Guideline sources The following summarized guidelines for the evaluation and management of priapism are prepared by our editorial team based on guidelines from the European Association of Urology (EAU 2023), the American Urological Association (AUA/SMSNA 2022), the National Heart, Lung, and Blood Institute (NHLBI 2014), and the American Urological Association (AUA 2003). 1 2 3 4 Pathway Priapism Management Guidelines https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 1/7 6/29/23, 11:28 PM Priapism Pathway 1. Classification and risk stratification Classification: determine whether the priapism is ischemic or non-ischemic in order to initiate appropriate management. Ischemic priapism is an emergency, while non-ischemic priapism does not require emergent treatment. E Show 2 more 2. Diagnostic investigations History and physical examination: As per EAU 2023 guidelines: Elicit a comprehensive history to establish the diagnosis to determine the priapism subtype. A Perform a physical examination of the genitalia, perineum and abdomen in the diagnostic evaluation of patients with priapism. Perform neurological examination if neurogenic non- ischemic priapism is suspected. A As per SMSNA 2022 guidelines, elicit a complete medical, sexual and surgical history and perform a physical examination including the genitalia and perineum in patients presenting with priapism. B Laboratory tests: As per EAU 2023 guidelines, obtain CBC, WBC count with blood cell differential, platelet count, and coagulation profile in patients with priapism. Obtain further laboratory testing depending upon history, clinical and laboratory findings. A Show 2 more As per SMSNA 2022 guidelines, obtain additional diagnostic testing to determine the etiology of diagnosed acute ischemic priapism while not delaying, rather obtaining simultaneously with, definitive treatment. E Doppler ultrasound: As per EAU 2023 guidelines, obtain color duplex ultrasound of the penis and perineum before aspiration to differentiate between ischemic and non-ischemic priapism. A As per SMSNA 2022 guidelines: Consider obtaining penile Doppler ultrasound if the diagnosis of acute ischemic versus non-ischemic priapism is indeterminate. E Consider obtaining penile duplex ultrasound for the assessment of fistula location and size in patients with non-ischemic priapism. E Magnetic resonance imaging: obtain MRI of the penis as an adjunct to predict smooth muscle viability in patients of prolonged ischemic priapism or refractory priapism. B Selective pudendal arteriography: obtain selected pudendal arteriography if embolization is planned for the management of patients with non-ischemic priapism. A 3. Diagnostic procedures https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 2/7 6/29/23, 11:28 PM Priapism Pathway Penile blood gas analysis: As per EAU 2023 guidelines, obtain an analysis of the blood gas parameters from blood aspirated from the penis to differentiate between ischemic and non-ischemic priapism. A As per SMSNA 2022 guidelines, obtain corporal blood gas analysis at the initial presentation of patients with priapism. B 4. Medical management General principles, ischemic priapism: As per EAU 2023 guidelines, initiate management in patients with ischemic priapism as early as possible (within 4-6 hours) and follow a stepwise approach. A As per SMSNA/AUA 2022 guidelines, do not delay definitive therapies in patients with diagnosed acute ischemic priapism, recognizing that conservative therapies (observation, oral medications, cold compresses, exercise) are unlikely to be successful. D As per AUA 2003 guidelines, use either therapeutic corporal aspiration (with or without irrigation) or intracavernous injection of sympathomimetics as the initial management strategy for ischemic priapism. E Show 3 more General principles, non-ischemic priapism: As per EAU 2023 guidelines, proceed to definitive management of non-ischemic priapism at the discretion of the treating physician, as it is not an emergency. B As per SMSNA/AUA 2022 guidelines, counsel patients that non-ischemic priapism is not an emergency condition and offer an initial period of observation. E As per AUA 2003 guidelines: Provide observation as the initial management strategy in patients with non-ischemic priapism should be observation. E Avoid performing corporal aspiration or intracavernous injection of sympathomimetic