Priapism

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Priapism
Pompeya erotica6.jpg
Fresco in Pompeii depicting Priapus
Pronunciation
Specialty Urology, emergency medicine
Symptoms Penis remains erect for hours [3]
Complications Permanent scarring of the penis [3]
Types Ischemic (low-flow), nonischemic (high-flow), recurrent ischemic (intermittent) [3]
Causes Sickle cell disease, antipsychotics, SSRIs, blood thinners, cocaine, trauma [3]
TreatmentIschemic: Removal of blood from the corpus cavernosum with a needle [3]
Non-ischemic: Cold packs and compression [3]
Frequency1 in 60,000 males per year [3]

Priapism is a condition in which a penis remains erect for hours in the absence of stimulation or after stimulation has ended. [3] There are three types: ischemic (low-flow), nonischemic (high-flow), and recurrent ischemic (intermittent). [3] Most cases are ischemic. [3] Ischemic priapism is generally painful while nonischemic priapism is not. [3] In ischemic priapism, most of the penis is hard; however, the glans penis is not. [3] In nonischemic priapism, the entire penis is only somewhat hard. [3] Very rarely, clitoral priapism occurs in women. [4]

Contents

Sickle cell disease is the most common cause of ischemic priapism. [3] Other causes include medications such as antipsychotics, SSRIs, blood thinners and prostaglandin E1, as well as drugs such as cocaine. [3] [5] Ischemic priapism occurs when blood does not adequately drain from the penis. [3] Nonischemic priapism is typically due to a connection forming between an artery and the corpus cavernosum or disruption of the parasympathetic nervous system resulting in increased arterial flow. [3] Nonischemic priapism may occur following trauma to the penis or a spinal cord injury. [3] Diagnosis may be supported by blood gas analysis of blood aspirated from the penis or an ultrasound. [3]

Treatment depends on the type. [3] Ischemic priapism is typically treated with a nerve block of the penis followed by aspiration of blood from the corpora cavernosa. [3] If this is not sufficient, the corpus cavernosum may be irrigated with cold, normal saline or injected with phenylephrine. [3] Nonischemic priapism is often treated with cold packs and compression. [3] Surgery may be done if usual measures are not effective. [3] In ischemic priapism, the risk of permanent scarring of the penis begins to increase after four hours and definitely occurs after 48 hours. [3] [6] Priapism occurs in about 1 in 20,000 to 1 in 100,000 males per year. [3]

Classification

Priapism is classified into three groups: ischemic (low-flow), nonischemic (high-flow), and recurrent ischemic. [3] The majority of cases (19 out of 20) are ischemic in nature. [3]

Some sources give a duration of four hours as a definition of priapism, but others give six. Per the University Hospital Schleswig Holstein, "The duration of a normal erection before it is classifiable as priapism is still controversial. Ongoing penile erections for more than 6 hours can be classified as priapism." [7]

In women

Priapism in women (continued, painful erection of the clitoris) is significantly rarer than priapism in men and is known as clitoral priapism or clitorism. [4] It is associated with persistent genital arousal disorder (PGAD). [8] Only a few case reports of women experiencing clitoral priapism exist. [4]

Signs and symptoms

Complications

Because ischemic priapism causes the blood to remain in the penis for unusually long periods of time, the blood becomes deprived of oxygen, which can cause damage to the penile tissue. Such damage can result in erectile dysfunction or disfigurement of the penis. [9] In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile gangrene. [10]

Low-flow priapism

Causes of low-flow priapism include sickle cell anemia (most common in children), leukemia, and other blood dyscrasias such as thalassemia and multiple myeloma, and the use of various drugs, as well as cancers. [11] A genome-wide association study on Brazilian patients with sickle cell disease identified four single nucleotide polymorphisms in LINC02537 and NAALADL2 significantly associated with priapism. [12]

Other conditions that can cause priapism include Fabry's disease, as well as neurologic disorders such as spinal cord lesions and spinal cord trauma (priapism has been reported in people who have been hanged; see death erection).

