[Priapism associated to zuclopentixol and the choice for an alternative]. / Priapisme bij gebruik van zuclopentixol en de keuze voor een alternatief.

A 29-year-old man developed priapism following the (re)administration of zuclopentixol. In the previous days, a significant amount of alcohol was consumed, presumably in combination with amphetamine and cannabis. Priapism is a rare but serious side effect of various psychoactive medications and recreational drugs, leading to permanent loss of erectile function if not treated in time. In this case the side effect was discovered in a late stage, at which curative treatment was no longer viable. A clear guideline for choosing an alternative antipsychotic agent is currently lacking, but an antipsychotic with low alfa-adrenergic affinity seems preferable. To prevent erectile disfunction following priapism, awareness of its severity is essential, for both doctor and patient.

Oral phosphodiesterase type 5 inhibitors and priapism: A VigiBase analysis.

PURPOSE: To explore the differences of priapism events among a diverse cohort taking erectogenic medicines (i.e., phosphodiesterase type 5 inhibitors [PDE5i] and intracavernousal drugs). METHODS: We queried the World Health Organization global database of individual case safety reports (VigiBase) for records of the adverse drug reactions (ADR) with sildenafil, tadalafil, avanafil, vardenafil, papaverine, and alprostadil. Disproportionality analyses (case/non-case approach) were performed to assess the reporting odds ratio (ROR) of priapism reporting in PDE5i consumers compared to intracavernousal drug recipients. RESULTS: From a total of 133 819 ADR events for erectogenic medications, 632 were priapism (PDE5is: n = 550, 0.41%; intracavernousal drugs: n = 82, 9.92%). Priapism disproportionality signals from intracavernousal drugs were 25 times stronger than PDE5is (ROR = 34.7; confidence interval [CI] 95%: 27.12-43.94 vs. ROR = 1.38; 95% CI: 1.24-1.54). For all PDE5i agents, the 12-17 years age group had the highest ROR (9.49, 95% CI: 3.76-19.93) followed by 2-11 years (4.31, 95% CI: 1.57-9.4). Disproportionality signals for consumers under 18 for both all PDE5is as a whole (ROR = 4.57, 95% CI: 2.48-7.73) and sildenafil (ROR = 4.89, 95% CI: 2.51-8.62) were stronger than individuals 18 or older (ROR = 1.06, 95% CI: 0.93-1.21 and ROR = 1.08, 95% CI: 0.91-1.26, respectively). CONCLUSIONS: PDE5i use shows disproportionate priapism signals which are higher in young patients.

Erectile response profiles of men using PDE5 inhibitors combined with intracavernosal injections as part of a penile rehabilitation program after radical prostatectomy.

BACKGROUND: Phosphodiesterase type 5 (PDE5) inhibitor labeling states that these agents should not be used in conjunction with other erectogenic medications for fear of priapism occurring. AIM: We explored the risk of priapism and prolonged erections in men in our post-radical prostatectomy (RP) penile injection program who were using regular PDE5 inhibitor and intracavernosal injections (ICIs) as part of their rehabilitation program. METHODS: The study cohort included men on penile injection therapy who (1) were taking tadalafil 5 mg daily or taking sildenafil 25 mg on noninjection days, (2) had an RP, (3) were using their respective PDE5 inhibitor regularly at the time of penile injection training, and (4) complied with the program instructions regarding penile injection use. Demographics, comorbidity details, PDE5 inhibitor dose and utilization, and injection dose and utilization data were collected. All patients underwent in-office injection training and used trimix (papaverine/phentolamine/prostaglandin E1) as the intracavernosal medication. OUTCOMES: Priapism was defined as a patient self-reported penetration hardness erection ≥4 hours in duration, while prolonged erection was defined as a penetration hardness erection lasting ≥2 hours. RESULTS: A total of 112 tadalafil users and 364 sildenafil users were compared. Mean age and duration post-RP were 62 ± 14 years and 5.2 ± 12 months, respectively, and there was no difference between tadalafil and sildenafil groups. The mean trimix dose was tadalafil 24 ± 24 units and sildenafil 31 ± 37 units (P < .05). Priapism occurred in 2 (1.7%) of 112 tadalafil users and 5 (1.4%) of 364 sildenafil users (P = .47). Excluding those men experiencing priapism on any occasion, those with any reported penetration hardness erection lasting ≥2 hours were 7 (6.3%) of 112 tadalafil users and 12 (3.3%) of 364 sildenafil users (P < .01). A total of 53% of these prolonged erections occurred within the first 6 injections at home (no difference between tadalafil and sildenafil groups). CLINICAL IMPLICATIONS: We emphasize the need for continued monitoring and education on proper injection techniques to minimize the risk of adverse events in ICI and PDE5 inhibitor combination therapy. STRENGTHS & LIMITATIONS: This study has a relatively large patient population with a considerable follow-up time. Additionally, the rigorous training, education, and monitoring of the participants, as well as the use of formal definitions for priapism and prolonged erections, enhances the accuracy and reliability of the results. However, there are some limitations, such as social desirability, confounding factors, and recall bias. CONCLUSION: There is no significant difference in the incidence of priapism in an ICI program in which men combine ICI with tadalafil or sildenafil. However, tadalafil patients had a higher rate of prolonged erections, which was found to occur mostly early during the titration phase.

