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.     

A pathophysiology-based approach to the management of early priapism

Priapism is a rare condition that involves persistent penile erection for greater than 4 h. Distinct variants exist, each with unique characteristics. Ischemic priapism is a painful medical emergency that may occur as a result of veno-occlusion leading to hypoxia and tissue death. Recurrent bouts of ischemic priapism, or stuttering priapism, require treatment for individual attacks as well as long-term prevention. Non-ischemic priapism is associated with trauma and may be managed conservatively. Recent advances into the pathophysiology of priapism have allowed the development of treatment algorithms that specifically target the mechanisms involved. In this review, we outline the basics of smooth muscle contraction and describe how derangement of these pathways results in priapism. A pathophysiological approach to the treatment of priapism is proposed with duration-based algorithms presented to assist in management.     

New aspects in the treatment of priapism

Recent advances in the understanding of erectile physiology have improved the prompt diagnosis and treatment of priapism. During initial assessment, the physician must distinguish between veno-occlusive low flow (ischemic) and arterial high flow (nonischemic) in order to choose the correct treatment option for each type of priapism. Patient history, physical examination, penile haemodynamics and corporeal metabolic blood quality assist the distinction between static and dynamic priapism. Normally, priapism is effectively treated with intracavernous vasoconstrictive agents or surgical shunting. However, when these two methods fail, subsequent treatment procedures are a matter for debate. Alternative options, such as intracavernous injection of methylene blue or selective penile arterial embolization, for the management of high and low flow priapism are described and a survey of current treatment modalities is presented.     

Unsatisfactory outcomes of prolonged ischemic priapism without early surgical shunts: our clinical experience and a review of the literature

Ischemic priapism is a rare occurrence which can cause severe erectile dysfunction (ED) without timely treatment. This retrospective study reports our experience in treating prolonged ischemic priapism and proposes our further considerations. In this paper, a total of nine patients with prolonged ischemic priapism underwent one to three types of surgical shunts, including nine Winter shunts, two Al-Ghorab shunts and one Grayhack shunt. During the follow-up visit (after a mean of 21.11 months), all patients'' postoperative characters were recorded, except one patient lost for death. Six postoperative patients accepted a 25-mg oral administration of sildenafil citrate. The erectile function of the patients was evaluated by their postoperative 5-item version of International Index of Erectile Function Questionnaire (IIEF-5), which were later compared with their premorbid scores. All patients had complete resolutions, and none relapsed. The resolution rate was 100%. Seven patients were resolved with Winter shunts, one with an Al-Ghorab shunt and one with a Grayhack shunt. The mean hospital stay was 8.22 days. There was only one urethral fistula, and the incidence of postoperative ED was 66.67%. Four patients with more than a 72-h duration of priapism had no response to the long-term phosphodiesterase type 5 (PDE-5) inhibitor treatment. These results suggest that surgical shunts are an efficient approach to make the penis flaccid after prolonged priapism. However, the severe ED caused by prolonged duration is irreversible, and long-term PDE-5 inhibitor treatments are ineffective. Thus, we recommend early penile prosthesis surgeries for these patients.     

Management of post-traumatic arterial priapism in children: Presentation of a case and review of the literature

In this article, a 9-year-old boy with arterial priapism is presented. The patient was managed with the conservative measures including imipramine hydrochloride and a favorable outcome was achieved after 2 months of follow-up. The pathophysiology, diagnostic tools and treatment alternatives are discussed.     

The use of high‐resolution magnetic resonance imaging in the management of patients presenting with priapism

Study Type – Diagnosis (exploratory cohort)
Level of Evidence 2b OBJECTIVE To investigate the use of magnetic resonance imaging (MRI) of the penis during an episode of priapism and assess the viability of the corpus cavernosum (CC) smooth muscle, as prolonged ischaemic priapism is associated with a high rate of long‐term erectile dysfunction (ED), and the viability of CC smooth muscle influences the subsequent management in ischaemic priapism. PATIENTS AND METHODS The study was set in a single centre based in a large university teaching hospital. We investigated the correlation of T2‐weighted gadolinium‐ enhanced MRI with the histology from CC biopsies in the same patients. In all, 38 patients (mean age 42 years) presenting with priapism over a 3‐year period had MRI of the penis. The scans were reported by two dedicated uro‐radiologists who graded the MR images as showing viable or nonviable erectile tissue. One pathologist assessed the CC biopsies for necrosis. The findings were then correlated. Where no biopsies were taken a clinical follow‐up was used to assess erectile function. RESULTS In 23 patients undergoing both a CC biopsy and MRI, the sensitivity of MRI in predicting nonviable smooth muscle was 100%. In a further 10 patients MRI showed nonviable CC smooth muscle, but no biopsy was taken in these patients; on clinical follow‐up all of these patients subsequently developed ED. In a further five patients the imaging showed viable smooth muscle and these patients subsequently maintained erectile function on clinical follow up. CONCLUSIONS Penile MRI provides an accurate imaging method to assess smooth muscle viability in patients presenting with priapism.     