drugs as a management strategy for non-ischemic priapism. Corporal aspiration has only a diagnostic role in the management of non-ischemic priapism. D Androgen deprivation therapy: consider initiating androgen deprivation therapy only in adult patients with arterial priapism. C 5. Nonpharmacologic interventions Perineal compression: offer conservative management with site-specific perineal compression as the first step in patients with arterial priapism. B 6. Therapeutic procedures Penile aspiration and irrigation: As per EAU 2023 guidelines, decompress the corpus cavernosum by penile aspiration and washout until fresh red blood is obtained. A https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 3/7 6/29/23, 11:28 PM Priapism Pathway As per SMSNA 2022 guidelines, perform corporal aspiration, with or without irrigation, as first-line therapy and before operative interventions in patients with acute ischemic priapism. B Intracavernous sympathomimetics: As per EAU 2023 guidelines, replace blood aspiration with intracavernous injection of sympathomimetic agents as the first step of the management of patients with priapism secondary to intracavernous injections of vasoactive agents. Administer intracavernous sympathomimetic agents as the next step if priapism persists despite aspiration. A Show 3 more As per SMSNA 2022 guidelines, administer intracavernous phenylephrine as the initial treatment in patients with prolonged erection of 4 hours following intracavernous injection pharmacotherapy for erectile dysfunction. E Show 4 more As per AUA 2003 guidelines, observe patients during and following intracavernous injection of sympathomimetic agents for subjective symptoms and objective findings consistent with known undesirable effects of these agents: acute hypertension, headache, reflex bradycardia, tachycardia, palpitations, and cardiac arrhythmia. Obtain BP and ECG monitoring in patients with high cardiovascular risk. E Selective arterial embolization: As per EAU 2023 guidelines: Perform selective arterial embolization in patients with arterial priapism failed conservative management. A Perform first selective arterial embolization using temporary material. Repeat the procedure with temporary or permanent material for recurrent non-ischemic priapism following selective arterial embolization. B As per SMSNA 2022 guidelines, offer embolization as first-line therapy after a trial of observation in patients with persistent non-ischemic priapism wishing to be treated. B Show 2 more As per AUA 2003 guidelines: Consider selective arterial embolization for the management of non-ischemic priapism in patients who request treatment. E Discuss the risks and benefits of selective arterial embolization thoroughly prior to performing this procedure, including the risks of treatment-related erectile dysfunction, chances of spontaneous resolution, and lack of significant consequences expected from delaying interventions. E 7. Perioperative care Perioperative anticoagulation: Consider administering peri- and postoperative anticoagulation to decrease recurrence in patients with ischemic priapism. C Consider administering the following anticoagulant prophylaxis for the prevention of recurrence in patients with ischemic priapism: https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 4/7 6/29/23, 11:28 PM Priapism Pathway Situation Guidance 325 mg acetylsalicylic acid Preoperatively 5,000 IU heparin Intraoperatively 81 mg acetylsalicylic acid and 75 mg clopidogrel. C 5 days postoperatively 8. Surgical interventions Surgical shunting: As per EAU 2023 guidelines, proceed to surgical treatment only if blood aspiration and intracavernous injection of sympathomimetic agents have failed. A Show 2 more As per SMSNA 2022 guidelines, perform placement of a distal corporo-glanular shunt, with or without tunneling, in patients with persistent acute ischemic priapism after intracavernous phenylephrine and corporal aspiration, with or without irrigation. B Show 3 more As per AUA 2003 guidelines, consider performing surgical shunting to treat ischemic priapism only after a trial of intracavernous injection of sympathomimetics has failed. Perform cavernoglanular shunting as the procedure of choice. E Surgical fistula ligation: As per EAU 2023 guidelines, reserve selective surgical ligation of a fistula as the final treatment in patients failed repeated