Priapism

What is Priapism?

Priapism is a condition where a man’s penis stays erect for a long period without any sexual stimulation. Generally, any erection that lasts for four hours or more is considered priapism.

This condition basically falls into three types: ischemic, non-ischemic, and recurrent ischemic. Priapism caused by ischemia, or the lack of blood flow, is a serious situation and needs immediate attention to prevent potential harm to the penis that could lead to permanent erectile dysfunction, i.e., difficulty achieving or maintaining an erection.

It’s of great importance to manage this condition as soon as possible in order to retain normal erectile function. If not treated, the tissue in the penis can die and eventually turn into scar tissue, which can result in permanent erectile dysfunction.

What Causes Priapism?

Priapism is a condition where a persistent, often painful erection occurs without sexual stimulation. Its causes can generally be divided into two groups: low flow (caused by less blood flow) and high flow (caused by too much blood flow).

Low flow, or ischemic priapism, can be caused by a large variety of factors. Some of these include certain blood disorders like sickle cell disease and thalassemia, as well as any condition that makes your blood clot more easily. Erectile dysfunction medications and injections are often linked to an increased occurrence of priapism, and they’re thought to cause at least 25% of all cases. Other drugs like antidepressants and cocaine can also cause priapism. More uncommonly, it can be caused by certain cancer types, nerve disorders, kidney dialysis, and even specific infections that cause your blood to clot more easily.

Often the causes of ongoing low flow priapism are the same as those that cause single episodes. However, there are cases where problems in the body’s regulation systems can cause repeated episodes of priapism.

Despite what you might expect, erectile dysfunction drugs, like Viagra and Cialis, aren’t the most commonly linked to causing priapism. Antipsychotics and certain antidepressants are more than twice as likely to cause it.

Certain male pelvic cancers can cause priapism by either directly invading the tissues there, or by blocking blood outflow. An MRI can be used to identify this. While fairly rare, there have been reported cases of priapism linked to several unusual causes. These include other drugs, leukemia, Covid-19, scorpion and spider bites, spinal cord injuries, and electronic cigarettes.

High flow, or non-ischemic priapism, is less common and usually results from a direct injury or trauma. It can also be caused by injuries during surgery, birth defects in the arteries, or cancer. Sometimes, there’s no obvious cause for this form of priapism to occur.

Risk Factors and Frequency for Priapism

Priapism, a condition that causes long-lasting, painful erections, can be triggered by several factors. In about two-thirds of patients, it is caused by using certain drugs to treat erectile dysfunction. Sickle cell disease is another common cause, especially among African Americans, and it accounts for between 40% and 80% of adult priapism cases.

Priapism affects people of all ages, but it is most common in children aged 7 to 10 and adults aged 20 to 50.
In younger people, it is usually due to sickle cell disease, while in older people, it is more often caused by the side effects of medication.
Overall, priapism is estimated to affect 0.73 to 5.4 per 100,000 men each year.
Rates following treatment for erectile dysfunction with drug injections into the penis are between 1.3% and 5.3%.
This kind of treatment is more likely to cause priapism in younger men or in patients with neurological or psychological forms of erectile dysfunction.
Priapism is 30% more common in the summer.
Of the people who go to the emergency department with priapism, 13% end up being admitted to the hospital.

Although priapism mainly affects males, there have been a few rare cases reported in females.

Signs and Symptoms of Priapism

Priapism is a condition where a man experiences abnormal and often uncomfortable prolonged erections. When a patient comes in with priapism, it’s crucial to find out how long the erection has been lasting. By understanding the duration and history of priapism, doctors can identify the cause and determine the specific type of priapism. Useful information includes how long the symptoms have lasted, any treatments or injections the person has had, how their erectile function was before the episode, and whether they’ve had priapism before and how it was treated.

Current medication usage and history of any related diseases, such as sickle cell, or injuries to the penis, pelvis, or perineum are also important. Sometimes, illegal drug use or alcohol intoxication can contribute to priapism, accounting for up to 21% of cases with the blood flow restricted type of priapism. Whether the person is feeling pain can help doctors tell the difference between the types of priapism, as absence of pain usually suggests a non-blood flow restricted type priapism. In very rare and serious cases, blood flow restricted priapism can result in gangrene, causing tissue death in the penis.

