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[Management of priapism in sickle-cell diseases with alpha-adrenergic agonists].

Priapism is a common complication of sickle cell anemia. Two different patterns are described: acute priapism, a prolonged painful erection generally lasting more than 6 hours, and stuttering priapism, which consist of brief repeated self-resolving episodes. Until 1990, priapism in sickle-cell patients has relied on measures aimed at lowering blood viscosity and acidosis and reducing the level of circulating hemoglobin S (alcalinization, hyperhydration, exsanguinotransfusion). But these means are not consistently successful. Surgical cavernous-venous shunt was proposed after 12 to 24 hours when conservative treatment failed. These therapeutic modalities are based on the pathophysiology of sickle-cell priapism. Priapism in sickle-cell disease may be due to sequestered sickled red cells in the corpus cavernosum with venous outflow obstruction. For some years, the treatment of priapism in sickle-cell anemia was changed by the use of alpha-adrenergic agonists. These therapeutics (mainly etilefrine and epinephrine) were first reserved for priapism resulting from intrapenile injections of vasoactive drugs which are used for the treatment of impotence. In acute priapism, alpha-adrenergic agonists are used in intracavernous injections (ICI). In stuttering priapism, treatment consists in an oral administration associated, if necessary, with self-administered ICI. ICI results mainly depend on when treatment occurs. Detumescence is achieved in patients treated within 30 hours, as opposed to the few patients treated beyond this delay. This finding is in agreement with experimental findings demonstrating histological evidence of necrosis of endothelial cells and cavernous smooth muscle fibers after 24 hours. Surgery is only used after failure of ICI. The result of oral treatment is not very satisfactory because many patients do not respond well or are dependent on ICI. However, self-administered ICI associated with the oral treatment protects patients with stuttering priapism against acute strokes. The safety of alpha-adrenergic agonists is good as both oral and ICI have few side-effects. The excellent efficacy of ICI in sickle-cell priapism leads to suggest that the pathogenic mechanism could involve a neuromuscular dysfunction.

Administration, Oral↗

Diagnosis and treatment of priapism: experience with 5 cases.

OBJECTIVES: Priapism is a rare disease, but needs early intervention and appropriate management. We present 5 cases, 2 of nonischemic high-flow priapism and 3 of ischemic low-flow priapism. METHODS: Focusing on the differential diagnosis of priapism between the nonischemic high-flow type and the ischemic low-flow type, we reviewed the medical records of 5 patients. RESULTS: Of the examinations carried out, cavernosography, blood gas analysis of cavernosal blood, color Doppler ultrasound, and internal pudendal arteriography were useful in differentiating the type of priapism. Complete detumescence of the penis in 2 cases of high-flow priapism and 3 cases of low-flow priapism was achieved by selective embolization with gelform and by glandular-cavernosal shunting, respectively. No recurrence was observed in any patient, and postoperative erectile function was preserved in 4 patients and is unknown in 1. CONCLUSIONS: These results indicate that angiographic studies provide the most reliable information for the differentiation of the type of priapism. However, color flow Doppler ultrasound and cavernosal blood gas determination can obviate the need for angiographic studies and are noninvasive. Although conservative treatment or even expectant management may be feasible with high-flow priapism, aggressive treatment should be carried out for low-flow priapism immediately after initial treatment fails to achieve detumescence of the penis. Selective embolization of the internal pudendal artery may be the treatment of choice for patients with high-flow priapism.

Adult↗

Priapism.

