Priapism
Encyclopedia
Priapism is a potentially harmful and painful medical condition in which the erect
penis
or clitoris
does not return to its flaccid state, despite the absence of both physical and psychological stimulation, within four hours. There are two types of priapism: low-flow and high-flow. Low-flow involves the blood not adequately returning to the body from the organ. High-flow involves a short-circuit of the vascular system partway along the organ. Treatment is different for each type. Priapism is considered a medical emergency
, which should receive proper treatment by a qualified medical practitioner. Early treatment can be beneficial for a functional recovery.
The name comes from the Greek god Priapus
, a fertility god often represented with a disproportionately large and permanent erection.
factors. 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 hanging victims; see death erection
). Priapism may also be associated with glucose-6-phosphate dehydrogenase deficiency, which leads to decreased NADPH. NADPH is a co-factor involved in the formation of nitric oxide, therefore glucose-6-phosphate dehydrogenase deficiency will lower nitric oxide levels, which may result in priapism. Recent breakthroughs in research of the disease have pointed to a raised level of the biochemical adenosine being the cause of the condition. This seems to cause blood vessels to dilate and has the potential to influence blood flow into the penis.
Sickle cell disease often presents special treatment obstacles. Hyperbaric oxygen therapy has also been used with success in some patients.
Priapism is also found to occur in extreme cases of rabies.
Priapism can also be caused by reactions to medication
s. The most common medications that cause priapism are intra-cavernous injections for treatment of erectile dysfunction
(papaverine
, alprostadil
). Other groups reported are antihypertensives, antipsychotic
s (e.g., chlorpromazine
, clozapine
), antidepressant
s (most notably trazodone
), anticoagulants, cantharides (Spanish Fly) and recreational drugs (alcohol
, heroin and cocaine
). Priapism has also been linked to achalasia
. Priapism is also known to occur from bites of the Brazilian wandering spider
and the black widow spider
. PDE-5 inhibitors have been evaluated as preventive treatment for recurrent priapism.
, clotting of the blood retained in the penis (thrombosis
), and damage to the blood vessels of the penis which may result in an impaired erectile function or impotence
. In serious cases, the ischemia may result in gangrene
, which could necessitate penis removal
.
. In sickle cell patients with priapism, the first step in management is a blood exchange transfusion, not a surgical intervention. For other patients, orally administered pseudoephedrine
may be effective. Likewise, other sympathomimetic drugs of the amphetamine
class have been observed to induce erectile dysfunction, although in a small number of cases they may have the opposite effect. Otherwise, the therapy at this stage is to aspirate blood from the corpus cavernosum
under local anaesthetic
. If this is still insufficient, then intracavernosal injection
s of phenylephrine
are administered. This should only be performed by a specialist trained in the procedure, with the patient under constant hemodynamic monitoring, as phenylephrine can cause severe hypertension
, bradycardia
, tachycardia
, and arrhythmia.
If aspiration fails and tumescence
recurs, surgical shunts
are next attempted. These attempt to reverse the priapic state by shunting blood from the rigid corpora cavernosa into the corpus spongiosum
(which contains the glans
and the urethra
). Distal shunts are the first step, followed by more proximal shunts.
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 hematologic induced priapism, as it is relatively simple and repeatable over time.
Proximal shunts, such as the Quackel's, 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.
Shunts created between corpora cavernosa and saphenous vein called Grayhack shunt can be done though rarely.
As the complication of shortened, indurated and non-erectile penis is high in prolonged priapism, early penile prosthesis implantation can be performed. Apart from early resumption of sexual activity, early implantation can avoid the formation of dense fibrosis and hence a shortened penis.
) is also known as clitorism
.
Erection
Penile erection is a physiological phenomenon where the penis becomes enlarged and firm. Penile erection is the result of a complex interaction of psychological, neural, vascular and endocrine factors, and is usually, though not exclusively, associated with sexual arousal...
penis
Penis
The penis is a biological feature of male animals including both vertebrates and invertebrates...
or clitoris
Clitoris
The clitoris is a sexual organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina. Unlike the penis, which is homologous to the clitoris, the clitoris does not...
does not return to its flaccid state, despite the absence of both physical and psychological stimulation, within four hours. There are two types of priapism: low-flow and high-flow. Low-flow involves the blood not adequately returning to the body from the organ. High-flow involves a short-circuit of the vascular system partway along the organ. Treatment is different for each type. Priapism is considered a medical emergency
Medical emergency
A medical emergency is an injury or illness that is acute and poses an immediate risk to a person's life or long term health. These emergencies may require assistance from another person, who should ideally be suitably qualified to do so, although some of these emergencies can be dealt with by the...
