Editing Priapism

{{short description|Medical condition where an erection lasts excessively long}}
{{Infobox medical condition (new)
| name            = Priapism
| image           = Pompeya erótica6.jpg
| caption         = Fresco in [[Pompeii]] depicting [[Priapus]]
| pronounce       = {{IPAc-en|ˈ|p|r|aɪ|.|ə|ˌ|p|ɪ|z|əm}}<ref>{{OED2|priapism}} (as {{IPA|/ˈpraɪəpɪz(ə)m/}})</ref><ref>{{cite Merriam-Webster|priapism |access-date=2017-03-07}} {{Cite web |url=https://www.merriam-webster.com/dictionary/priapism |title=Definition of PRIAPISM |access-date=2017-09-10 |archive-date=2017-06-06 |archive-url=https://web.archive.org/web/20170606061141/https://www.merriam-webster.com/dictionary/priapism |url-status=bot: unknown }}.</ref>
| field           = [[Urology]], emergency medicine
| symptoms        = [[Human penis|Penis]] remains [[erection|erect]] for hours<ref name=EM2017/>
| complications   = Permanent scarring of the penis<ref name=EM2017/>
| onset           = 
| duration        = 
| types           = [[Ischemic]] (low-flow), nonischemic (high-flow), recurrent ischemic (intermittent)<ref name=EM2017/>
| causes          = [[Sickle cell disease]], [[antipsychotic]]s, [[SSRIs]], [[anticoagulants|blood thinners]], [[cocaine]], trauma<ref name=EM2017/>
| risks           = 
| diagnosis       = 
| differential    = 
| prevention      = 
| treatment       = '''Ischemic''': Removal of blood from the [[Corpus cavernosum penis|corpus cavernosum]] with a [[hypodermic needle|needle]]<ref name=EM2017/><br />'''Non-ischemic''': Cold packs and compression<ref name=EM2017/>
| medication      = 
| prognosis       = 
| frequency       = 1 in 60,000 males per year<ref name=EM2017/>
| deaths          = 
}}
<!-- Definition and symptoms -->
'''Priapism''' is a condition in which a [[human penis|penis]] remains [[erection|erect]] for hours in the absence of [[Sexual stimulation|stimulation]] or after stimulation has ended.<ref name=EM2017/> There are three types: [[ischemic]] (low-flow), nonischemic (high-flow), and recurrent ischemic (intermittent).<ref name=EM2017/> Most cases are ischemic.<ref name=EM2017/> Ischemic priapism is generally painful while nonischemic priapism is not.<ref name=EM2017/> In ischemic priapism, most of the penis is hard; however, the [[glans penis]] is not.<ref name=EM2017/> In nonischemic priapism, the entire penis is only somewhat hard.<ref name=EM2017/> Very rarely, [[clitoral]] priapism occurs in women.<ref name=Lehmiller2014>{{cite book |first=Justin J. |last=Lehmiller |title=The Psychology of Human Sexuality |publisher=[[John Wiley & Sons]] |isbn=978-1-119-16470-8 |year=2014 |page=545 |access-date=February 8, 2018 |url=https://books.google.com/books?id=ytk5DwAAQBAJ&pg=PT545}}</ref>

<!-- Cause, mechanism, and diagnosis -->
[[Sickle cell disease]] is the most common cause of ischemic priapism.<ref name=EM2017/> Other causes include medications such as [[antipsychotic]]s, [[SSRIs]], [[anticoagulants|blood thinners]] and [[prostaglandin E1]], as well as drugs such as [[cocaine]].<ref name=EM2017/><ref>{{cite web|title=Alprostadil|url=https://www.drugs.com/monograph/alprostadil.html|publisher=The American Society of Health-System Pharmacists|access-date=8 January 2017|url-status=live|archive-url=https://web.archive.org/web/20170116185111/https://www.drugs.com/monograph/alprostadil.html|archive-date=16 January 2017}}</ref> Ischemic priapism occurs when blood does not adequately drain from the penis.<ref name=EM2017/> Nonischemic priapism is typically due to a connection forming between an [[artery]] and the [[Corpus cavernosum penis|corpus cavernosum]] or disruption of the [[parasympathetic nervous system]] resulting in increased arterial flow.<ref name=EM2017/> Nonischemic priapism may occur following trauma to the penis or a [[spinal cord injury]].<ref name=EM2017/> Diagnosis may be supported by [[blood gas]] analysis of blood aspirated from the penis or an [[ultrasound]].<ref name=EM2017/>

