Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

ischaemic or recurrent (stuttering) priapism

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observation

For ischaemic or recurrent (stuttering) priapism lasting up to 4 hours, observation or treatment are both acceptable options for management, depending on clinician or patient preference.[21] However, delays in treatment predispose the patient to tissue injury that places the patient at risk for the development of erectile dysfunction. Therefore, prompt treatment of all episodes of ischaemic or stuttering priapism are encouraged.

Ischaemic or recurrent (stuttering) priapism lasting >4 hours is an emergency and requires prompt treatment.[1]

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aspiration ± irrigation

For ischaemic or recurrent (stuttering) priapism lasting up to 4 hours, observation or treatment are both acceptable options for management, depending on clinician or patient preference.[21] However, delays in treatment predispose the patient to tissue injury that places the patient at risk for the development of erectile dysfunction. Therefore, prompt treatment of all episodes of ischaemic or stuttering priapism are encouraged.

Anaesthesia with a penile nerve block is given before aspiration or intracavernosal injection.[5]

Penile blood is aspirated using a non-heparinised syringe. Therapeutic aspiration may be performed simultaneously with cavernous blood gas sampling after insertion of a scalp vein needle (16 or 18 gauge) directly into the corpus cavernosum.[4] 

Irrigation/flushing of the cavernosa with normal saline or phenylephrine diluted with normal saline to a concentration of 100-500 micrograms/mL may be used in conjunction with aspiration. If phenylephrine is unavailable, other sympathomimetics may be used.

Use of downward pressure on the glans of the erect penis also helps to evacuate blood out of the needles placed for irrigation and aspiration more readily. Careful attention should be made to not dislodge the needles with downward pressure and compression of the glans. If dislodged, additional needle sticks will be required for evacuation of blood.

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Plus – 

intracavernosal injection of sympathomimetic agent

Treatment recommended for ALL patients in selected patient group

Phenylephrine is the preferred sympathomimetic agent because it has a lower risk of cardiovascular adverse effects than other agents and is associated with a higher rate of detumescence.[1] However, if phenylephrine is unavailable, other alpha adrenergic agonists may be used. 

For intracavernous injections in adult patients, phenylephrine should be diluted with normal saline to a concentration of 100-500 micrograms/mL and 1 mL injections given every 5 minutes or more until detumescence, or for approximately 1 hour, before deciding that the treatment will not be successful.

During and following intracavernous injection of any sympathomimetic, the patient should be monitored for known adverse effects (e.g., acute hypertension, headache, reflex bradycardia, tachycardia, palpitations, and cardiac arrhythmia). In all patients undergoing aspiration with irrigation, especially patients with high cardiovascular risk, blood pressure and ECG monitoring are recommended.

If after a reasonable duration (some suggest 1 hour) and dose escalation of phenylephrine (some suggest 1000 micrograms of diluted phenylephrine over 1 hour) the penis is still tumesced, then a Doppler ultrasound should be considered to evaluate the status of the cavernosal arterial flow in the penis.[2][5] Swelling and oedema after appropriate detumescence with aspiration and irrigation may present like ischaemic priapism, and therefore an ultrasound can rule in or out whether blood has been appropriately evacuated from the corpora cavernosa.

Primary options

phenylephrine injection: (100-500 micrograms/mL) 1 mL intracavernosally every 5 minutes or more, maximum 1000 micrograms

Secondary options

ephedrine: consult specialist for guidance on dose

OR

adrenaline (epinephrine): consult specialist for guidance on dose

OR

noradrenaline (norepinephrine): consult specialist for guidance on dose

OR

metaraminol: consult specialist for guidance on dose

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aspiration ± irrigation

Anaesthesia with a penile nerve block is given before aspiration or intracavernosal injection.[5]

Penile blood is aspirated using a non-heparinised syringe. Therapeutic aspiration may be performed simultaneously with cavernous blood gas sampling after insertion of a scalp vein needle (16 or 18 gauge) directly into the corpus cavernosum.[4]

Irrigation/flushing of the cavernosa with normal saline or phenylephrine diluted with normal saline to a concentration of 100-500 micrograms/mL may be used in conjunction with aspiration. If phenylephrine is unavailable, other sympathomimetics may be used.

Use of downward pressure on the glans of the erect penis also helps to evacuate blood out of the needles placed for irrigation and aspiration more readily. Careful attention should be made not to dislodge the needles with downward pressure and compression of the glans. If dislodged, additional needle sticks will be required for evacuation of blood.

US and European guidelines differ on the implementation of first-line therapies during ischaemic priapism episodes of extended duration (>48 hours).[1][4] First-line treatments are unlikely to be successful in this circumstance and should be attempted at the surgeon's discretion.

