History and exam

Key diagnostic factors

common

history of current systemic disease

The following have been reported as predisposing to ischaemic priapism: sickle cell disease, thalassaemia, assorted haematological dyscrasias, parenteral hyperalimentation, haemodialysis, heparin-induced platelet aggregation, and local primary (penile carcinoma/squamous cell carcinoma, prostatic adenocarcinoma) or metastatic neoplasia (metastases to the penis from prostate, rectosigmoid colon, kidney, urothelial carcinoma of the urinary bladder, chronic myeloid leukaemia).[13][14][15][16]

history of vasoactive medication or drug use

Medications implicated include alpha-adrenergic receptor antagonists (prazosin, terazosin, doxazosin, tamsulosin), anti-anxiety agents (hydroxyzine), anticoagulants (heparin, warfarin), antidepressants and antipsychotics (trazodone, bupropion, fluoxetine, sertraline, lithium, clozapine, risperidone, olanzapine, chlorpromazine, thioridazine), and antihypertensives (hydralazine, guanethidine, propranolol).[1][10]

Second-generation antipsychotics (33.8%), other medications (11.3%), and alpha-adrenergic antagonists (8.8%) account for most reported cases of drug-induced priapism.[10]

Alcohol and cocaine may predispose to ischaemic priapism.[29][30][31]

prolonged erection of >4 hours' duration

Typical of ischaemic and non-ischaemic priapism.

Episodes <4 hours are more often seen in recurrent (stuttering) priapism.[1]

painful rigid penis

Characteristic of ischaemic priapism.[1]

uncommon

penis not fully rigid and not painful

Characteristic of non-ischaemic priapism.[1][4]

history of perineal or genitourinary trauma

May predispose to non-ischaemic priapism.[1][4]

Trauma may create an arteriovenous fistula within the penis, leading to excessive arterial inflow.

Risk factors

strong

haemoglobinopathy

In haemoglobinopathies, most importantly sickle cell disease but also thalassaemia, venous stasis within the penis as a result of the associated hypercoagulable state is common.

Sickle cell disease is the most common aetiology of priapism in childhood, accounting for 65% of cases.[6] Among a large multi-centre cohort of patients with sickle cell disease (n=1314), 14.3% of patients had experienced priapism (n=188).[7] The mean age of the first priapism episode was 16 years. Significant predictors of priapism in the cohort included increasing age (51 of 745 males younger than 18 years [6.9%] vs. 137 of 568 males 18 years old or older [24.1%]) and more severe sickle cell disease genotype (e.g., homozygous SS).[7]

In sickle cell disease, most priapism episodes begin during sleep in boys.[27] In a survey of adults with sickle cell disease, 17% of episodes of priapism were related to sexual activity and 3% each were related to excessive ethanol intake or were spontaneous.[28]

vasoactive drugs

Medications implicated include alpha-adrenergic receptor antagonists (prazosin, terazosin, doxazosin, tamsulosin), anti-anxiety agents (hydroxyzine), anticoagulants (heparin, warfarin), antidepressants and antipsychotics (trazodone, bupropion, fluoxetine, sertraline, lithium, clozapine, risperidone, olanzapine, chlorpromazine, thioridazine), and antihypertensives (hydralazine, guanethidine, propranolol).[1][10] 

Second-generation antipsychotics (33.8%), other medications (11.3%), and alpha-adrenergic antagonists (8.8%) account for most reported cases of drug-induced priapism.[10]

Alcohol and cocaine may predispose to ischaemic priapism.[29][30][31]

weak

perineal or penile trauma

Major cause of non-ischaemic priapism (associated with excessive arterial penile inflow).[17][32]

malignancies or local infection

A variety of malignant diseases have been associated with ischaemic priapism. These include a local primary (e.g., prostatic adenocarcinoma) or metastatic (e.g., urothelial carcinoma of the urinary bladder or urethra, chronic myeloid leukaemia) disease.[16][33]

Rarely, ischaemic priapism may be seen in pelvic infection.

spinal cord disease

Injury (e.g., trauma, cauda equina syndrome) or lesions that interfere with penile stimulatory pathways may cause ischaemic priapism.

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