<!-- Treatment, prognosis, and epidemiology-->
Treatment depends on the type.<ref name=EM2017/> Ischemic priapism is typically treated with a [[nerve block]] of the penis followed by aspiration of blood from the corpora cavernosa.<ref name=EM2017/> If this is not sufficient, the corpus cavernosum may be irrigated with cold, [[normal saline]] or injected with [[phenylephrine]].<ref name=EM2017/> Nonischemic priapism is often treated with cold packs and compression.<ref name=EM2017/> Surgery may be done if usual measures are not effective.<ref name=EM2017/> In ischemic priapism, the risk of permanent scarring of the penis begins to increase after four hours and definitely occurs after 48 hours.<ref name=EM2017/><ref>{{cite book|last1=Salam|first1=Muhammad A.|title=Principles & Practice of Urology: A Comprehensive Text|date=2003|publisher=Universal-Publishers|isbn=978-1-58112-411-8|page=342|url=https://books.google.com/books?id=QPSWilXhECkC&pg=PA342|language=en|url-status=live|archive-url=https://web.archive.org/web/20170427100327/https://books.google.ca/books?id=QPSWilXhECkC&pg=PA342|archive-date=2017-04-27}}</ref> Priapism occurs in about 1 in 20,000 to 1 in 100,000 males per year.<ref name="EM2017">{{cite journal |last1=Podolej |first1=GS |last2=Babcock |first2=C |date=January 2017 |title=Emergency Department Management Of Priapism. |journal=Emergency Medicine Practice |volume=19 |issue=1 |pages=1–16 |pmid=28027457}}</ref>

==Classification==
Priapism is classified into three groups: ischemic (low-flow), nonischemic (high-flow), and recurrent ischemic.<ref name=EM2017/> The majority of cases (19 out of 20) are ischemic in nature.<ref name=EM2017/>

Some sources give a duration of four hours as a definition of priapism, but others give six. Per the [[University Medical Center Schleswig Holstein| 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."<ref>C. VAN DER HORST, HENRIK STUEBINGER, CHRISTOPH SEIF, DIETHILD MELCHIOR, F.J. MARTÍNEZ-PORTILLO, K.P. JUENEMANN; {{cite web |url=http://www.scielo.br/pdf/ibju/v29n5/18554.pdf |title=Priapism: Etiology, Pathophysiology and Management|access-date=2011-12-07 |url-status=live |archive-url=https://web.archive.org/web/20130429234259/http://www.scielo.br/pdf/ibju/v29n5/18554.pdf |archive-date=2013-04-29 }}</ref>

===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''.<ref name="Lehmiller2014" /> It is associated with [[persistent genital arousal disorder]] (PGAD).<ref>{{cite book |author=Helen Carcio |author2=R. Mimi Secor |title=Advanced Health Assessment of Women, Third Edition: Clinical Skills and Procedures|publisher=[[Springer Publishing Company]]|isbn=978-0-8261-2309-1|year=2014|page=85|access-date=February 8, 2018|url=https://books.google.com/books?id=Q4QbBQAAQBAJ&pg=PA85}}</ref> Only a few case reports of women experiencing clitoral priapism exist.<ref name="Lehmiller2014" />

