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Priapism is painful, persistent, abnormal erection unaccompanied by sexual desire or excitation. It is most common in boys 5 to 10 yr and in men age 20 to 50 yr.
The penis is composed of 3 corporeal bodies: 2 corpora cavernosa and 1 corpus spongiosum. Erection is the result of smooth muscle relaxation and increased arterial flow into the corpora cavernosa, causing engorgement and rigidity.
Most cases of priapism involve failure of detumescence and are most commonly due to failure of venous outflow (ie, low flow), also known as ischemic priapism. Severe pain from ischemia occurs after 4 h. If prolonged> 4 h, priapism can lead to corporeal fibrosis and subsequent erectile dysfunction or even penile necrosis and gangrene.
Stuttering priapism is a recurrent form of ischemic priapism with repeated episodes and intervening periods of detumescence.
In adults, the most common cause (see Table: Some Causes of Priapism) is
In children, the most common causes are
In many cases, priapism may be idiopathic and recurrent.
Some Causes of Priapism
Priapism requires urgent treatment to prevent chronic complications (primarily erectile dysfunction). Evaluation and treatment should be done simultaneously.
History of present illness should cover the duration of erection, presence of partial or complete rigidity, presence or absence of pain, and any recent or past genital trauma. The drug history should be reviewed for offending drugs, and patients should be directly asked about the use of recreational drugs and drugs used to treat erectile dysfunction.
Review of systems should seek symptoms suggesting a cause, including dysuria (UTIs), urinary hesitancy or frequency (prostate cancer), fever and night sweats (leukemia), and lower-extremity weakness (spinal cord pathology).
Past medical history should identify known conditions associated with priapism (see Table: Some Causes of Priapism), particularly hematologic disorders. Patients should be asked about a family history of hemoglobinopathies (sickle cell disease or thalassemia).
A focused genital examination should be done to evaluate extent of rigidity and tenderness and determine whether the glans and corpus spongiosum are also affected. Penile or perineal trauma and signs of infection, inflammation, or gangrenous change should be noted.
The general examination should note any psychomotor agitation, and the head and neck examination should look for pupillary dilation associated with stimulant use. The abdomen and suprapubic area should be palpated to detect any masses or splenomegaly, and a digital rectal examination should be done to detect prostatic enlargement or other pathology. Neurologic examination is useful to detect any signs of lower-extremity weakness or saddle paresthesias that might indicate spinal pathology.
In most cases, the clinical history reveals a history of drug treatment for erectile dysfunction, illicit drug use, or a history of sickle cell disease or trait; in these cases, no testing is indicated.
In patients with ischemic priapism, physical examination typically reveals complete rigidity with pain and tenderness of the corpus cavernosa and sparing of the glans and corpus spongiosum. By contrast, nonischemic priapism is painless and nontender, and the penis may be partially or completely rigid.
If the cause is not obvious, screening is done for hemoglobinopathies, leukemia, lymphoma, UTI, and other causes:
Many clinicians also do drug screening, intracavernosal ABG testing, and duplex ultrasonography. Penile duplex ultrasonography will show little or absent cavernosal blood flow in men with ischemic priapism and normal to high cavernosal blood flow in men with nonischemic priapism. Ultrasonography may also reveal anatomic abnormalities, such as cavernous arterial fistula or pseudoaneurysm, which usually indicate nonischemic priapism. Occasionally, MRI with contrast is useful to demonstrate arteriovenous fistulas or aneurysms.
Treatment is often difficult and sometimes unsuccessful, even when the etiology is known. Whenever possible, patients should be referred to an emergency department; patients should preferably be seen and treated urgently by a urologist. Other disorders should be treated. For example, priapism often resolves when sickle cell crisis is treated. Measures used to treat priapism itself depend on the type.
Treatment should begin immediately, typically with aspiration of blood from the base of one of the corpora cavernosa using a nonheparinized syringe, often with saline irrigation and intracavernous injection of the α-receptor agonist phenylephrine. For phenylephrine injections, 1 mL of 1% phenylephrine (10 mg/mL) is added to 19 mL of 0.9% saline to make 500 mcg/mL; 100 to 500 mcg (0.2 to 1 mL) is injected every 5 to 10 min until relief occurs or a total dose of 1000 mcg is given. Before aspiration or injection, anesthesia is provided with a dorsal nerve block or local infiltration.
If these measures are unsuccessful or if priapism has lasted > 48 h (and is thus unlikely to resolve with these measures), a surgical shunt can be created between the corpus cavernosum and glans penis or corpus spongiosum and another vein.
Stuttering priapism, when acute, is treated in the same way as other forms of ischemic priapism. There is a report of several cases caused by sickle cell disease that responded to a single oral dose of sildenafil. Treatments that may help prevent recurrences of stuttering priapism include antiandrogen therapy with gonadotropin-releasing hormone agonists, estrogen, bicalutamide, flutamide, phosphodiesterase type-5 inhibitors, and ketoconazole. The goal of antiandrogen therapy is to decrease the plasma testosterone level to < 10% of normal. Digoxin, terbutaline, gabapentin, and hydroxyurea have also been tried with some success.
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