Priapism is defined as a prolonged and persistent penile erection

lasting >4 hours, unassociated with sexual interest or stimulation. [1] It

is a true medical emergency with complications potentially resulting in
permanent erectile dysfunction. [2] In stuttering or recurrent priapism,
individual priapic episodes may last <4 hours.

Aetiology
Ischaemic priapism
Thromboembolic/hypercoagulable states:
o

Haemoglobinopathies (e.g., sickle cell

disease, [14] thalassaemia), thrombocystosis, assorted
haematological dyscrasias, [15] [16] parenteral
hyperalimentation, [17] haemodialysis, [18] and heparininduced platelet aggregation. [19]

Neoplastic disease:
o

Local primary (penile carcinoma, squamous cell

carcinoma, prostatic adenocarcinoma) or metastatic
neoplasia (metastases to the penis from, e.g., urothelial
carcinoma of the urinary bladder, cancer of the prostate,
rectosigmoid, kidney, or colon, and chronic myeloid
leukaemia). [20] [21][22] [23]

Neurological disease:
o

As a consequence of 'spinal shock' in acute spinal cord

injury, cauda equina syndrome, multiple sclerosis, spinal cord
tumour with compression, or neurotoxins.

Infection:

Rarely, ischaemic priapism may be seen in pelvic infection.

Vasoactive agents, medications, and legal/illegal drugs: [1]

o

These include erectile dysfunction pharmacotherapies

(e.g., phosphodiesterase 5 [PDE5] inhibitors and
intracavernous alprostadil), antihypertensives (e.g.,
hydralazine, prazosin), antipsychotics (e.g., chlorpromazine),
and antidepressants (e.g., trazodone). In addition, alcohol
and cocaine may predispose to ischaemic priapism.

Non-ischaemic priapism
This is much rarer than ischaemic priapism, and is
predominantly caused by traumatic injuries. [1] However, in
some patients no underlying cause is found. The most common
injuries reported are to the corporal bodies or perineum.
Mechanisms include straddle injury, coital trauma, kicks to the
penis or perineum, pelvic fractures, birth canal trauma to the
newborn male, and vascular erosions complicating metastatic
cancer infiltration of the corpora
cavernosa. [24] [23] [25] [26] [27]
While blunt trauma is still the most commonly reported aetiology,
high-flow priapism has also been described following surgical
interventions such as cold-knife urethrotomy, Nesbit's
corporoplasty, and deep dorsal vein arterialisations. [3]
Stuttering or recurrent priapism
Stuttering priapism can result from episodes of ischaemic
priapism and vice versa. Therefore, it shares many of the same
aetiological factors as those previously discussed for ischaemic
priapism. [1]

 While the cause of stuttering priapism remains idiopathic in

some cases, studies have shown recurrent priapism to be
related to a defective PDE5 regulatory function in the penis,
resulting from altered nitric oxide and cyclic guanosine
monophosphate (cGMP) signalling mechanisms that control
erectile function. [28] [3]
Pathophysiology

Ischaemic priapism is thought to be the result of an imbalance in the

vasoconstrictive and vasorelaxatory mechanisms governing penile
erection. Studies suggest that both vascular stasis within the penis
and reduced venous outflow are the primary pathophysiological
features in ischaemic priapism. [14] Deoxygenated blood in the penis
becomes trapped, creating venous congestion. The tissue ischaemia
and increased pressure generated within the corporal bodies lead to
the pain and penile rigidity clinically seen with ischaemic
priapism. [28]
Vascular stasis may result from an underlying hypercoagulable state.
Venous outflow obstruction may be a consequence of tumour,
infection, or reduced neural function. It is believed that these
ischaemic changes cause apoptosis, failure of corporal smooth
muscle contraction when activated by appropriate stimulus, and
upregulation of hypoxia-induced growth factors in the penis. This
results in damage to the corporal smooth muscle and vascular
endothelium leading to progressive hypoxia, hypercarbia, and
acidosis, which can be seen on cavernous blood gas analysis.
Permanent erectile dysfunction can follow. [1] [28] [3] [2] For these
reasons, ischaemic priapism is a medical emergency that demands
prompt medical treatment.
Non-ischaemic priapism is much rarer and has a different
pathophysiological basis. It is the result of unregulated cavernous

