Priapism is a condition in which a

penis A penis (; : penises or penes) is a sex organ through which male and hermaphrodite animals expel semen during copulation (zoology), copulation, and through which male placental mammals and marsupials also Urination, urinate. The term ''pen ...

remains erect for hours in the absence of

stimulation Stimulation is the encouragement of development or the cause of activity in general. For example, "The press provides stimulation of political discourse." An interesting or fun activity can be described as "stimulating", regardless of its physic ...

or after stimulation has ended. There are three types:

ischemic Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems ...

(low-flow), nonischemic (high-flow), and recurrent ischemic (intermittent). Most cases are ischemic. Ischemic priapism is generally painful while nonischemic priapism is not. In ischemic priapism, most of the penis is hard; however, the

glans penis In male human anatomy, the glans penis or penile glans, commonly referred to as the glans, (; from Latin ''glans'' meaning "acorn") is the bulbous structure at the Anatomical terms of location#Proximal and distal, distal end of the human penis ...

is not. In nonischemic priapism, the entire penis is only somewhat hard. Very rarely, clitoral priapism occurs in women.

Sickle cell disease Sickle cell disease (SCD), also simply called sickle cell, is a group of inherited Hemoglobinopathy, haemoglobin-related blood disorders. The most common type is known as sickle cell anemia. Sickle cell anemia results in an abnormality in the ...

is the most common cause of ischemic priapism. Other causes include medications such as

antipsychotic Antipsychotics, previously known as neuroleptics and major tranquilizers, are a class of Psychiatric medication, psychotropic medication primarily used to manage psychosis (including delusions, hallucinations, paranoia or disordered thought), p ...

s, SSRIs,

blood thinners An anticoagulant, commonly known as a blood thinner, is a chemical substance that prevents or reduces the coagulation of blood, prolonging the clotting time. Some occur naturally in blood-eating animals, such as leeches and mosquitoes, which h ...

and

prostaglandin E1 Prostaglandin E1 (PGE1) is a naturally occurring prostaglandin with various medical uses. Alprostadil and misoprostol are synthetic forms of prostaglandin E1 used as medications. Lubiprostone, a derivative of prostaglandin E1, is also used as ...

, as well as drugs such as

cocaine Cocaine is a tropane alkaloid and central nervous system stimulant, derived primarily from the leaves of two South American coca plants, ''Erythroxylum coca'' and ''Erythroxylum novogranatense, E. novogranatense'', which are cultivated a ...

. Ischemic priapism occurs when blood does not adequately drain from the penis. Nonischemic priapism is typically due to a connection forming between an

artery An artery () is a blood vessel in humans and most other animals that takes oxygenated blood away from the heart in the systemic circulation to one or more parts of the body. Exceptions that carry deoxygenated blood are the pulmonary arteries in ...

and the corpus cavernosum or disruption of the

parasympathetic nervous system The parasympathetic nervous system (PSNS) is one of the three divisions of the autonomic nervous system, the others being the sympathetic nervous system and the enteric nervous system. The autonomic nervous system is responsible for regulat ...

resulting in increased arterial flow. Nonischemic priapism may occur following trauma to the penis or a

spinal cord injury A spinal cord injury (SCI) is damage to the spinal cord that causes temporary or permanent changes in its function. It is a destructive neurological and pathological state that causes major motor, sensory and autonomic dysfunctions. Symptoms of ...

. Diagnosis may be supported by

blood gas A blood gas test or blood gas analysis tests blood to measure blood gas tension values and blood pH. It also measures the level and base excess of bicarbonate. The source of the blood is reflected in the name of each test; arterial blood gases ...

analysis of blood aspirated from the penis or an

ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...

. Treatment depends on the type. Ischemic priapism is typically treated with a

nerve block Nerve block or regional nerve blockade is any deliberate interruption of signals traveling along a nerve, often for the purpose of pain relief. #Local anesthetic nerve block, Local anesthetic nerve block (sometimes referred to as simply "nerve b ...

of the penis followed by aspiration of blood from the corpora cavernosa. If this is not sufficient, the corpus cavernosum may be irrigated with cold,

normal saline Saline (also known as saline solution) is a mixture of sodium chloride (salt) and water. It has a number of uses in medicine including cleaning wounds, removal and storage of contact lenses, and help with dry eyes. By intravenous therapy, inje ...

or injected with

phenylephrine Phenylephrine, sold under the brand names Neosynephrine and Sudafed PE among others, is a medication used as a decongestant for uncomplicated nasal congestion in the form of a nasal spray or oral tablet, to dilate the pupil, to increase bloo ...

. Nonischemic priapism is often treated with cold packs and compression. Surgery may be done if usual measures are not effective. In ischemic priapism, the risk of permanent scarring of the penis begins to increase after four hours and definitely occurs after 48 hours. Priapism occurs in about 1 in 20,000 to 1 in 100,000 males per year.

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."

In women

Priapism in women (continued, painful erection of the

clitoris In amniotes, the clitoris ( or ; : clitorises or clitorides) is a female sex organ. In humans, it is the vulva's most erogenous zone, erogenous area and generally the primary anatomical source of female Human sexuality, sexual pleasure. Th ...