Priapism can also be caused by reactions to medications. The most common medications that cause priapism are intra-cavernous injections for the treatment of erectile dysfunction (papaverine, alprostadil). Other medication groups reported are antihypertensives (e.g. Doxazosin), antipsychotics (e.g., chlorpromazine, clozapine), antidepressants (most notably trazodone), anti-convulsant and mood stabilizer drugs such as sodium valproate. [13] Anticoagulants, cantharides (Spanish Fly) and recreational drugs (alcohol, heroin and cocaine) have been associated. Priapism is also known to occur from bites of the Brazilian wandering spider. [14]

High-flow priapism

Causes of high-flow priapism include:

Diagnosis

The diagnosis is often based on the history of the condition as well as a physical exam. [3]

Blood gas testing the blood from the cavernosa of the penis can help in the diagnosis. [3] If the low-flow type of priapism is present, the blood typically has a low pH, while if the high-flow type is present, the pH is typically normal. [3] Color Doppler ultrasound may also help differentiate the two. [3] Testing a person to make sure they do not have a hemoglobinopathy may also be reasonable. [3]

Ultrasonography

Color Doppler ultrasound demonstrating a hypoechoic collection that corresponds to hematoma with arteriovenous fistula secondary to traumatic injury of the penis due to impact with bicycle handlebars, resulting in high-flow priapism Ultrasonography of traumatic arteriovenous fistula of the penis.jpg
Color Doppler ultrasound demonstrating a hypoechoic collection that corresponds to hematoma with arteriovenous fistula secondary to traumatic injury of the penis due to impact with bicycle handlebars, resulting in high-flow priapism

Penile ultrasonography with Doppler is the imaging method of choice, because it is noninvasive, widely available, and highly sensitive. By means of this method, it is possible to diagnose priapism and differentiate between its low- and high-flow forms. [11]

In low-flow (ischemic) priapism the flow in the cavernous arteries is reduced or absent. As the condition progresses, there is an increase in echogenicity of the corpora cavernosa, attributed to tissue edema. Eventually, changes in the echotexture of the corpora cavernosa can be observed due to the fibrotic transformation generated by tissue anoxia. [11]

In high-flow priapism normal or increased, turbulent blood flow in the cavernous arteries is seen. The area surrounding the fistula presents a hypoechoic, irregular lesion in the cavernous tissue. [11]

Treatment

Medical evaluation is recommended for erections that last for longer than four hours. Pain can often be reduced with a dorsal penile nerve block or penile ring block. [3] For those with nonischemic priapism, cold packs and pressure to the area may be sufficient. [3]

Pseudoephedrine

Orally administered pseudoephedrine is a first-line treatment for priapism. [15] Erection is largely a parasympathetic response, so the sympathetic action of pseudoephedrine may serve to relieve this condition. Pseudoephedrine is an alpha-agonist agent that exerts a constriction effect on smooth muscle of corpora cavernosum, which in turn facilitates venous outflow. Pseudoephedrine is no longer available in some countries.

Aspiration

For those with ischemic priapism, the initial treatment is typically aspiration of blood from the corpus cavernosum. [3] This is done on either side. [3] If this is not sufficiently effective, then cold normal saline may be injected and removed. [3]

Medications

If aspiration is not sufficient, a small dose of phenylephrine may be injected into the corpus cavernosum. [3] Side effects of phenylephrine may include: high blood pressure, slow heart rate, and arrhythmia. [3] If this medication is used, it is recommended that people be monitored for at least an hour after. [3] For those with recurrent ischemic priapism, diethylstilbestrol (DES) or terbutaline may be tried. [3]

Surgery

Distal shunts, such as the Winter's,[ clarification needed ] involve puncturing the glans (the distal part of the penis) into one of the cavernosa, where the old, stagnant blood is held. This causes the blood to leave the penis and return to the circulation. This procedure can be performed by a urologist at the bedside. Winter's shunts are often the first invasive technique used, especially in hematologically induced priapism, as it is relatively simple and repeatable. [16]

Proximal shunts, such as the Quackel's,[ clarification needed ] are more involved and entail operative dissection in the perineum where the corpora meet the spongiosum while making an incision in both and suturing both openings together. [17] Shunts created between the corpora cavernosa and great saphenous vein called a Grayhack shunt can be done though this technique is rarely used. [18]

As the complication rates with prolonged priapism are high, early penile prosthesis implantation may be considered. [3] As well as allowing early resumption of sexual activity, early implantation can avoid the formation of dense fibrosis and, hence, a shortened penis.