INTRODUCTION: There are three different types of priapism: low-flow, ischemic, anoxic or veno-occlusive priapism; high-flow, arterial or nonischemic priapism; and recurrent or stuttering priapism. AIM: To provide recommendations/guidelines concerning state-of-the-art knowledge for the diagnosis and treatment of priapism. METHODS: An International Consultation in collaboration with the major urology and sexual medicine associations assembled over 200 multidisciplinary experts from 60 countries into 17 committees. Committee members established specific objectives and scopes for various male and female sexual medicine topics. The recommendations concerning state-of-the-art knowledge in the respective sexual medicine topic represent the opinion of experts from five continents developed in a process over a 2-year period. Concerning the Priapism Committee, there were 10 experts from six countries. MAIN OUTCOME MEASURE: Expert opinion was based on grading of evidence-based medical literature, widespread internal committee discussion, public presentation and debate. RESULTS: Concerning ischemic priapism, persistent cavernous smooth muscle relaxation and failure of contraction is a compartment syndrome with increasing intracavernosal anoxia, rising pCO2 and acidosis. Urgent medical attention should be sought for an erection lasting >4 hours; 90% with priapism >24 hours develop complete erectile dysfunction. After diagnosis and counselling, intracavernosal aspiration and alpha-blockers should precede surgical shunting. Concerning high-flow priapism (congenital, traumatic or iatrogenic), intervention is not urgent and often unnecessary. Definitive management is by selective embolization with autologous blood clot. Concerning recurrent/stuttering priapism, the pathophysiology may be central or local (sickle cell disease). Management needs to be individualized; androgen deprivation has proved useful but has adverse effects. CONCLUSIONS: There is need for prospective, clinical trials to define safe and effective management strategies for patients with low-flow, high-flow or recurrent priapism.

Consensus↗

Arterial embolization in the treatment of post-traumatic priapism.

Priapism is a prolonged penile erection not associated with sexual arousal. Two types of priapism have been described: the more common one is the "veno-occlusive" priapism and can be frequently observed as the consequence of an intracavernosal injection of vaso-active drugs for the treatment of erectile dysfunction. The less common type of priapism is known as "high flow" priapism and usually follows perineal or direct penile trauma. The clinical presentation in case of high flow priapism is quite typical: hystory of recent penile or perineal trauma followed, by the onset of a painless, incomplete and constant erection of the penis. A color-flow Doppler sonogram should be performed as first diagnostic step: this examination allows to identify the presence of patent cavernous arteries and prominent venous drainage with focal area of high flow turbulence along the pathway of one or both the cavernous arteries. An arterial blood sample taken from the corpora will confirm the diagnosis. At first, conservative therapeutical attempts can be suggested, with mechanical external compression of the perineum, the use of ice packs, corporeal aspiration and irrigation with saline. Besides, intracorporeal administration of alpha-agonists and methylene blue should be performed. Unfortunately, these conservative measures often result unsuccessful, and more invasive approaches must be considered. The radiological superselective transcatheter embolization of the proximal artery supplying arterial-lacunar fistula should be the present treatment of choice in these cases of high-flow priapism refractory to conservative and medical treatments. The first successful management of high flow priapism by selective arterial embolization was reported by Wear and coworkers in 1977. Autologous clots and gelatine sponge have been extensively used and become very popular as the embolic agent. More recently, platinum microcoils have been proposed with the aim to achieve more precise and selective embolization. In our single-case-experience on the treatment of high flow priapism by arterial embolization, we used the recently introduced tungsten microcoils. At the time of the follow-up, 2 months later, patient reported satisfactory intercourse with an approximately 75% of penile rigidity. By comparison with microsurgical ligature of the damaged vessel, selective embolization is, at least theoretically, a less invasive procedure, particularly with reference to the trauma caused to the erectile tissue. High-flow priapism is a fairly rare urological pathology which does not require immediate and emergency treatment (as is the case, instead, with venous-occlusive priapism), since the risk of post-ischaemic fibrosis is excluded thanks to the fact that oxygen is supplied to the cavernous tissue. Once the diagnosis has been established with certainty, therefore, the specialist has the necessary time at disposal to arrange for the most appropriate therapeutic steps. When, as is frequently the case, conservative measures prove ineffective, the current treatment of choice for cases of fistula of the cavernous artery would appear to be superselective embolization of the artery, provided same can be performed at specialized centres and by experienced personnel.