==Signs and symptoms==
===Complications===
Because [[ischemia|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.<ref>{{cite web | title= Priapism - Symptoms and Causes | publisher= [[Mayo Clinic]] | url= http://www.mayoclinic.org/diseases-conditions/priapism/basics/complications/con-20029378 | access-date= 2014-08-30 | url-status= live | archive-url= https://web.archive.org/web/20140806005205/http://www.mayoclinic.org/diseases-conditions/priapism/basics/complications/con-20029378 | archive-date= 2014-08-06}}</ref> In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile [[gangrene]].<ref>{{cite journal | title= Penile Gangrene: An Unusual Complication of Priapism in a Patient with Bladder Carcinoma | last1= Ajape | first1= A. A. | last2= Bello | first2= A. | journal= J Surg Tech Case Rep | year= 2011 | volume= 3 | issue= 1 | pages= 37–9 | pmc= 3192523 | pmid= 22022653 | doi= 10.4103/2006-8808.78470 | doi-access= free }}</ref>

===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.<ref name="FernandesSouza2018"/> 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.<ref>{{Cite journal |doi=10.1016/j.jsxm.2019.09.012 |pmc=6904926 |pmid=31668730|title=Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study |year=2019 |last1=Cintho Ozahata |first1=Mina |last2=Page |first2=Grier P. |last3=Guo |first3=Yuelong |last4=Ferreira |first4=João Eduardo |last5=Dinardo |first5=Carla Luana |last6=Carneiro-Proietti |first6=Anna Bárbara F. |last7=Loureiro |first7=Paula |last8=Mota |first8=Rosimere Afonso |last9=Rodrigues |first9=Daniela O.W. |last10=Belisario |first10=André Rolim |last11=Maximo |first11=Claudia |last12=Flor-Park |first12=Miriam V. |last13=Custer |first13=Brian |last14=Kelly |first14=Shannon |last15=Sabino |first15=Ester Cerdeira |author16=International Component of the NHLBI Recipient Epidemiology Donor Evaluation Study (REDS-III) |journal=The Journal of Sexual Medicine |volume=16 |issue=12 |pages=1988–1999 }}</ref>

Other conditions that can cause priapism include [[Fabry's disease]], as well as [[neurology|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 [[medication]]s. 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]]), [[antipsychotic]]s (e.g., [[chlorpromazine]], [[clozapine]]), [[antidepressant]]s (most notably [[trazodone]]), [[anti-convulsant]] and [[mood stabilizer]] drugs such as [[sodium valproate]].<ref>{{cite journal |vauthors=Bansal S, Gupta SK | title = Sodium Valproate induced priapism in an adult with bipolar affective disorder | journal = [[Indian Journal of Pharmacology]] | volume = 45 | issue = 6 | pages = 629–30 |date=November 2013 | doi = 10.4103/0253-7613.121383| pmc = 3847259 | pmid=24347777 | doi-access = free }}</ref> [[Anticoagulants]], [[cantharides]] (''Spanish Fly'') and recreational drugs ([[alcohol (drug)|alcohol]], [[heroin]] and [[cocaine]]) have been associated. Priapism is also known to occur from bites of the [[Brazilian wandering spider]].<ref>{{cite web|url=http://www.webmd.com/erectile-dysfunction/news/20090924/spider-venom-for-erectile-dysfunction|title=Spider Venom for Erectile Dysfunction?|work=webmd.com|access-date=11 February 2015|url-status=live|archive-url=https://web.archive.org/web/20150211214317/http://www.webmd.com/erectile-dysfunction/news/20090924/spider-venom-for-erectile-dysfunction|archive-date=11 February 2015}}</ref>

===High-flow priapism=== 
Causes of high-flow priapism include:
*blunt trauma to the perineum or penis, with laceration of the cavernous artery, which can generate an arterial-lacunar fistula.<ref name="FernandesSouza2018"/>
*Anticoagulants (heparin and warfarin).
*Antihypertensives (i.e., hydralazine, guanethidine and propranolol).
*Hormones (i.e., gonadotropin releasing hormone and testosterone).