Successful Treatment of SCD-Related Priapism With Crizanlizumab: A Case Series.

Priapism, an unwanted, painful, prolonged erection that is unrelated to sexual stimulation, is a common complication of sickle cell disease (SCD). Priapic events in SCD are stuttering, meaning they occur repeatedly with intervening periods of detumescence. Without health care intervention, repeated episodes can lead to erectile dysfunction. There are limited treatment options for SCD-related priapism and no approved targeted therapies. Crizanlizumab is a monoclonal antibody that binds to P-selectin and is used to reduce the frequency of vaso-occlusive crises in patients with SCD. Here, we report the cases of 3 patients with SCD-related priapism who were treated with crizanlizumab. All patients were African American men who experienced numerous priapic episodes that interfered with their daily lives. Upon treatment with crizanlizumab, priapic events were reduced in all 3 patients. These successful cases suggest a potential role for crizanlizumab in the prevention of SCD-related priapism.

[Refractory ischemic priapism due to trazodone]. / L'image du mois. Priapisme ischémique réfractaire sur prise de trazodone.

Priapism is a prolonged erection lasting more than four hours. In most cases, it is a surgical emergency. Trazodone is one of the molecules that can cause priapism. This constitutes a real challenge in the management of these patients, who are often young, to avoid too bad impact on erectile function. We report the case of a 34-year-old man.

Le priapisme est une érection prolongée de plus de quatre heures. Il constitue, dans la majorité des cas, une urgence chirurgicale. La trazodone fait partie des molécules pouvant entraîner un priapisme. Ceci constitue un vrai challenge dans la prise en charge de ces patients, souvent jeunes, pour éviter un impact trop important sur la fonction érectile. Nous rapportons le cas d'un homme de 34 ans.

Probable drug-induced clitoral priapism due to potentiating effects of pregabalin and duloxetine.

PURPOSE: The following case report discusses probable clitoral priapism secondary to duloxetine and pregabalin. While this is a rare adverse effect, it is possible given the mechanism of action and potentiating effects of the combined therapy. This adverse drug reaction was reported to MedWatch and shows that additional research into the physiology of clitoral erection is warranted given the scarcity of information on how drugs influence this reaction. SUMMARY: A 53-year-old African American female with uncontrolled anxiety was started on duloxetine. Pregabalin was added 1 month later due to continued feelings of anxiety. Three weeks later, the patient reported symptoms of clitoral pain, as well as a swollen, tender, and erect clitoris. These adverse effects remained for 4 days, prompting the patient to present to the emergency department where a physical exam was completed with no significant finding except as noted above. Pregabalin was immediately discontinued by the attending physician based on the probability that the swelling was likely drug-induced clitoral priapism. During follow-up, the patient continued to note clitoral erection and pain. The psychiatric pharmacist tapered off duloxetine over 2 weeks with resolution of symptoms. In an examination of the mechanism of action of both drugs, pregabalin can amplify duloxetine's inhibitory effects on voltage-dependent calcium channels. It is likely this mechanism that causes smooth muscle relaxation and led to clitoral priapism. CONCLUSION: This case suggests that pharmacological agents affecting vasoconstriction through serotonergic receptors or calcium-dependent channels can also influence clitoral erection.