缺血性阴茎异常勃起病因和危险因素分析

目的:分析缺血性阴茎异常勃起病因和危险因素。方法:回顾性分析25例患者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:有明确病因15例(60%),其中口服或注射药物史患者12例(48%),包括静脉滴注藻酸双酯钠5例(20%),阴茎海绵体注射血管活性药物4例(16%),静脉滴注肝素2例(8%),口服"补肾"保健品1例(4%);慢性粒细胞性白血病患者2例(8%),直肠癌转移患者1例(4%),15例患者中,并发糖尿病7例,高血压病6例,高脂血症6例,肾病综合征1例。无明确病因10例(40%)。结论:肝素、藻酸双酯钠以及阴茎海绵体注射血管活性药物剂量过大,慢性粒细胞性白血病是缺血性阴茎异常勃起的常见病因。系统性疾病如高血压病、糖尿病和高脂血症导致的血液高粘滞状态可能是缺血性阴茎异常勃起的危险因素。     

An alternative noninvasive approach for the treatment of high-flow priapism in a child: duplex ultrasound-guided compression

We are presenting a 5-year-old boy with a traumatic high-flow priapism developed after a straddle injury and successfully treated by compression and simultaneous monitoring with a duplex ultrasound probe. We believe that this may be an alternative method against conventional treatment modalities including conservative follow-up, sympathomimetic drug administration, percutaneous embolization of the fistula, and surgical ligation.     

Partial priapism secondary to tamsulosin: a case report and review of the literature

Partial priapism is also called partial segmental thrombosis of the corpus cavernosum. It is a rare pathology, and its aetiology, physiopathology and treatment are still not completely understood. To our knowledge, partial priapism due to alpha blockers has not been reported previously in the literature. In this study, a successfully treated case of partial priapism occurring after the usage of alpha blocker is presented and discussed in light of the related literature.     

高血流量阴茎异常勃起(5例报告及文献复习)

目的探讨高血流量阴茎异常勃起临床诊疗程序。方法5例阴茎异常勃起患者，经病史和体检，海绵体穿刺血气分析，彩色多普勒超声和超选择血管造影确诊为高血流量阴茎异常勃起，超选择阴部内动脉造影监视下动脉栓塞治疗，IIEF-5评分随访远期效果。结果4例有骑跨伤或会阴部钝性外伤史，血气分析结果接近动脉血。超声显示5例患者患侧海绵体动脉血流速度显著增加。超选择阴部内动脉造影4例患1者在阴部内动脉海绵体支末端形成动静脉瘘(2例左侧和2例右侧)；1例非外伤患者发现为海绵体血管瘤自发破裂。5例患者即时行明胶海绵动脉栓塞治疗，4例成功。1例失败患者改用微钢圈栓塞成功。随访结果无阴茎异常勃起复发，IIEF-5评分1例有中度勃起功能障碍。结论超选择性阴部内动脉栓塞是治疗高血流量阴茎异常勃起首选治疗方法。     

Priapism in the newborn: management and review of literature

Priapism is a pathological condition of a penile erection that persists beyond or is unrelated to sexual stimulation. Priapism is broadly classified into two types: (i) ischaemic priapism (veno-occlusive) (low-flow), (ii) nonischaemic priapism (arterial) (high-flow). We report the case of a newborn presenting with priapism on the first day of life and also review published data on the management and follow-up of this condition.     

Structural analysis of the corpora cavernosa in patients with ischaemic priapism

Study Type – Therapy (case series)
Level of Evidence 4

OBJECTIVE

To evaluate, using quantitative and qualitative methods, the changes in the corpora cavernosa of patients with ischaemic priapism.

PATIENTS AND METHODS

We obtained samples of corpora cavernosa from seven patients with ischaemic priapism (mean age 38 years, range 28–44) who had a cavernous‐glandular shunt. The control tissues were fragments of corpora cavernosa obtained from autopsies of seven age‐matched men who died from causes unrelated to the urogenital tract. Histochemical and immunohistochemical techniques were used to assess and quantify the extracellular matrix and smooth muscle fibres. The volumetric density of smooth muscle, elastic fibres and collagen were determined in corpora cavernosa.

RESULTS

From the stereological analysis the mean (sd ) values of volumetric density were: for collagen, control 34.76 (4.64), priapism 39.64 (2.91) (P = 0.002); elastic system fibres, controls 28.10 (2.85), priapism 36.10 (3.06) (P = 0.001); smooth muscle fibres, controls 43.37 (4.96), priapism 26.48 (5.00) (P < 0.001). There were significantly more fibrous elements of the connective tissue and significantly fewer smooth muscle fibres in the corpora cavernosa of patients with ischaemic priapism than in controls.

CONCLUSION

Ischaemic priapism is associated with early and significant changes in the components of the extracellular matrix and smooth muscle fibres of the corpora cavernosa. This could explain the frequent occurrence of erectile dysfunction found in patients with ischaemic priapism.     