==Diagnosis==
The diagnosis is often based on the history of the condition as well as a [[physical exam]].<ref name=EM2017/>

[[Blood gas]] testing the blood from the cavernosa of the penis can help in the diagnosis.<ref name=EM2017/> 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.<ref name=EM2017/> Color Doppler [[ultrasound]] may also help differentiate the two.<ref name=EM2017/> Testing a person to make sure they do not have a [[hemoglobinopathy]] may also be reasonable.<ref name=EM2017/>

===Ultrasonography===
[[File:Ultrasonography of traumatic arteriovenous fistula of the penis.jpg|thumb|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<ref name="FernandesSouza2018"/>]]
[[Penile ultrasonography]] with [[Doppler ultrasonography|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.<ref name="FernandesSouza2018">Originally copied from:<br />{{cite journal|last1=Fernandes|first1=Maitê Aline Vieira|last2=Souza|first2=Luis Ronan Marquez Ferreira de|last3=Cartafina|first3=Luciano Pousa|title=Ultrasound evaluation of the penis|journal=Radiologia Brasileira|volume=51|issue=4|year=2018|pages=257–261|issn=1678-7099|doi=10.1590/0100-3984.2016.0152|pmc=6124582|pmid=30202130}}<br />[[Creative Commons|CC-BY]] license</ref>

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.<ref name="FernandesSouza2018"/>

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.<ref name="FernandesSouza2018"/>

==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]].<ref name=EM2017/> For those with nonischemic priapism, cold packs and pressure to the area may be sufficient.<ref name=EM2017/>

===Pseudoephedrine===

Orally administered [[pseudoephedrine]] is a first-line treatment for priapism.<ref>{{cite web|title=Priapism (An Erection that Lasts Too Long)|url=https://www.mskcc.org/cancer-care/patient-education/priapism|website=mskcc.org|access-date=22 July 2021|language=en}}</ref> 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 [[Suction (medicine)|aspiration]] of blood from the [[Corpus cavernosum penis|corpus cavernosum]].<ref name=EM2017/> This is done on either side.<ref name=EM2017/> If this is not sufficiently effective, then cold [[normal saline]] may be injected and removed.<ref name=EM2017/>

===Medications===
If aspiration is not sufficient, a small dose of [[phenylephrine]] may be injected into the corpus cavernosum.<ref name=EM2017/> Side effects of phenylephrine may include: [[hypertension|high blood pressure]], [[bradycardia|slow heart rate]], and [[Heart arrhythmia|arrhythmia]].<ref name=EM2017/> If this medication is used, it is recommended that people be monitored for at least an hour after.<ref name=EM2017/> For those with recurrent ischemic priapism, [[diethylstilbestrol]] (DES) or [[terbutaline]] may be tried.<ref name=EM2017/>

===Surgery===
Distal shunts, such as the [[George D. Winter|Winter's]],{{clarify|date=November 2009}} 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.<ref>{{cite journal |vauthors=Macaluso JN, Sullivan JW | title = Priapism: A review of 34 cases | journal = Urology | volume = 26 | pages = 233–236 | year = 1985 | doi = 10.1016/0090-4295(85)90116-5 | pmid = 4035837 | issue = 3 }}</ref>

Proximal shunts, such as the Quackel's,{{clarify|date=November 2009}} 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.<ref>{{cite journal |vauthors=Montague DK, Jarow J, Broderick GA, Dmochowski RR, Heaton JP, Lue TF, Nehra A, Sharlip ID | title = American Urological Association guideline on the management of priapism | journal = [[J. Urol.]] | volume = 170 | issue = 4 Pt 1 | pages = 1318–24 |date=October 2003 | pmid = 14501756 | doi = 10.1097/01.ju.0000087608.07371.ca }}</ref> Shunts created between the corpora cavernosa and [[great saphenous vein]] called a Grayhack shunt can be done though this technique is rarely used.<ref>{{Cite journal |last1=Bassett |first1=Jeffrey |last2=Rajfer |first2=Jacob |date=Winter 2010 |title=Diagnostic and Therapeutic Options for the Management of Ischemic and Nonischemic Priapism |journal=Reviews in Urology |language=en |volume=12 |issue=1 |pages=56–63 |pmid=20428295|pmc=2859143 }}</ref>

