Editing Priapism (section)

==== Special Scenarios ====

===== Priapism refractory to shunting =====
*'''<span style="color:#ff0000">A vascular study (such as a PDUS) or cavernosal blood gas should be performed prior to performing additional interventions (repeat distal or proceeding to proximal shunting).</span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
**Goal is to assess penile hemodynamic characteristics and extent of necrosis/fibrosis to differentiate persistent acute ischemic priapism from reactive hyperemia or conversion to non-ischemic priapism and inform secondary treatment decisions
***Penile corporal blood gas can help with decision making about proceeding to additional surgical procedures including placement of an immediate penile prosthesis.
***Penile duplex ultrasound 
****Can be used to 
*****Differentiate between acute ischemic and non-ischemic priapism
*****Determine shunt patency by showing restoration of cavernosal arterial inflow in a patient who has undergone a distal shunt
**Further surgical decisions should not be based only on exam
**'''Evaluating the status of a patient with refractory priapism is particularly important in the event that a patient is referred from another institution and/or the clinician is seeing a patient who had been previously treated elsewhere and a complete patient history may not be available.'''

===== Acute Ischemic Priapism >36 hours =====
*'''<span style="color:#ff0000">Options</span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
*#'''<span style="color:#ff0000">Observation, pain control, and outpatient follow-up</span>'''
*#*May bypass more invasive procedures (e.g., surgical shunting).
*#'''<span style="color:#ff0000">Distal shunting, with or without tunneling</span>''' 
*#'''<span style="color:#ff0000">Early implantation of penile prosthesis</span>'''
*'''<span style="color:#ff0000">Unlikely the acute ischemic event will resolve with ICI therapy of phenylephrine and aspiration.</span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
**'''The response to phenylephrine decreases with increased duration of priapism'''

*'''It is important to note that before considering conservative management or penile prosthesis placement in men with a priapism >36 hours, the timeline should be sufficiently confirmed.''' 
**Patient histories relating to an exact timeline may often be unreliable, particularly in cases of concomitant substance use, episodes of intermittent detumescence, recurrent priapism (e.g., SCD), or partial (not fully rigid) erections. In these settings, clinical judgment is required to identify the true timeline for onset of ischemia (i.e., onset of severe, persistent penile pain). 
**If the timeline is in question, clinicians should preferentially attempt to decompress the priapism, particularly in younger men or those with high baseline erectile function.

===== Early implantation (within 2 weeks) of penile prosthesis =====
* '''<span style="color:#ff0000">Indications</span>'''
**'''<span style="color:#ff0000">May be considered in a patient with untreated acute ischemic priapism > 36 hours or in those who are refractory to shunting, with or without tunneling. </span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
*'''Advantages to prostheses placed in the setting of acute ischemic priapism (5):<span style="color:#ff0000">[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]</span>'''
*# '''Detumescence'''
*# '''Relief of pain'''
*#'''Preservation of penile length'''
*#'''Return to sexual activity'''
*#'''Overall satisfaction'''
* '''Disadvantages to immediate implantation (3):'''
*# '''Urologist involved for may lack the experience, comfort level, or materials to render device placement practical and/or possible<span style="color:#ff0000">[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]</span>'''
*#Repetitive bedside irrigation procedures may, in theory, increase the chances for bacterial entry into the corpora that could threaten an implant with infection. 
*##'''Infection rates''' < 10%, '''higher with delayed implantation<span style="color:#ff0000">[https://pubmed.ncbi.nlm.nih.gov/35536142/ ,★]</span>'''
*#'''Distal shunts may have compromised the integrity of the tunica albuginea that would surround an implant, possibly predisposing to erosion.'''