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Plus – 

intracavernosal injection of sympathomimetic agent

Treatment recommended for ALL patients in selected patient group

Phenylephrine is the preferred sympathomimetic agent because it has a lower risk of cardiovascular adverse effects than other agents and is associated with a higher rate of detumescence.[1] However, if phenylephrine is unavailable, other alpha adrenergic agonists may be used. 

For intracavernous injections in adult patients, phenylephrine should be diluted with normal saline to a concentration of 100-500 micrograms/mL and 1 mL injections given every 5 minutes or more until detumescence, or for approximately 1 hour, before deciding that the treatment will not be successful.

During and following intracavernous injection of any sympathomimetic, the patient should be monitored for known adverse effects (e.g., acute hypertension, headache, reflex bradycardia, tachycardia, palpitations, and cardiac arrhythmia). In all patients undergoing aspiration with irrigation, especially patients with high cardiovascular risk, blood pressure and ECG monitoring are recommended.

If after a reasonable duration (some suggest 1 hour) and dose escalation of phenylephrine (some suggest 1000 micrograms of diluted phenylephrine over 1 hour) the penis is still tumesced, then a Doppler ultrasound should be considered to evaluate the status of the cavernosal arterial flow in the penis.[2][5] Swelling and oedema after appropriate detumescence with aspiration and irrigation may present like ischaemic priapism, and therefore an ultrasound can rule in or out whether blood has been appropriately evacuated from the corpora cavernosa.

Primary options

phenylephrine injection: (100-500 micrograms/mL) 1 mL intracavernosally every 5 minutes or more, maximum 1000 micrograms

Secondary options

ephedrine: consult specialist for guidance on dose

OR

adrenaline (epinephrine): consult specialist for guidance on dose

OR

noradrenaline (norepinephrine): consult specialist for guidance on dose

OR

metaraminol: consult specialist for guidance on dose

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penile shunt surgery

Surgical shunts for ischaemic priapism should be considered only if intracavernous injection of sympathomimetics has failed.[1][4]

A percutaneous distal corporoglanular shunt is the first choice, as it is simpler and has a lower complication rate than other approaches.[1] In a Winter shunt procedure, a large-bore biopsy needle or biopsy gun is placed percutaneously through the glans penis. If percutaneous distal shunts fail, proceeding with an open distal shunt is the next step. In the Al-Ghorab procedure, a piece of the tunica albuginea may be excised from the tip of the corpus cavernosum. Modifications to Al-Ghorab corporoglanular shunt surgery have been described.[40]

Consideration should be given to corporal tunnelling (e.g., the Burnett snake manoeuvre) in patients with persistent ischaemic priapism following distal corporoglanular shunt.[1] Distal shunts with corporal tunnelling are associated with considerable success in relieving priapism, but may impinge upon post-procedure erectile function to a greater extent than distal shunting alone.[1]

If distal shunting procedures have failed to relieve the priapism, alternative (proximal shunt) approaches using either the Quackels or Sacher shunts (creating a window between the corpus cavernosum and corpus spongiosum) may be considered. However, proximal shunting is considered by some experts to be a historical procedure, having largely been replaced by distal shunts with tunnelling procedures.[1]

As a last resort, venous anastomoses/shunts, such as the Grayhack shunt (creating a window in the corpus cavernosum anastomosing the saphenous vein) or Barry shunt (creating a window in the corpus cavernosum anastomosing the deep dorsal vein), should be performed.

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penile prosthesis

US guidelines state that penile prosthesis placement can be considered for untreated acute ischaemic priapism >36 hours or in those who are refractory to shunting, with or without tunnelling.[1] European guideline relative indications for penile prosthesis in patients with ischaemic priapism include duration >48 hours, failure of medical management, MRI or corporal biopsy with evidence of smooth muscle necrosis, or failure of shunt procedures.[4]

Penile implant placement should occur within 3 weeks after an episode of acute ischaemic priapism, although patients who have undergone distal penile shunts may need to wait longer for proper healing of distal corporal tissue.[4]

Penile implant should depend on the patient’s clinical scenario and the surgeon’s experience level. A malleable penile implant may offer less surgical and post-operative risk.[4]

For men who have presented with recurrent refractory episodes of ischaemic priapism and have undergone surgical management for priapism, whether it was repeated aspiration and irrigation or more invasive repetitive shunt procedures, a penile prosthesis may be an appropriate alternative rather than subjecting the patient to additional future shunt procedures.[41][42][43][44][45]

non-ischaemic priapism

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observation ± conservative treatment

The initial management of non-ischaemic priapism is observation with an option for conservative treatment.[1][4]

