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Showing posts with label priapism. Show all posts
Showing posts with label priapism. Show all posts

Sunday, February 1, 2009

Priapism

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Priapism is the presence of a persistent, usually painful, erection of the penis unrelated to sexual stimulation or desire. It is a true urologic emergency that may lead to permanent erectile dysfunction and penile necrosis if left untreated. Priapism is frequently idiopathic in etiology but is associated with a number of important medical conditions and pharmacologic agents.

Pathophysiology

Priapism is the result of persistent engorgement of the corpora cavernosa of the penis, originating from a disturbance in the mechanisms that control normal penile detumescence. In most cases, the ventral corpora spongiosum and glans penis remain flaccid.
Two types of priapism are generally described. Arterial high-flow priapism usually is secondary to a rupture of a cavernous artery and unregulated flow into the lacunar spaces. This rare type of priapism is usually not painful and results from penetrating penile trauma or a blunt perineal injury. Low-flow priapism is usually due to full and unremitting corporeal veno-occlusion where venous stasis and deoxygenated blood pools within the cavernous tissue. Prolonged veno-occlusive priapism results in fibrosis of the penis and a loss of the ability to achieve an erection. Significant changes at the cellular level are noted within 24 hours in veno-occlusive priapism, whereas arterial priapism is not associated with fibrotic change.

Frequency

The overall incidence of priapism is 1.5 cases per 100,000 person-years, which increases to 2.9 cases per 100,000 person-years for men older than 40 years. In one study, 38-42% of adult patients with sickle cell disease reported at least one episode of priapism.

Mortality/Morbidity

Deaths have been reported in patients with sickle cell disease presenting with priapism, but the cause of death usually is not related to the priapism per se but to complications from the underlying disease process.
Morbidity is related to long-term impotence, primarily with veno-occlusive priapism, when diagnosis and therapy have been delayed.

Race
No racial predilection exists. Sickle cell disease, which predisposes to the development of priapism, occurs more frequently in the African American population.

Sex
Priapism is primarily a disease of males. Priapism of the clitoris has been reported but is extremely rare.

Age
Priapism has been described at nearly all ages, from infancy through old age. A bimodal distribution between 5 and 10 years in children and 20-50 years in adults is noted.
Younger groups are more often associated with sickle cell disease, while older groups tend to be secondary to pharmacologic agents.


History

-History of thromboembolic (eg, sickle cell disease) or neoplastic disease
-Drug history, including injectable medications used for erectile dysfunction such as papaverine, phentolamine, and prostaglandin E1; antipsychotic oral medications use (eg, trazodone)
-Recent illicit drug use (Cocaine, ecstasy, and marijuana use have been associated.)
-History of trauma or activities that may result in the formation of an arterial-venous fistula or shunt (eg, bicycle riding)
-Degree of pain may help to differentiate between high and low flow varieties of priapism.
-Arterial high-flow priapism
Priapism secondary to arterial causes also may be significantly less painful than venous priapism.
Onset of priapism may be delayed after the acute injury. The delay may be due to vessel spasm initially or to the formation of a clot that is gradually reabsorbed over a period of days.
Priapism secondary to arterial causes usually is less tumescent when compared with venous priapism.
-Veno-occlusive priapism
Patients with veno-occlusive priapism present with a painful erection.
Erection may have been present for hours to days.

Physical
-Presence of priapism should be confirmed by the finding of an erect or semierect penis. The ventral glans and corpus spongiosum are rarely rigid.
-Carefully examine for evidence of trauma or unreported injection sites to the genital region.
-Examine the patient for evidence of an underlying condition that may predispose to priapism.
-Piesis sign - Perineal compression with thumb in young children causes prompt detumescence in high-flow priapism.

Causes

-Medications
Only rare case reports of selective cyclic guanosine monophosphate (cGMP) inhibitors such as sildenafil have been associated with priapism. In fact, several case reports suggest sildenafil as a means to treat priapism and may be able to prevent full-blown episodes from occurring in patients with sickle cell disease.
Some patients may use injectable medications to induce an erection. In these patients, excessive use may produce priapism. Examples of agents used to induce an erection include papaverine, phentolamine, and prostaglandin E1.
Many psychotropic medications such as chlorpromazine, trazodone, quetiapine, and thioridazine have been associated with priapism. The newer agents are not immune to this complication. Priapism has been described with citalopram, a selective serotonin reuptake inhibitor.
Rebound hypercoagulable states with anticoagulants such as heparin and warfarin have been associated. Hydralazine, metoclopramide, omeprazole, hydroxyzine, prazosin, tamoxifen, and androstenedione for athletic performance enhancement.
Cocaine, marijuana, and ethanol abuse - Recently, the complication has been described in patients using ecstasy.

