[Priapism]. / Priapismus.

Priapism is defined as penile erection lasting more than four hours that is unrelated to sexual arousal. Priapism is classified based on the oxygenation of the penile tissue into ischemic and non-ischemic subtypes. As the most common form, ischemic priapism is usually associated with pain and carries a significant risk of permanent loss of erectile function; thus, rapid intervention is necessary. Initial therapy consists of corporal aspiration and injection of sympathomimetic agents. If detumescence is not achieved, a cavernosal shunt is necessary. Non-ischemic priapism is less common than the ischemic type and is usually the result of perineal trauma. In this subtype, there is usually no pain and treatment is initially conservative. Recurrent (stuttering) priapism is a variant of the ischemic subtype, but is self-limiting and usually occurs during sleep with a duration of less than three to four hours. In the case of prolonged erection, therapy is analogous to that of the ischemic subtype.

Management of Priapism: 2021 Update.

Priapism is defined as a persistent penile erection lasting more than 4 hours. Priapism is a rare condition but when present it requires prompt evaluation and definitive diagnosis. Priapism has 2 pathophysiologic subtypes: ischemic and nonischemic. Ischemic priapism accounts for a majority of cases reported. Ischemic priapism is a urologic emergency and requires intervention to alleviate pain and prevent irreversible damage to erectile tissues. This article highlights current guidelines and the contemporary literature on priapism.

Acute Ischemic Priapism: An AUA/SMSNA Guideline.

PURPOSE: Priapism is a persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation and results in a prolonged and uncontrolled erection. Given its time-dependent and progressive nature, priapism is a situation that both urologists and emergency medicine practitioners must be familiar with and comfortable managing. Acute ischemic priapism, characterized by little or no cavernous blood flow and abnormal cavernous blood gases (ie, hypoxic, hypercarbic, acidotic) represents a medical emergency and may lead to cavernosal fibrosis and subsequent erectile dysfunction. MATERIALS AND METHODS: A comprehensive search of the literature was performed by Emergency Care Research Institute for articles published between January 1, 1960 and May 1, 2020. Searches identified 2948 potentially relevant articles, and 2516 of these were excluded at the title or abstract level for not meeting inclusion criteria for any key question. Full texts for the remaining 432 articles were reviewed, and ultimately 137 unique articles were included in the report. RESULTS: This Guideline was developed to inform clinicians on the proper diagnosis and surgical and non-surgical treatment of patients with acute ischemic priapism. This Guideline addresses the role of imaging, adjunctive laboratory testing, early involvement of urologists when presenting to the emergency room, discussion of conservative therapies, enhanced data for patient counseling on risks of erectile dysfunction and surgical complications, specific recommendations on intracavernosal phenylephrine with or without irrigation, the inclusion of novel surgical techniques (eg, tunneling), and early penile prosthesis placement. CONCLUSIONS: All patients with priapism should be evaluated emergently to identify the sub-type of priapism (acute ischemic versus non-ischemic) and those with an acute ischemic event should be provided early intervention. Treatment of the acute ischemic patient must be based on patient objectives, available resources, and clinician experience. As such, a single pathway for managing the condition is oversimplified and no longer appropriate. Using a diversified approach, some men may be treated with intracavernosal injections of phenylephrine alone, others with aspiration/irrigation or distal shunting, and some may undergo non-emergent placement of a penile prosthesis.

Veno-Occlusive Priapism in COVID-19 Disease.

Infection by COVID-19, being a respiratory disease caused by SARS-CoV-2, can predispose to arterial and venous thrombotic disease, in response to excessive inflammation, platelet activation, endothelial dysfunction, and venous stasis. During the COVID-19 pandemic period, the technological and resource availability for the care of these patients with thrombotic disease is critical, marking a factor of morbidity and poor prognosis in these cases. We describe a case of priapism in a patient with COVID-19, during the course of systemic inflammatory response syndrome and respiratory distress syndrome with a procoagulant state, seeking to relate the pathophysiological factors of ischemic priapism in patients with infection with SARS-Cov-2.

