Approach

The most common complication of priapism is complete erectile dysfunction (ED), which has been reported to occur in >50% of patients with priapism lasting 24-48 hours.[36] Therefore, the most critical factor in maintaining erectile function is immediate treatment of men presenting with priapism and prevention of future episodes. The primary goals of medical therapy for ischaemic priapism are to relieve the pain and decompress the corporal bodies, thus reducing ischaemia and the risk of tissue necrosis or injury.[1]

Management depends on differentiating between ischaemic, non-ischaemic, and stuttering forms of priapism. While ischaemic priapism lasting for >4 hours is a medical emergency and demands immediate treatment, episodes of ischaemic or stuttering priapism lasting for <4 hours should not be ignored. Delays in treatment and repetitive episodes of stuttering priapism lead to cellular, molecular, and morphological changes in the corpus cavernosum, which over time result in tissue injury that places the patient at risk for the development of ED.[21] Prompt treatment of all ischaemic priapism episodes should be the goal.

Ischaemic priapism

For ischaemic 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 ED. Therefore, prompt treatment of all episodes of ischaemic or stuttering priapism are encouraged.

Ischaemic priapism lasting for >4 hours is an emergency and requires immediate treatment.[1] Management of ischaemic priapism is approached in a step-wise fashion to achieve prompt resolution.[4]

Medical management

  • Definitive first-line treatment consists of evacuation/aspiration of blood and irrigation of the corpora cavernosa along with intracavernous injection of an alpha-adrenergic sympathomimetic agent.[1] For anaesthetic purposes, a preceding dorsal nerve block or local penile shaft block should be given.[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] Concurrent irrigation with normal saline or a sympathomimetic agent to flush out blood from the cavernosum may be carried out.[1]

  • 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.

  • Repeated aspirations or irrigations and sympathomimetic injections over several hours may be necessary and should be performed before initiating surgical intervention.[34]

  • Many clinicians elect to perform intracavernosal injections without previous aspiration.

  • 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, such as ephedrine, adrenaline, noradrenaline, or metaraminol.

  • 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, BP 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.

  • For priapism specifically related to sickle cell disease, medical therapies such as intravenous hydration, oxygenation, alkalinisation, and exchange transfusion may be performed. However, these interventions should not precede the first-line treatment for all episodes of ischaemic priapism: aspiration/irrigation in combination with an intracavernous alpha-agonist injection.[1][4][37][38]

  • 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.

Surgical shunt procedures

  • Ischaemic or recurrent priapism refractory to medical therapy may require surgical intervention. Surgical shunts for ischaemic priapism should be considered only if intracavernous injection of sympathomimetics has failed.[1][4]

  • The goal of surgery is to create a channel or fistula that allows the deoxygenated blood to drain from the corpora cavernosa.[1] There are four sub-divisions of shunts: percutaneous distal shunts, open distal shunts, open proximal shunts, and vein anastomoses/shunts.[39] Distal shunts should be performed first with proximal shunts potentially following if a distal shunt fails.[4]

  • A percutaneous distal corporoglanular shunt is the first choice, as it is simpler to perform and has lower complication rates 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 the previously mentioned distal shunts are unsuccessful, an open distal shunt is the next step. The Al-Ghorab shunt involves excising a piece of the tunica albuginea 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]

  • When distal shunts fail, an open proximal shunt is the next line of treatment. However, proximal shunting is considered by some experts to be a historical procedure, having largely been replaced by distal shunts with tunnelling procedures.[1] Proximal shunts such as the Quackels or Sacher shunt (creating a window between the corpus cavernosum and corpus spongiosum) may be considered.

  • Vein anastomoses/shunts should be performed as a last resort. The Grayhack shunt creates a window in the corpus cavernosum anastomosing the saphenous vein, whereas the Barry shunt creates a window in the corpus cavernosum anastomosing the deep dorsal vein.

  • For all shunt procedures, the patient should receive peri-operative and post-operative antibiotics.[21][Figure caption and citation for the preceding image starts]: The technique of penile blood aspiration (corpora cavernosal needle placement for maximal corporal body irrigation)Arthur L. Burnett, MD, FACS [Citation ends].com.bmj.content.model.Caption@677e8f72[Figure caption and citation for the preceding image starts]: Types of surgical shunt procedures for the treatment of ischaemic priapismHelen R. Levey, DO, MPH [Citation ends].com.bmj.content.model.Caption@2e20b3f4

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 tunneling.[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

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

Conservative approaches

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 of some form may occur in some patients.[46]

Embolisation

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.[1][4] 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]

Surgery

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

Recurrent (stuttering) priapism

Treatment should focus on preventing future episodes; management of individual episodes should follow that for ischaemic priapism.

Preventative strategies

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.

Home self-injection

Intracavernosal self-injection of phenylephrine or other sympathomimetic (e.g., ephedrine, epinephrine [adrenaline], norepinephrine [noradrenaline], or metaraminol) 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 the administration and adverse effects of sympathomimetic agents. If an episode of priapism occurs, the patient can perform the injection at home after being shown how to do so in the clinic. However, it is essential that he be instructed to seek medical attention if the priapism lasts >3 hours, because prompt medical treatment is necessary.

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