arterial embolizations. B As per AUA 2003 guidelines, consider surgical management (with intraoperative color duplex sonography) only as a last resort in patients with non-ischemic priapism. E Penile prosthesis: As per EAU 2023 guidelines, consider preferring penile prosthesis over proximal shunting particularly in patients with delayed (> 48 hours) or refractory priapism. C Show 5 more As per SMSNA 2022 guidelines: Consider performing implantation of penile prosthesis in patients with untreated acute ischemic priapism > 36 hours or in patients refractory to shunting, with or without tunneling. E Discuss the risks and benefits of early versus delayed placement in patients with acute ischemic priapism being candidates for prosthesis implantation. B 9. Specific circumstances Patients with stuttering priapism: As per EAU 2023 guidelines, manage each episode of stuttering priapism similar to that for ischemic priapism. A https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 5/7 6/29/23, 11:28 PM Priapism Pathway Show 3 more As per SMSNA 2022 guidelines: Inform patients with recurrent ischemic priapism that optimal strategies to prevent subsequent episodes are unknown. B Inform patients with recurrent ischemic priapism that hormonal regulators may impair fertility and sexual function. B As per AUA 2003 guidelines, consider administering a trial of GnRH agonists or anti- androgens in patients with recurrent priapism. E Show 2 more Patients with sickle cell disease: As per EAU 2023 guidelines, treat ischemic priapism associated with sickle cell disease in the same fashion as idiopathic ischemic priapism. Provide other supportive measures (IV hydration, oxygen administration with alkalization with bicarbonate, blood exchange transfusions), but do not delay initial treatment to the penis. A As per SMSNA 2022 guidelines: Do not delay the standard management of acute ischemic priapism for disease-specific systemic interventions in patients with hematologic and oncologic disorders such as sickle cell disease or chronic myelogenous leukemia. D Do not use exchange transfusion as the primary treatment in patients with acute ischemic priapism associated with sickle cell disease. D As per NHLBI 2014 guidelines, initiate vigorous oral or IV hydration and oral or IV analgesia if an episode of priapism lasts 4 hours. B Show 3 more As per AUA 2003 guidelines, do not use systemic treatment of the underlying disorder as the only treatment for ischemic priapism in patients with an underlying disorder, such as sickle cell disease or hematologic malignancy. Initiate intracavernous treatment for ischemic priapism concurrently with the treatment of the underlying disorder. D 10. Patient education General counseling: Counsel all patients with persistent ischemic priapism about the chance of erectile dysfunction. B Counsel patients with an acute priapism event > 36 hours that the likelihood of erectile function recovery is low. B Clinical findings Symptoms Past medical history Erection lasting 4 hours unrelated to sexual stimulation Amyloidosis Gout Penile pain Leukemia https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 6/7 6/29/23, 11:28 PM Priapism Pathway Multiple myeloma Past events Sickle cell disease Scorpion sting Spinal cord injury Syphilis Genitourinary exam Thalassemia Rigid penile shaft Social history Soft glans of penis Alcohol consumption Exposure to spider bite Illicit drug use Imaging findings Spinal cord compression References 1. Trinity J. Bivalacqua, MD PhD, Bryant K. Allen et al. The Diagnosis and Management of Priapism: an AUA/SMSNA Guideline 2022 . J Urol. 2022. Open 2. A. Salonia, C. Bettocchi, J. Carvalho et al. EAU Guidelines on Sexual and Reproductive Health. EAU. 2023. Open 3. Montague DK, Jarow J, Broderick GA et al. American Urological Association guideline on the management of priapism. J Urol. 2003 Oct;170 4 Pt 1 1318 24. Open 4. Yawn BP, Buchanan GR, Afenyi-Annan AN et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. JAMA. 2014 Sep 10;312 10 1033 48. Open 5. Trinity J Bivalacqua, Bryant K Allen, Gerald Brock et al. Acute Ischemic Priapism: An AUA/SMSNA Guideline. J Urol. 2021 Nov;206 5 1114 1121. Open 6. Andrea Salonia, Ian Eardley, Fran ois Giuliano et al. European Association of Urology guidelines on priapism. Eur Urol. 2014 Feb;65 2 480 9. Open https://web.pathway.md/diseases/recXQTuAsr9rLS1ZW 7/7