US guidelines recommend 4 weeks as a reasonable observation period, unless the patient is experiencing significant discomfort. After the 4-week monitoring period, the fistula should be re-evaluated with colour duplex ultrasonography to assess if it has started to close. If the fistula is unchanged, or if the patient is experiencing ongoing discomfort, intervention may be considered.[1]

Conservative treatment methods include applying ice to the perineum or perineal compression, with or without ultrasound guidance.[4]

Spontaneous resolution is seen in the majority of cases, although erectile dysfunction may occur in some patients.[46]

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Consider – 

counselling and cavernosal artery embolisation

Additional treatment recommended for SOME patients in selected patient group

Invasive interventions can be performed at the patient's request, but the likelihood of spontaneous resolution, risks of treatment-related erectile dysfunction, and the relatively low risk of complications if no active treatment is performed should all be discussed with the patient before any procedure is performed.

Failure of observation or conservative management warrants discussion of treatment with selective arterial embolisation.[4][46]

Both resorbable (i.e., autologous clot, gel foam) and non-resorbable (i.e., microcoils, polyvinyl alcohol [PVA] particles) embolisation materials are available for use and achieve similar results.[1] Some studies suggest that autologous clot is associated with the highest recurrence rate, and that PVA particles provide the best recovery of erectile function; however, data remain inconclusive.[1][4]

If an initial attempt at embolisation fails, patients should be offered a second attempt, ideally with non-resorbable PVA particles.[1]

Studies suggest that embolisation leads to resolution of non-ischaemic priapism in 85% of patients, with 80% retaining functional erections. However, embolisation carries a risk of erectile dysfunction; priapism may recur.[1]

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Consider – 

surgical ligation

Additional treatment recommended for SOME patients in selected patient group

Surgical management of non-ischaemic priapism should be considered if attempts at repeat embolisation have failed. Usually this involves direct surgical ligation of cavernosal sinusoidal fistulae or pseudoaneurysms. Surgery should be performed with intraoperative colour duplex ultrasonography.[1]

ONGOING

recurrent (stuttering) priapism

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treatment of underlying condition

Any underlying medical condition should be appropriately managed.

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Consider – 

preventative therapy

Additional treatment recommended for SOME patients in selected patient group

Efficacy and safety data remain insufficient to recommend optimal preventative strategies with certainty.[1]

Oral baclofen, dutasteride (a 5-alpha-reductase inhibitor), tadalafil or sildenafil (phosphodiesterase-5 inhibitors), ketoconazole with prednisolone, pseudoephedrine, cyproterone (an antiandrogen), and aspirin have all been used with varying degrees of success. Etilefrine, hydroxycarbamide, and automated exchange transfusion may be considered in addition to these therapies in individuals with recurrent priapism and sickle cell disease (SCD).[1]

One meta-analysis of different drug treatments, including sildenafil and ephedrine, found no significant effect attributable to any of the treatments, compared with placebo, in relation to reduction in frequency of stuttering priapism in patients with SCD.[47]

Ketoconazole with prednisolone appears to be the most effective pharmacological intervention for the prevention of recurrent (stuttering) ischaemic priapism.[1] Expert guidance is required; ketoconazole may cause severe liver injury and adrenal insufficiency.[1] Liver and adrenal function should be monitored before and during treatment.

Hormonal agents such as ketoconazole and cyproterone should not be used in patients who have not achieved full sexual maturation and adult stature.

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Consider – 

intracavernosal self-injection of phenylephrine or other sympathomimetic agent

Additional treatment recommended for SOME patients in selected patient group

May be considered in patients refractory to or who reject systemic treatment; however, it is not a preventative strategy.[1]

The patient should be counselled regarding administration and adverse effects of sympathomimetic agents. If an episode of priapism occurs, the patient can perform the injection after being shown how to do so in the clinic.

It is essential that the patient be instructed to seek medical attention if the priapism lasts >3 hours, because prompt medical treatment is necessary.

The drug is injected with a 30-gauge needle at the lateral aspect of the penis near the base, after the skin is cleansed with an alcohol swab. Injection site compression for 7-10 minutes is recommended to prevent haematoma formation. Mild bruising and pain at the injection site are common.

For intracavernous injections in adult patients, phenylephrine should be diluted with normal saline to a concentration of 100-500 micrograms/mL and 1 mL injections administered every 5 minutes or more until detumescence, or for approximately 1 hour, before deciding that the treatment will not be successful.

Primary options

phenylephrine injection: (100-500 micrograms/mL) 1 mL intracavernosally every 5 minutes or more, maximum 1000 micrograms

Secondary options

ephedrine: consult specialist for guidance on dose

OR

adrenaline (epinephrine): consult specialist for guidance on dose

OR

noradrenaline (norepinephrine): consult specialist for guidance on dose

OR

metaraminol: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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