Adult↗

[Physiopathologic aspects of priapism. Disease or symptom?].

The frequency of the iatrogenic priapism complicating the intracavernous injections of drugs for impotence has greatly improved the conceptions about priapism resulting in several novelties: a. definition: priapism may be defined as a pathological erection provoked by various anomalies of the erectile hemodynamics; a. pathogeny: at first, priapism is only the penile symptom of various diseases. Secondary and inconstantly, the priapism symptom become a priapism disease featured by an acute cavernous ischemia; c. physiopathology with two different priapisms: an unusual high flow priapism due to an acquired arteriocavernous fistula. A most usual stasis priapism due to a neuromuscular or hematological blockade of the cavernous detumescence of several origin; d. treatment: the stasis priapism alone is an genuine emergency. Puncture must be always the first treatment. If unsuccessful, the choice between pharmacological detumescence (new treatment for priapism) and surgery depends from both the physiopathological mechanisms and mainly the severity of the cavernous tissular suffering.

Hemodynamics↗

Priapism: a historical and update review.

Early this century, the aetiology of priapism was solely attributed to "systemic disease and local irritation of lower genital tract and neurologic lesion". Corpora cavernosa incision described by Young was the only form of treatment. However, the aetiology, diagnosis and treatment options for the management of priapism and prolonged erections have evolved significantly in the past several years. Before the use of pharmacological agents for the production of erections, idiopathic priapism became the most common aetiology. Causes of priapism from newer psychotropic medications such as trazodone to intra-cavernosal injection therapy with pharmacological agents have increased the number of patients with priapism presenting to the urologist. The management of priapism has remained controversial and has perplexed and continued to frustrate many urologists. A recent and more thorough knowledge of the pathophysiological basis of priapism and the clear differentiation between the low flow veno- occlusive priapism and high flow arterial priapism have significantly improved the diagnostic protocol for patients with priapism. Colour doppler ultrasound evaluation and cavernosal blood gas determinations have become mandatory and greatly improved specific diagnosis. Priapism must be considered a urological emergency and early surgical intervention with corpus cavernosum aspiration and pharmacological lavage with normal saline alpha-adrenegic agonists should be instituted immediately. This will avoid the risk of erectile impotence with considerable medico-legal consequences. Precious time must not be wasted in the older unproven conservative methods including hot and cold water enemas, and vigorous prostatic massage.

Adrenergic alpha-Agonists↗

Embolization therapy in two subtypes of priapism.

Two subtypes of priapism have been described based on the pathophysiologic mechanism. The more common type, termed stasis priapism, is characterized by a low flow state in which inadequate venous outflow creates an acidotic hypoxic environment leading to a painful prolonged erection. The other less common subtype, high flow priapism, is arteriogenic. We used embolization therapy in one case with long lasting stasis priapism and in the other with high flow priapism due to bilateral arteriosinusoidal fistulae in the penis. In both cases we used polyvinyl alcohol for embolization and sexual potency preservation. Priapism is the persistence of erection that does not result from sexual desire. Hauri et al. described two variants of priapism. In high flow priapism (non-ischaemic) there is unregulated arterial inflow to the lacunar spaces due to a lacerated cavernous artery associated with previous perineal and penile trauma. In stasis priapism, the second type, the basic abnormality could be due to a more pronounced or prolonged blood entrapment inside the vascular spaces of the corpora cavernosa sustained by an unknown cause. There are many treatment methods especially for low flow ischaemic variant. We report two different kinds of priapism and embolization therapy in both of them with polyvinyl alcohol.

Adult↗

Drug-induced priapism. Its aetiology, incidence and treatment.

