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Priapism.

Background

Priapism in sub-Saharan Africa is common due to the high prevalence of sickle cell disease (SCD). In SCD, sickle-shaped red blood cells block penile blood flow, causing ischemic priapism. The use of drugs like cocaine, methylphenidate and phosphodiesterase (type 5 inhibitors used for erectile dysfunction) have been rarely linked with priapism.  Limited healthcare access delays treatment, leading to complications like fibrosis and erectile dysfunction. Timely medical intervention is crucial to prevent long-term issues.

Discussion
Symptoms 

In sub-Saharan Africa, the most important symptom of priapism is a sustained erection lasting over four hours without sexual stimulation, often accompanied by significant pain. This condition is particularly prevalent due to the high incidence of sickle cell disease (SCD), where rigid, sickle-shaped red blood cells obstruct penile blood flow. Recurring episodes of priapism are common in SCD patients, leading to repeated tissue ischemia and pain. If untreated, this can cause permanent penile tissue damage, fibrosis, and chronic erectile dysfunction. Additionally, the psychological impact of recurrent priapism, including anxiety and distress, significantly affects the quality of life for affected individuals.

Clinical findings

Clinical findings of priapism in sub-Saharan Africa often include a prolonged erection lasting more than four hours, characterized by a rigid penile shaft with a soft glans. Patients typically report severe penile pain and tenderness. On examination, there is usually a lack of detumescence even after attempts to urinate or perform physical activity. Blood gas analysis of the corporal aspirate often shows hypoxia, hypercarbia, and acidosis, indicating ischemic priapism. In cases associated with sickle cell disease, laboratory tests might reveal haemolytic anaemia and the presence of sickle-shaped red blood cells.

Differential diagnoses

The differential diagnoses of priapism in sub-Saharan Africa include:

  1. Ischemic (Low-Flow) Priapism: The most common form, often related to sickle cell disease, characterized by painful, prolonged erections.
  2. Non-Ischemic (High-Flow) Priapism: Less common, typically resulting from trauma or injury to the perineum or penis, leading to unregulated arterial blood flow and a less painful, partially rigid erection.
  3. Drug-Induced Priapism: Caused by medications such as phosphodiesterase inhibitors (e.g., sildenafil), antidepressants, antipsychotics, or recreational drugs.
  4. Neurologic Conditions: Disorders like spinal cord injuries or multiple sclerosis can disrupt the normal neurological control of erections.
  5. Malignancies: Penile, pelvic, or haematological cancers (such as leukaemia) that can cause blood flow obstruction or increased blood viscosity.
Investigations

Relevant investigations for priapism in sub-Saharan Africa include:

  1. Medical History and Physical Examination: This is the most effective way of investigating priapism in low-income settings. Assessing the duration of the erection, pain level, and presence of any underlying conditions like sickle cell disease.
  2. Blood Gas Analysis: Analysing aspirated corporal blood for oxygen, carbon dioxide, and pH levels to differentiate between ischemic (low-flow) and non-ischemic (high-flow) priapism.
  3. Complete Blood Count (CBC): To detect hematologic abnormalities such as sickle cell disease or leukaemia.
  4. Haemoglobin Electrophoresis: Specifically used to diagnose sickle cell disease or other hemoglobinopathies.
  5. Penile Doppler Ultrasound: Evaluating blood flow in the penile arteries and veins to distinguish between low-flow and high-flow priapism and to identify any vascular abnormalities or injuries.
  6. Toxicology Screening: Identifying any drugs or substances that might have induced priapism.
Treatment

Treatment of priapism in sub-Saharan Africa involves:

  1. Initial Management: Pain relief, hydration, and oxygenation, particularly for sickle cell patients.
  2. Aspiration and Irrigation: Removing blood from the corpora cavernosa and flushing with saline.
  3. Pharmacological Treatment: Injecting sympathomimetic agents like phenylephrine into the penis.
  4. Management of Underlying Conditions: Treating sickle cell disease and discontinuing causative drugs.
  5. Surgical Intervention: Performing shunting or penile prosthesis for severe or recurrent cases.
Follow up.

Long-Term Management: Providing patient education, counselling, and regular follow-up to monitor for complications.

Prevention and control

Prevention and control of priapism in sub-Saharan Africa involve:

  1. Managing sickle cell disease with regular monitoring, hydroxyurea therapy, and ensuring proper hydration and oxygenation.
  2. Education and awareness for patients and healthcare providers about the signs, symptoms, and importance of early treatment.
  3. Avoiding triggers by managing medications and advising against recreational drug use.
  4. Early intervention by seeking prompt medical attention and improving access to healthcare.
  5. Psychosocial support through counselling services and support groups for affected individuals.