Sickle cell anemia

In sickle cell anemia, treatment is initially with intravenous fluids, pain medication, and oxygen therapy. [19] [3] The typical treatment of priapism may be carried out as well. [3] Blood transfusions are not usually recommended as part of the initial treatment, but if other treatments are not effective, exchange transfusion may be done. [19] [3]

History

Persistent semi-erections and intermittent states of prolonged erections have historically been sometimes called semi-priapism. [20]

Terminology

The name comes from the Greek god Priapus (Ancient Greek : Πρίαπος), a fertility god, often represented with a disproportionately large phallus. [21] [22]

Related Research Articles

Erectile dysfunction (ED), also referred to as impotence, is a form of sexual dysfunction in males characterized by the persistent or recurring inability to achieve or maintain a penile erection with sufficient rigidity and duration for satisfactory sexual activity. It is the most common sexual problem in males and can cause psychological distress due to its impact on self-image and sexual relationships.

<span class="mw-page-title-main">Penis enlargement</span> Technique aimed to increase the size of a human penis

Penis enlargement, or male enhancement, is any technique aimed to increase the size of a human penis. Some methods aim to increase total length, others the shaft's girth, and yet others the glans size. Techniques include surgery, supplements, ointments, patches, and physical methods like pumping, jelqing, and traction.

Erectile tissue is tissue in the body with numerous vascular spaces, or cavernous tissue, that may become engorged with blood. However, tissue that is devoid of or otherwise lacking erectile tissue may also be described as engorging with blood, often with regard to sexual arousal.

<span class="mw-page-title-main">Penile fracture</span> Rupture of the fibrous coverings that envelop the peniss erectile tissue

Penile fracture is rupture of one or both of the tunica albuginea, the fibrous coverings that envelop the penis's corpora cavernosa. It is caused by rapid blunt force to an erect penis, usually during vaginal intercourse, or aggressive masturbation. It sometimes also involves partial or complete rupture of the urethra or injury to the dorsal nerves, veins and arteries.

<span class="mw-page-title-main">Corpus spongiosum (penis)</span> Spongy tissue within the human penis

The corpus spongiosum is the mass of spongy tissue surrounding the male urethra within the penis. It is also called the corpus cavernosum urethrae in older texts.

Tumescence is the quality or state of being tumescent or swollen. Tumescence usually refers to the normal engorgement with blood of the erectile tissues, marking sexual excitation, and possible readiness for sexual activity. The tumescent sexual organ in males is the penis and in females is the clitoris and other parts of the genitalia like the vestibular bulbs. Arteries in the penis dilate to increase blood volume.

<span class="mw-page-title-main">Corpus cavernosum penis</span> Sponge-like region of erectile tissue

A corpus cavernosum penis (singular) is one of a pair of sponge-like regions of erectile tissue, which contain most of the blood in the penis during an erection.

<span class="mw-page-title-main">Corpus cavernosum of clitoris</span> One of a pair of regions in the clitoris that contain the blood during erection

The corpus cavernosum of the clitoris is one of a pair of sponge-like regions of erectile tissue that engorge with blood during an erection. This is homologous to the corpus cavernosum of the penis. The term corpora cavernosa literally means "cave-like bodies".

Nocturnal penile tumescence (NPT) is a spontaneous erection of the penis during sleep or when waking up. Along with nocturnal clitoral tumescence, it is also known as sleep-related erection. Colloquially, the term morning wood, or less commonly, morning glory is also used, although this is more commonly used to refer specifically to an erection beginning during sleep and persisting into the period just after waking. Men without physiological erectile dysfunction or severe depression experience nocturnal penile tumescence, usually three to five times during a period of sleep, typically during rapid eye movement sleep. Nocturnal penile tumescence is believed to contribute to penile health.

Venous leak, also called venogenic erectile dysfunction and penile venous insufficiency, is one category of vasculogenic impotence — a cause of erectile dysfunction in males. It affects all ages, being particularly awkward in young men. Much about venous leaks has not reached a consensus among the medical community, and many aspects of the condition, particularly its treatment strategies, are controversial. The prevalence of the condition is still unknown, although some sources claim it to be a common cause of erectile dysfunction.

<span class="mw-page-title-main">Penile frenulum</span> Band of tissue under the glans penis connecting the foreskin to the ventral mucosa

The frenulum of the penis, often known simply as the frenulum or frenum, is a thin elastic strip of tissue on the underside of the glans and the neck of the human penis. In men who are not circumcised, it also connects the foreskin to the glans and the ventral mucosa. In adults, the frenulum is typically supple enough to allow manual movement of the foreskin over the glans and help retract the foreskin during erection. In flaccid state, it tightens to narrow the foreskin opening.

<span class="mw-page-title-main">Helicine arteries of penis</span> Arteries of the penis

The helicine arteries of penis are arteries in the penis. They are found in the corpora cavernosa penis.