) is significantly rarer than priapism in men and is known as ''clitoral priapism'' or ''clitorism''. It is associated with persistent genital arousal disorder (PGAD). Only a few case reports of women experiencing clitoral priapism exist.

Signs and symptoms

Complications

Because

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 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 Human penis, penile erection with sufficient rigidity and durat ...

or disfigurement of the penis. In extreme cases, if the penis develops severe vascular disease, the priapism can result in penile

gangrene Gangrene is a type of tissue death caused by a lack of blood supply. Symptoms may include a change in skin color to red or black, numbness, swelling, pain, skin breakdown, and coolness. The feet and hands are most commonly affected. If the ga ...

Low-flow priapism

Causes of low-flow priapism include

sickle cell anemia Sickle cell disease (SCD), also simply called sickle cell, is a group of inherited haemoglobin-related blood disorders. The most common type is known as sickle cell anemia. Sickle cell anemia results in an abnormality in the oxygen-carrying ...

(most common in children),

leukemia Leukemia ( also spelled leukaemia; pronounced ) is a group of blood cancers that usually begin in the bone marrow and produce high numbers of abnormal blood cells. These blood cells are not fully developed and are called ''blasts'' or '' ...

, and other blood dyscrasias such as

thalassemia Thalassemias are a group of Genetic disorder, inherited blood disorders that manifest as the production of reduced hemoglobin. Symptoms depend on the type of thalassemia and can vary from none to severe, including death. Often there is mild to ...

and

multiple myeloma Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibody, antibodies. Often, no symptoms are noticed initially. As it progresses, bone ...

, 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. Other conditions that can cause priapism include Fabry's disease, as well as neurologic disorders such as

spinal cord The spinal cord is a long, thin, tubular structure made up of nervous tissue that extends from the medulla oblongata in the lower brainstem to the lumbar region of the vertebral column (backbone) of vertebrate animals. The center of the spinal c ...

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 Medication (also called medicament, medicine, pharmaceutical drug, medicinal product, medicinal drug or simply drug) is a drug used to medical diagnosis, diagnose, cure, treat, or preventive medicine, prevent disease. Drug therapy (pharmaco ...

s. The most common medications that cause priapism are intra-cavernous injections for the treatment of

(

papaverine Papaverine (Latin '' papaver'', "poppy") is an opium alkaloid antispasmodic drug, used primarily in the treatment of visceral spasms and vasospasms (especially those involving the intestines, heart, or brain), occasionally in the treatment of ...

, alprostadil). Other medication groups reported are antihypertensives (e.g.

Doxazosin Doxazosin, sold under the brand name Cardura among others, is a medication used to treat symptoms of benign prostatic hyperplasia (enlarged prostate), hypertension (high blood pressure), and post-traumatic stress disorder (PTSD). For high bloo ...

s (e.g.,

chlorpromazine Chlorpromazine (CPZ), marketed under the brand names Thorazine and Largactil among others, is an antipsychotic medication. It is primarily used to treat psychotic disorders such as schizophrenia. Other uses include the treatment of bipolar d ...

clozapine Clozapine, sold under the brand name Clozaril among others, is a psychiatric medication and was the first atypical antipsychotic to be discovered. It is used primarily to treat people with schizophrenia and schizoaffective disorder who have ...

antidepressant Antidepressants are a class of medications used to treat major depressive disorder, anxiety disorders, chronic pain, and addiction. Common side effects of antidepressants include Xerostomia, dry mouth, weight gain, dizziness, headaches, akathi ...

s (most notably

trazodone Trazodone is an antidepressant medication used to treat major depressive disorder, anxiety disorders, and insomnia. It is a phenylpiperazine compound of the serotonin antagonist and reuptake inhibitor (SARI) class. The medication is taken or ...

anti-convulsant Anticonvulsants (also known as antiepileptic drugs, antiseizure drugs, or anti-seizure medications (ASM)) are a diverse group of pharmacological agents used in the treatment of epileptic seizures. Anticonvulsants are also used in the treatment ...

and

mood stabilizer A mood stabilizer is a psychiatric medication used to treat mood disorders characterized by intense and sustained mood shifts, such as bipolar disorder and the bipolar type of schizoaffective disorder. Uses Mood stabilizers are best known for t ...

drugs such as sodium valproate.

Anticoagulants An anticoagulant, commonly known as a blood thinner, is a chemical substance that prevents or reduces the coagulation of blood, prolonging the clotting time. Some occur naturally in blood-eating animals, such as leeches and mosquitoes, which h ...

, cantharides (''Spanish Fly'') and recreational drugs (

alcohol Alcohol may refer to: Common uses * Alcohol (chemistry), a class of compounds * Ethanol, one of several alcohols, commonly known as alcohol in everyday life ** Alcohol (drug), intoxicant found in alcoholic beverages ** Alcoholic beverage, an alco ...

heroin Heroin, also known as diacetylmorphine and diamorphine among other names, is a morphinan opioid substance synthesized from the Opium, dried latex of the Papaver somniferum, opium poppy; it is mainly used as a recreational drug for its eupho ...

and

) have been associated. Priapism is also known to occur from bites of the Brazilian wandering spider.