Reviewer’s Reflections: Phosphodiesterase 5 (PDE5) inhibitors - sildenafil, vardenafil, tadalafil - is an uncommon cause of priapism. In fact, phosphodiesterase type 5 (PDE5) inhibitors (e.g., sildenafil), though off-label, is used in treatment. Oral pseudoephedrine can be started following diagnosis while setting up for corporal aspiration and saline irrigation.

Interesting patient case

Mr J.M, a 28-year-old with sickle cell disease from rural Kenya, presented with a painful erection lasting over 12 hours. Examination revealed a rigid penis and hypoxic, acidic blood, indicating ischemic priapism. Treatment included intravenous fluids, oxygen, corporal aspiration, saline irrigation, and a phenylephrine injection, leading to resolution of the priapism. John was educated on early symptom recognition and the importance of prompt medical attention and was scheduled for regular follow-ups and connected to a support group.

Further readings
  1. Borhade MB, Kondamudi NP. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Aug 29, 2022. Sickle Cell Crisis. [PubMed]
  2. Greiner T, Schneider M, Regente J, Toto S, Bleich S, Grohmann R, Heinze M. Priapism induced by various psychotropics: A case series. World J Biol Psychiatry. 2019 Jul;20(6):505-512. [PubMed]  
  3. Karayagmurlu A, Coskun M. Successful Management of Methylphenidate or Atomoxetine-Related Priapism During Attention-Deficit Hyperactivity Disorder Treatment. J Clin Psychopharmacol. 2020 May/Jun;40(3):314-315. [PubMed]
  4. James Johnson M, Hallerstrom M, Alnajjar HM, Frederick Johnson T, Skrodzka M, Chiriaco G, Muneer A, Ralph DJ. Which patients with ischaemic priapism require further investigation for malignancy? Int J Impot Res. 2020 Mar;32(2):195-200. [PubMed]

Author's details

Reviewer's details

Priapism.

Priapism in sub-Saharan Africa is common due to the high prevalence of sickle cell disease (SCD). In SCD, sickle-shaped red blood cells block penile blood flow, causing ischemic priapism. The use of drugs like cocaine, methylphenidate and phosphodiesterase (type 5 inhibitors used for erectile dysfunction) have been rarely linked with priapism.  Limited healthcare access delays treatment, leading to complications like fibrosis and erectile dysfunction. Timely medical intervention is crucial to prevent long-term issues.

  1. Borhade MB, Kondamudi NP. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Aug 29, 2022. Sickle Cell Crisis. [PubMed]
  2. Greiner T, Schneider M, Regente J, Toto S, Bleich S, Grohmann R, Heinze M. Priapism induced by various psychotropics: A case series. World J Biol Psychiatry. 2019 Jul;20(6):505-512. [PubMed]  
  3. Karayagmurlu A, Coskun M. Successful Management of Methylphenidate or Atomoxetine-Related Priapism During Attention-Deficit Hyperactivity Disorder Treatment. J Clin Psychopharmacol. 2020 May/Jun;40(3):314-315. [PubMed]
  4. James Johnson M, Hallerstrom M, Alnajjar HM, Frederick Johnson T, Skrodzka M, Chiriaco G, Muneer A, Ralph DJ. Which patients with ischaemic priapism require further investigation for malignancy? Int J Impot Res. 2020 Mar;32(2):195-200. [PubMed]

Content

Author's details

Reviewer's details

Priapism.

Priapism in sub-Saharan Africa is common due to the high prevalence of sickle cell disease (SCD). In SCD, sickle-shaped red blood cells block penile blood flow, causing ischemic priapism. The use of drugs like cocaine, methylphenidate and phosphodiesterase (type 5 inhibitors used for erectile dysfunction) have been rarely linked with priapism.  Limited healthcare access delays treatment, leading to complications like fibrosis and erectile dysfunction. Timely medical intervention is crucial to prevent long-term issues.

  1. Borhade MB, Kondamudi NP. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Aug 29, 2022. Sickle Cell Crisis. [PubMed]
  2. Greiner T, Schneider M, Regente J, Toto S, Bleich S, Grohmann R, Heinze M. Priapism induced by various psychotropics: A case series. World J Biol Psychiatry. 2019 Jul;20(6):505-512. [PubMed]  
  3. Karayagmurlu A, Coskun M. Successful Management of Methylphenidate or Atomoxetine-Related Priapism During Attention-Deficit Hyperactivity Disorder Treatment. J Clin Psychopharmacol. 2020 May/Jun;40(3):314-315. [PubMed]
  4. James Johnson M, Hallerstrom M, Alnajjar HM, Frederick Johnson T, Skrodzka M, Chiriaco G, Muneer A, Ralph DJ. Which patients with ischaemic priapism require further investigation for malignancy? Int J Impot Res. 2020 Mar;32(2):195-200. [PubMed]
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