<span class="mw-page-title-main">Penile implant</span> Medical device

A penile implant is an implanted device intended for the treatment of erectile dysfunction, Peyronie's disease, ischemic priapism, deformity and any traumatic injury of the penis, and for phalloplasty or metoidioplasty, including in gender-affirming surgery. Men also opt for penile implants for aesthetic purposes. Men's satisfaction and sexual function is influenced by discomfort over genital size which leads to seek surgical and non-surgical solutions for penis alteration. Although there are many distinct types of implants, most fall into one of two categories: malleable and inflatable transplants.

<span class="mw-page-title-main">Human penis</span> Human male external reproductive organ

In human anatomy, the penis is an external male sex organ that serves as a passage for urine during urination and semen during ejaculation. The main parts are the root, body, the epithelium of the penis including the shaft skin, and the foreskin covering the glans. The body of the penis is made up of three columns of tissue: two corpora cavernosa on the dorsal side and corpus spongiosum between them on the ventral side. The urethra passes through the prostate gland, where it is joined by the ejaculatory ducts, and then through the penis. The urethra goes across the corpus spongiosum and ends at the tip of the glans as the opening, the urinary meatus.

<span class="mw-page-title-main">Erection</span> Physiological phenomenon involving the hardening and enlargement of the penis

An erection is a physiological phenomenon in which the penis becomes firm, engorged, and enlarged. Penile erection is the result of a complex interaction of psychological, neural, vascular, and endocrine factors, and is often associated with sexual arousal, sexual attraction or libido, although erections can also be spontaneous. The shape, angle, and direction of an erection vary considerably between humans.

<span class="mw-page-title-main">Diphallia</span> Genital medical condition

Diphallia, penile duplication (PD), diphallic terata, or diphallasparatus is an extremely rare developmental abnormality in which a male is born with two penises. The first reported case was by Johannes Jacob Wecker in 1609. Its occurrence is 1 in 5.5 million boys in the United States.

<span class="mw-page-title-main">Clitoral erection</span> Physiological phenomenon involving the engorgement of the clitoris

Clitoral erection is a physiological phenomenon where the clitoris becomes enlarged and firm.

A penile injury is a medical emergency that afflicts the penis. Common injuries include fracture, avulsion injury, strangulation, entrapment, and amputation.

Penile ultrasonography is medical ultrasonography of the penis. Ultrasound is an excellent method for the study of the penis, such as indicated in trauma, priapism, erectile dysfunction or suspected Peyronie's disease.

<span class="mw-page-title-main">Glans insufficiency syndrome</span> Medical condition

Glans insufficiency syndrome, also known as the soft glans, cold glans, or glans insufficiency, is a medical condition that affects male individuals. This condition is characterized by the persistent inability of the glans penis to achieve and maintain an erect or turgid state during sexual arousal, remaining soft and cold. This condition can have an impact on a person's sexual function, including decreased sensitivity, difficulty in maintaining an erection, and overall quality of life.