Priapismo: abordaje clínico y manejo de urgencia / Priapism: clinical approach and emergency management

Priapism is a persistent erection, often painful, lasting more than 4 hours and unrelated to sexual stimulation. Based on clinical and pathophysiological features, priapism can be classified as ischemic, nonischemic and stuttering. Ischemic priapism is the most frequent form and represents a urological emergency. Although it is described as a low frequency entity, its timely diagnosis and immediate intervention are essential in the reestablishment of cavernous blood flow and in the prevention of necrosis and permanent erectile dysfunction. Intracavernous blood aspiration and injection of a-adrenergic agents correspond to the first-line options for the management of cases of ischemic priapism. Surgical shunts continue to be the most widely used surgical option for the management of prolonged ischemic priapism refractory to non-invasive management, with emerging evidence supporting the early implantation of a penile prosthesis. The objective of this review article is to describe the keys to the clinical approach and acute management of priapism in the emergency department. (AU)

Superselective embolization for high-flow priapism refractory to medical and surgical treatments.

BACKGROUND: This study aimed to report long-term outcome of superselective embolization in patients with high-flow priapism refractory to medical and surgical treatments. METHODS: From August 2011 until July 2016, 14 patients with high-flow priapism refractory to local treatments were treated and their charts were retrospective reviewed. Clinical evaluation, color Doppler ultrasonography, arteriography and selective embolization were performed. Follow up was performed in all patients. Fourteen men (18-63 years old) were enrolled, with priapism duration of 14 h to 28 days. Internal pudendal arteries or glutaea inferior arteriae were successfully embolized with gelatin sponge particles, polyvinyl alcohol particles or microcoils. RESULTS: Pseudoaneurysm in right femoral artery was found in one case after intervention. The follow-up 1 week later showed that 13 patients were in good condition, the priapism diminished 1-7 days (mean 3.2 ± 0.5 days) after intervention, and 1 patient received second intervention. Mean follow-up was (range 10.8-69.6) months. One patient had recurrent priapism months after embolization and had his penis surgically removed for severe necrosis. CONCLUSIONS: Superselective embolization is safe and effective in high-flow priapism refractory local treatments, with a good long-term prognosis.

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function.

OBJECTIVE: To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. METHODS: This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. RESULTS: Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. CONCLUSION: Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.

Mechanisms underlying priapism in sickle cell disease: targeting and key innovations on the preclinical landscape.

Introduction: Priapism is prolonged penile erection in the absence of sexual arousal or desire and is a devastating condition affecting millions of patients with sickle cell disease (SCD) globally. Available drug treatments for SCD-related priapism remain limited and have been primarily reactive rather than preventive. Hence, there is an unmet need for new drug targets and pharmacologic therapies.Areas covered: We examine the molecular mechanisms underlying SCD-associated priapism evaluated mostly in animal models. In mouse models of SCD, molecular defects of priapism operating at the cavernous tissue level include reduced tonic NO/cGMP signaling, elevated oxidative/nitrosative stress, vascular adhesion molecule derangements, excessive adenosine and opiorphin signaling, dysregulated vasoconstrictive RhoA/ROCK signaling, and testosterone deficiency. We discuss the consequences of downregulated cGMP-dependent phosphodiesterase type 5 (PDE5) activity in response to these molecular signaling derangements, as the main effector mechanism causing unrestrained cavernous tissue relaxation that results in priapism.Expert opinion: Basic science studies are crucial for understanding the underlying pathophysiology of SCD-associated priapism. Understanding the molecular mechanisms could unearth new therapeutic targets for this condition based on these mechanisms. Treatment options should aim to improve deranged erection physiology regulatory signaling to prevent priapism and potentially restore or preserve erectile function.

Rare disorders of penile erection.

This literature review presents two unusual and mystifying disorders of penile erection: painful nocturnal erections, alternatively termed sleep-related painful erections, and idiopathic stuttering priapism, a variant of recurrent ischemic priapism in which no cause is discernible. The disorders are closely related although they are distinct clinically and pathologically. The main subject areas of discussion are recognition, clinical evaluation and management although current concepts surrounding their causes and mechanisms are also addressed. It is acknowledged that despite the perceived rarities of these disorders they are impactful in terms of their disease profiles and consequences. Future advances in their management will require continued development of evidence-based treatments.

