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Priapism

BASICS

  • Definition: Penile (or rarely clitoral) erection lasting >4 hours, unrelated to sexual stimulation.
  • Classification:
  • Ischemic (low-flow, veno-occlusive): 95% of cases; emergent, painful, risk of tissue necrosis.
  • Nonischemic (high-flow, arterial): Less common, often painless, not emergent.
  • Stuttering (recurrent ischemic): Episodic, short-lived, may not need intervention.
  • Malignant: Rare, usually due to metastasis (bladder, prostate, rectosigmoid, renal).

EPIDEMIOLOGY

  • Incidence: ~5.3/100,000 men/year
  • Age: Shift toward >40 years
  • Race: 61% African American (SCD association)
  • Children: SCD is the most common cause (63%), others include leukemia, trauma, drugs.

ETIOLOGY AND PATHOPHYSIOLOGY

  • Anatomy: 2 corpora cavernosa (erection), 1 corpus spongiosum (glans).
  • Ischemic priapism: Decreased venous outflow β†’ blood stasis, hypoxia, acidosis, compartment syndrome β†’ tissue necrosis/fibrosis if >24 hr.
  • Mechanisms: NO/cGMP dysregulation, RhoA/Rho kinase, adenosine, opiorphin.
  • Nonischemic: Increased arterial inflow, usually due to trauma (arteriocavernous fistula).
  • Causes:
  • Ischemic: Idiopathic (50%), SCD/hemoglobinopathies, leukemia, drugs (erectile agents, Ξ±-blockers, antidepressants, antipsychotics, antihypertensives, hormones, anticoagulants), infections, neurogenic, neoplastic, metabolic, recreational drugs.
  • Nonischemic: Penile/perineal trauma, acute spinal injury.
  • Risk Factors: SCD (lifetime risk 29–42%), dehydration, prior priapism.

GENERAL PREVENTION

  • Avoid dehydration (esp. SCD)
  • Avoid causative drugs, trauma, excessive stimulation

COMMONLY ASSOCIATED CONDITIONS

  • SCD (most important)
  • Drug abuse, G6PD deficiency, leukemia, neoplasm

DIAGNOSIS

History

  • Prior episodes, pain degree, duration, trauma, urination issues, hemoglobinopathy history, drug use

Physical Exam

  • Ischemic: Fully erect, painful, rigid corpora cavernosa; flaccid glans/corpus spongiosum.
  • Nonischemic: Partially erect, nontender, semirigid corpora; flaccid glans/spongiosum.
  • Assess for trauma, gangrene, prosthesis, underlying disease.

Diagnostic Tests

  • Labs: CBC (reticulocyte), sickling test, hemoglobin electrophoresis, coag profile, urinalysis/toxicology.
  • Corporal Blood Gas: Ischemic: pH <7.25, pO2 <30 mmHg, pCO2 >60 mmHg.
  • Imaging:
  • Doppler US: Ischemic = low flow; Nonischemic = high flow
  • MRI: Consider if >48–72 hr (to assess necrosis/prosthesis candidacy)
  • Arteriography: For nonischemic fistula/pseudoaneurysm

TREATMENT

Ischemic Priapism (Emergent)

  • Initial: Attempt ejaculation, vigorous exercise, ice/cold bath (do not delay definitive therapy!)
  • Definitive:
  • Aspiration of cavernosal blood (large-bore needle)
  • Intracavernosal phenylephrine: 200 ΞΌg q3–5min (max 1,500 ΞΌg) with BP monitoring
  • If failure: Shunt procedures (distal/proximal)
  • SCD: IV hydration, O2, exchange transfusion only after failed initial therapy
  • Analgesia: Opioids PRN
  • Second Line: Intracavernosal etilefrine, methylene blue, or epinephrine
  • Refer: Urology (ALL cases)

Nonischemic Priapism

  • Not emergent: Observe, ice, local compression
  • If persistent: Selective arterial embolization or surgery

Stuttering Priapism

  • Acute attacks: Manage as ischemic
  • Prevention:
  • Adrenergic agonists (pseudoephedrine, etilefrine)
  • Androgen suppression (antiandrogens, finasteride, ketoconazole)
  • PDE5 inhibitors (sildenafil)
  • Digoxin, terbutaline, gabapentin, baclofen, hydroxyurea

SURGERY/PROCEDURES

  • Shunt procedures: Distal (Ebbehoj, Winter, T, Al-Ghorab), proximal (Quackles, Sacher), venous (Grayhack, Barry)
  • Immediate penile prosthesis: For failed medical/surgical treatment or tissue necrosis (early surgery avoids fibrosis/curvature)
  • Nonischemic: Selective embolization, surgical ligation if needed

PROGNOSIS

  • Ischemic >24 hr: Up to 90% risk permanent ED
  • Detumescence may take weeks if prolonged priapism
  • Recurrence or stuttering common in SCD

COMPLICATIONS

  • Erectile dysfunction, tissue necrosis, mental health issues (depression, suicidality)
  • Avoid SSRIs, trazodone, fluoxetine, citalopram in at-risk patients

ICD-10 CODES

  • N48.30 Priapism, unspecified
  • N48.31 Priapism due to trauma
  • N48.39 Other priapism

CLINICAL PEARLS

  • Priapism >24 hr likely causes permanent sexual impairment.
  • In children, especially SCD, address both acute episode and underlying disease.
  • Always distinguish ischemic (emergent) from nonischemic by history, exam, cavernosal blood gas, and imaging.
  • Immediate urologic evaluation for ischemic priapism is essential.