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).

Diagnosis

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

physical exam In a physical examination, medical examination, clinical examination, or medical checkup, a medical practitioner examines a patient for any possible medical signs or symptoms of a medical condition. It generally consists of a series of questions ...

Blood gas A blood gas test or blood gas analysis tests blood to measure blood gas tension values and blood pH. It also measures the level and base excess of bicarbonate. The source of the blood is reflected in the name of each test; arterial blood gases ...

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

may also help differentiate the two. Testing a person to make sure they do not have a

hemoglobinopathy Hemoglobinopathy is the medical term for a group of inherited blood disorders involving the hemoglobin, the major protein of red blood cells. They are generally single-gene disorders and, in most cases, they are inherited as Autosome, autosomal R ...

may also be reasonable.

Ultrasonography

Penile ultrasonography with

Doppler The Doppler effect (also Doppler shift) is the change in the frequency of a wave in relation to an observer who is moving relative to the source of the wave. The ''Doppler effect'' is named after the physicist Christian Doppler, who described ...

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:

CC-BY license 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.

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

Pseudoephedrine

Orally administered

pseudoephedrine Pseudoephedrine, sold under the brand name Sudafed among others, is a sympathomimetic medication which is used as a decongestant to treat nasal congestion. It has also been used off-label for certain other indications, like treatment of lo ...

is a first-line treatment for priapism. 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. This is done on either side. If this is not sufficiently effective, then cold

may be injected and removed.

Medications

If aspiration is not sufficient, a small dose of

may be injected into the corpus cavernosum. Side effects of phenylephrine may include:

high blood pressure Hypertension, also known as high blood pressure, is a long-term medical condition in which the blood pressure in the arteries is persistently elevated. High blood pressure usually does not cause symptoms itself. It is, however, a major ri ...

, slow heart rate, and

arrhythmia Arrhythmias, also known as cardiac arrhythmias, are irregularities in the cardiac cycle, heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – ab ...

. 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 Diethylstilbestrol (DES), also known as stilbestrol or stilboestrol, is a nonsteroidal estrogen medication, which is presently rarely used. In the past, it was widely used for a variety of indications, including pregnancy support for those with ...

(DES) or

terbutaline Terbutaline, sold under the brand names Bricanyl and Marex among others, is a β2 adrenergic receptor agonist, used as a "reliever" inhaler in the management of asthma symptoms and as a tocolytic (anti- contraction medication) to delay preterm ...

may be tried.

Surgery

Distal shunts, such as the Winter's, 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. Proximal shunts, such as the Quackel's, are more involved and entail operative dissection in the

perineum The perineum (: perineums or perinea) in placentalia, placental mammals is the space between the anus and the genitals. The human perineum is between the anus and scrotum in the male or between the anus and vulva in the female. The perineum is ...

where the corpora meet the spongiosum while making an incision in both and suturing both openings together. Shunts created between the corpora cavernosa and

great saphenous vein The great saphenous vein (GSV; ) or long saphenous vein is a large, subcutaneous, superficial vein of the human leg, leg. It is the longest vein in the body, running along the length of the lower limb, returning blood from the foot, human leg, le ...

called a Grayhack shunt can be done though this technique is rarely used. As the complication rates with prolonged priapism are high, early

penile prosthesis A penile implant is an Implant (Medicine), 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, inclu ...

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.

Sickle cell anemia

In sickle cell anemia, treatment is initially with

intravenous fluids Intravenous therapy (abbreviated as IV therapy) is a medical technique that administers fluids, medications and nutrients directly into a person's vein. The intravenous route of administration is commonly used for rehydration or to provide nutr ...

pain medication An analgesic drug, also called simply an analgesic, antalgic, pain reliever, or painkiller, is any member of the group of drugs used for pain management. Analgesics are conceptually distinct from anesthetics, which temporarily reduce, and in so ...

, and

oxygen therapy Oxygen therapy, also referred to as supplemental oxygen, is the use of oxygen as medical treatment. Supplemental oxygen can also refer to the use of oxygen enriched air at altitude. Acute indications for therapy include hypoxemia (low blood o ...

. The typical treatment of priapism may be carried out as well.

Blood transfusions Blood transfusion is the process of transferring blood products into a person's circulation intravenously. Transfusions are used for various medical conditions to replace lost components of the blood. Early transfusions used whole blood, but ...

are not usually recommended as part of the initial treatment, but if other treatments are not effective, exchange transfusion may be done.

History

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

Terminology

The name comes from the Greek god

Priapus In Greek mythology, Priapus (; ) is a minor rustic fertility god, protector of livestock, fruit plants, gardens, and male genitalia. Priapus is marked by his oversized, permanent erection, which gave rise to the medical term priapism. He becam ...

(), a fertility god, often represented with a disproportionately large phallus.

References

External links

Guideline on the Management of Priapism (2003)
- American Urological Association website
The unabridged 275-page version of this guideline.
* {{Male diseases of the pelvis and genitals Penis disorders Medical emergencies Sexual health Wikipedia emergency medicine articles ready to translate