Priapism is characterised by a persistent erection that cannot be relieved by sexual intercourse or masturbation. Although priapism subsides spontaneously in a few days, impotence frequently follows. Both vascular and neural mechanisms are implicated in the pathophysiology of priapism, but it is not clear which initiates the process. Idiopathic cases of priapism are the most frequent (near 50%); other medical conditions that can result in priapism are haematological diseases (mainly sickle cell anaemia and leukaemia), traumatism, and neoplastic processes. Drug-induced priapism comprises about 30% of cases. The drugs most frequently implicated are psychotropic drugs (phenothiazines and trazodone), antihypertensives (mainly prazosin) and heparin. Recently, the intracavernosal injection of vasoactive drugs (papaverine and phentolamine) has been described in patients treated for impotence. With the exception of heparin, an alpha-adrenergic blocking mechanism has been suggested in the priapism-inducing action of these drugs. A significant number of anecdotal case reports link priapism and drugs, and it is possible that certain cases of idiopathic priapism could be reclassified if accurate pharmacological anamnesis were to be performed. Priapism must be considered a urological emergency. Surgical procedures are the most preferred treatment for this condition but, in selected cases, drug treatment seems to be an alternative approach.

Anticoagulants↗

Conservative management of priapism in acute spinal cord injury.

OBJECTIVES: To perform a retrospective chart review of priapism as a complication of spinal cord injury and review the management and follow-up. Priapism is a known complication of acute spinal cord injury, but little has been written concerning the management of this condition. METHODS: A retrospective chart review (1992 through 2002) was performed for all patients with a diagnosis of priapism. Of these patients, 6 had priapism in the setting of acute spinal cord injury without pelvic trauma. We reviewed the management of the priapism in these cases, and follow-up was attempted in each case. RESULTS: Of the 6 patients with spinal cord injury-related priapism, 4 had spinal cord injury located at C5-C7, 1 at C5-C6, and 1 at T12. The prolonged erections were managed conservatively in 4 patients and irrigated with intracorporeal phenylephrine in 2. All patients with corporal blood gas measurement (n = 4) had nonischemic priapism. All 4 patients who underwent no intervention had the priapism resolve within 5 hours. Four patients (two treated conservatively and two who underwent irrigation) had recurrent episodes during the same admission that resolved spontaneously. Long-term outcomes were obtained by telephone from all 6 patients. Of the 6 patients, 5 had maintained spontaneous erections to date (range 3 to 10 years). CONCLUSIONS: The results of our study have shown that priapism related to acute spinal cord injury is nonischemic and may be managed conservatively because of the high likelihood of resolution. Corporal blood gas measurement is important because the results can guide further management decisions. Our results suggests that conservative management of priapism related to spinal cord injury has a low rate of causing long-term erectile dysfunction.

Acute Disease↗

Follow-up of sickle cell disease patients with priapism treated by hydroxyurea.

Hydroxyurea is one of the most successfully used therapies for sickle cell disease. Results of many clinical trials point to hydroxyurea administration for patients with frequent painful crises and acute chest syndrome. Priapism is one of the complications that could be prevented by hydroxyurea, but there are few reports demonstrating the results. Since November 1993, hydroxyurea has been used in our clinic for preventing priapism in patients with stuttering or major attacks who are still capable of achieving intercourse on demand. Five patients were enrolled in the study, and 4 cases benefited by this treatment. After the initial treatment for the acute attack, all five patients developed stuttering priapism. Hydroxyurea was then introduced at the initial dose of 10 mg/kg, and as the hydroxyurea dosage increased, the number or length of priapism episodes decreased. One to two months after the maximal dose (20-35 mg/kg) was introduced, the episodes disappeared. In two patients, we were forced to administer over 30 mg hydroxyurea/kg to abort the episodes, and, in another patient, 25 mg/kg was necessary. All patients present normal sexual activity. Hydroxyurea was discontinued in two patients, but stuttering priapism reappeared. Hydroxyurea was then re-introduced, and priapism disappeared. One patient, using 20 mg hydroxyurea/kg, had a 6-year remission of priapism after hydroxyurea administration; however, he experienced stuttering priapism, 1 month before a major attack, that progressed to impotence. During that month, he did not seek medical attention. In conclusion, the data here presented suggests that hydroxyurea may prevent priapism attacks in sickle cell disease, probably at higher doses than usually prescribed for painful crisis prevention.

