Clitoral Priapism

What is Clitoral Priapism 

• In health, an integral part of the human sexual response is tumescence of the penis in males and the clitoris and vulva in females, known as erection.
• The physiological process that results in tumescence is the result of a complex interplay of the parasympathetic and sympathetic nervous system and vasoactive neurotransmitters, including prostaglandin E ₁ and the vasoactive intestinal polypeptide, as well as nitric oxide.
• Rarely, tumescence of the penis and clitoris can occur in the absence of sexual arousal and can be the result of systemic disease, for example, sickle cell disease, or drugs such as sildenafil and trazodone.
• Occasionally, no causative factor can be identified. If the duration of tumescence is prolonged, it is termed priapism. Although most attention has been paid to cases of priapism occurring in males, more recently cases of clitoral priapism have been identified as an uncommon cause of female pelvic pain.
• Priapism of the clitoris is a condition that may develop during therapy with certain medications, specifically those possessing a strong alpha-adrenergic blockade.
• Conditions altering blood flow to the clitoris may also predispose to developing this condition.
• Familiarity with this condition and a high index of suspicion are paramount in establishing a diagnosis.

Drugs Implicated in Priapism in Men and Women

• Trazodone
• Bupropion
• Risperidone
• Olanzapine
• Fluoxetine
• Bromocriptine
• Nefazodone
• Citalopram
• Papaverine
• Cocaine
• Sildenafil
• Vardenafil
• Tadalafil

What are the Symptoms of Clitoral Priapism

• Priapism of the clitoris is defined as a painful and often prolonged erection of the clitoris in the absence of sexual arousal.
• The erection may last from minutes to hours and is often described as painful, with the pain being characterized as burning and often involving not only the clitoris but also the vulva.
• The patient may be hesitant to describe the exact nature or location of the painful erection because of embarrassment or a lack of understanding as to what is actually causing the pain.
• Often, the patient may report a painful swelling of her vagina and attribute her symptoms to an insect bite, urinary tract or vaginal infection, or allergic reaction.
• On physical examination, the examiner will note that the clitoris is erect and firm, with the glans of the clitoris retracted beneath the engorged clitoral hood. Rubor is often present, as well as significant allodynia. Tenderness to palpation is also a common finding.
• Vaginal transudation, which is seen as part of female sexual arousal, is usually absent. It should be noted that enlargement of the clitoris has other causes, some of which are painful and some not, such as infiltrative tumors.

Causes of Clitoromegaly

Congenital

• • Congenital adrenal hyperplasia, classical
• • Ambiguous genitalia, isolated or in syndromic conditions

Acquired

• • Hormonal
  • • Congenital adrenal hyperplasia, late onset
  • • Ovarian or adrenal tumors (androgen secreting)
  • • Iatrogenic androgen exposure
• • Nonhormonal
  • 1. Neurofibromatosis
  • 2. Epidermoid cyst (spontaneous or traumatic, female genital mutilation)
  • 3. Hemangioma of the clitoris or the prepuce
  • 4. Metastatic infiltration
  • 5. Idiopathic

How is Clitoral Priapism diagnosed?

• Pelvic examination is the cornerstone of the diagnosis of patients with vulvodynia. Careful examination for infection, cutaneous or mucosal abnormalities, tenderness, muscle spasm, or tumor is crucial to avoid overlooking clitoral, vulvar, or pelvic malignancy. Ultrasound examination of the pelvis is indicated in all patients with clitoral priapism.
• Color Doppler imaging may be used to quantify cavernosal artery velocity values, as baseline cavernosal artery velocity values in the normal clitoris increase significantly after sexual stimulation, but in women with clitoral priapism, cavernosal artery velocity values are minimal, consistent with priapism-induced ischemia.
• If a question exists regarding occult malignancy of the vulva or pelvic contents, magnetic resonance imaging (MRI) or computed tomography (CT) of the pelvis is mandatory to rule out malignancy or disease of the pelvic organs that may be responsible for the symptoms.
• Urinalysis to rule out urinary tract infection also is indicated in all patients with vulvodynia. Culture for sexually transmitted diseases, including herpes, is indicated in the evaluation of all patients thought to have clitoral priapism.
• Based on the patient’s clinical presentation, additional tests, including complete blood cell count, erythrocyte sedimentation rate, and antinuclear antibody testing, may be indicated. MRI and electromyography of the lumbar plexus are indicated if tumor or hematoma is suspected.

