Craven de Kere wrote:
Priapism (prolonged erection) has been a contributing cause to some deaths.
Background: Priapism is the presence of a persistent, usually painful, erection of the penis. Although frequently idiopathic, priapism is associated with some systemic diseases. Priapism also is associated with the use of intracavernosal injections of medications to treat impotence.
Pathophysiology: Priapism is a persistent erection of the corpora cavernosa of the penis, originating from disturbances to the mechanisms that control penile detumescence. This process affects only the corpora cavernosa. The corpora spongiosum of the glans penis and surrounding the urethra remain flaccid.
Two types of priapism are described. Arterial high-flow priapism usually is secondary to a rupture of a cavernous artery and unregulated flow into the lacunar spaces. This type of priapism is usually not painful. Veno-occlusive priapism is usually due to full and unremitting corporeal veno-occlusion. Prolonged veno-occlusive priapism results in fibrosis of the penis and a loss of the ability to achieve an erection. Significant changes at the cellular level are noted within 24 hours in veno-occlusive priapism, whereas arterial priapism is not associated with fibrotic change.
Frequency: In the US: Arterial high-flow priapism is a rare entity and is usually secondary to penetrating penile trauma or a blunt perineal injury. Patients with sickle cell disease are prone to veno-occlusive priapism. In one study, 38-42% of patients with sickle cell disease reported at least one episode of priapism.
Mortality/Morbidity: Deaths have been reported in patients with sickle cell disease presenting with priapism, but cause of death usually is not related to the priapism per se but to complications from the underlying disease process. Morbidity is related to long-term impotence, primarily with veno-occlusive priapism, when diagnosis and therapy have been delayed.
Race: Sickle cell disease, which predisposes to the development of priapism, is a disease of the African American population.
Sex: Priapism is primarily a disease of males. Priapism of the clitoris has been described very rarely.
Age: Priapism has been described at nearly all ages, from infancy through old age. In patients with sickle cell disease, the peak incidence seems to occur in persons aged 19-21 years.