Background

Priapism is a medical condition characterized by prolonged and often painful erections that can last for several hours or even longer without sexual arousal or stimulation. This condition is considered a urologic emergency and requires prompt medical attention because if left untreated, it can lead to serious complications, including erectile dysfunction and tissue damage to the penis.

• Ischemic (low flow) priapism: This is the most common type of priapism and occurs when there is impaired blood flow out of the penis. It often results in a painful and prolonged erection and can lead to tissue damage if not treated promptly.
• Non-ischemic (high flow) priapism: This type is less common and usually not painful. It is caused by an abnormal connection between an artery and a vein in the penis, leading to increased blood flow. Non-ischemic priapism is less likely to result in tissue damage.

Epidemiology

• Priapism is considered a rare condition, and its prevalence varies among different populations. It is more commonly reported in certain medical conditions, such as sickle cell disease and leukemia.
• The annual incidence of priapism is estimated to be between 0.34 and 1.5 cases per 100,000 males. Incidence rates may be higher in certain subpopulations, such as individuals with sickle cell disease or those who use medications that can increase the risk of priapism.

Anatomy

Pathophysiology

• In a normal flaccid state, the smooth muscle within the erectile tissue of the penis is contracted, restricting blood flow. In ischemic priapism, this smooth muscle fails to relax, leading to sustained constriction of the penile arteries and reduced oxygen levels in the erectile tissue.
• The failure of the smooth muscle to relax causes blood to become trapped in the erectile chambers (corpora cavernosa), leading to engorgement and increased pressure within these structures.
• An abnormal communication between an artery and a vein in the penis allows for unregulated blood flow into the erectile tissue.
• In non-ischemic priapism, there is no obstruction of venous outflow. Blood continuously flows into and out of the penis, resulting in a prolonged, non-painful erection.

Etiology

• Sickle cell disease is one of the most common underlying causes of priapism, particularly ischemic priapism.
• In SCD, abnormal hemoglobin causes red blood cells to become misshapen and block blood flow, including blood flow out of the penis.
• Priapism can occur as a complication of certain types of leukemia, particularly acute lymphoblastic leukemia (ALL).

Genetics

Prognostic Factors

• This type of priapism is a significant prognostic factor. Ischemic (low-flow) priapism is associated with a higher risk of complications and poorer outcomes compared to non-ischemic (high-flow) priapism.
• Ischemic priapism can lead to tissue damage, fibrosis (scarring), and long-term erectile dysfunction if not treated promptly. The duration of the priapism episode is a crucial prognostic factor. Delay in seeking medical attention can lead to more severe complications and worse outcomes.
• The underlying cause of priapism plays a significant role in prognosis. Identifying and addressing the cause is essential for optimal management.
• Individuals who experience recurrent episodes of priapism may have a higher risk of complications and poorer long-term outcomes.
• Managing the underlying cause and implementing preventive measures can help reduce the risk of recurrence.

Clinical History

• Priapism can occur in children, including infants and adolescents, although it is relatively rare in this age group.
• Paediatric priapism is often associated with conditions like sickle cell disease, which can be present from birth or develop in early childhood.
• Priapism may be more commonly seen in adolescents and young adults, particularly in cases of idiopathic or ischemic priapism.
• This age group may also experience priapism related to trauma, drug use, or other factors.

Physical Examination

• General Assessment: The general assessment of the patient’s overall condition, including vital signs such as blood pressure, heart rate, and temperature.
• Genital Examination: The primary focus of the physical examination is the genital area, specifically the penis.
• Palpation: The healthcare provider may gently palpate (touch) the penis to assess for tenderness, firmness, and the presence of any areas of tissue induration (hardening).
• Evaluation of Color and Temperature: Assessing the color and temperature of the penis can help distinguish between ischemic and non-ischemic priapism.
• Ischemic priapism may present with a dusky or cyanotic (bluish) color, indicating poor oxygenation and tissue hypoxia.
• Non-ischemic priapism is often associated with normal or near-normal coloration and temperature.
• Neurological Assessment: In some cases, a neurological assessment may be performed to rule out underlying neurological conditions or injuries that could contribute to priapism.

Age group

Associated comorbidity

• Sickle Cell Disease (SCD) is one of the most significant comorbidities associated with priapism.
• Individuals with SCD have a higher risk of developing ischemic priapism due to the occlusion of blood vessels by sickle-shaped red blood cells.
• Priapism can occur as a complication of certain types of leukemia, particularly acute lymphoblastic leukemia.
• Physical trauma or injury to the genital area can be a comorbidity that leads to priapism, particularly non-ischemic priapism. Trauma may result in arteriovenous fistulas in the penis, allowing unregulated blood flow.

Associated activity

Acuity of presentation

• Acute priapism is characterized by a sudden and severe onset of a prolonged and painful erection. This type of presentation is typically associated with ischemic priapism, which is considered a medical emergency.
• Acute priapism often leads to intense pain and discomfort in the affected individual, and it requires immediate medical attention. Subacute priapism is characterized by a less sudden but still relatively rapid onset of priapism.
• While the pain and discomfort may not be as severe as in acute cases, subacute priapism still warrants prompt medical evaluation and intervention, as it can progress to a more severe form.