During a physical exam, the doctor will touch the penis to find out if there’s any pulse that might suggest high-flow priapism. In blood flow restricted priapism, the penis is hard and fully erect, often with some tenderness. The penis head will most likely feel soft or only partially hard. If there’s little to no tenderness, or if the penis is not fully hard, the patient might have a non-blood flow restricted priapism.

Non-blood flow restricted priapism usually comes with less rigid erections and less pain compared to blood flow restricted priapism. This condition can be triggered by a needle procedure, injury to the perineum, or a recent procedure by a urologist. The doctor will not only examine the genital area, but also the perineum and abdomen for signs of injury, bruising, or possible cancer. Getting a complete medical history and conducting a thorough physical exam will help to identify the cause behind priapism.

Testing for Priapism

If you go to the doctor because you’re suffering from priapism, which is a painful and prolonged erection not associated with sexual arousal, the doctor will start by asking you questions about your symptoms and examining you physically. If they can’t find the cause of your priapism this way, they will then check the blood flow in your penis and test the gases in your blood. They might do this by using a small needle to draw some blood from your penis.

The levels of acidity (pH), oxygen (pO2), and carbon dioxide (pCO2) in your blood can give the doctor clues about what’s causing your priapism. For example, in ischemic priapism, the most common type caused by blood not being able to leave the penis, the blood drawn tends to be dark or blackened, the pH less than 7.0, pO2 less than 30 mmHg, and pCO2 greater than 60 mmHg. On the other hand, if your priapism is caused by too much blood flowing to your penis (non-ischemic priapism), the blood will be red and have more normal gas levels – pH near 7.4, and pO2/pCO2 levels closer to 90 mmHg and 40 mmHg, respectively.

Additional laboratory tests may be conducted to identify any underlying or unknown diseases that could be triggering your priapism. These tests might include a complete blood count, checks of reticulocytes (young red blood cells), and checks for abnormal hemoglobin, a protein found in red blood cells. Screening for sickle cell disease, which is a common cause of priapism, is also recommended for anyone at risk. If alcohol or drug misuse is suspected, your blood alcohol levels and/or drug toxicology might be checked.

Imaging of the penis can also be used to aid in working out the cause of the priapism. For example, a colorduplex ultrasound can assess blood flow in the penis, and can help doctors distinguish between ischemic and non-ischemic priapism, as well as identify any abnormal structures. On this type of ultrasound, low blood flow can suggest ischemic priapism, whereas normal or high blood flow may point to non-ischemic priapism. Magnetic Resonance Imaging (MRI) or Magnetic Resonance Angiography (MRA) can also be used to investigate for other causes like cancer or blood clots. It may also predict if the penile tissue can be saved in cases of prolonged priapism.

If the doctor suspects sickle cell disease, they might measure the level of hemoglobin S in your blood. You might also need a ‘type and screen test’ (to determine blood type and check for antibodies), as some patients with sickle cell disease and priapism may need a procedure called a plasma exchange. Blood alcohol levels and/or a drug screen could be relevant too.

A condition similar to priapism, known as “Sleep-Related Painful Erections”, is also worth mentioning here, although it is quite rare. It involves painful erections during REM sleep that cause frequent awakenings and disrupt sleep. The erections last between 15 to 60 minutes – not long enough to be classified as priapism. This condition doesn’t affect normal daytime sexual activities, testosterone levels, or sexual desire, and it isn’t associated with blood disorders – unlike priapism. Its cause is unclear, but it may be linked to overly tight pelvic floor muscles. Physical therapy can help in some cases, and the drug baclofen appears to be the most effective treatment. Surgical interventions and penile prostheses – artificial devices placed inside the penis to enable an erection – don’t tend to reduce pain for these patients.

Treatment Options for Priapism

Ischemic priapism is a condition in which blood flow to the penis is blocked, leading to a persistent erection that lasts for four hours or longer. If left untreated for such a long time, it can cause long-term damage and may result in future erectile dysfunction. Hence, it’s treated as a medical emergency.

Initially, oral medicines like pseudoephedrine could be used while preparing for more advanced treatments. However, oral therapies often have high failure rates, necessitating the need for more invasive procedures.