How is clitoral priapism treated?

The foundation of treatment of clitoral priapism is to first identify the factor responsible for the symptoms and then immediately remove it.

Because most cases of both male and female priapism are drug induced, a careful drug history looking at both legal and illegal drugs is mandatory.

A history of spider bite or painful insect stings also should be ascertained because the venom of both black widow spiders and scorpions can cause priapism.

Empiric treatment with alpha-adrenergic drugs such as phenylephrine and phenylpropanolamine should be initiated with careful monitoring of the patient’s cardiovascular status.

Oral pseudoephedrine has also been reported to treat clitoral priapism. Intracavernosal injection of phenylephrine may be effective in the treatment of refractory clitoral priapism. If the clitoral priapism persists, corporal-spongiosum shunt surgery may be required on an emergent basis.

Complications

The major pitfalls in the care of a patient with clitoral priapism are threefold:

1. The misdiagnosis of extraclitoral pathological processes responsible for the pain
2. The failure to identify clitoral or vulvar or pelvic malignancy or both
3. The failure to address the psychological issues surrounding the pain

Differential diagnosis

The differential diagnosis of clitoral priapism includes various conditions that can cause clitoral enlargement or engorgement.

1. Sexual Arousal Disorder: Excessive sexual arousal or persistent genital arousal disorder (PGAD) can lead to clitoral engorgement and discomfort. It is essential to differentiate between clitoral priapism and PGAD as the management approaches may differ.
2. Trauma or Injury: Physical injury or trauma to the clitoral area may lead to localized swelling and engorgement, mimicking priapism.
3. Infection: Certain infections, such as cellulitis or Bartholin’s gland abscess, can cause inflammation and swelling of the clitoral area.
4. Clitoral Cyst or Tumor: A cyst or tumor in or around the clitoral area can lead to clitoral enlargement and pain.
5. Vascular Disorders: Some vascular conditions, such as arteriovenous malformation or clitoral varices, can cause clitoral engorgement and pain.
6. Medications: Certain medications, such as selective serotonin reuptake inhibitors (SSRIs) or medications that affect blood flow, may have clitoral engorgement as a side effect.
7. Neurological Disorders: Neurological conditions that affect the pelvic nerves can sometimes lead to clitoral priapism.
8. Idiopathic: In some cases, the cause of clitoral priapism may not be apparent, and it is considered idiopathic.

Clinical Pearls

• The most common cause of clitoral priapism is drug-induced clitoral dysfunction.
• The clinician should be aware that the relationship of the genitalia to the female psyche presents some unique challenges when treating patients with clitoral priapism.
• The behavioral and psychological issues must be addressed concurrently with the medical issues if treatment is to be successful.
• The possibility for vulvar or pelvic malignancy is ever present and should be carefully sought in all patients thought to have clitoral priapism.

Summary

• Clitoral priapism is a rare cause of vulvar pain and can be excruciating to the patient. Diagnosis may be difficult given embarrassment, vague history and inconclusive physical exam.
• Several causes has been linked to clitoral priapism including, alpha blocking agents, antidepressants, psychotropics, and 5H2 blockers.
• Other causes, such as cancer, have also been implicated. Treatment involves alpha agonists for acute attacks, imipramine, and symptomatic relief with NSAIDS and ice packs. Gynecology follow up is necessary for further evaluation.

Sources

Modified from Bruni V, Pontello V, Dei M, et al. Hemangioma of the clitoris presenting as clitoromegaly: a case report. J Pediatr Adolesc Gynecol. 2009;22:e137–e138.

Gharahbaghian L. Clitoral priapism with no known risk factors. West J Emerg Med. 2008 Nov;9(4):235-7. PMID: 19561754; PMCID: PMC2672283. https://pmc.ncbi.nlm.nih.gov/articles/PMC2672283/