-Thromboembolic
Sickle cell disease and thalassemia
Leukemia and multiple myeloma

-Trauma (pelvic, genital, or perineal)

-Neoplastic (may be primary or metastatic)

-Neurologic
Spinal cord injury and anesthesia
Cauda equina compression syndrome

-Infection
Recent infection with Mycoplasma pneumoniae (Mechanism is thought to be a hypercoagulable state induced by the infection.)
Malaria

Other causes
Fabry disease (rare association, occasionally noted to be priapism of the high-flow type) and amyloidosis
Carbon monoxide poisoning, black widow spider bites, and vigorous sexual exercise have been implicated.
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Priapism

Priapism is a persistent, usually painful, erection that lasts for more than four hours and occurs without sexual stimulation. The condition develops when blood in the penis becomes trapped and unable to drain. If the condition is not treated immediately, it can lead to scarring and permanent erectile dysfunction.

It can occur in all age groups, including newborns. However, it usually affects men between the ages of 5 to 10 years and 20 to 50 years.

There are two categories of priapism:
low-flow and high-flow.

Low flow:
This type of priapism is the result of blood being trapped in the erection chambers. It often occurs without a known cause in men who are otherwise healthy, but also affects men with sickle-cell disease, leukemia (cancer of the blood) or malaria.

High flow:
High flow priapism is more rare than low-flow and usually less painful. It is the result of a ruptured artery from an injury to the penis or the perineum (area between the scrotum and anus), which prevents blood in the penis from circulating normally.

What causes priapism?

Sickle cell anemia:
Some adult cases of priapism are the result of sickle-cell disease and approximately 42% of all adults with sickle-cell will eventually develop priapism.

Medications:
A common cause of priapism is the use and/or misuse of medications. Drug-related priapism includes drugs such as Desyrel used to treat depression or Thorazine, used to treat certain mental illnesses. For people who have erectile dysfunction, injection therapy medications to treat the condition may also cause priapism.

Other causes of priapism include:
Trauma to the spinal cord or to the genital area
Black widow spider bites
Carbon monoxide poisoning
Illicit drug use, such as marijuana and cocaine

In rare cases, priapism may be related to cancers that can affect the penis and prevent the outflow of blood.

How is priapism diagnosed?

If you experience priapism, it is important that you seek medical care immediately. Tell your doctor:

The length of time you have had the erection
How long your erection usually lasts
Any medication or drugs, legal or illegal, which you have used. Be honest with your doctor, illegal drug use is particularly relevant since both marijuana and cocaine have been linked to priapism.
Whether or not priapism followed trauma to that area of the body.

Your doctor will review your medical history and perform a thorough physical examination to determine the cause of priapism. This will include checking the rectum and the abdomen for evidence of unusual growths or abnormalities that may indicate the presence of cancer.

After the physical exam is complete, the doctor will take a blood-gas measurement of the blood from the penis. During this test, a small needle is placed in the penis, some of the blood is drawn and then it is sent to a lab for analysis. This provides a clue as to how long the condition has been present and how much damage has occurred.

How is priapism treated?

The goal of all treatment is to make the erection go away and preserve future erectile function. If a person receives treatment within four to six hours, the erection can almost always be reduced with medication.
If the erection has lasted less than four hours, decongestant medications, which may act to decease blood flow to the penis, may be very helpful. Other treatment options include:

Ice packs: Ice applied to the penis and perineum may reduce swelling.
Surgical ligation: Used in cases where an artery has been ruptured, the doctor will ligate (tie off) the artery that is causing the priapism in order to restore normal blood flow.
Intracavernous injection: Used for low-flow priapism, during this treatment drugs known as alpha-agonists are injected into the penis that cause the veins to narrow reducing blood flow to the penis causing the swelling to subside.
Surgical shunt: Also used for low-flow priapism, a shunt is a passageway that is surgically inserted into the penis to divert the blood flow and allow circulation to return to normal.
Aspiration: After numbing the penis, doctors will insert a needle and drain blood from the penis to reduce pressure and swelling.
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