Page 124 - Remedial Andrology

in priapism and may result in a change in the NO pathway, with down-regulation of cavernosal PDE5 thereby
preventing the complete degradation of cGMP in the corpus cavernosum [1260, 1295, 1356, 1382].

9.2.1.4.9 Intracavernosal injections
Some patients with stuttering priapism, who have been started on systemic treatment to prevent recurrence
of unwanted erections, may not see therapeutic benefits immediately and temporarily require intracavernous
self-injections at home with sympathomimetic agents [1260, 1295]. The most commonly used drugs are
phenylephrine and etilephrine (as described in the treatment of ischaemic priapism) [1243, 1280, 1351, 1365]
(LE: 3). Adverse effects include hypertension, coronary ischaemia and cardiac arrhythmias.

Tissue plasminogen activator (TPA) is a secreted serine protease that converts the pro-enzyme plasminogen to
plasmin, which acts as a fibrinolytic enzyme. Limited clinical data have suggested that a single intracavernous
injection of TPA can successfully treat patients with recalcitrant priapism [1367, 1387] (LE: 3). Mild bleeding is
the most commonly observed adverse effect.

9.2.1.4.10 Penile prosthesis
Patients with medically refractory stuttering priapism require frequent visits to the emergency department and
are always at risk of a major ischaemic episode, which can be mitigated with insertion of a penile prosthesis
[1342, 1388, 1389]. Nevertheless, penile prosthesis for preventing stuttering priapism should not be offered
before medical treatment and a penile prosthesis should be performed only in carefully selected patients as a
last resort [1389]. In patients with permanent ED due to stuttering priapism, medical treatments for ED (PDE5Is
or intracavernosal injection) should be used cautiously because of the risk of inducing an ischaemic episode
and a penile prosthesis can be considered [1389, 1390].

9.2.1.5 Summary of evidence for treatment of stuttering priapism

Summary of evidence LE
The primary goal in the management of patients with stuttering priapism is prevention of future 2b
episodes, which can generally be achieved pharmacologically.
Phosphodiesterase type 5 inhibitors have a paradoxical effect in alleviating and preventing stuttering 3
priapism, mainly in patients with idiopathic and sickle cell disease-associated priapism.
The evidence with other systemic drugs (digoxin, α-adrenergic agonists, baclofen, gabapentin and 3
terbutaline, hydroxyurea) is limited.

9.2.1.6 Recommendations for treatment of stuttering priapism

Recommendations Strength rating
Manage each acute episode similar to that for ischaemic priapism. Strong
Use hormonal therapies (mainly gonadotropin-receptor hormone agonists or antagonists) Weak
and/or antiandrogens for the prevention of future episodes in patients with frequent
relapses. Do not use them before sexual maturation is reached.
Initiate treatment with phosphodiesterase type 5 inhibitors only when the penis is in its Weak
flaccid state.
Use digoxin, α-adrenergic agonists, baclofen, gabapentin or terbutaline only in patients with Weak
frequent and uncontrolled relapses.
Use intracavernous self-injections of sympathomimetic drugs at home for treatment of Weak
acute episodes on an interim basis until ischaemic priapism has been alleviated.

9.2.1.7 Follow-up
Follow-up for stuttering priapism includes history and clinical examination to assess the efficacy of treatment in
preventing or alleviating erectile events as well as assessing erectile function and penile fibrosis.

9.2.2 Priapism in children
The classification of priapism in children is similar to that in adults. In addition to ischaemic, stuttering and
non-ischaemic priapism, a fourth type, neonatal priapism is also described [1243]. Priapism in children is
considered rare as no data on its prevalence exist. SIckle cell disease is the major cause of priapism in
children, followed by leukaemia (10%), trauma (10%), idiopathic causes (19%) and drugs (5%) [1391]. One
study showed that 25% of children experienced SCD-related priapism in a pre-pubertal period [1392]. Another

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