References

  1. ↑ "priapism". Oxford English Dictionary (2nd ed.). Oxford University Press. 1989. (as /ˈpraɪəpɪz(ə)m/)
  2. ↑ "priapism". Merriam-Webster.com Dictionary . Merriam-Webster. Retrieved 2017-03-07. "Definition of PRIAPISM". Archived from the original on 2017-06-06. Retrieved 2017-09-10.{{cite web}}: CS1 maint: bot: original URL status unknown (link).
  3. 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 Podolej, GS; Babcock, C (January 2017). "Emergency Department Management Of Priapism". Emergency Medicine Practice. 19 (1): 1–16. PMID   28027457.
  4. 1 2 3 Lehmiller, Justin J. (2014). The Psychology of Human Sexuality. John Wiley & Sons. p. 545. ISBN   978-1-119-16470-8 . Retrieved February 8, 2018.
  5. ↑ "Alprostadil". The American Society of Health-System Pharmacists. Archived from the original on 16 January 2017. Retrieved 8 January 2017.
  6. ↑ Salam, Muhammad A. (2003). Principles & Practice of Urology: A Comprehensive Text. Universal-Publishers. p. 342. ISBN   978-1-58112-411-8. Archived from the original on 2017-04-27.
  7. ↑ C. VAN DER HORST, HENRIK STUEBINGER, CHRISTOPH SEIF, DIETHILD MELCHIOR, F.J. MARTÍNEZ-PORTILLO, K.P. JUENEMANN; "Priapism: Etiology, Pathophysiology and Management" (PDF). Archived (PDF) from the original on 2013-04-29. Retrieved 2011-12-07.
  8. ↑ Helen Carcio; R. Mimi Secor (2014). Advanced Health Assessment of Women, Third Edition: Clinical Skills and Procedures. Springer Publishing Company. p. 85. ISBN   978-0-8261-2309-1 . Retrieved February 8, 2018.
  9. ↑ "Priapism - Symptoms and Causes". Mayo Clinic. Archived from the original on 2014-08-06. Retrieved 2014-08-30.
  10. ↑ Ajape, A. A.; Bello, A. (2011). "Penile Gangrene: An Unusual Complication of Priapism in a Patient with Bladder Carcinoma". J Surg Tech Case Rep. 3 (1): 37–9. doi: 10.4103/2006-8808.78470 . PMC   3192523 . PMID   22022653.
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    Fernandes, Maitê Aline Vieira; Souza, Luis Ronan Marquez Ferreira de; Cartafina, Luciano Pousa (2018). "Ultrasound evaluation of the penis". Radiologia Brasileira. 51 (4): 257–261. doi:10.1590/0100-3984.2016.0152. ISSN   1678-7099. PMC   6124582 . PMID   30202130.
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  12. ↑ Cintho Ozahata, Mina; Page, Grier P.; Guo, Yuelong; Ferreira, João Eduardo; Dinardo, Carla Luana; Carneiro-Proietti, Anna Bárbara F.; Loureiro, Paula; Mota, Rosimere Afonso; Rodrigues, Daniela O.W.; Belisario, André Rolim; Maximo, Claudia; Flor-Park, Miriam V.; Custer, Brian; Kelly, Shannon; Sabino, Ester Cerdeira; International Component of the NHLBI Recipient Epidemiology Donor Evaluation Study (REDS-III) (2019). "Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study". The Journal of Sexual Medicine. 16 (12): 1988–1999. doi:10.1016/j.jsxm.2019.09.012. PMC   6904926 . PMID   31668730.
  13. ↑ Bansal S, Gupta SK (November 2013). "Sodium Valproate induced priapism in an adult with bipolar affective disorder". Indian Journal of Pharmacology . 45 (6): 629–30. doi: 10.4103/0253-7613.121383 . PMC   3847259 . PMID   24347777.
  14. ↑ "Spider Venom for Erectile Dysfunction?". webmd.com. Archived from the original on 11 February 2015. Retrieved 11 February 2015.
  15. ↑ "Priapism (An Erection that Lasts Too Long)". mskcc.org. Retrieved 22 July 2021.
  16. ↑ Macaluso JN, Sullivan JW (1985). "Priapism: A review of 34 cases". Urology. 26 (3): 233–236. doi:10.1016/0090-4295(85)90116-5. PMID   4035837.
  17. ↑ Montague DK, Jarow J, Broderick GA, Dmochowski RR, Heaton JP, Lue TF, Nehra A, Sharlip ID (October 2003). "American Urological Association guideline on the management of priapism". J. Urol. 170 (4 Pt 1): 1318–24. doi:10.1097/01.ju.0000087608.07371.ca. PMID   14501756.
  18. ↑ Bassett, Jeffrey; Rajfer, Jacob (Winter 2010). "Diagnostic and Therapeutic Options for the Management of Ischemic and Nonischemic Priapism". Reviews in Urology. 12 (1): 56–63. PMC   2859143 . PMID   20428295.
  19. 1 2 Evidence Based Management of Sickle Cell Disease (PDF). NHLBI. 2014. pp. 39–40. Archived from the original (PDF) on 2017-01-25. Retrieved 2017-03-07.
  20. ↑ Newman Herbert F., Northup Jane D. (1981). "Mechanism of human penile erection: an overview". Urology. 17 (5): 399–408. doi:10.1016/0090-4295(81)90177-1. PMID   7015666.
  21. ↑ Munarriz, Ricardo; Kim, Noel N.; Traish, Abdul; Goldstein, Irwin (2005), Wessells, Hunter; McAninch, Jack W. (eds.), "Priapism", Urological Emergencies: A Practical Guide, vol. 29, no. 10, Totowa, NJ: Humana Press, pp. 213–224, doi:10.1385/1-59259-886-2:213, ISBN   978-1-59259-886-1, PMID   16447594
  22. ↑ Papadopoulos, I.; Kelâmi, A. (1988). "Priapus and priapism. From mythology to medicine". Urology. 32 (4): 385–386. doi:10.1016/0090-4295(88)90252-x. ISSN   0090-4295. PMID   3051631.
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