Adolescent↗

Multisystem damage associated with tricorporal priapism in sickle cell disease.

PURPOSE: Priapism is an uncommon but debilitating complication of sickle cell disease (SCD). Recent observations among adult males regarding the abysmal failure of medical and surgical therapy encouraged us to review our 25-year experience identifying the prognostic features that might determine outcome. PATIENTS AND METHODS: As part of a prospective 25-year longitudinal demographic and clinical cohort study, a subset of 38 (8.2%) patients with priapism were identified among a cohort of 461 men with SCD. The patients with priapism were compared with the nonaffected men with respect to severity of disease expression, hematologic status, beta s globin gene haplotype, and the incidence of sickle-related major organ failure. The influence of the treatment modalities on outcome was also evaluated. RESULTS: Priapism occurred as a single episode in 24 patients, and in 14 as temporally clustered repeat episodes. Eighty-seven percent of those with priapism had sickle cell anemia (SS), an increased risk as compared with other variants of SCD (p = < 0.05). There were two distinct age-related patterns of disease expression. Eight patients were prepubertal; they experienced shorter episodes, involvement of the corpora cavernosa only, few recurrent episodes, and a good prognosis for future erectile function. Non-surgical therapy in children was associated with excellent results. In contrast, the 29 postpubertal adults often had involvement of the corpora cavernosa and corpus spongiosa (tricorporal disease) and half had prolonged episodes that lasted longer than 8 days. One pubescent patient had repeated episodes and became impotent. Prolonged or repeated episodes eventuated in impotence in 56%. Surgical intervention was not beneficial. Sickle cell-related organ failure such as stroke, chronic restrictive lung disease, chronic renal failure, and nonhealing leg ulcers was observed more frequently in men who had priapism. Death occurred in nine adult patients (25%) within 5 years of the first episode of priapism. CONCLUSION: Priapism in adult males identifies those at high risk for other sickle cell-related organ failure syndromes and, as such, is another complication indicative of severe disease. The dismal prognosis in SS adults requires better understanding of the precise pathophysiology of low-flow tricorporal priapism. Clarification of the mechanisms inducing the priapic state should lead to specific therapeutic maneuvers and an improved prognosis for this disabling condition.

Adolescent↗

The ultrastructure of the erectile tissue in priapism.

The ultrastructure of erectile tissue from the corpora cavernosa penis of patients suffering from stasis priapism and high-flow priapism has been studied. Trabecular interstitial edema was confirmed as the first reaction of the tissue to the hemodynamic impairment. At the cellular level trabecular smooth muscle cells were found to be the first affected by the altered environmental conditions. Their reaction consisted of structural and functional transformation to fibroblast-like cells. Severe cellular damage and widespread necrosis were not seen in high flow priapism; such damage existed in stasis priapism, but only when the priapic episode lasted more than 24 hours. Blood clot formation within the cavernae and destruction of the endothelial lining occurred in stasis priapism lasting over 48 hours. At this time trabecular inflammation became conspicuous and most of the smooth muscle cells were either transformed to fibroblast-like cells or had undergone necrosis. This stage was not reached in high flow priapism, a fact supporting the view that high flow priapism is a more benign and prognostically more favorable form of priapism. Massive smooth muscle cell transformation and the loss of contractile trabecular elements may play an important role in the evolution of irreversible erectile failure following stasis priapism persisting longer than 24 hours.

Adult↗

Alternative approaches to the management of priapism.