Lamotrigine induced priapism in children: case analysis and literature review.

Lamotrigine is an antiepileptic drug that can be used to control many types of seizures as a single-agent or an add-on therapy in patients over 2 years of age. In addition to common adverse reactions, this current case report describes a paediatric male patient with a rare side-effect of persistent penile erectile due to lamotrigine. Previous studies have shown that it can improve sexual function in adult male patients. This patient suffered from refractory epilepsy and pneumonia. He had taken a variety of antiepileptic drugs for a long time and developed priapism after the dosage of lamotrigine had been increased. The priapism improved after drug withdrawal and sedation. Further research is needed to elucidate the mechanism of this rare side-effect.

Buprenorphine/naloxone (Suboxone®) withdrawal may facilitate antipsychotic-induced priapism. A case report.

INTRODUCTION: Priapism is defined as a prolonged penile erection in absence of sexual arousal, leading also to serious sexual and urological problems such as erectile dysfunction and penile fibrosis. Amongst many different etiologies, priapism may be caused by a wide range of antipsychotic medications, mainly due to the α1-adrenergic receptor antagonism. On the other hand, only a couple of cases of opioid compounds have been linked to the onset of priapism, with evidence coming only from methadone and buprenorphine. Here we describe the case of a patient treated with antipsychotics who developed priapism four times following rapid discontinuation of buprenorphine/naloxone (Suboxone®). CASE PRESENTATION: S.C. is a 30-year-old Caucasian man suffering from chronic buprenorphine/naloxone (Suboxone®) abuse, borderline personality disorder, antisocial traits, and multiple suicide attempts. During the acute and the first part of post-acute Suboxone® withdrawal, four episodes of priapism developed while he was treated with clotiapine, clozapine, and chlorpromazine. However, after the last episode of priapism, despite he was either on haloperidol or zuclopenthixol and chlorpromazine, no other urological event occurred during the following 6 months of observation. CONCLUSIONS: As opioids may have dampened the patient's sexual function due to chronic consumption, a rapid drug suspension coupled with an antipsychotic therapy might have created the conditions to facilitate the occurrence of close clustered priapism events.

Safety and Efficacy of Phenylephrine Administration for the Treatment of Ischemic Priapism: An Opportunity for Quality Improvement in Periprocedural Safety Assessment.

OBJECTIVE: To determine the safety and efficacy of hourly, high dose phenylephrine (>1000 µg) for acute ischemic priapism (AIP) through monitoring adverse hemodynamic events amongst risk profiles. METHODS: An IRB-approved retrospective review of patients with AIP from 2010 to 2020. Patients were stratified to a low or high dose phenylephrine group based on cumulative, hourly dose of ≤1000 µg and > 1000 µg respectively and examined for successful resolution of their AIP. The safety profile of phenylephrine for patients at risk for adverse hemodynamic events was examined. RESULTS: A total of 123 patients were identified with a median age of 40 (range: 7-76) years with median time from AIP onset to presentation of 11 (2-168) hours. A total of 97 men received phenylephrine (78.9%) and detumescence was achieved nonoperatively in 62 of these men (63.9%) with a mean priapism duration of 8.7 hours. Those resolving with phenylephrine administration had a mean duration of 8.8 ± 5.6 vs 57.3 ± 37.1 hours without resolution P < .001. Among low and high dose phenylephrine groups (500 and 2000 µg respectively), the median duration of AIP was 10 and 12 hours respectively without a difference in AIP resolution (P > .05). Twenty-one patients (17.1%) were deemed at risk for phenylephrine complication of which 4 (4.1%) had phenylephrine discontinued due to hemodynamic changes. CONCLUSION: Nonoperative resolution of AIP with phenylephrine does not appear to be dose-dependent and hemodynamic changes secondary to phenylephrine administration may be underreported. Future work should utilize standardized risk assessment and periprocedural monitoring for hemodynamic change.