The management of stuttering priapism.

INTRODUCTION: Stuttering priapism is a variation of ischemic priapism, generally transient and self-limiting, occurring during sleep and lasting less than 3-4 hours. It may progress to episodes of complete ischemic priapism in approximately one third of cases, necessitating emergent intervention. EVIDENCE ACQUISITION: This review aims to provide an up-to-date picture of the pathophysiology and management of stuttering priapism. A search using Medline and EMBASE for relevant publications using the terms "priapism", "stuttering", "diagnosis", "treatment", "fibrosis", was performed. EVIDENCE SYNTHESIS: Stuttering priapism shares its etiologies with ischemic priapism and a large number of diseases or clinical situations have risk association for developing the disorder. The most common causes are sickle cell disease or other hematologic and coagulative dyscrasias especially in children. In the adult population, idiopathic priapism occurring without any discernible cause is considered to be the most common form in adults. The medical management of priapism represents a therapeutic challenge to urologists. Unfortunately, although numerous medical treatment options have been reported, the majority are through small trials or anecdotal reports. Understanding the underlying pathophysiology and understanding the current and emerging future agents and therapeutic options are mandatory in order to provide the best solution for each patient. CONCLUSIONS: The goal of management of priapism is to achieve detumescence of the persistent erection in order to preserve erectile function. To achieve successful management, urologists should address this emergency clinical condition. In the present article, we review the diagnosis and clinical management of the three types of priapism.

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function / Prevalência de priapismo em indivíduos com doença falciforme e suas implicações na função sexual masculina

ABSTRACT Objective To evaluate epidemiological aspects of priapism in patients with sickle cell disease, and these aspects impact on adult sexual function. Methods This was a cross-sectional study including individuals with sickle cell disease who were evaluated at a reference center for sickle cell. Participants completed a structured questionnaire about their sociodemographic characteristics and priapism events. Sexual function was assessed using validated two instruments, the Erection Hardness Score and one about the sex life satisfaction. Results Sixty-four individuals with median aged of 12 (7 to 28) years were interviewed. The prevalence of priapism was 35.9% (23/64). The earliest priapism episode occurred at 2 years of age and the latest at 42 years. The statistical projection was that 71.1% of individuals of the study would have at least one episode of priapism throughout life. Patients with episodes of priapism (10/23) had significantly worse erectile function Erection Hardness Score of 2 [1-3]; p=0.01 and were less satisfied with sexual life 3 [3-5]; p=0.02. Conclusion Priapism is usually present in childhood, and severe episodes are associated with cavernous damage, impairment in the quality of the erection, and lower sexual satisfaction.

RESUMO Objetivo Avaliar aspectos epidemiológicos do priapismo em pacientes com doença falciforme e o impacto desses aspectos na função sexual de adultos. Métodos Trata-se de estudo transversal, que incluiu indivíduos com doença falciforme acompanhados em um centro de referência. Os participantes responderam a um questionário estruturado acerca das características sociodemográficas e eventos de priapismo. A função sexual foi avaliada por meio de dois instrumentos validados, a Escala de Rigidez de Ereção e um sobre satisfação com a vida sexual. Resultados Foram entrevistados 64 indivíduos com média de idade de 12 (7-28) anos. A prevalência de priapismo foi de 35,9% (23/64). O episódio mais precoce ocorreu aos 2 anos de idade e o mais tardio, aos 42 anos. A projeção estatística foi de que 71,1% desses sujeitos teriam pelo menos um episódio de priapismo ao longo da vida. Pacientes adultos com episódios de priapismo (10/23) apresentaram função erétil significativamente pior Escala de Rigidez de Ereção de 2 [1-3]; p=0,01 e estavam menos satisfeitos com a vida sexual 3 [3-5]; p=0,02. Conclusão O priapismo manifesta-se desde a infância, e episódios graves estão associados a dano cavernoso, prejuízo na qualidade da ereção e menor satisfação sexual.