One of these procedures is blood aspiration where a needle is used to draw out blood from the penis, combined with a saline solution to help flush out remaining blood. During this process, oxygenated blood (bright red in color) usually begins to appear, which is a good sign. However, aspiration alone is only effective in about a third of patients and typically needs to be combined with other treatments.

If the condition persists, drugs may be directly injected into the penis. The preferred choice for this is phenylephrine because it tends to be both effective and safe. During this treatment, vital signs, including heart rate and blood pressure, are carefully monitored. In rare cases where these treatments don’t work, surgical intervention might be required.

Non-ischemic priapism, on the other hand, happens when blood flow to the penis isn’t blocked but still causes prolonged erections. Unlike ischemic priapism, this is usually not painful and doesn’t pose a risk of long-term damage. Therefore, non-invasive treatments like applying ice packs or simple observation are often prescribed. If these fail, minimally invasive treatments like tying off an abnormal blood vessel using specialized medical imaging or, less commonly, surgical intervention might be needed.

It’s important to note that the longer the condition persists before receiving medical intervention, the more damage it can cause. Therefore, if you experience an erection lasting four hours or longer, you should seek immediate medical attention.

There are several conditions and circumstances that could lead to pain or discomfort in the genital area, including:

Peyronie’s disease, a condition where fibrous scar tissue grows inside the penis
Physical injury or trauma to the genitals
An installed penile implant
Cancers in the pelvic region
Use of illegal drugs such as cocaine
Insertion of a foreign object into the penis or urethra
Painful erections during the night
Paraphimosis, a condition where the foreskin cannot be pulled back from the tip of the penis
Penile fracture, or a rupture in one of the tubes in the penis
A partial or complete rupture of the urethra, the tube that carries urine out of the body

What to expect with Priapism

The severity of a patient’s condition and recovery prospects depend on various factors such as how long they have been experiencing symptoms, their overall health, other existing health conditions they may have, and their age. Generally, the longer someone has been experiencing symptoms, the worse their outcome may be.

One of the significant risks to consider is the high chance of long-term or even permanent problems with erectile function, especially in prolonged cases of priapism, even when treated appropriately. Priapism is a condition where one experiences a persistent and often painful erection lasting longer than 4 hours. It’s crucial to know that patients who have had an episode of priapism are likely to experience it again in the future.

Infections are a common cause of less desirable outcomes. Therefore, it is vital for younger men, especially those with sickle cell disease, to be educated about priapism early. Sickle cell disease is a genetic disorder that affects the red blood cells and can occasionally lead to priapism. Quick treatment when priapism occurs can help minimize permanent damage and prevent long-term erectile dysfunction.

Possible Complications When Diagnosed with Priapism

: It’s possible for long-term erectile dysfunction to occur as a result of damage from priapism, a condition where an erection lasts too long. The longer the erection, the more damage potentially done to the tissues in the penis.

One rare complication of priapism is the death of tissue in the glans, or the head of the penis. However, non-surgical treatments, such as a Winter or similar type of shunt (a technique to reduce blood flow) along with continuous flushing with normal or blood-thinning saline solutions, have reportedly been more successful than immediate surgery.

Common Risks:

Long-term erectile dysfunction
Damage to penile tissues
Death of tissue in the head of the penis (very rare)

Treatments:

Non-surgical options such as a Winter or similar type of shunt
Continuous flushing with normal or blood-thinning saline solutions
Surgical excision (less preferred)

Preventing Priapism

Regular check-ups are really important to make sure the treatment is working as it should. Patients who have a chance of their condition returning might be given one or more medications that have been found to help keep it under control. The medications used most often include bicalutamide, finasteride, sildenafil, baclofen, gabapentin, hydroxyurea (but only for patients with a blood disorder called sickle cell), phenylephrine, pseudoephedrine, and IM leuprolide. Doctors will decide whether these should be given alone or in combination, based on the individual patient’s needs.

Frequently asked questions

Priapism is a condition where a man's penis stays erect for a long period without any sexual stimulation.

Priapism is estimated to affect 0.73 to 5.4 per 100,000 men each year.