Priapism – A rare side effect of alpha blockers: Report of 2 cases and literature review / Priapismo – Un efecto secundario raro de los alfabloqueantes: informe de dos casos y revisión de la literatura

Priapism is a prolonged unintended erectile state unrelated to sexual stimulation or sexual desire. There is a very rare relationship between the use of alpha blockers and the development of priapism. Here, we describe 2 cases of alpha blocker induced priapism and a literature review. One of these cases is related to the use of silodosin and the other is related to the use of tamsulosin. So far, 18 alpha blocker induced priapism cases have been reported. We are presenting the first case of silodosin induced priapism and the eighth case of priapism secondary to tamsulosin. Despite silodosin having a much greater affinity for the α1-a receptor than the α1-b receptor, as represented in this case it can cause this rare side effect. Before starting alpha blocker treatment, side effects such as priapism, which may be very rare but may cause serious problems, should be kept in mind. (AU)

El priapismo es un estado eréctil prolongado no intencionado y no relacionado con la estimulación o el deseo sexual. Existe una relación muy infrecuente entre el uso de alfabloqueantes y el desarrollo de priapismo. Describimos aquí dos casos de priapismo inducido por alfabloqueantes y una revisión de la literatura. Uno de estos casos guarda relación con el uso de silodosina, y el otro con el uso de tamsulosina. Hasta el momento se han reportado 18 casos de priapismo inducido por alfabloquantes. Presentamos aquí el primer caso de priapismo inducido por silodosina y el octavo caso de priapismo secundario a tamsulosina. A pesar de que silodosina tiene mucha mayor afinidad por el receptor α1-a que el receptor α1-b, según lo representado en este caso, puede causar este efecto secundario raro. Antes de iniciarse tratamiento con alfabloquantes deben tenerse en cuenta los efectos secundarios, tales como priapismo, que pueden ser muy raros pero pueden causar problemas graves. (AU)

Resveratrol-nitric oxide donor hybrid effect on priapism in sickle cell and nitric oxide-deficient mouse.

BACKGROUND: Children and adult with sickle cell disease (SCD) display priapism associated with low nitric oxide (NO) bioavailability and oxidative stress in penis. AIM: This study aimed to evaluate the effects of hybrid compound RVT-FxMe, derived from resveratrol bearing a NO-donor subunit, on two murine model that display priapism phenotype, SCD transgenic mice and endothelial NO synthase gene-deficient (eNOS-/-) mice. METHODS: Wild-type, SCD, and eNOS-/- mice were treated with RVT-FxMe (25 mg/kg/d, 2 weeks). OUTCOMES: Hematological parameters, concentration-response curves to acetylcholine (ACh) and sodium nitroprusside (SNP), as well as to electrical field stimulation (EFS), were obtained in mice corpus cavernosum strips. RESULTS: Corpus cavernosum relaxations to SNP and EFS were increased in eNOS-/- group, which were normalized by RVT-FxMe treatment. SCD mice exhibited an excessive CC relaxant response induced by ACh, EFS and SNP RVT-FxMe treatment did not change the increased relaxant responses to ACh, EFS and SNP in corpus cavernosum from SCD group. CLINICAL TRANSLATION: Excess of plasma hemoglobin in SCD may interfere in pharmacological activity of NO donors compounds. STRENGTH/LIMITATIONS: While mechanistic data with promising potential is showed, the current study is not without limitations. RVT-FxMe effects in the mid- and long-term warrant complementary studies. CONCLUSION: Treatment with RVT-FxMe reversed the enhanced NO-cGMP-mediated CC relaxations in eNOS-/- mice, but not in SCD mice; it is likely that excess of plasma hemoglobin in SCD mice act to inactivate NO before it reaches soluble guanylyl cyclase, avoiding restoration of NO bioavailability in penis.

Acute Management of Prolonged, Recalcitrant Priapism in a Pediatric Patient.

Although rare in pediatrics, ischemic priapism carries significant risk of erectile dysfunction if not treated promptly. We report a case of idiopathic ischemic priapism in a male aged 14 years, refractory to traditional therapies, including aspiration/irrigation, phenylephrine injection, and distal shunt. However, the priapism resolved after intracavernosal injections of tissue plasminogen activator (tPA) with preservation of normal erectile function. To our knowledge, this is the first reported case of intracavernosal tPA for treatment of pediatric priapism. tPA may be a useful management option for recalcitrant ischemic priapism in pediatric patients and may prevent devastating outcomes such as lifelong erectile dysfunction.

A case of priapism following intrathecal morphine injection in a dog.