Differential Diagnoses

• Penile Fracture: Penile fracture occurs when there is a tear in the tunica albuginea, the fibrous covering of the erectile tissue, usually due to trauma during sexual activity.
• It can present with sudden pain, swelling, and a “popping” sound but typically involves the loss of an erection rather than a prolonged one.
• Compartment Syndrome: In rare cases, compartment syndrome involving the penis (penile compartment syndrome) can cause significant swelling, pain, and discoloration.
• Vasculitis: Vasculitis, an inflammatory condition affecting blood vessels, can theoretically lead to prolonged erections if it affects penile blood vessels.
• Neurogenic Erections: Neurological conditions or spinal cord injuries may lead to uncontrolled, prolonged erections.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

• Immediate Measures: Ischemic priapism is a medical emergency. The primary goal is to relieve the painful, prolonged erection and restore normal blood flow to prevent tissue damage.
• Continuous Monitoring: Patients with ischemic priapism may require close monitoring, including vital signs and oxygen saturation, as well as periodic evaluation of the penis, to ensure that the erection does not recur.
• Identification and Evaluation: Non-ischemic priapism is usually not painful. It is important to confirm the diagnosis through clinical evaluation, including imaging studies such as Doppler ultrasound.
• Conservative Management: In many cases of non-ischemic priapism, especially those resulting from trauma or injury, observation and conservative management may be sufficient.
• Embolization: In cases where conservative management is ineffective or when an arteriovenous fistula (abnormal connection between an artery and a vein) is identified on imaging, embolization may be considered.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-non-pharmacological-approach-for-treating-priapism

• Avoiding Triggers: For individuals with known triggers for priapism, such as certain medications or recreational drugs, avoiding these triggers can be important.
• Hydration: Staying adequately hydrated can help reduce the risk of priapism in individuals with sickle cell disease, as dehydration can exacerbate Vaso-occlusive crises, which may lead to priapism.
• Avoiding Extreme Temperatures: Extremely cold or hot temperatures can affect blood flow. Individuals at risk of priapism may find it beneficial to avoid prolonged exposure to extreme temperatures.
• Stress Management: High levels of stress and anxiety can potentially contribute to priapism in some cases. Stress management techniques, such as relaxation exercises, may be helpful.
• Regular Physical Activity: Engaging in regular physical activity and exercise can contribute to overall cardiovascular health and may help reduce the risk of priapism.
• Smoking Cessation: Smoking can have a negative impact on blood circulation. Quitting smoking may be beneficial for individuals at risk of priapism.
• Decrease Alcohol Use: Reducing or eliminating the use of alcohol and recreational drugs, especially substances known to contribute to priapism, can be important.
• Good Sleep Quality: Ensuring good sleep hygiene and addressing sleep disorders, such as sleep apnea, can contribute to overall health and well-being, potentially reducing the risk of priapism.

Use of Adrenergic Agonists

• Adrenergic agonists, such as phenylephrine, are commonly used in the treatment of ischemic priapism, which is characterized by a prolonged and painful erection caused by trapped blood in the penis. These medications work by constricting blood vessels in the erectile tissue of the penis.
• Phenylephrine: It is administered directly into the corpus cavernosum of the penis. The choice of concentration and dosage may vary depending on the specific protocol used by the healthcare provider.
• Phenylephrine: It is an alpha-adrenergic agonist that acts by constricting blood vessels in the erectile tissue, increasing the outflow of blood, and reducing blood flow into the penis.

use-of-intervention-with-a-procedure-in-treating-priapism

• Selective Arterial Embolization: In some cases of non-ischemic (high flow) priapism, particularly those involving arteriovenous fistulas (abnormal connections between arteries and veins), selective arterial embolization may be performed.
• Tunica Albuginea Plication: Tunica albuginea plication is a surgical procedure that may be considered in cases of recurrent priapism.
• It involves creating small incisions in the tunica albuginea (the fibrous covering of the erectile tissue) to reduce its tension and prevent prolonged erections.
• Penile Implant (Prosthesis): In cases of recurrent or refractory priapism, where other interventions have failed, a penile implant (penile prosthesis) may be considered.

use-of-phases-in-managing-priapism

• Emergency Assessment and Stabilization Phase: Priapism is considered a medical emergency, especially ischemic priapism. The initial phase involves a prompt assessment to determine the type, duration, and severity of the priapism.
• Once the acute episode is managed, healthcare providers work to identify and address the underlying cause of the priapism. This may involve a thorough medical history, physical examination, and diagnostic tests.
• Treatment of Underlying Conditions: Treatment plans are developed to manage the specific underlying condition contributing to priapism. This may include interventions such as transfusions, medication adjustments, or treatment of sickle cell crises.
• Conservative Management for Non-Ischemic Priapism: In cases of non-ischemic priapism, which is typically painless, a conservative approach may be appropriate.
• Monitoring Phase: Patients are monitored over time to assess if the condition resolves spontaneously. Follow-up appointments are scheduled to track progress.
• Follow-Up and Preventive Measures: Patients who have experienced priapism should have regular follow-up appointments to monitor for complications and assess the effectiveness of treatment.

Medication

Future Trends

Media Gallary

References

• Priapism – StatPearls – NCBI Bookshelf (nih.gov)
• Priapism: Symptoms, Causes, and Diagnosis (healthline.com)
• Priapism: Symptoms, Diagnosis & Treatment – Urology Care Foundation (urologyhealth.org)