Sexual function after highly selective embolization of cavernous artery in patients with high flow priapism: long-term followup

PURPOSE: We present 15 cases of high flow priapism treated by selective embolization and evaluate erectile function at long-term followup. MATERIALS AND METHODS: Between 1995 and 2001, 15 patients underwent highly selective embolization of the cavernous artery for high flow priapism. Trauma was reported by 12 of the 15 patients, and no etiologic causes were evident in the other 3. The fistula was unilateral in 13 patients and bilateral in 2. All patients underwent embolization during arteriography. Erectile function was determined using the International Index of Erectile Function (IIEF) at followup after an average of 55 months (range 18 to 93). RESULTS: Postoperative color Doppler ultrasonography showed no recurrence in 11 patients (73%). Repeat pelvic angiography with selective embolization was required at 1 month postoperatively in 3 patients (20%). In 1 case (7%) 3 consecutive embolizations were not conclusive and a surgical operation was required. The IIEF results showed that sexual function was in the normal range in 80% of patients. Three patients (20%) reported a slight change in the quality of erection. Mean postoperative IIEF score was 26.3 (range 18 to 30). CONCLUSIONS: Highly selective embolization of the fistula is an effective and safe treatment option for high flow priapism because it ensures a high level of preservation of pretreatment erectile function.     

The investigation of putative agents, using an in vitro model, to prevent cavernosal smooth muscle dysfunction during low-flow priapism

OBJECTIVE

To investigate the effect of putative agents for preventing irreversible smooth muscle dysfunction, using an in vitro model of low‐flow priapism (a condition conventionally managed using a combination of corporal blood aspiration and instillation of α‐adrenergic agonists), as failure of detumescence results in a high incidence of erectile dysfunction.

MATERIALS AND METHODS

We investigated the effects of several agents (N‐acetylcysteine, BayK 8644, glutathione, digoxin, calcium and N^ω‐nitro‐l ‐arginine methyl ester) on the recovery of smooth muscle tone after exposure to 4 h of a combination of hypoxia, glucopenia and acidosis in corpus cavernosum isolated from rabbit.

RESULTS

After 4 h of ischaemia, none of the agents were able to prevent irreversible smooth muscle dysfunction.

CONCLUSION

Prolonged low‐flow priapism leads to smooth muscle dysfunction and fibrosis within the corpus cavernosum. When α‐adrenergic agents fail to reverse the condition, surgical intervention is required. We showed that the administration of novel agents, including antioxidants, does not prevent smooth muscle dysfunction.     

Spontaneous detumescence and full restoration of erection in a patient with high‐flow priapism with persistent arterial–lacunar fistula after repeated selective embolisation—A case report

High‐flow priapism is a rare condition mainly caused by perineal trauma. Laceration of cavernosal artery results in a formation of arterial–lacunar fistula with unregulated blood flow causing prolonged erection. We present a case of a 25‐year‐old man with high‐flow priapism and concurrent erectile dysfunction treated with repeated selective embolisation with only a partial effect. When no further embolisation was possible, we assumed on conservative management even through the fistula was still present. Spontaneous detumescence occurred 9 months, and erectile function has fully restored 24 months after the injury. To the best of our knowledge, spontaneous detumescence with full restoration of erection even through the persistent arterial–lacunar fistula has not been reported previously. Therefore, we propose conservative approach after embolisation to be an option.     

Triple negative breast cancer: Clinical characteristics in the different histological subtypes

PurposeTo investigate the clinical behavior of triple negative breast cancer (TNC), including age distribution, occurrence of LN (lymph node) invasion and prognosis in different histological subtypes.MethodsFor this cohort study we used data on 476 patients with newly diagnosed TNC at the University Hospitals Leuven (Belgium) between 1999 and 2009. Of these, 395 received upfront surgery, 68 neoadjuvant chemotherapy and 21 had metastases at diagnosis.ResultsApocrine and invasive lobular TNC occur more often in older patients compared to IDC-NOS. Of the primarily operated patients with TNC, 35.1% has pathological LN involvement. There were no significant differences in nodal invasion between different histological subtypes, but most subtypes contained few patients. In contrast to previous reports, 6/14 of apocrine TNC had LN involvement. Disease free survival (DFS) was different in different histological subtypes, but group sizes were insufficient to be able to draw firm conclusions. Within the histologically ‘homogeneous’ IDC-NOS group with primary surgery and outcome data (n = 300), DFS with 3.5 year median follow-up decreased with increasing age, but chemotherapy and radiotherapy were much less frequently given with increasing age. In multivariable analysis, lower age, presence of LN involvement, lack of administration of chemotherapy and radiotherapy were significant predictors of relapse.ConclusionTNC is not a uniform disease. Different histological subtypes have different age distribution and behavior. The prognosis of the most common histological subgroup, IDC-NOS, is better in older patients, but this is counterbalanced by significantly decreased use of chemotherapy and radiotherapy.     

Priapism: new concepts in medical and surgical management

Advances have recently been made in both medical and surgical management of priapism, and these offer improvements in the level of care afforded such patients. Further developments can be expected based on ongoing progress, particularly in the area of molecular science, which is the primary source for driving novel therapeutic approaches. Continued action to address the health care administrative concerns of those most commonly affected by priapism, specifically individuals with sickle cell disease, is also appropriate. All successes in these arenas ensure that afflicted individuals avoid the health burdens of priapism and preserve sexual function.