A 19-year-old man comes to the emergency department with penile pain. He started to get an erection about 2 hours ago, and it has not subsided. He took pain medications without relief. The patient has had no manipulation or trauma to his genitourinary tract. His only medical problem is sickle cell disease (Hb SS), with his usual vasoocclusive crisis being joint and bone pain. His last pain crisis was about 4 months ago. His medications include hydroxyurea, folate, oxycodone, and acetaminophen.
His temperature is 37.8 C (100 F), blood pressure is 140/90 mm Hg, pulse is 120/min, and respirations are 32/min. Pulse oximetry is 95% on room air. Examination shows a very uncomfortable man with an erect penis.
Laboratory results are as follows:

His chest x-ray shows no infiltrates.
Which of the following is the most appropriate next step in management?

Question

A 19-year-old man comes to the emergency department with penile pain.  He started to get an erection about 2 hours ago, and it has not subsided.  He took pain medications without relief.  The patient has had no manipulation or trauma to his genitourinary tract.  His only medical problem is sickle cell disease (Hb SS), with his usual vasoocclusive crisis being joint and bone pain.  His last pain crisis was about 4 months ago.  His medications include hydroxyurea, folate, oxycodone, and acetaminophen.
His temperature is 37.8 C (100 F), blood pressure is 140/90 mm Hg, pulse is 120/min, and respirations are 32/min.  Pulse oximetry is 95% on room air.  Examination shows a very uncomfortable man with an erect penis.
Laboratory results are as follows:

His chest x-ray shows no infiltrates.
Which of the following is the most appropriate next step in management?

Quizplus · Accepted Answer

This patient with sickle cell disease (SCD) presents with priapism, a serious SCD complication defined as persistent (>2-4 hours) penile or clitoral erection not associated with sexual stimulation or desire. Priapism has a prevalence of up to 45% in SCD patients, with the first episode usually occurring before age 20. The erection may last >2 hours and is likely due to decreased oxygen levels in the corpora cavernosa, which lead to sickling and blockage of penile venous outflow. Irreversible ischemic changes may occur in the penile tissue if the priapism is not corrected after 4 hours, resulting in fibrosis and erectile dysfunction. Recurrent priapism with more frequent and longer episodes may also lead to similar changes. Priapism with milder symptoms lasting <2 hours usually responds to increased fluid intake, oral analgesics, and attempted urination once the erection subsides. Priapism lasting >2 hours with more significant symptoms requires intravenous pain medication and hydration, blood aspiration from the corpus cavernosum with saline irrigation, and intracavernosal injection of sympathomimetics (eg, phenylephrine, epinephrine) to restore penile venous outflow. This procedure is usually done by an urologist. Persistent priapism (>12 hours) may require surgical shunting (glans-spongiosum shunt) to bypass blood from the corpus cavernosum to the corpus spongiosum and relieve the occlusion (Choice E). (Choice B) Epidural pain control can be used if oral and intravenous pain medications have not provided relief. (Choice C) Exchange transfusions are controversial as they can rapidly increase the hemoglobin (Hgb) level. If exchange transfusion is used, the target goal of Hgb should be <10 g/dL. Higher target Hgb after exchange transfusion has been associated with neurologic complications (eg, headaches, seizures, obtundation requiring ventilatory support). Aspiration is recommended as first line treatment. (Choice D) Hydroxyurea has been used to prevent priapism but is not effective for acute treatment. Educational objective: Priapism is a serious complication associated with sickle cell disease and defined as an erection lasting >2-4 hours. Blood aspiration from the corpus cavernosum, followed by saline irrigation and use of adrenergic agonists (eg, epinephrine), is first-line therapy. If priapism still persists, surgical shunting may be required. __________ References: Priapism: a review. (http://www.ncbi.nlm.nih.gov/pubmed/11665875) Priapism in sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/16214652) Priapism in sickle cell disease: incidence, risk factors and complications -an international multicentre study. (http://www.ncbi.nlm.nih.gov/pubmed/12460353)

A 19-Year-Old Man Comes to the Emergency Department with Penile