure. Purtscher's retinopathy may be caused by extensive fractures of the long bones.


References


External links

{{Eye pathology Disorders of choroid and retina