===== Recurrent Ischemic Priapism =====
* '''Options''' '''to prevent subsequent episodes<span style="color:#ff0000">[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]</span>'''
**'''Idiopathic (7):'''
**#'''<span style="color:#ff0000">Ketoconazole with prednisone'''
**#*'''Highest success rate'''
**#*'''<span style="color:#ff0000">Adverse effects'''
**#*#'''<span style="color:#ff0000">Liver toxicity, thus warranting frequent assessment of liver function tests</span>'''
**#'''<span style="color:#ff0000">Pseudoephedrine</span>''', an oral α-adrenergic agonist, promotes muscle contraction within the erectile tissue
**#'''<span style="color:#ff0000">Phosphodiesterase type 5 inhibitors</span>'''
**#'''Aspirin'''
**#'''Baclofen'''
**#'''Dutasteride'''
**#'''Cyproterone acetate'''
**##Anti-androgen
**##Not available in the United States
**'''Associated with sickle cell disease'''
**#'''Same options as idiopathic'''
**#'''Etilefrine'''
**#'''Hydroxyurea'''
**#'''Automated exchange transfusion'''
**'''Drug therapy is typically initiated at bedtime'''
**Patients with recurrent ischemic priapism should be informed that hormonal regulators (ketoconazole, cyproterone acetate) may impair fertility and sexual function
***Adverse effects, due to manipulation of hypothalamic-pituitary-gonadal axis
****Fatigue
****Hot flashes
****Breast tenderness
****Changes in mood
****ED
****Negatively impact sperm parameters
**'''Home self-injection of intracavernous α-adrenergic agent'''
*** Not a preventative strategy; may avert a full-blown episode of ischemic priapism when administered at home for prolonged morning erections
**'''Optimal strategy is unknown'''

===== Sickle Cell Disease and other Hematologic Disorders =====
* '''<span style="color:#ff0000">The best intervention is to relieve episodes with prompt intracavernosal phenylephrine and corporal aspiration, with or without irrigation, as in other acute ischemic priapism patients, before proceeding to systemic therapies specific to the underlying disorder</span>[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]'''
*'''Standard sickle cell assessment and interventions should be considered concurrent with initiation of urologic intervention. Specifically, disease specific systemic care should address:'''
** '''Hydration with IV fluid only if made NPO (maintenance rate) or dehydrated (replace deficit plus maintenance rate)'''
*** '''Hyperhydration is not indicated and may predispose to acute chest syndrome.'''
** '''Supplemental oxygenation only if hypoxic.'''
** '''Pain management with oral or parenteral opioids as per usual painful events (remembering that some patients with SCD may be tolerant to analgesia because of those prior experiences).'''
** '''Hematologic status comparison of CBC and reticulocyte count to baseline values'''
*** '''Best done in consultation with the patient’s hematologist.''' 
*** '''Transfusion is not indicated if hemoglobin is near usual value, and over-transfusion may be associated with neurologic events.'''
*** '''Acute exchange transfusion is not indicated.'''
*** If operative shunting procedures are required, consideration should be given to a simple transfusion of packed red blood cells to raise the hemoglobin to 9-10 g/dl prior to general anesthesia
*** Rarely are blood products required before an aspiration and irrigation procedure, the one exception may be with a very low platelet count (<20,000/uL).
** '''Presence of other acute sickle cell events: neurologic disorders including acute stroke, acute chest syndrome, biliary colic, renal insufficiency which while not associated with a higher frequency of priapism may present at the same time.'''
* '''Ice packs and other cold compresses should never be used in SCD patients as they may worsen painful events by precipitating intravascular sickling.'''
*Most patients with SCD experience recurrent short ischemic priapism events, (lasting <4 hours and commonly referred to as stuttering priapism) but acute episodes and particularly recurrent acute episodes occur commonly enough (both before and after shorter, stuttering events) that education about when to seek urologic attention is a critical part of the patient education in SCD disorders.
*Drugs that have been tried to prevent subsequent priapism episodes
**Etilefrine
**Ephedrine
**Pseudoephedrine
**Terbutaline
**PDE5is (e.g., sildenafil, tadalafil)
**5 alpha reductase inhibitors (dutasteride or finaseride)
**Anti-androgens (cyproterone, bicalutamide, leuprolide, stilboesterol)
**Ketoconazole/prednisone
**Hydroxyurea
*Ongoing chronic (monthly) exchange transfusions do appear to be associated with a reduction in acute and stuttering priapism episodes.
**Exchange transfusion should not be as the primary treatment in patients with acute ischemic priapism associated with sickle cell disease.
**For prolonged acute priapism events that cannot be relieved with intracavernosal phenylephrine and corporal aspiration, exchange transfusion can be considered
*