Background: Priapism refers to prolonged erection unrelated to sexual stimulation, with severe sequelae unless treated. In humans, it is a rare complication associated with epidural or spinal opioid administration. Its pathophysiology is unclear. This is the first report of priapism following neuraxial anesthesia in dog. Case Description: An intrathecal morphine injection (30 mcg/kg) at L5-L6 for postoperative analgesia was given at the end of surgery for removal of cutaneous mastocytomas of the abdomen and left axillary lymphadenectomy. Painless penile erection occurred 2 hours later and lasted 6 hours, before spontaneously resolving 7-8 hours after the injection. No pain or other adverse events (e.g., nausea, urinary retention, and itching) were recorded. Recovery was complete without treatment. Conclusion: Painless, self-resolving priapism is a rare complication associated with intrathecal morphine injection in dogs.

Recurrent Priapism From Cabergoline and Bromocriptine in a Hypogonadal Man With Prolactinoma.

Dopamine agonists are generally well tolerated and represent the first-line therapy for prolactinomas. We report a case of a 20-year-old man with a macroprolactinoma who developed recurrent priapism with cabergoline and bromocriptine. Transsphenoidal pituitary adenoma resection was done with normalization of the prolactin level. Priapism is a rare side effect of dopamine agonists that warrants discontinuation of therapy. Patients should be educated about this potential side effect at the time of prescribing the medication.

Medical treatment of recurrent ischaemic priapism: a review of current molecular therapeutics and a new clinical management paradigm.

OBJECTIVES: To examine the current molecular therapeutics in the medical treatment of recurrent ischemic priapism (RIP). To propose a stepwise clinical management paradigm for the treatment of RIP. METHODS: We performed a literature search using the PubMed database for the terms 'recurrent ischemic priapism' and 'stuttering priapism' up until December 2020. We assessed pre-clinical and clinical studies regarding medical management of RIP and molecular pathophysiology. Case series and randomized trials were evaluated by study quality and patient outcomes to determine a potential clinical management scheme. RESULTS: Recent research has fostered an improved understanding of the underlying molecular pathophysiology of RIP that has paved the way forward for developing new therapeutic agents. Medications targeting neurovascular, hormonal and haematological mechanisms associated with RIP show great promise towards remedying this condition. A host of therapeutic agents operating across different mechanistic directions may be implemented according to a clinical management scheme to potentially optimize RIP outcomes. CONCLUSION: RIP remains a medically neglected condition with current management focused on treating the acute condition rather than modulating the course of disease. Continued research into the molecular mechanisms of RIP and standardized clinical pathways can improve the quality of care for patients suffering from this condition.

Using cyproterone acetate to treat recurrent ischemic priapism in a patient with sickle cell anemia as a comorbidity: a case report.

INTRODUCTION: The management of recurrent ischemic priapism is unclear in contemporary practice. Yet, if left untreated, the condition may evolve into an acute ischemic priapism and in some cases result in erectile dysfunction. This report documents the results of successful management of recurrent ischemic priapism using cyproterone acetate in a 30-year-old Saudi man with sickle cell anemia as a comorbidity. CASE PRESENTATION: A 30-year-old Saudi man denoted visited the emergency room with a painful erection which had lasted for more than four hours. The patient has sickle cell anemia and a family history of sickle cell disease. He is married and has two children. His first priapism case occurred when he was 7 years old. At the age of 15, the condition recurred, and the patient's doctor prescribed cyproterone acetate 50 mg twice daily for 5 days. The doctor had told him that whenever he was experiencing priapism, he should adhere to this regimen for 5 days. The doctor could not find any guidelines for the prescription of cyproterone acetate. CONCLUSION: Priapism cases represent a significant challenge in therapeutic management because of the elevated risk of structural damage to the penis. The fact that there lacks a clinically approved standard approach to managing the condition make it difficult for physicians to effectively manage the condition. Management of the condition is further complicated by existence of comorbidities such as sickle cell anemia. This patient's case demonstrates that cyproterone acetate prescription is a great preventative strategy that limits priapism recurrences.

Clinical Outcomes of Periprocedural Antithrombotic Therapy in Ischemic Priapism Management.