As the complication rates with prolonged priapism are high, early [[Penile implant|penile prosthesis]] implantation may be considered.<ref name=EM2017/> 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]].<ref name=NIH2014/><ref name=EM2017/> The typical treatment of priapism may be carried out as well.<ref name=EM2017/> [[Blood transfusions]] are not usually recommended as part of the initial treatment, but if other treatments are not effective, [[exchange transfusion]] may be done.<ref name=NIH2014>{{cite book|title=Evidence Based Management of Sickle Cell Disease|date=2014|publisher=NHLBI|pages=39–40|url=https://www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/sickle-cell-disease-report.pdf|archive-url=https://web.archive.org/web/20170125155233/https://www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/sickle-cell-disease-report.pdf|archive-date=2017-01-25|access-date=2017-03-07}}</ref><ref name=EM2017/>

==History==
Persistent semi-erections and intermittent states of prolonged erections have historically been sometimes called semi-priapism.<ref>{{cite journal | author = Newman Herbert F., Northup Jane D. | year = 1981 | title = Mechanism of human penile erection: an overview | journal = Urology | volume = 17 | issue = 5| pages = 399–408 | doi=10.1016/0090-4295(81)90177-1| pmid = 7015666 }}</ref>

===Terminology===
The name comes from the Greek god [[Priapus]] ({{langx|grc|Πρίαπος}}), a fertility god, often represented with a disproportionately large phallus.<ref>{{Citation |last1=Munarriz |first1=Ricardo |title=Priapism |date=2005 |work=Urological Emergencies: A Practical Guide |pages=213–224 |editor-last=Wessells |editor-first=Hunter |place=Totowa, NJ |publisher=Humana Press |language=en |doi=10.1385/1-59259-886-2:213 |isbn=978-1-59259-886-1 |last2=Kim |first2=Noel N. |last3=Traish |first3=Abdul |last4=Goldstein |first4=Irwin |volume=29 |issue=10 |pmid=16447594 |editor2-last=McAninch |editor2-first=Jack W.}}</ref><ref>{{Cite journal |last1=Papadopoulos |first1=I. |last2=Kelâmi |first2=A. |date=1988 |title=Priapus and priapism. From mythology to medicine |journal=Urology |volume=32 |issue=4 |pages=385–386 |doi=10.1016/0090-4295(88)90252-x |issn=0090-4295 |pmid=3051631}}</ref>

==References==
{{reflist}}

==External links==
{{Medical resources
| DiseasesDB      = 25148
| ICD10           = {{ICD10|N|48|3|n|40}}
| ICD9            = {{ICD9|607.3}}
| ICDO            = 
| OMIM            = 
| MedlinePlus     = 
| eMedicineSubj   = med
| eMedicineTopic  = 1908
| MeshID          = D011317}}

* [http://www.auanet.org/education/guidelines/priapism.cfm Guideline on the Management of Priapism (2003)] {{Webarchive|url=https://web.archive.org/web/20140521181122/http://www.auanet.org/education/guidelines/priapism.cfm |date=2014-05-21 }} - American Urological Association website - [https://web.archive.org/web/20140521195552/http://www.auanet.org/common/pdf/education/clinical-guidance/Priapism.pdf The unabridged 275-page version of this guideline.]
*{{Commons category-inline}}

{{Male diseases of the pelvis and genitals}}

[[Category:Priapism| ]]
[[Category:Penis disorders]]
[[Category:Medical emergencies]]
[[Category:Sexual health]]
[[Category:Wikipedia emergency medicine articles ready to translate]]