Herein we describe the use of intracavernous methylene blue (MB), a guanylate cyclase inhibitor, or internal pudendal artery embolization for the treatment of priapism. Eleven patients with priapism were treated from 1993-1996. Etiologies of priapism included PGE1/papaverine (3), trazodone (2), and sickle cell disease (1), in the other five cases the causes the cause was unknown. The average duration of priapism was 27 h for all patients (6-72 h). Five patients who failed intracavernous MB or an alpha-adrenergic agonist, underwent unilateral or bilateral pudendal artery embolization. The average duration of priapism for patients undergoing embolization was 43 h. Sixty-seven percent of the patients treated with MB responded with immediate detumescence. One-hundred percent of patients with priapism secondary to intracavernous injection therapy or trazodone responded. Of the five patients who underwent embolization, 40% achieved immediate pain relief and subsequent detumescence. The three non-responders exhibited a partial detumescence over 47-72 h. After follow-up of one year embolization available for only two patients revealed that one regained potency while the other remained impotent. These results confirmed that MB is effective for pharmacologically-induced priapism. Embolization is a less invasive option for refractory priapism, although results are less than satisfactory in men with priapism of several days duration.

Adult↗

The prediction of papaverine induced priapism by color Doppler sonography.

PURPOSE: We identified color Doppler sonography findings that are useful for predicting priapism in patients after the intracavernous injection of diagnostic papaverine. MATERIALS AND METHODS: We evaluated 72 men with erectile dysfunction by color Doppler sonography after the diagnostic injection of papaverine. Cavernous artery waveforms were recorded, and peak systolic and diastolic velocity of the recorded waveforms was measured. In cases of priapism color Doppler sonography findings were retrospectively evaluated to identify any finding that would predict priapism. RESULTS: Priapism was observed in 8 of the 72 patients (11.1%). In 6 of 8 priapism cases there was no blood flow in the cavernous artery during or after adequate erection, including after minutes 5, 10 and 20 in 3, 1 and 2, respectively. This absence of encoding was not relieved even after all stimuli were removed. In 2 patients who later presented with complaints of priapism the Doppler study was completed normally and all parameters were acquired without any cessation of blood flow in the cavernous artery. The persistent disappearance of color and pulse encoding in the cavernous artery predicted priapism with 75% sensitivity, 100% specificity, a positive and negative predictive value of 100% and 96.9%, respectively, and 97% accuracy. CONCLUSIONS: The persistence of absent blood flow in the cavernous artery even after the elimination of all stimuli during penile color Doppler ultrasound is a reliable predictor of priapism. The finding is accurate enough to initiate treatment for priapism to avoid further delay.

Humans↗

Feasibility of the use of phosphodiesterase type 5 inhibitors in a pharmacologic prevention program for recurrent priapism.

INTRODUCTION: Recurrent ischemic priapism is an enigmatic erectile disorder in need of improved clinical interventions to avert its known, potentially serious complications. AIM: To evaluate the use of a long-term, continuous phosphodiesterase type 5 (PDE5) inhibitor therapeutic regimen in controlling recurrent ischemic priapism and its feasibility in a clinical management program for the disorder. MAIN OUTCOME MEASURES: The main outcome measure was reduction in frequency or duration of priapism episodes. A secondary outcome measure was preservation of erectile ability. METHODS: We retrospectively evaluated the clinical progress of seven patients (age 22-37 years) with sickle cell disease-associated "stuttering" priapism (N = 4) and idiopathic recurrent priapism (N = 3), who were counseled and consented to the "off-label" use of the PDE5 inhibitors sildenafil citrate and tadalafil. The medications were administered according to a specified therapeutic regimen based on scientific evidence that chronic PDE5 inhibitor administration in priapism contexts effectively reconditions PDE5 regulatory function in the penis. The duration of clinical follow-up extended through 2 years. RESULTS: All seven patients were confirmed to have recurrent ischemic priapism without identifiable pharmacologic, traumatic, or neoplastic disease associations based on clinical history, physical examination, laboratory testing, and penile diagnostics. PDE5 inhibitor treatment was successful in alleviating or resolving priapism recurrences in six of the seven patients. Erectile function was unchanged in six patients and improved in one patient at last follow-up compared with baseline status. All the patients reported that PDE5 inhibitor therapy was well tolerated and did not cause any adverse effects limiting their continued use of the medication. CONCLUSIONS: Because of their efficacy, safety, and tolerability as shown in this case series, PDE5 inhibitors would appear to have a possible role in a rigorously implemented clinical management program to control recurrent priapism. However, completion of a controlled clinical trial is necessary to confirm the utility of this treatment.