, which should receive proper treatment by a qualified medical practitioner. Early treatment can be beneficial for a functional recovery.
The name comes from the Greek god Priapus
Priapus
In Greek mythology, Priapus or Priapos , was a minor rustic fertility god, protector of livestock, fruit plants, gardens and male genitalia. Priapus is marked by his absurdly oversized, permanent erection, which gave rise to the medical term priapism...
, a fertility god often represented with a disproportionately large and permanent erection.
Causes
The causative mechanisms are poorly understood but involve complex neurological and vascularVascular
Vascular in zoology and medicine means "related to blood vessels", which are part of the circulatory system. An organ or tissue that is vascularized is heavily endowed with blood vessels and thus richly supplied with blood....
factors. Priapism may be associated with haematological
Hematology
Hematology, also spelled haematology , is the branch of biology physiology, internal medicine, pathology, clinical laboratory work, and pediatrics that is concerned with the study of blood, the blood-forming organs, and blood diseases...
disorders, especially sickle-cell disease
Sickle-cell disease
Sickle-cell disease , or sickle-cell anaemia or drepanocytosis, is an autosomal recessive genetic blood disorder with overdominance, characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various...
, sickle-cell trait, and other conditions such as leukemia
Leukemia
Leukemia or leukaemia is a type of cancer of the blood or bone marrow characterized by an abnormal increase of immature white blood cells called "blasts". Leukemia is a broad term covering a spectrum of diseases...
, thalassemia
Thalassemia
Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region. In thalassemia the genetic defect, which could be either mutation or deletion, results in reduced rate of synthesis or no synthesis of one of the globin chains that make up hemoglobin...
, and Fabry's disease
Fabry's disease
Fabry disease is a rare X-linked recessive lysosomal storage disease, which can cause a wide range of systemic symptoms...
, and neurologic disorders
Neurology
Neurology is a medical specialty dealing with disorders of the nervous system. Specifically, it deals with the diagnosis and treatment of all categories of disease involving the central, peripheral, and autonomic nervous systems, including their coverings, blood vessels, and all effector tissue,...
such as spinal cord
Spinal cord
The spinal cord is a long, thin, tubular bundle of nervous tissue and support cells that extends from the brain . The brain and spinal cord together make up the central nervous system...
lesions and spinal cord trauma (priapism has been reported in hanging victims; see death erection
Death erection
A death erection, angel lust, or terminal erection is a post-mortem erection, technically a priapism, observed in the corpses of human males who have been executed, particularly by hanging.-Overview:...
). Priapism may also be associated with glucose-6-phosphate dehydrogenase deficiency, which leads to decreased NADPH. NADPH is a co-factor involved in the formation of nitric oxide, therefore glucose-6-phosphate dehydrogenase deficiency will lower nitric oxide levels, which may result in priapism. Recent breakthroughs in research of the disease have pointed to a raised level of the biochemical adenosine being the cause of the condition. This seems to cause blood vessels to dilate and has the potential to influence blood flow into the penis.
Sickle cell disease often presents special treatment obstacles. Hyperbaric oxygen therapy has also been used with success in some patients.
Priapism is also found to occur in extreme cases of rabies.
Priapism can also be caused by reactions to medication
Medication
A pharmaceutical drug, also referred to as medicine, medication or medicament, can be loosely defined as any chemical substance intended for use in the medical diagnosis, cure, treatment, or prevention of disease.- Classification :...
s. The most common medications that cause priapism are intra-cavernous injections for treatment of erectile dysfunction
Erectile dysfunction
Erectile dysfunction is sexual dysfunction characterized by the inability to develop or maintain an erection of the penis during sexual performance....
(papaverine
Papaverine
Papaverine is an opium alkaloid antispasmodic drug, used primarily in the treatment of visceral spasm, vasospasm , and occasionally in the treatment of erectile dysfunction...
, alprostadil
Alprostadil
Prostaglandin E1 , known pharmaceutically as alprostadil, is a prostaglandin. It is a drug used in the treatment of erectile dysfunction and has vasodilatory properties.-Sexual dysfunction:...
