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Ischemic Priapism

Introduction
Signs and symptoms
Causes
Risk Factors
Screening and diagnosis
Treatment
Prevention

Ischemic Priapism is a condition characterized by a sustained, persistent, frequently painful erection. It's not associated with sexual stimulation or desire.

In normal sexual functioning, an erection occurs when your penis fills with blood, and it subsides upon orgasm as excess blood leaves your penis. But with priapism, this blood doesn't drain from the penile shaft upon orgasm.

Priapism can occur at any age, although it's most prevalent in boys between ages 5 and 10 years old, and men from ages 20 to 50 years. An uncommon condition, priapism is usually an emergency that needs immediate medical attention. Delaying treatment can result in permanent scarring of your penis, interfering with the ability to achieve a normal erection. However, when treated promptly, priapism has no lasting negative effects.

The word "priapism" is derived from Latin mythology, and specifically from Priapus, the name of a male fertility god.

Priapism causes abnormally persistent erections. The signs and symptoms may include:

• An erection lasting at least four to six hours
• An erection not associated with sexual desire, or that continues even after sexual stimulation or orgasm ends
• A painful erection
• A firm penile shaft, but a soft tip of the penis

Some cases of priapism can cause "stuttering" - intermittent symptoms lasting for several minutes to as long as three hours, and then resolving on their own. In people with stuttering priapism, painful erections can occur repeatedly.

The cause of priapism is a malfunctioning of the blood vessels and nerves, in which blood becomes trapped in, and thus cannot drain from the tissues of the penis that are associated with erections.

Use or misuse of certain drugs may lead to priapism. Most people can use these medications with an extremely low risk of priapism. However, in certain people, signs and symptoms can begin within weeks to months after starting these medications. These drugs include:

• Oral medications used to manage erectile dysfunction, such as sildenafil (Viagra) and vardenafil (Levitra)
• Drugs injected directly into the penis to treat erectile dysfunction, such as papaverine
• Antidepressants, such as trazodone (Desyrel)
• Drugs used to treat psychotic disorders, such as chlorpromazine (Thorazine), risperidone (Risperdal) and olanzapine (Zyprexa)
• Anti-anxiety medications, such as diazepam (Valium)
• Blood thinners, such as heparin and warfarin (Coumadin)
• Blood pressure lowering medications

Other causes of priapism include:

• Trauma or injury to your genitals, groin or spinal cord
• Blood clots
• Leukemia
• Inflammation of the tube through which urine flows out of your body (urethra)
• A tumor located in your pelvis
• Abuse of alcohol, cocaine or marijuana
• Sickle cell anemia, an inherited disease characterized by a deficiency in healthy red blood cells, and which can interfere with blood flowing out of an erect penis
• Fabry's disease, a metabolic disorder in which fats are not properly broken down in your digestive system
• Carbon monoxide poisoning
• Black widow spider bites

In some cases, doctors are unable to find a specific cause for priapism.

Doctors divide priapism into two subtypes:

• Ischemic (or low-flow) priapism. In this type, blood flow out of the penis is impaired or obstructed, often in otherwise healthy people. This form accounts for about 90 percent of cases of priapism.
• Nonischemic (or high-flow) priapism. This less common type tends to be less painful or pain-free. It's most often associated with a ruptured artery due to trauma and injury to the penis or to the area between the scrotum and the anus (perineum).

Your doctor will begin the diagnostic process by taking a medical history, asking questions such as:

• How long have you had an erection?
• How does this compare with how long your erections normally last?
• Have you had previous episodes of lengthy erections?
• Have you recently experienced an injury or trauma to the groin or nearby areas of the body?
• Are you feeling pain? Ischemic priapism is a painful condition, while nonischemic priapism is more likely not to cause pain.
• What medications or drugs are you taking - prescription, over-the-counter or illegal?
• Do you consume alcohol?

Doctor will conduct a physical examination, looking for evidence of trauma or inflammation of the urethra. Your doctor will examine your abdomen, perineum and genitals, and check for signs of tumors that could be related to priapism.

Your doctor may also recommend or perform the following tests to help make the diagnosis:

• Blood gas measurement. In this test, a tiny needle is inserted into your penis to remove a small sample of blood, which is sent to the laboratory. The lab analysis can provide information that may help your doctor determine the extent of any damage to your penis, how long priapism has existed, and whether it's ischemic or nonischemic.
• Blood tests. These tests, providing a complete blood count and platelet count, can help detect acute infections that might be causing symptoms, or blood abnormalities such as sickle cell disease.
• Ultrasound. You may undergo a color duplex ultrasonography, which is a non-radiation medical imaging procedure using sound waves. Or, your doctor may have you undergo a penile angiogram, which involves injecting dye into the vessels of your penis to improve the quality of imaging. This test can be used to evaluate blood flow within your penis. Ultrasound can differentiate between ischemic and nonischemic priapism, as well as detect any other abnormalities in your penis that could suggest another medical condition.

If you believe that you may have priapism, do not try self-treating the condition, but rather contact your doctor or go to an emergency room.

Nonischemic priapism often improves on its own without treatment, and thus your doctor may recommend a period of nontreatment and observation. Applying ice packs and compression to the penis and perineum may minimize any swelling and improve an occurrence of nonischemic priapism.

Ischemic priapism is an emergency situation that requires immediate treatment. In its management, the goal of doctors is to bring the erection to an end.

The most commonly used therapeutic choices for ischemic priapism include:

• Intracavernous injection. When priapism has been caused by drug injections into the penis to treat impotence, other medications called sympathomimetic agents (epinephrine, norepinephrine, phenylephrine, ephedrine, metaraminol) can be injected to constrict or narrow the blood vessels in the penis, allowing less blood to flow into the penis and more to flow out; thus the priapism subsides. This approach has shown varying degrees of success. Side effects of sympathomimetic drugs may include headaches, heart palpitations and acute high blood pressure.
• Aspiration. After your penis is numbed with local anesthetic, excess blood is drained from it, using a small needle and syringe. As part of this procedure, the penile veins are also irrigated with saline solution. Aspiration is sometimes performed as an adjunct to intracavernous injections. This treatment is generally used only for treating ischemic priapism.
• Surgery. If other treatments are unsuccessful, surgery is an option. Surgery is most appropriate if you have a ruptured penile artery due to trauma. Your surgeon can restore normal blood flow by tying off the artery associated with priapism. In some surgical procedures, the surgeon will implant a shunt, which is a device that reconfigures blood flow so that it moves through your penis normally.

If a condition like sickle cell anemia or a tumor is the cause of priapism, treatment focuses on managing the underlying disease.

If you've experienced one or more episodes of priapism, you may be able to avoid further incidents by staying away from triggering factors such as certain medications, drugs or alcohol.

As a preventive measure, doctors sometimes prescribe the drugs pseudoephedrine and terbutaline (Brethine) for people who have had episodes of stuttering priapism. You can take these medications orally or self-inject them in the earliest stages of an abnormal erection. A number of other medications may be effective in preventing priapism, including hormonal drugs and the muscle relaxant baclofen (Lioresal).

Proper treatment of sickle cell disease, including the use of the drug hydroxyurea (Hydrea), may help prevent priapism.