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== Classification (3) == # '''<span style="color:#ff0000">Ischemic</span>''' # '''<span style="color:#ff0000">Non-ischemic</span>''' #'''<span style="color:#ff0000">Recurrent non-ischemic (stuttering)</span>''' === Ischemic priapism (veno-occlusive, low-flow) === * '''<span style="color:#ff0000">Majority of cases</span>''' * '''Characterized by little or no cavernous blood flow''' *'''<span style="color:#ff0000">Associated with</span>''' (features that distinguish this from non-ischemic priapism) *# '''<span style="color:#ff0000">Pain</span>''' *# '''<span style="color:#ff0000">Fully rigid and tender corpora cavernosa</span>''' *# '''<span style="color:#ff0000">Abnormal blood gas</span> (i.e., hypoxic, hypercarbic, acidotic)''' *#'''<span style="color:#ff0000">Requires prompt evaluation and may require emergency management</span>''' *#'''<span style="color:#ff0000">Non-traumatic etiology</span>''' * Natural history of untreated acute ischemic priapism includes days to weeks of painful erections followed by permanent loss of erectile function *'''<span style="color:#ff0000">Duration of ischemic priapism is associated with the risk of future erectile dysfunction</span>''' ** In sickle cell disease patients in whom priapism was reversed, spontaneous erections (with or without use of sildenafil) were reported in: *** '''100% when priapism was reversed by 12 hours''' *** β'''75% when reversed by 12-24 hours''' *** '''β50% when reversed by 24-36 hours''' *** '''0% when reversed β₯36 hours''' ***[https://pubmed.ncbi.nlm.nih.gov/18312286/ Bennett, Nelson, and John Mulhall. "Sickle cell disease status and outcomes of African-American men presenting with priapism." ''The journal of sexual medicine'' 5.5 (2008): 1244-1250.] ** '''A more recent study suggested that the cutoff for irreversible restoration of erectile tissue is 48 hours[https://pubmed.ncbi.nlm.nih.gov/23892191/]''' ** '''<span style="color:#ff0000">Interventions beyond 48-72 hours of onset may relieve erection and pain but have little benefit in preserving potency</span>''' === Non-ischemic priapism (arterial, high-flow) === * '''<span style="color:#ff0000">Relatively rare</span>''' * May last hours to weeks and is frequently recurrent *'''<span style="color:#ff0000">Pathogenesis:</span>''' ** Likely results from unregulated control of arterial inflow and cavernous smooth muscle tone **'''<span style="color:#ff0000">Usually involves perineal or penile trauma</span>''' '''resulting in laceration of the cavernous artery (or one of its branches within the corpora)''' '''and unregulated cavernous arterial inflow.''' *** '''<span style="color:#ff0000">Most common cause is a straddle injury</span>''' to the crura **** '''Other mechanisms of traumatic arterial laceration include''' coital trauma, kicks to the penis or perineum, pelvic fractures, birth canal trauma to the male newborn, needle lacerations, iatrogenic needle injury, complications of penile diagnostics, vascular erosions complicating metastatic infiltration of the corpora, and after iatrogenic trauma from coldknife urethrotomy, corporoplasty, and penile revascularization procedures ***** '''Sustained partial erection may develop 24 hours after perineal or penile blunt trauma''' ***'''The arteriolacunar fistula is typically unilateral'''. ** '''<span style="color:#ff0000">After either aggressive medical management of ischemic priapism or surgical shunting, priapism may rapidly recur with conversion from ischemic (low-flow) to non-ischemic (high-flow)''' ** '''Other causes include Fabry disease and sickle cell anemia''' * '''<span style="color:#ff0000">Associated with</span>''' (features that distinguish this from ischemic priapism) *# '''<span style="color:#ff0000">Lack of pain</span>''' (beyond pain from potential acute traumatic etiology) *# '''<span style="color:#ff0000">Tumescent but less rigid corpora cavernosa</span>''' *#* '''<span style="color:#ff0000">Erection is partial and bendable because there is no restriction of venous outflow,</span>''' *#* '''Patients do report additional engorgement with sexual stimulation, with return to partial erection after climax''' *# '''<span style="color:#ff0000">Normal penile blood gas</span>''' (features that distinguish this from non-ischemic priapism) *#'''<span style="color:#ff0000">Once properly diagnosed, does not require emergent intervention</span>''' *#'''<span style="color:#ff0000">Traumatic etiology, usually</span>''' === Recurrent ischemic priapism === * Also known as "stuttering" priapism *'''<span style="color:#ff0000">Definition: Recurrent ischemic episodes, with or without meeting the 4-hour time criteria for priapism</span>''' **Unwanted painful erections occur repeatedly with intervening periods of detumescence **The key differentiating factor between the current definition of recurrent ischemic priapism and other recurrent priapism-like conditions is the requirement of confirmed penile ischemia. ***Priapism-like conditions include sleep-related painful erections, undesired prolonged erections, and recurrent non-ischemic priapism *'''Both acute ischemic priapism and non-ischemic priapism may recur over time''' *'''<span style="color:#ff0000">Commonly associated with sickle cell disease</span>''' ** '''<span style="color:#ff0000">Priapism in children and adolescents is most commonly related to sickle cell disease</span>''' *** The sickle cell genetic mutation is the result of a single amino acid substitution in the beta-globin subunit of hemoglobin S. *** Clinical features seen in homozygous sickle cell disease: chronic hemolysis, vascular occlusion, tissue ischemia, and end-organ damage. *** '''Sickle cell trait can be a predisposing factor to ischemic priapism.''' ** Pathogenesis is related to hemolysis and reduced nitric oxide * '''<span style="color:#ff0000">Management focuses on</span>''' **'''<span style="color:#ff0000">Treatment of acute episodes</span>''' **'''<span style="color:#ff0000">Prevention of future events</span>''' *
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