A 22-year-old man comes to the emergency department with a persistent, painful erection he has had for the past 6 hours. The patient has had similar episodes that lasted 1-2 hours but resolved spontaneously. He says his symptoms started this morning without provocation when he woke up. The patient recently started a summer job working outside pouring concrete. Medical history includes sickle cell disease; he has not taken any medications other than his usual folic acid. The patient does not use alcohol or illicit drugs. On examination, the penis is rigid, and the glans is soft. The scrotum is normal. Which of the following is the most appropriate next step in management?

Question

A 22-year-old man comes to the emergency department with a persistent, painful erection he has had for the past 6 hours.  The patient has had similar episodes that lasted 1-2 hours but resolved spontaneously.  He says his symptoms started this morning without provocation when he woke up.  The patient recently started a summer job working outside pouring concrete.  Medical history includes sickle cell disease; he has not taken any medications other than his usual folic acid.  The patient does not use alcohol or illicit drugs.  On examination, the penis is rigid, and the glans is soft.  The scrotum is normal.  Which of the following is the most appropriate next step in management?

Quizplus · Accepted Answer

11ec25c8_d84a_56e9_bc81_5d64de977206_MD0003_00 This patient with sickle cell disease has priapism, a persistent, painful erection in the absence of ongoing sexual stimulation. Penile erections are normally terminated by sympathetic activation, which constricts arterial flow to the corpora cavernosa; blood subsequently exits the corpora via the emissary veins, leading to detumescence. However, in priapism, abnormal autonomic or vascular tone or obstruction of venous outflow can lead to a prolonged erection. Sickle cell disease (SCD) is associated with an increased risk of priapism due to altered blood viscosity and resulting venous obstruction. Priapism in SCD can be precipitated by fever, cold exposure, alcohol, medications, dehydration (the likely trigger in this case), or cocaine use. Patients with SCD commonly experience brief, spontaneously terminating priapism events (ie, stuttering priapism) leading up to a more severe event that requires medical care. Priapism is a medical emergency that requires prompt treatment to avoid long-term complications (eg, erectile dysfunction). Initial management of priapism includes aspiration of blood from the corpora cavernosa. Blood gas analysis of aspirated blood typically shows an ischemic pattern with acidosis, hypoxemia, and hypercarbia. In addition to aspiration, additional treatment may include intracavernous injection of a sympathomimetic drug (eg, phenylephrine) to stimulate contraction of cavernous smooth muscle and facilitate venous emptying. (Choice A) Blood transfusion is generally performed in SCD with uncomplicated severe anemia; in case of complicated anemia (eg, acute chest syndrome), exchange transfusion may be performed. Packed red blood cell transfusion is typically not beneficial in priapism caused by SCD and would not be the first step in management. (Choices B and C) Detumescence occurs because sympathetic nervous system input causes cavernous smooth muscle contraction and vasoconstriction (not vasodilation) of the blood vessels in the penis. Nitroglycerin is a potent vasodilator, and beta blockers have little effect on penile vascular tone; neither has a role in the treatment of priapism. (Choice E) Insertion of a urinary catheter is necessary only if a patient with priapism is unable to void. It does not treat the underlying pathology. Educational objective: Priapism is a persistent, painful erection in the absence of ongoing sexual stimulation. Sickle cell disease is associated with an increased risk of priapism due to altered blood viscosity and resulting venous obstruction. Initial management of priapism includes aspiration of blood from the corpora cavernosa, often followed by intracavernous injection of phenylephrine.

A 22-Year-Old Man Comes to the Emergency Department with a Persistent