Priapism: An Overview

What Is Priapism?

Priapism refers to the persistent or prolonged, painful erection of the penis that occurs without sexual arousal or stimulation. Depending on the type of priapism, it may last for hours and pose dangers for the affected individual.

How Does Priapism Occur?

Priapism results from blood being trapped in the penis with no draining outlet. Left untreated, it can lead to permanent erectile dysfunction (inability to get or maintain an erection for sexual intercourse).

Who Is Affected by Priapism?

Priapism generally affects males 30 years of age and over. However, it can also affect young males when it occurs secondary to blood-related chronic diseases like sickle cell anemia or leukemia.

The commonly affected age groups depending on the type, are:

• 5 - 10 years.

• 20 - 50 years.

What Are the Types of Priapism?

• Ischemic or Low-Flow Priapism: Blood cannot flow out of the veins of the penis and is trapped; it could also occur due to penile smooth muscle contraction problems.

• Stuttering or Intermittent Priapism: Stuttering priapism is a little-known, rarer form of priapism that usually occurs in sickle cell patients - presents as repeated episodes of erections and ischemic priapism.

• Non-ischemic or High-Flow Priapism: Artery rupture due to trauma is the usual cause for this type of priapism - arterial flow in the penis is disrupted. However, there may still be some oxygenation and blood flow.

What Are the Causes of Priapism?

The causative factors responsible for priapism include:

1. Illnesses:

• Blood Disorders: Sickle cell anemia, leukemia, and other blood disorders like multiple myeloma.

• Metabolic Diseases: Gout.

• Neurogenic Diseases: Spinal cord injury.

• Malignancies or Cancers: Cancers of the penis.

2. Substance Abuse: Misuse of alcohol and drugs. Drugs that commonly cause priapism include:

• Cocaine.

• Marijuana.

3. Medications: Priapism may occur as a side effect of various drugs.

4. Other:

• Infections from animal bites or stings (spider and scorpion).

• Carbon monoxide poisoning.

• Accidents or trauma to the penile area is the most typical cause of high-flow priapism, which is quite rare.

What Is Clitoral Priapism?

While priapism has commonly been attributed to a male condition, the rare occurrence of priapism has been noted in females. It is called clitoral priapism and is characterized by prolonged clitoral erection with swelling and pain of the clitoris and surrounding areas. It usually develops secondary to the intake of certain medications (particularly antidepressants like Trazodone) or due to illnesses like sickle cell anemia or other blood disorders. Case studies have shown that termination of the causative agent or drug and replacement with a different drug, prescription pain medication, and decongestants like Pseudoephedrine has provided relief. The rarity of this condition means that available information on it is limited.

What Are the Signs and Symptoms of Priapism?

Low-Flow Priapism:

• Erection has been present for over four hours and is not associated with sexual stimulation or arousal.

• Rigid erection.

• Painful in nature.

• Trauma is not a causative factor.

• Restricted blood and oxygen flow (ischemia) in spongy blood-filled spaces called the corpora are present in the erectile tissue of the penis.

High-Flow Priapism:

• Long-standing history of the condition.

• Soft erection.

• Painless in nature.

• Trauma is the commonest reason for high-flow priapism.

• No evidence of ischemia is seen.

What Are the Consequences of Priapism?

When left untreated, priapism may result in the following complications:

• Permanent Erectile Dysfunction: Permanent inability to get and keep an erection during sexual activity.

• Scarring: Scar tissue formation in the penis.

• Cavernosal Tissue Ischemia: Cavernosal tissue refers to blood-filled spaces lined by endothelium (a membrane) and smooth muscle. It is present in the penile and clitoral erectile tissue. Cavernosal tissue ischemia refers to restricted blood flow and oxygen in the cavernosal tissue area, which may occur secondary to priapism and other causes like atherosclerosis (fat and cholesterol buildup on the walls of arteries).

Is Priapism Dangerous?

Priapism can result in severe life-long consequences, and low-level priapism is considered a medical emergency.

• Erectile Dysfunction: Priapism can cause life-long erectile dysfunction if not treated immediately and correctly.

• Tissue Damage: The lack of oxygen circulation to the penis can permanently damage it.

• Death: While deaths have been reported in priapism patients whose primary illness is a blood disorder like sickle cell anemia, this has been attributed to the effects of the underlying condition rather than as a direct consequence of priapism.

How Is Priapism Managed and Treated?

Basic Life Support: Management begins with treating the condition as an emergency and providing basic life support (BLS) if necessary.

History and Examination: Gleaning a detailed patient history is crucial to understanding the type of priapism (low-level, high-level, or secondary to sickle cell anemia or other blood disorders). Visual examination will also help with this. The history will also provide details on medications, drug abuse, and other features that may be the causative factors.

Investigations:

Special investigations that can be conducted include:

• Screening for Sickle Cells.

• Doppler Ultrasonography: A non-invasive test to estimate blood flow using high-frequency sound waves to view blood moving through the blood vessels in the penis.

• Urinary Drug Screens: To check the urine for the presence of drugs that may cause priapism.

• Penile Blood Gas Measurement: A blood sample is collected from the penis using a needle, and the presence of oxygen is determined. Lack of oxygen indicates ischemic or low-level priapism.

Treatment:

Ischemic or Low-Level Priapism:

• Decongestants: For erections lasting fewer than four hours, decongestants like Pseudoephedrine can decrease blood flow to the penis and provide some relief.

• Ice Packs: Penile engorgement and swelling may be reduced by applying ice.

• Aspiration Decompression: A small needle and syringe may be inserted into the penis, and the blood is drawn out, reducing pain. The erection may also go down.

• Medications: When injected into the penis, medications such as phenylephrine can constrict the blood vessels and prevent the flow and collection of oxygen-poor blood in the penis. Instead, it causes the blood to flow out of the penis.

• Surgical Shunting: A shunt is inserted into the penis - it helps divert the blood flow and restore proper circulation.

Non-ischemic or High-Level Priapism:

• Ice Packs: Applying ice packs to the penis may reduce swelling and cause the erection to go down.

• Pressure: Applying pressure in the perineal region (the region between the base of the penis and the anus) can also help to reduce swelling and erection.

• Surgical Ligation: In cases where the arterial rupture has occurred, the artery may be tied off or ligated, and blood flow is restored.

• Absorbable Gel: An absorbable gel may be surgically inserted into the penis to temporarily block blood flow to the penis.

Sickle Cell-Related Priapism: The treatment of priapism that is a result of sickle cell disease is different from low-level or high-level priapism:

• Hydration.

• Analgesia or pain relief medications.

• Blood Alkalization: Reducing the acidity of the blood.

• Partial Exchange Transfusion: Exchanging the patient’s blood with other blood components or products.

• Red blood cell transfusion.

• Drug Therapy: Drugs such as Sildenafil and Stilboestrol have been suggested as useful in treatment.

Conclusion:

Priapism has side effects, but prompt treatment (within 4 to 6 hours in ischemic priapism) will usually help revert to normal, with no long-lasting effects. It need not cause worry but is undoubtedly a reason for caution and quick treatment.