arterial inflow. Most episodes are trauma-induced, whereby the

cavernous artery or one of its tributaries within the corpora becomes
lacerated, creating an arteriolar-sinusoidal fistula. [28] The disruption
of penile arterial circulation results in excessive arterial inflow to the
penis. [24] [29] This produces unregulated pooling of blood in the
sinusoidal spaces, with consequent erection. [3] Cavernous blood
gases do not show hypoxia, hypercarbia, or acidosis. [1] [2]Therefore,
non-ischaemic priapism is not a medical emergency.
Stuttering priapism shares many of the same pathophysiological
mechanisms as ischaemic priapism. Studies also suggest that
dysregulation at the level of the penis, and at other regulatory levels,
including central or peripheral levels of the nervous system, have a
role in abnormal penile erection.[30] Recurrent priapism is thought to
be related to a defective PDE5 regulatory function in the penis,
resulting from altered nitric oxide (NO) and cGMP signalling
mechanisms that control erectile function. [30] [31] [32] NO is a
chemical released from the endothelium lining the trabeculae of the
corpora cavernosa that, together with its neuronal sources, results in
vasodilation during erection. A leading proposal in this regard is that
NO becomes dysfunctional in association with underlying disease
states. [31] From this understanding, novel modalities of treatment are
evolving. Research now suggests that factors involved in pathways
affecting inflammation, cellular adhesion, NO metabolism, vascular
reactivity, and coagulation may have roles in the pathophysiology of
SCD-associated stuttering priapism.
Classification

Physiological/clinical classification [1]

A classification system has been developed to assist with the practical

understanding of priapism and facilitate its clinical management.
Priapism has been divided into 3 main categories: ischaemic, nonischaemic, and stuttering priapism. [1] Ischaemic priapism, also

termed veno-occlusive or low-flow priapism, is a persistent erection

marked by rigidity of the corpora cavernosa and little or no cavernous
arterial inflow. The lack of arterial inflow creates an ischaemic
environment in the penis whereby abnormalities in the cavernous
blood gases are seen. Ischaemic priapism is painful and considered a
medical emergency. [1] [3] Non-ischaemic priapism, also termed
arterial or high-flow priapism, is a persistent erection caused by
unregulated cavernous arterial inflow. [1] The penis is tumescent but
not fully rigid. The continual arterial inflow does not predispose to an
ischaemic environment; therefore, cavernous blood gases are normal
and typically the erection is not painful. [1] [3] Non-ischaemic priapism
is not considered a medical emergency but should be treated in a
timely manner. The third type, stuttering or recurrent priapism, is
characterised by recurring episodes of ischaemic priapism. These
episodes generally last <4 hours before remission, but they may
increase in frequency and/or duration, potentially developing into full
ischaemic priapism episodes. [1] [3] Episodes are painful and
commonly arise during nocturnal sleep, preceding or following sexual
stimulation, and after morning erections. All stuttering episodes that
progress to >4 hours in duration should be treated the same as
ischaemic episodes and considered a medical
emergency. [2] Episodes <4 hours should be treated with the goal of
preventing future recurrences in order to decrease ischaemic damage
to the penis. Ischaemic and stuttering priapism resolve when the penis
returns to a flaccid and non-painful state, although in some cases
penile oedema and partial erection may remain. [1] Resolution of nonischaemic priapism constitutes return to a flaccid state. [1]
Ischaemic priapism
Veno-occlusive or low-flow priapism.
Typically features little or absent intracorporal blood flow.

 Cavernous blood gases are abnormal.

May be associated with haematological abnormalities (e.g.,
sickle cell disease), malignancy, medication or illicit drug use
(psychiatric medicines, cocaine, amphetamines), and vasoactive
drug use.
Is a true compartment syndrome involving the penis, in which
there are characteristic changes in cavernous blood gases and
excessive increases in intracorporal pressure.
Penis is fully rigid.
Penile pain is present.
Is a medical emergency and must be treated.
Non-ischaemic priapism
Arterial or high-flow priapism.
Typically features elevated vascular flow within the corpora
cavernosa.
Not associated with systemic disease or pharmacotherapy.
Commonly follows an episode of trauma to the perineum or the
genitalia.
Penis shows a chronic tolerated tumescence without full rigidity.
Penile pain is not typically present.
Is not a medical emergency.
Recurrent (stuttering) priapism
Usually ischaemic/low-flow in nature.

 Typically features recurrent attacks of short-lived (<4 hours)

priapic episodes.
Often occurs during nocturnal sleep, after morning erections, or
preceding or following sexual stimulation.
May be associated with haematological abnormalities (sickle cell
disease).
Is considered a medical emergency and must be treated with a
goal of prevention in mind.
Secondary prevention

Treatment of any associated medical condition may reduce the risk of

subsequent episodes of priapism occurring. Most available treatment
options do not prevent episodes of priapism and their pathological
consequences. It is here that phosphodiesterase (PDE5) inhibitors
offer promising new treatment possibilities for preventing stuttering
priapism in this patient population. Consequently, multi-centre,
randomised, placebo-controlled trials are under way to further
evaluate the potential use and benefit of PDE5 inhibitors for the
treatment of stuttering priapism. The use of PDE5 inhibitors is
investigational. [3] In some patients with recurrent priapism, the use of
anti-androgen therapy may also reduce recurrence.