Priapism#Classification

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

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."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 }}

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

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.{{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}} In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile gangrene.{{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 }}

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. 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.{{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 }}

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.{{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 }} 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.{{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}}

===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.
• Anticoagulants (heparin and warfarin).
• Antihypertensives (i.e., hydralazine, guanethidine and propranolol).
• Hormones (i.e., gonadotropin releasing hormone and testosterone).

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

Blood gas testing the blood from the cavernosa of the penis can help in the diagnosis. 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. Color Doppler ultrasound may also help differentiate the two. Testing a person to make sure they do not have a hemoglobinopathy may also be reasonable.

File:Ultrasonography of traumatic arteriovenous fistula of the penis.jpg

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.Originally copied from:
{{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}}
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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.

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.

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. For those with nonischemic priapism, cold packs and pressure to the area may be sufficient.

Orally administered pseudoephedrine is a first-line treatment for priapism.{{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}} 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.

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

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

Distal shunts, such as the 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.{{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 }}

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.{{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 }} Shunts created between the corpora cavernosa and great saphenous vein called a Grayhack shunt can be done though this technique is rarely used.{{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 }}

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

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

Persistent semi-erections and intermittent states of prolonged erections have historically been sometimes called semi-priapism.{{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 }}

The name comes from the Greek god Priapus ({{langx|grc|Πρίαπος}}), a fertility god, often represented with a disproportionately large phallus.{{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.}}{{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}}

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• [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.]
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