). Other groups reported are antihypertensives, antipsychotic
Antipsychotic
An antipsychotic is a tranquilizing psychiatric medication primarily used to manage psychosis , particularly in schizophrenia and bipolar disorder. A first generation of antipsychotics, known as typical antipsychotics, was discovered in the 1950s...
s (e.g., chlorpromazine
Chlorpromazine
Chlorpromazine is a typical antipsychotic...
, clozapine
Clozapine
Clozapine is an antipsychotic medication used in the treatment of schizophrenia, and is also used off-label in the treatment of bipolar disorder. Wyatt. R and Chew...
), antidepressant
Antidepressant
An antidepressant is a psychiatric medication used to alleviate mood disorders, such as major depression and dysthymia and anxiety disorders such as social anxiety disorder. According to Gelder, Mayou &*Geddes people with a depressive illness will experience a therapeutic effect to their mood;...
s (most notably trazodone
Trazodone
Trazodone is an antidepressant of the serotonin antagonist and reuptake inhibitor class. It is a phenylpiperazine compound...
), anticoagulants, cantharides (Spanish Fly) and recreational drugs (alcohol
Alcohol
In chemistry, an alcohol is an organic compound in which the hydroxy functional group is bound to a carbon atom. In particular, this carbon center should be saturated, having single bonds to three other atoms....
, heroin and cocaine
Cocaine
Cocaine is a crystalline tropane alkaloid that is obtained from the leaves of the coca plant. The name comes from "coca" in addition to the alkaloid suffix -ine, forming cocaine. It is a stimulant of the central nervous system, an appetite suppressant, and a topical anesthetic...
). Priapism has also been linked to achalasia
Achalasia
Achalasia , also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder involving the smooth muscle layer of the esophagus and the lower esophageal sphincter...
. Priapism is also known to occur from bites of the Brazilian wandering spider
Brazilian wandering spider
Phoneutria, commonly known as Brazilian wandering spiders, armed spiders , or banana spiders , are a genus of aggressive and highly venomous spiders found in tropical South and Central America...
and the black widow spider
Black widow spider
Latrodectus mactans, the Southern black widow, is a highly venomous species of spider in the genus Latrodectus. They are well known for the distinctive black and red coloring of the female of the species and for the fact that she will occasionally eat her mate after reproduction. The species is...
. PDE-5 inhibitors have been evaluated as preventive treatment for recurrent priapism.
Complications
Potential complications include ischemiaIschemia
In medicine, ischemia is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. It may also be spelled ischaemia or ischæmia...
, clotting of the blood retained in the penis (thrombosis
Thrombosis
Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...
), and damage to the blood vessels of the penis which may result in an impaired erectile function or impotence
Erectile dysfunction
Erectile dysfunction is sexual dysfunction characterized by the inability to develop or maintain an erection of the penis during sexual performance....
. In serious cases, the ischemia may result in gangrene
Gangrene
Gangrene is a serious and potentially life-threatening condition that arises when a considerable mass of body tissue dies . This may occur after an injury or infection, or in people suffering from any chronic health problem affecting blood circulation. The primary cause of gangrene is reduced blood...
, which could necessitate penis removal
Penectomy
Penectomy is the surgical removal of the penis for medical or personal reasons.- Medical Reasons for Penectomy :Cancer, for example, sometimes necessitates removal of part or of the penis. The amount of the penis removed depends on the severity of the cancer. Some men have only the tip of their...
.
Treatment
Medical advice should be sought immediately for cases of erection beyond four hours. Generally, this is done at an emergency departmentEmergency department
An emergency department , also known as accident & emergency , emergency room , emergency ward , or casualty department is a medical treatment facility specialising in acute care of patients who present without prior appointment, either by their own means or by ambulance...
. In sickle cell patients with priapism, the first step in management is a blood exchange transfusion, not a surgical intervention. For other patients, orally administered pseudoephedrine
Pseudoephedrine
Pseudoephedrine is a sympathomimetic drug of the phenethylamine and amphetamine chemical classes. It is used as a nasal/sinus decongestant and stimulant, or as a wakefulness-promoting agent....
may be effective. Likewise, other sympathomimetic drugs of the amphetamine
Amphetamine
Amphetamine or amfetamine is a psychostimulant drug of the phenethylamine class which produces increased wakefulness and focus in association with decreased fatigue and appetite.Brand names of medications that contain, or metabolize into, amphetamine include Adderall, Dexedrine, Dextrostat,...
class have been observed to induce erectile dysfunction, although in a small number of cases they may have the opposite effect. Otherwise, the therapy at this stage is to aspirate blood from the corpus cavernosum
Corpus cavernosum penis
The corpus cavernosum penis is one of a pair of sponge-like regions of erectile tissue which contain most of the blood in the penis during penile erection...
under local anaesthetic
Local anesthetic
A local anesthetic is a drug that causes reversible local anesthesia, generally for the aim of having local analgesic effect, that is, inducing absence of pain sensation, although other local senses are often affected as well...
