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Priapism

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Priapism
Fresco in Pompeii depicting Priapus
Pronunciation
SpecialtyUrology, emergency medicine
SymptomsPenis remains erect for hours[3]
ComplicationsPermanent scarring of the penis[3]
TypesIschemic (low-flow), nonischemic (high-flow), recurrent ischemic (intermittent)[3]
CausesSickle cell disease, antipsychotics, SSRIs, blood thinners, cocaine, cannabis, 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]

Sickle cell disease is the most common cause of ischemic priapism.[3] Other causes include medications such as antipsychotics, SSRIs, and blood thinners, as well as drugs such as cocaine and cannabis.[3] 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 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][5] 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: "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."[6]

Priapism in females (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).[7] 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 and can cause damage to the penile tissue itself. Should the penile tissue become damaged, it can result in erectile dysfunction or disfigurement of the penis.[8] In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile gangrene.[9]

Causes

Priapism may be associated with haematological disorders, especially sickle-cell disease, sickle-cell trait, and other conditions such as leukemia, thalassemia, and Fabry's disease, and 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 may also be associated with glucose-6-phosphate dehydrogenase deficiency, which leads to decreased NADPH levels. NADPH is a co-factor involved in the formation of nitric oxide, which may result in priapism.[10]

Sickle cell disease often presents special treatment obstacles. Hyperbaric oxygen therapy has also been used with success in some patients.[11] Priapism is also found to occur in extreme cases of rabies. Priapism also occurs due to encephalitic rabies.

Medications

Priapism can also be caused by reactions to medications. The most common medications that cause priapism are intra-cavernous injections for treatment of erectile dysfunction (papaverine, alprostadil). Other groups reported are antihypertensives, antipsychotics (e.g., chlorpromazine, clozapine), antidepressants (most notably trazodone), anti-convulsant and mood stabilizer drugs such as sodium valproate,[12] anticoagulants, cantharides (Spanish Fly) and recreational drugs (alcohol, heroin and cocaine). Priapism is also known to occur from bites of the Brazilian wandering spider and the black widow spider.[13]

Mechanism

The mechanisms are poorly understood but involve complex neurological and vascular factors.

Diagnosis

The diagnosis is often based on the history of the condition and the 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]

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]

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.[14]

Proximal shunts, such as the Quackel's,[clarification needed] are more involved and entail operative dissection in the perineum to where the corpora meet the spongiosum, making an incision in both, and suturing both openings together.[15] Shunts created between corpora cavernosa and saphenous vein called Grayhack shunt can be done though this technique is rarely used.[citation needed]

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.[16][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.[16][3]

History

Persistent semi-erections or intermittent states of prolonged erections have historically been sometimes called semi-priapism.[17]

Terminology

The name comes from the Greek god Priapus (Ancient Greek: Πρίαπος), a fertility god often represented with a disproportionately large and permanent erection.

References

  1. ^ OED 2nd edition, 1989 as /ˈpraɪəpɪz(ə)m/.
  2. ^ "Definition of PRIAPISM". www.merriam-webster.com. Archived from the original on 6 June 2017. Retrieved 7 March 2017. {{cite web}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)
  3. ^ a b c d e f g h i j k l m n o p q r s t u v w x y z aa ab ac ad ae af ag ah ai aj ak al am an ao ap aq ar as at Podolej, GS; Babcock, C (January 2017). "Emergency Department Management Of Priapism". Emergency medicine practice. 19 (1): 1–16. PMID 28027457.
  4. ^ a b c The Psychology of Human Sexuality. John Wiley & Sons. 2014. p. 545. ISBN 1119164702. Retrieved February 8, 2018. {{cite book}}: Unknown parameter |authors= ignored (help)
  5. ^ Salam, Muhammad A. (2003). Principles & Practice of Urology: A Comprehensive Text. Universal-Publishers. p. 342. ISBN 9781581124118. Archived from the original on 2017-04-27. {{cite book}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)
  6. ^ PRIAPISM – ETIOLOGY, PATHOPHYSIOLOGY AND MANAGEMENT, C. VAN DER HORST, HENRIK STUEBINGER, CHRISTOPH SEIF, DIETHILD MELCHIOR, F.J. MARTÍNEZ-PORTILLO, K.P. JUENEMANN; "Archived copy" (PDF). Archived from the original (PDF) on 2013-04-29. Retrieved 2011-12-07. {{cite web}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)CS1 maint: archived copy as title (link)
  7. ^ Advanced Health Assessment of Women, Third Edition: Clinical Skills and Procedures. Springer Publishing Company. 2014. p. 85. ISBN 0826123090. Retrieved February 8, 2018. {{cite book}}: Unknown parameter |authors= ignored (help)
  8. ^ "Archived copy". Archived from the original on 2014-08-06. Retrieved 2014-08-30. {{cite web}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)CS1 maint: archived copy as title (link)
  9. ^ Ajape AA, Bello A (2011). "Penile Gangrene: An Unusual Complication of Priapism in a Patient with Bladder Carcinoma". J Surg Tech Case Rep. 3: 37–9. doi:10.4103/2006-8808.78470. PMC 3192523. PMID 22022653.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  10. ^ Finley DS (December 2008). "Glucose-6-phosphate dehydrogenase deficiency associated stuttering priapism: report of a case". J Sex Med. 5 (12): 2963–6. doi:10.1111/j.1743-6109.2008.01007.x. PMID 18823322.
  11. ^ Macaluso JN (1985). "Priapism: Update for the non-urologist". Sexual Medicine Today. 9: 11–15.
  12. ^ Bansal S, Gupta SK (November 2013). "Sodium Valproate induced priapism in an adult with bipolar affective disorder". Indian Journal of Pharmacology. 45 (6): 629. doi:10.4103/0253-7613.121383.{{cite journal}}: CS1 maint: unflagged free DOI (link)
  13. ^ "Spider Venom for Erectile Dysfunction?". webmd.com. Archived from the original on 11 February 2015. Retrieved 11 February 2015. {{cite web}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)
  14. ^ 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.
  15. ^ 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.
  16. ^ a b Evidence Based Management of Sickle Cell Disease (PDF). NHLBI. 2014. pp. 39–40. Archived from the original (PDF) on 2017-01-25. {{cite book}}: Unknown parameter |deadurl= ignored (|url-status= suggested) (help)
  17. ^ Newman, Herbert F., and Jane D. Northup. "Mechanism of human penile erection: an overview." Urology 17.5 (1981): 399-408.

Further reading

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