Insights of priapism mechanism and rationale treatment for recurrent priapism

Priapism is defined as abnormal prolonged penile erection occurring beyond or unrelated to sexual interest. The disorder is enigmatic yet devastating because of its elusive etiology, irreversible erectile tissue damage, and resultant erectile dysfunction （ED）. Current management strategies suffer from a poor understanding of the pathophysiology, especially at the molecular level. The traditional treatments are based more on empirical rather than evidence-based knowledge. The outcomes for restoration of normal erectile function are poor, especially for stuttering priapism. Therefore, it is critical to understand priapism from a molecular level, to formulate treatment strategies and to establish rational prevention strategies for high-risk populations, such as sickle cell disease （SCD） patients and cases of the stuttering variant. This review focuses on the recent advances at the molecular level in priapism and penile erection, and applies the recent knowledge to the treatment of stuttering priapism.     

Medical management of ischemic stuttering priapism: a contemporary review of the literature

Priapism is defined as a prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process with little information on the pathophysiology of this erectile disorder. Complications from this disorder are devastating due to the irreversible erectile damage and resultant erectile dysfunction (ED). Stuttering priapism, though relatively rare, affects a high prevalence of men with sickle-cell disease (SCD) and presents a challenging problem with guidelines for treatment lacking or resulting in permanent ED. The mechanisms involved in the development of priapism in this cohort are poorly characterized; therefore, medical management of priapism represents a therapeutic challenge to urologists. Additional research is warranted, so we can effectively target treatments for these patients with prevention as the goal. This review gives an introduction to stuttering priapism and its clinical significance, specifically with regards to the patient with SCD. Additionally, the proposed mechanisms behind its pathophysiology and a summary of the current and future targets for medical management are discussed.     

Idiopathic stuttering priapism treated with salbutamol orally: a case report

Recurrent ischaemic priapism also known as stuttering priapism is an uncommon form of ischaemic priapism, and its treatment is not yet clearly defined. If left untreated, it may evolve into classic form of acute ischaemic priapism and lead to erectile dysfunction due to fibrosis of corpora cavernosa. Several drugs have been proposed with variable results and only supported with level three or four of evidence. Hormonal therapy such as cyproterone acetate, oestrogen, bicalutamide or Lh‐Rh agonist are often effective but can cause side effects such as hypogonadal state and infertility. Other medical options are 5‐alpha‐reductase and phosphodiesterase‐5 inhibitors, ketoconazole, baclofen, digoxin, gabapentin and beta‐2‐agonist terbutaline. We report the first case of stuttering priapism treated with beta‐2‐agonist salbutamol.     

Clinical course linkage among different priapism subtypes: Dilemma in the management strategies

Objectives: Priapism is a rare condition whose management differs according to the etiology. We report the clinical course of three forms of priapism to assess the feasibility and safety of recent management strategies. Methods: The study included eight patients complaining of persistent erection for ≥4 h who were treated in our institution between January 1996 and July 2007. Results: Overall, we categorized 12 cases of priapism in eight patients divided as follows: five cases of ischemic priapism (IP), three of stuttering priapism (SP), and four of non‐ischemic priapism (NIP). Two of five IP patients needed a shunt procedure, which led to the subsequent erectile dysfunction. The other three were treated successfully with a corporal injection of sympathomimetic agents and subsequently suffered from SP. One of the three SP patients suffered from mimicked NIP with increased arterial blood flow during the initial treatment for IP. Four of the NIP patients including the mimicked one achieved complete detumescence, through arterial embolization in two and conservative management in two. Conclusions: Current management seems effective and safe in the short‐term. However, the long‐term outcome of the treatment for IP is still disappointing. Careful long‐term observation is needed for an appropriate management.     

反复发作缺血性阴茎异常勃起(1例报告并文献复习)

目的探讨反复发作缺血性阴茎异常勃起的临床特点。方法分析复发性阴茎异常勃起1例,结合相关文献复习。结果反复发作夜间阴茎异常勃起16个月患者,临床检查确诊为复发性缺血性异常勃起,采用5a还原酶抑制剂保列治联合5羟色胺再摄取抑制剂左洛复治疗5d后,异常勃起未复发。继续服用保列治治疗,随访4个月异常勃起无复发,勃起功能正常。结论复发性缺血性阴茎异常勃起临床罕见,采用5a还原酶抑制剂联合5羟色胺再摄取抑制剂治疗具有一定的效果。     

高流量性阴茎异常勃起1例报告及文献复习

目的通过病例报告及复习相关文献,探讨高流量性阴茎异常勃起的诊断及治疗。方法回顾性分析兰州军区兰州总医院近期收治的1例外伤后高流量性阴茎异常勃起患者的症状、体征、检查以及治疗、随访情况,结合既往报道的文献资料进行综述分析。结果患者入院后保守治疗无效,行超选择性阴部内动脉造影检查明确双侧阴茎海绵体动脉瘘,使用明胶海绵栓塞后治愈,随访6月患者恢复佳,无勃起功能障碍。结论通过典型的临床表现、阴茎海绵体血气分析以及彩色多普勒超声检查可初步诊断,而高选择性血管造影检查及栓塞术则为高流量性阴茎异常勃起的确定诊断及治疗的最常用及有效的方法。     

Temporary cavernosal-cephalic vein shunt in low-flow priapism treatment

Background

Surgical shunting might be considered as the only option in the treatment of extended ischemic priapism that does not respond to aspiration and medication.