Isolated intermittent neurogenic priapism: an unusual presentation in degenerative lumbar spinal stenosis.

Priapism is a relatively uncommon clinical presentation. The well-recognised causes are sickle cell anaemia and the use of medications, such as vasoactive erectile agents. Infrequently, it could be the result of lumbar spinal stenosis. The authors reported an elderly man with 1-year history of isolated intermittent priapism aggravated by walking. MRI showed lumbosacral spondylosis with severe stenosis at the level of L4-L5. Total laminectomy of L4 and L5 was done, resulting in a rapid and complete resolution of symptom without recurrence during the follow-up time of 10 years.

Intractable acute ischaemic priapism occurring secondary to newly commenced olanzapine.

We present a case of intractable acute ischaemic priapism occurring secondary to newly commenced olanzapine. It demonstrates rapid intervention in a stepwise approach aiming to restore penile flaccidity in order to prevent chronic damage to the corpora cavernosa. After an unsuccessful conservative approach, our patient underwent two formal distal penile shunt procedures with no effective penile detumescence. Subsequently, bilateral proximal penile shunts were performed comprising a right corpus cavernosum to corpus spongiosum anastomosis and a left saphenous vein to left corpus cavernosum anastomosis. The patient remained an inpatient for observation, and detumescence was gradually achieved over several days after this procedure. However, follow-up revealed erectile dysfunction, and it was explained to the patient that he was unlikely to achieve further erections and that a penile implant was the only realistic option.

A Systematic Review on Ischemic Priapism and Immediate Implantation: Do We Need More Data?

INTRODUCTION: Ischemic priapism (IP) is the most common form of priapism. In cases of priapism persisting for >36 hours, conservative management usually fails, and the patients' erectile tissue will be inevitably compromised, resulting in corporal fibrosis, shortening of the penis, and refractory erectile dysfunction. In this subset of patients, early implantation of a penile prosthesis (PP) could be a solution for both the priapic episode and the erectile dysfunction. AIM: To analyze the current literature with regard to the correlation between refractory ischemic priapism and immediate implantation of PP. METHODS: An extensive literature research was conducted to retrieve studies focusing on immediate PP implantation in patients suffering from refractory ischemic priapism (RIP). MAIN OUTCOME MEASURES: We evaluated the functional outcomes of patients who have undergone the immediate insertion of a PP as treatment for an acute episode of IP refractory to medical therapy or shunt surgery. RESULTS: Nine studies were included in this systematic review, including 3 case reports and 6 retrospective analyses. All studies agreed that in patients with RIP, early insertion of a PP is a safe and effective procedure, and all studies but 1 preferred malleable implants to inflatable implants. CONCLUSION: The systematic review does not demonstrate superiority of immediatePP implantation over delayed PP implantation, because none of the studies was designed with this purpose. However, considering the reduced complication rate and the ease of the procedure, all studies are in favor of early implantation over delayed implantation. Capece M, La Rocca R, Mirone V, et al. A Systematic Review on Ischemic Priapism and Immediate Implantation: Do We Need More Data? Sex Med Rev 2018;7:530-534.

Ischemic priapism as a model of exhausted metabolism.

In vivo metabolic studies typically concern complex open systems. However, a closed system allows better assessment of the metabolic limits. Ischemic priapism (IP) constitutes a special model of the compartment syndrome that allows direct sampling from a relatively large blood compartment formed by the corpora cavernosa (CC). The purpose of our study was to measure metabolic changes and the accumulation of end products within the CC during IP. Blood gas and biochemical analyses of aspirates of the CC were analyzed over an 8-year period. Mean ± SD pH, pCO2 , pO2 , O2 -saturation, lactate, and glucose of the aspirated blood were determined with a point-of-care analyzer. Forty-seven initial samples from 21 patients had a pH of 6.91 ± 0.16, pCO2 of 15.3 ± 4.4 kPa, pO2 of 2.4 ± 2.0 kPa, and an O2 -saturation of 19 ± 24% indicating severe hypoxia with severe combined respiratory and metabolic acidosis. Glucose and lactate levels were 1.1 ± 1.5 and 14.6 ± 4.8 mmol/L, respectively. pH and pCO2 were inversely correlated (R2  = 0.86; P < 0.001), glucose and O2 -saturation were positively correlated (R2  = 0.83; P < 0.001), and glucose and lactate were inversely correlated (R2  = 0.72; P < 0.001). The positive correlation of CO2 and lactate (R2  = 0.69; P < 0.001) was similar to that observed in vitro, when blood was titrated with lactic acid. The observed combined acidosis underscores that IP behaves as a closed system where severe hypoxia and glycopenia coexist, indicating that virtually all energy reserves have been consumed.