Signs and symptoms of priapism include: - Abnormal and prolonged erections that are often uncomfortable. - Duration of the symptoms, as it is crucial to determine how long the erection has been lasting. - History of priapism, including previous episodes and how they were treated. - Current medication usage and history of related diseases or injuries, such as sickle cell or trauma to the penis, pelvis, or perineum. - Possible contribution of illegal drug use or alcohol intoxication, accounting for up to 21% of cases with the blood flow restricted type of priapism. - Presence or absence of pain, as the absence of pain usually suggests a non-blood flow restricted type of priapism. - Rare and serious cases of blood flow restricted priapism can result in gangrene and tissue death in the penis. - Physical examination findings, such as the presence or absence of a pulse in the penis, tenderness, rigidity of the erection, and the condition of the penis head. - Non-blood flow restricted priapism may have less rigid erections and less pain compared to blood flow restricted priapism. - Possible triggers for non-blood flow restricted priapism, including needle procedures, perineal injury, or recent urological procedures. - Examination of the genital area, perineum, and abdomen for signs of injury, bruising, or possible cancer.

Priapism can be caused by various factors, including certain blood disorders like sickle cell disease and thalassemia, conditions that increase blood clotting, certain medications (such as erectile dysfunction drugs, antidepressants, and antipsychotics), certain cancers, nerve disorders, kidney dialysis, specific infections, injuries or trauma to the penis, birth defects in the arteries, and sometimes there is no obvious cause.

The doctor needs to rule out the following conditions when diagnosing Priapism: 1. Peyronie's disease, a condition where fibrous scar tissue grows inside the penis. 2. Physical injury or trauma to the genitals. 3. An installed penile implant. 4. Cancers in the pelvic region. 5. Use of illegal drugs such as cocaine. 6. Insertion of a foreign object into the penis or urethra. 7. Painful erections during the night. 8. Paraphimosis, a condition where the foreskin cannot be pulled back from the tip of the penis. 9. Penile fracture, or a rupture in one of the tubes in the penis. 10. A partial or complete rupture of the urethra, the tube that carries urine out of the body.

The types of tests that may be needed to diagnose priapism include: 1. Physical examination: The doctor will ask questions about symptoms and perform a physical examination to assess the condition. 2. Blood tests: These tests can provide information about the underlying cause of priapism. They may include: - Blood gas analysis: Measures the levels of acidity (pH), oxygen (pO2), and carbon dioxide (pCO2) in the blood. - Complete blood count: Checks for any abnormalities in the blood. - Reticulocyte count: Measures the number of young red blood cells. - Hemoglobin screening: Tests for abnormal hemoglobin, a protein found in red blood cells. - Sickle cell disease screening: Recommended for anyone at risk. 3. Imaging tests: These tests can help identify the cause of priapism and assess blood flow in the penis. They may include: - Color duplex ultrasound: Assesses blood flow in the penis and distinguishes between ischemic and non-ischemic priapism. - Magnetic Resonance Imaging (MRI) or Magnetic Resonance Angiography (MRA): Used to investigate other causes like cancer or blood clots. 4. Additional tests: Depending on the suspected cause, additional tests may be conducted, such as measuring the level of hemoglobin S in the blood for sickle cell disease or checking blood alcohol levels and drug toxicology for alcohol or drug misuse. It's important to note that the specific tests ordered may vary depending on the individual case and the doctor's clinical judgment.

Priapism can be treated through various methods depending on the type of priapism. For ischemic priapism, initial treatment may involve oral medicines like pseudoephedrine, but if these fail, more invasive procedures such as blood aspiration or direct injection of drugs like phenylephrine may be necessary. In some rare cases, surgical intervention might be required. On the other hand, non-ischemic priapism is usually treated with non-invasive methods like applying ice packs or simple observation. If these methods fail, minimally invasive treatments or surgical intervention may be considered. It is important to seek immediate medical attention if an erection lasts for four hours or longer.

The side effects when treating Priapism include: - Long-term erectile dysfunction - Damage to penile tissues - Death of tissue in the head of the penis (very rare)

The prognosis for priapism depends on various factors, including the duration of symptoms, overall health, existing health conditions, and age of the patient. Generally, the longer someone has been experiencing symptoms, the worse their outcome may be. There is a high chance of long-term or permanent problems with erectile function, especially in prolonged cases of priapism, even when treated appropriately. Patients who have had an episode of priapism are likely to experience it again in the future. Quick treatment can help minimize permanent damage and prevent long-term erectile dysfunction.

You should see a urologist for priapism.