===== Prolonged Erection Following Intracavernosal Vasoactive Medication =====

* '''<span style="color:#ff0000">A prolonged erection (≤4 hours in duration) following iatrogenic- or patient self-administration of erectogenic medications into the corpus cavernosum (ICI) represents a distinct pathology when compared to acute ischemic priapism or non-ischemic priapism.</span>'''
**Much more common and may be managed differently than acute ischemic priapism
*'''<span style="color:#ff0000">Management</span>'''
**See [https://www.auanet.org/documents/Guidelines/PDF/priapism/NIP%20JU%20SUMMARY%20Figure%20Three%20Prolonged%20Erections.pdf AUA/SMSNA Guideline Flowchart on Management of Prolonged Erection]
**'''If erection > 4 hours, treat according to ischemic priapism algorithm'''
***'''All patients should be instructed at the time of ICI training, or after receiving an in-office erectogenic therapy, that they should return to either the office or emergency department if they experience an erection lasting > 4 hours.<span style="color:#ff0000">[https://pubmed.ncbi.nlm.nih.gov/35536142/ ★]</span>'''
**'''<span style="color:#ff0000">If prolonged erection 1-4 hours after home ICI or following an in-office injection</span>'''
***'''<span style="color:#ff0000">Options</span>'''
***#'''<span style="color:#ff0000">Conservative options (in the case of home ICI)</span>''' including ice compresses to the penis, laying supine, ejaculation, exercise, penile compresses and oral medications such as pseudoephedrine, in the absence of any clinical data demonstrating efficacy
***#*Utilize intracavernosal phenylephrine if conservative management is ineffective in the treatment of a prolonged erection.
***#'''<span style="color:#ff0000">In-office phenylephrine</span>'''
***#* '''Not appropriate for clinicians who administer in-office erectogenic medications to refer the patient to the emergency department as a matter of routine following an in-office injection, rather, the patient should return to the office for detumescence whenever possible.''' 
***#*'''Intracavernosal aspiration and irrigation likely represents too aggressive of a therapy for this specific clinical scenario to be used as a first-line therapy'''
***#** Intracavernosal phenylephrine can be delivered through a small needle (27G) compared to needle typically used for aspiration/irrigation (16 or 18G)
***#**The physiologic rationale for aspiration and irrigation is to remove intracavernosal clots and permit entry of fresh blood in an attempt to restore smooth muscle function and vascular drainage.  As the pathologic state of intracavernosal clotting and ischemia likely is not present with prolonged erections <4 hours, aspiration and irrigation is rarely warranted. 
***#*** Persistent, prolonged erections may be considered for aspiration and irrigation if phenylephrine alone is unsuccessful.
***'''Duration of a persistent erection requiring intervention is not clearly defined. Generally,'''
**** '''Erection lasting''' 
***** '''<1 hour post injection would not require intervention'''
***** '''>4 hours would warrant treatment, regardless of underlying etiology'''
***** '''Decision to intervene in the time-period between 1 and 4 hours would depend on several clinical factors:'''
*****# '''Penile rigidity'''
*****#* Mild erection (i.e., not sufficient to penetrate without assistance) would not require treatment, whereas a fully rigid erection might
*****#* Intermittently rigid erection is considered differently than a fully rigid erection, which has remained persistent since the original injection
*****# '''Specific medication used and dosage'''
*****#* Alprostadil alone is likely associated with shorter durations of erections and likely has a lower risk of ischemic priapism compared to combination therapies, which include  papaverine and/or phentolamine
*****#* Higher dosages are empirically more likely to result in a prolonged erection compared to lower ones
*****# '''Age'''
*****# '''Baseline erectile function'''
*****# '''Reliability/capacity'''
*****# '''Comorbid conditions'''
*****# Pain as an indicator for treatment is not relevant in many scenarios, as the intracavernosal medications themselves are often associated with penile pain