. If this is still insufficient, then intracavernosal injection
Intracavernosal injection
An intracavernous injection is an injection into the base of the penis. This injection site is often used to administer medications to check for or treat erectile dysfunction in adult men .-References:...
s of phenylephrine
Phenylephrine
Phenylephrine is a selective α1-adrenergic receptor agonist used primarily as a decongestant, as an agent to dilate the pupil, and to increase blood pressure...
are administered. This should only be performed by a specialist trained in the procedure, with the patient under constant hemodynamic monitoring, as phenylephrine can cause severe hypertension
Hypertension
Hypertension or high blood pressure is a cardiac chronic medical condition in which the systemic arterial blood pressure is elevated. What that means is that the heart is having to work harder than it should to pump the blood around the body. Blood pressure involves two measurements, systolic and...
, bradycardia
Bradycardia
Bradycardia , in the context of adult medicine, is the resting heart rate of under 60 beats per minute, though it is seldom symptomatic until the rate drops below 50 beat/min. It may cause cardiac arrest in some patients, because those with bradycardia may not be pumping enough oxygen to their heart...
, tachycardia
Tachycardia
Tachycardia comes from the Greek words tachys and kardia . Tachycardia typically refers to a heart rate that exceeds the normal range for a resting heart rate...
, and arrhythmia.
If aspiration fails and tumescence
Tumescence
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...
recurs, surgical shunts
Shunt (medical)
In medicine, a shunt is a hole or a small passage which moves, or allows movement of fluid from one part of the body to another. The term may describe either congenital or acquired shunts; and acquired shunts may be either biological or mechanical.* Cardiac shunts may be described as...
are next attempted. These attempt to reverse the priapic state by shunting blood from the rigid corpora cavernosa into the corpus spongiosum
Corpus spongiosum
Corpus spongiosum is the mass of spongy tissue surrounding the male urethra within the penis...
(which contains the glans
Glans penis
The glans penis is the sensitive bulbous structure at the distal end of the penis. The glans penis is anatomically homologous to the clitoral glans of the female...
and the urethra
Urethra
In anatomy, the urethra is a tube that connects the urinary bladder to the genitals for the removal of fluids out of the body. In males, the urethra travels through the penis, and carries semen as well as urine...
). Distal shunts are the first step, followed by more proximal shunts.
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 hematologic induced priapism, as it is relatively simple and repeatable over time.
Proximal shunts, such as the Quackel's, are more involved and entail operative dissection in the perineum
Perineum
In human anatomy, the perineum is a region of the body including the perineal body and surrounding structures...
to where the corpora meet the spongiosum, making an incision in both, and suturing both openings together.
Shunts created between corpora cavernosa and saphenous vein called Grayhack shunt can be done though rarely.
As the complication of shortened, indurated and non-erectile penis is high in prolonged priapism, early penile prosthesis implantation can be performed. Apart from early resumption of sexual activity, early implantation can avoid the formation of dense fibrosis and hence a shortened penis.
In females
Priapism in females (continued, painful erection of the clitorisClitoris
The clitoris is a sexual organ that is present only in female mammals. In humans, the visible button-like portion is located near the anterior junction of the labia minora, above the opening of the urethra and vagina. Unlike the penis, which is homologous to the clitoris, the clitoris does not...
) is also known as clitorism
Clitorism
Clitorism is a continued, painful condition in the female with recurring erection of the clitoris. The word is also used to describe an abnormal enlargement of the clitoris. Clitorism is painful, much like its male counterpart, priapism....
.
Further reading
- Beers MH, Berkow R (Eds.) (1999). The Merck Manual of Diagnosis and Therapy (17 ed.). Whitehouse Station: Merck Research Laboratories. ISBN 0-911910-10-7
- Therapeutic Guidelines Limited (2001). Therapeutic Guidelines: Endocrinology (2 ed.). North Melbourne: Therapeutic Guidelines Limited. ISSN 1327-9505
- Guidelines on management of priapism - American Urological Association website
- Priapism Primer: Priapism
- Macaluso JN: Priapism (letter). Urology. 25:335, 1985