BACKGROUND: Priapism is a urologic emergency consisting of a painful erection lasting greater than 4 hours; antithrombotic therapy (ATT) have recently been recommended as an adjunct in the treatment of ischemic priapism. AIM: To determine the short- and long-term outcomes of periprocedural ATT in the management of acute ischemic priapism. METHODS: A retrospective review of patients seen at the University of California, San Francisco, from 2008 to 2019 was carried out to identify those evaluated for acute priapism. Information regarding duration of priapism, etiology, treatment, periprocedural and postprocedural ATT type and dose, and follow-up data was collected. OUTCOMES: ATT use was the exposure of interest; outcome variables included priapism resolution, repeat episodes, long-term complications, and follow-up. RESULTS: 70 patients with at least 1 detailed record of an acute priapism episode between 2008 and 2019 were identified. Of the 70 patients who underwent management for an acute episode of priapism, 59 (84%) received intracavernous injection of phenylephrine with or without corporal aspiration. Of the 4 patients who received ATT at the same time as intracavernous injection, none had additional priapism episodes. In the 55 patients who did not receive immediate ATT, 22 (40%) required at least 1 shunting procedure. The 9 patients who received ATT concurrently with shunting experienced less recurrence than the 13 patients who did not receive ATT (11% vs 69%, respectively P = .012). There were no significant differences in long-term erectile dysfunction (P = .627), fibrosis (P = .118), genitourinary pain (P = .474), and urinary issues (P = .158) between those who received ATT and those who did not. CLINICAL IMPLICATIONS: Our findings suggest that ATT has a role in preventing priapism recurrence; we observed that long-term repeat priapism episodes are less frequent in those who received periprocedural ATT compared with those who did not and that ATT may especially reduce recurrence in cases when shunting was required STRENGTHS & LIMITATIONS: This is the first study looking at the clinical outcomes of periprocedural ATT in the management of ischemic priapism. It is limited by the fact that it is a single-center study, types of ATT were heterogenous, and the exact timing of priapism management could not be measured for everyone. CONCLUSION: In spite of its limitations, these preliminary findings are promising and warrant further exploration of the use of ATT in the management of ischemic priapism. Ramstein JJ, Lee A, Cohen AJ, et al. Clinical Outcomes of Periprocedural Antithrombotic Therapy in Ischemic Priapism Management. J Sex Med 2020;17:2260-2266.

Treatments for priapism in boys and men with sickle cell disease.

BACKGROUND: Sickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally 'sickle-shaped' red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however, there is no consistency in treatment. We therefore need to know the best way of treating this complication in order to offer an effective interventional approach to all affected individuals. This is an update of a previously published review. OBJECTIVES: To assess the benefits and risks of different treatments for stuttering (repeated short episodes) and fulminant (lasting for six hours or more) priapism in sickle cell disease. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched trial registries. Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 09 September 2019. Date of most recent search of trial registries and of Embase: 01 October 2019. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing non-surgical or surgical treatment with placebo or no treatment, or with another intervention for stuttering or fulminant priapism. DATA COLLECTION AND ANALYSIS: The authors independently extracted data and assessed the risk of bias of the trials. MAIN RESULTS: Three trials with 102 participants were identified and met the criteria for inclusion in this review. These trials compared stilboestrol to placebo, sildenafil to placebo and a four-arm trial which compared ephedrine or etilefrine to placebo and ranged in duration from two weeks to six months. All of the trials were conducted in an outpatient setting in Jamaica, Nigeria and the UK. None of the trials measured our first primary outcome, detumescence. However, all three trials reported on the reduction in frequency of stuttering priapism, our second primary outcome; and from the evidence included in this review, we are uncertain whether stilboestrol, etilefrine or ephedrine reduce the frequency of stuttering priapism as the certainty of the evidence has been assessed as very low. Additionally, we conclude that sildenafil may make little or no difference (low-certainty evidence). Two trials reported on immediate side effects and we are uncertain whether etilefrine or ephedrine reduce the occurrence of these (very low-certainty of evidence) and also conclude that sildenafil may make little or no difference in side effects (low-quality evidence). Given that all of the trials were at risk of bias and all had low participant numbers, we considered the certainty of the evidence to be low to very low. AUTHORS' CONCLUSIONS: There is a lack of evidence for the benefits or risks of the different treatments for both stuttering and fulminant priapism in sickle cell disease. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions for priapism in sickle cell disease.