Appropriate management of high-flow priapism based on color Doppler ultrasonography findings in pediatric patients: four case reports and a review of the literature.

INTRODUCTION: High-flow priapism in children is a very rare condition, and there is no clear consensus on its management. High-flow priapism is associated with increased cavernosal blood flow and broadly divided into two groups based on the presence or absence of arteriocavernous fistula in the corpora cavernosa. OBJECTIVE: This study aimed to determine the appropriate management of high-flow priapism based on the existence of arteriocavernous fistula using penile color Doppler ultrasonography (CDU) findings in the pediatric population. STUDY DESIGN: The cases of four boys aged between 6 and 11 years with high-flow priapism treated between 2009 and 2017 are reported. Two boys had prior perineal trauma, one boy had blunt penile glans trauma, and one had no obvious cause for the condition. All boys initially underwent penile CDU and were treated conservatively or via selective arterial embolization depending upon the presence or absence of an arteriocavernous fistula. RESULTS: Penile CDU revealed an arteriocavernous fistula inside the corpus cavernosum penis in two of four boys and increased blood flow inside the corpus spongiosum in the remaining boys. The former two boys underwent selective arterial embolization and one boy underwent repeated embolization because of remaining arteriocavernous fistula feeding from the contralateral cavernosal artery, whereas the boys with no arteriocavernous fistula on CDU were managed conservatively. All boys were successfully treated within 1 month, and they had normal morning erection and no evidence of recurrent priapism at the follow-up. DISCUSSION: Unlike low-flow priapism, high-flow priapism is not a medical emergency. Therefore, conservative therapy is an appropriate initial treatment, although selective arterial embolization can be effective for high-flow priapism with arteriocavernous fistula, with a success rate of 97% and no reported complications to date. Penile CDU is an imaging technique that can detect focal areas of turbulent flow with sensitivity close to 100%. This study has several limitations including a small number of cases, limited follow-up duration, and possibility of spontaneous arteriocavernous fistula closure in cases treated by arterial embolization. CONCLUSION: Penile CDU could be a reliable tool to diagnose high-flow priapism and detect the presence or absence of arteriocavernous fistula. Although conservative therapy remains the first choice, selective arterial embolization may be an early treatment option when CDU reveals an arteriocavernous fistula.

Management of priapism with a trial of exercise in the emergency department.

Priapism is characterized by persistent penile erection in the absence of sexual arousal or desire that does not subside with orgasm. Although relatively uncommon, it is a genitourinary emergency that necessities prompt work-up and appropriate management, as there is a time-dependent relationship between total duration of erection and an increasing risk of permanent erectile dysfunction. Confirming the type of priapism is key to proper management, but the majority of cases presenting to the emergency department are ischemic in nature. Conservative management strategies for ischemic priapism are sparsely described in the literature but generally include ice pack application to the area, cold showers, masturbation and rarely, exercise. These strategies lack sound evidence, but the risks of attempting them are minimal as long as access to more definitive treatment is not delayed. Lower-limb exercise as a first-line treatment warrants further study in the undifferentiated emergency department priapism population. The case we present and discuss here illustrates the potential benefits of a trial of acute lower-limb exercise, specifically stair climbing, as a treatment for medication-induced priapism. If effective, this simple non-invasive management strategy may decrease the time to effective treatment, requires minimal resource utilization, and ultimately, avoids the need for more invasive treatment.