Objective

A modified, simple, minimally invasive, and easily applicable artificial cavernosal–venous shunt technique for treating priapism is described in this report.

Design, setting, and participants

A total of 15 patients with extended and nonresponsive low-flow priapism were treated with this technique between January 1998 and February 2007.

Intervention

When the conservative treatment of low-flow priapism does not yield the expected results, then the temporary cavernosal–cephalic vein shunt should be applied. The standard equipment required for this modified technique includes three angiocaths, two shorn blood serum sets, and saline solution with heparin. The blood in the cavernosa and the saline solution infusion are incorporated into the systemic circulation with the aid of serum sets and angiocaths.

Measurements

Priapism duration, history, causes, its relation with sexual stimulation, pain, and any prior management of priapism were assessed in all patients. A complete blood count and blood gases assessment were conducted in corporal aspirates, and duplex penile ultrasonography was performed, which showed attenuated blood flow in the cavernosal artery.

Results and limitations

This technique was applied in 15 patients. Complete detumescence was achieved in 13. In the two cases in whom the technique did not yield the expected results, there was a need for a sapheno–cavernosal shunt. These patients later complained of erectile dysfunction and penile pain that continued for 6 mo. Of the 13 patients in whom detumescence was achieved, 3 reported erectile dysfunction according to International Index of Erectile Function (IIEF) scores at the 12-mo follow-up.

Conclusions

Artificial cavernosal–cephalic vein shunt in the treatment of priapism is simple, safe, effective, easily applicable, and warrants primary consideration when the second-line treatment of priapism is initiated.     

Acenocoumarol associated priapism: report of a case

We report on a 20-year-old man presenting with priapism while receiving oral acenocoumarol for the treatment of deep vein thrombosis. To the authors' knowledge, it is the first reported case of acenocoumarol-associated priapism.     

A rare cause of recurrent priapism: hereditary angioedema

Hereditary angioedema is a rare disease, which is caused by deficiency of compleman c1 esterase inhibitor regulatory protein in the compleman system. Priapism is involuntary, painful and prolonged erection of penis more than 4 h without sexual desire. In this case report, we elucidated a patient diagnosed with hereditary angioedema while he had recurrent priapism.     

9例阴茎异常勃起诊治体会

目的 探讨阴茎异常勃起的诊断和治疗方法.方法 回顾性分析9例阴茎异常勃起患者的诊治过程.结果 9例患者均通过病史、体检、海绵体抽吸血气分析及多普勒超声检查确诊.经镇静、局部冷敷、海绵体抽吸血液、海绵体内注射药物及手术等不同方法治疗后,8例缺血性阴茎异常勃起患者中5例完全消退,3例部分消退;1例非缺血性阴茎异常勃起患者部分消退.5例随访6个月,其中4例出现ED.结论 阴茎海绵体血气分析、海绵体彩色多普勒检查对阴茎异常勃起有重要诊断价值,早期及正确应用海绵体注射药物和阴茎海绵体尿道海绵体分流术加术中冲洗是治疗缺血性阴茎异常勃起的可靠方法,但是由于大多数病人持续勃起时间较长,ED的发生率偏高.     

High-flow priapism: colour-Doppler ultrasound-guided supraselective embolization therapy

High flow priapism is mainly caused by traumatic arteriocavernous fistulas. The standard therapy is a transcatheter embolization of the fistula. This study analyzes the combined approach of arteriography and color Doppler ultrasound during the embolization procedure. During the arteriography procedure, a perineal color Doppler ultrasound examination of the fistula was performed to achieve an optimal positioning of the catheter tip in the fistula with a minimum of radiation exposure. To visualize the correct localization, ultrasound contrast medium or saline solution was injected through the catheter. The flow-pattern of the contrast medium allowed evaluation of the successful occlusion of the fistula and preservation of the unaffected penile arteries. In six patients (unilateral fistula: three, bilateral fistulas: three) with a posttraumatic high-flow priapism, this technique was performed for embolization of the fistulas. A total of nine embolization sessions were performed. Only one case required a second session due to dislocation of a microcoil. In all cases, the priapism disappeared immediately after the final session while erectile function was restored within 4 weeks after embolization. The combined approach of x-ray and ultrasound imaging facilitates the supraselective embolization of the arteriocavernous fistula, leading to an optimal success rate, while reducing the radiation exposure and the applied dose of contrast medium.     

