Stuttering (Recurrent or

intermittent) Priapism
Epidemiology/ aetiology:
Recurrent priapism episodes are common in men with
sickle cell disease (42-64%) while in adolescents and young
men the incidence of priapism is 35%, of whom 72% have a
stuttering priapism
The aetiology of stuttering priapism is similar to that of
ischaemic priapism. Men has a risk for developing
stuttering priapism when he has suffered from an acute
ischaemic priapic event especially more than 4 hours
Stuttering (Recurrent or
intermittent) Priapism
Classification:
Stuttering priapism also termed intermittent or
recurrent priapism is a distinct condition that is
characterised by repetitive and painful episodes of
prolonged erections
These are analogous to repeated episodes of low flow
(ischaemic) priapism: duration of erectile episodes is
shorter than in ischaemic priapism, frequency and/ or
duration of these episodes is variable and a single
episode can sometimes progress into a major
ischaemic priapic episode
Stuttering (Recurrent or
intermittent) Priapism
Diagnostic evaluation:
The priapic episodes occurs during sleep and
detumescence does not occur upon waking. Priapic
episodes are painful.
Erections are painful, the penis is rigid, but duration of
events is usually shorter. Some cases, signs of fibrosis
can be found. And rarely the penis may become enlarged
(megalophallus).
Laboratory testing to identify possible causes and should
be directed by history, clinical, and laboratory finding.
Colour duplex US of the penis, perineum, and MRI to
differentiate arterial from ischaemic type of priapism.
Stuttering (Recurrent or
intermittent) Priapism
Disease Management:
Primary goal : the prevention of future episodes can be
achieved pharmacologically.
The management of each acute episodes : aspiration/
irrigation in combination with intracavernous injections of
alpha-adrenergic agonists.
Daily dosing of oral alpha-adrenergic agonists as effective
prevention. Side effects include tachycardia and
palpitations. First line treatment: pseudoephedrine as an
oral decongestant. Etilefrine has been used to prevent
stuttering priapism due to sickle cell anaemia, at doses of
50-100mg daily, with response rates of up to 72%
Stuttering (Recurrent or
intermittent) Priapism
The aim of hormonal manipulation is to down-regulate
circulating testosterone levels to suppress the action of
androgens on penile erection, using gonadotropin-
releasing hormone (GnRH) agonists or antagonist,
antiandrogens or oestrogens. Side effects: hot flushes,
gynaecomastia, impaired erectile function, loss of libido.
Primary treatment for adult men : GnRH agonists and
anti-androgens
Hormonal treatment have a contraceptive effect and
interfere with normal sexual maturation. Caution to
prepubertal boys, adolescents or men who are trying for
their female partner to conceive.
Stuttering (Recurrent or
intermittent) Priapism
Terbutaline is a beta-agonists that causes vasodilation,
resulting in smooth muscle relaxation, used to prevent
stuttering priapism with detumescence rates of 36% in
patients. Side effects: nervousness, shakiness,
drowsiness, heart palpitations, headache, dizziness, hot
flashes, nausea and weakness.
Gabapentin has anticonvulsant, antinociceptive and
anxiolytic properties, used as an analgesic and
antiepileptic agent. Doses : 400mg, 4 times a day, up to
2400mg daily, until complete penile detumescence
occurs, with subsequent maintenance administrations
of gabapentin 300mg daily. Side effects: anorgasmia,
impaired erectile function.
Stuttering (Recurrent or
intermittent) Priapism
Baclofen is a gamma-aminobutyric acid (GABA)
derivative that acts as a muscle relaxant and
anti-muscle spasm agent. Intrathecal baclofen
dosing is more effective in stuttering priapism.
Hydroxyurea blocks the synthesis of
deoxyribonucleic acid (DNA) by inhibiting
ribonucleotide reductase, which has the effect
of arresting cells in the S-phase. These are
treatment for ameliorating sickle cell disease
and improving their life expectancy. Side
effects: oligozoospermia, leg ulcers.
Stuttering (Recurrent or
intermittent) Priapism
Phosphodiesterase type 5 inhibitors
(PDE5Is) probably act in priapism by
increasing the concentration of cGMP in
the smooth muscle in a NO dysfunctional
state. This can occur in priapism and may
result in a change in the nitric oxide
pathway, with down-regulation of
cavernosal PDE5 thereby preventing the
complete degradation of cGMP in the
corpora cavernosa
Stuttering (Recurrent or
intermittent) Priapism