A case report on idiopathic recurrent priapism

Idiopathic recurrent priapism is a difficult problem to treat and a potentially devastating condition that may result in irreversible penile fibrosis. We present the case of a patient who, during a period of 10 years, had recurrent episodes of idiopathic priapism and we show that therapeutic options do exist for the management.     

The 10-year priapism experience: identifying clearer targets for intervention

BackgroundThere is a paucity of data on the clinical experience of priapism. Moreover, little work has explored differences in practice patterns between urologists and emergency medicine (EM) physicians. Our primary objective was to understand the priapism patient population and identify targets that may guide clinical translational efforts.MethodsA retrospective chart review was performed on two priapism datasets from June 2008–July 2018—one focused on patients managed by urology and another on patients managed exclusively by EM physicians. Primary areas of interest included the duration of priapism and acute interventions during the consultation. Time to presentation, prior interventions and evaluation was also documented.ResultsOver the course of 10 years, there were 396 encounters for priapism in 95 unique patients. Urology was consulted 199 times in 83 unique patients and EM physicians managed 197 encounters in 15 unique patients. In the urology cohort, median duration of priapism was 6 hours, and 72% of patients required further intervention. For the EM cohort, median duration of priapism was 4 hours and 89% of patients required further intervention. Amongst all patients, nine patients presented 4 or more times for a total of 294 encounters.ConclusionsUrology and EM managed a similar number of encounters, but EM patients had a shorter duration of priapism. Understanding the role of the EM physician and the urologist can help tailor joint curriculum efforts for initial priapism management while focusing on more complex management for urology trainees. A small proportion of patients accounted for the majority of visits secondary to recurrent ischemic priapism indicating a need to target prevention of these episodes on an outpatient basis.     

阴茎异常勃起的诊断和治疗(附12例报告)

目的提高阴茎异常勃起的诊治水平。方法统计临床所见12例阴茎异常勃起患者，年龄20-62岁，平均44岁。持续勃起时间13-162h，平均25．4h。其中血管活性药物所致5例，膀胱癌转移至阴茎异常勃起1例，白血病1例，有外伤史者3例，不明诱因者2例。对异常勃起分型，治疗及预后进行分析。结果12例患者中9例为低流量型，3例为高流量型。随访2—26个月，9例低流量型患者中，5例经阴茎海绵体根部注射间羟胺2—6mg，必要时在阴茎头及阴茎海绵体根部置9号针头灌注肝素化生理盐水对冲治愈，其中2例发生勃起功能障碍（ED）；2例行阴茎海绵体与阴茎头血管分流术治愈，其中一例术后出现ED；因白血病引起的阴茎异常勃起1例，膀胱癌转移至阴茎1例，预后均不佳。3例高流量型患者中1例行选择性阴部内动脉栓塞后治愈，无ED；2例行保守治疗出院，均有ED发生。结论详细的病史、海绵体血气分析和彩色双功能超声等检查有助于阴茎异常勃起准确及时的诊断。阴茎异常勃起如保守治疗无效，应立刻进行手术治疗。关键词阴茎异常勃起，诊断，治疗     

Ten-year-old boy with post-traumatic priapism

Summary The case of a 10-year-old boy with post-traumatic priapism after perineal trauma is presented. Interdisciplinary treatment using angiography and selective catheter embolization for juvenile high-flow priapism is demonstrated. The literature and the diagnostic possibilities resulting from color duplex sonography are discussed.      

阴茎异常勃起的病因和危险因素

目的分析阴茎异常勃起的病因和危险因素。方法回顾性分析2009年5月至2013年5月北京大学第一医院男科中心收治的41例阴茎异常勃起患者的病例资料,了解其既往异常勃起发作史、用药史、性生活史、发病诱因和临床实验室检查结果。结果明确病因者15例(15/41,36.59%),其中药物造成11例(11/15,73.33%),包括静脉滴注藻酸双酯钠8例(8/15,53.33%)、抗抑郁药1例(1/15,6.67%)、中成药2例(2/15,13.33%);暴力性生活2例(2/15,13.33%);骑跨伤2例(2/15,13.33%)。并发疾病直肠癌转移患者1例(4%),并发糖尿病2例、高血压病4例、高脂血症6例、抑郁症2例、脑梗塞1例、肝炎1例,反复阴茎异常勃起2例。无明确病因26例(63.41%)。结论骑跨伤造成的阴茎异常勃起均为动脉型,而药物引起的、如藻酸双酯钠,均为静脉型。高血压病、糖尿病和高脂血症导致的血液高黏滞状态可能是阴茎异常勃起的危险因素。     

Pediatric high-flow priapism and super-selective angiography--an Australian perspective

High-flow priapism is an uncommon entity in the pediatric and adolescent population. It is usually caused by perineal trauma. Here we describe the experience of our institution in this condition over the past 10 years, the various treatment options available, and the successful application of super-selective angiographic embolization as our treatment modality of choice. Included here is the case of a 4-year-old boy (case 3) who, to our knowledge, is the youngest patient described with this condition in the literature.