3',5'-Cyclic-GMP Phosphodiesterases↗

Methylene blue as a successful treatment alternative for pharmacologically induced priapism.

OBJECTIVE: Priapism is defined as prolonged and persistent erection of the penis without sexual stimulation. Etiologies of this condition are numerous. Treatment of priapism varies from a conservative medical to a drastic surgical approach. Recent findings indicate methylene blue (MB), a guanylate cyclase inhibitor, to be a potential inhibitor of endothelial-mediated cavernous relaxation. This prompted us to assess the feasibility, use and effectiveness of MB in the treatment of priapism. METHODS: 12 patients were treated for priapism. Etiologies were: 10 drug-mediated (PGE(1) or papaverine/phentolamine mixture) after corpus cavernosum injection therapy (CCIT); 1 leukemia-induced, and 1 idiopathic high-flow priapism. The age range for all patients was 13-67 years, the average duration of priapism was 5.5 h after CCIT. MB was administered after blood aspiration of the corpora cavernosa. 5 ml of MB was injected intracavernously (i.c.) and left for 5 min. MB was then aspirated and the penis compressed for an additional 5 min. RESULTS: All patients with CCIT-induced priapism were cured with MB alone. The 2 patients who did not respond to MB underwent i.c. phenylephrine administration and embolization of the pudendal artery, respectively. The etiology and duration of priapism were the strongest predictors for success with i.c. administered MB. The primary side effects were a transient burning sensation and blue discoloration of the penis on injection of MB. The initial baseline erectile status was restored in all patients cured by MB. CONCLUSION: These results confirm that MB is a safe and highly effective treatment agent for short-term pharmacologically induced priapism. The application of MB shows virtually no significant side effects compared to the systemic and local complications induced by alpha-adrenergic agonists.

Adolescent↗

Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism.

OBJECTIVES: Recurrent ischemic priapism describes a disorder of repeated episodes of prolonged penile erection that frequently leads to devastating complications of erectile tissue damage and erectile dysfunction. A mechanistic role for dysregulated phosphodiesterase 5 (PDE5) in the deranged smooth muscle response of the corpus cavernosum of the penis offers new understanding about the pathogenesis of the disorder and suggests that PDE5 may serve as a molecular target for its treatment and prevention. We explored the use of PDE5 inhibitors to treat recurrent priapism, based on the hypothesis that the erection regulatory function of PDE5 would be regularized by this treatment and protect against further episodes. METHODS: We administered PDE5 inhibitors using a long-term therapeutic regimen to 3 men with sickle cell disease-associated priapism recurrences and 1 man with idiopathic priapism recurrences. RESULTS: Long-term PDE5 inhibitor treatment alleviated priapism recurrences. CONCLUSIONS: These observations support the hypothesis that PDE5 dysregulation exerts a pathogenic role for priapism associated with hematologic dyscrasias, as well as idiopathic priapism. Although these preliminary findings suggest that continuous, long-term PDE5 inhibitor therapy may be useful as a preventative strategy for priapism, additional evaluation in the form of a controlled clinical trial is needed.

3',5'-Cyclic-GMP Phosphodiesterases↗

Management strategy for arterial priapism: therapeutic dilemmas.

PURPOSE: We present 7 cases of arterial high flow priapism and propose management algorithms for the condition. MATERIALS AND METHODS: We studied 2 children and 5 adults with posttraumatic arterial priapism. Blood gas analysis and color Doppler ultrasonography of the corpora cavernosa confirmed the diagnosis in 4 adults, while 1 patient had already undergone cavernous artery ligation in elsewhere. In the children perineal compression resulted in detumescence, a sign that is proposed to be indicative of the diagnosis of arterial priapism (piesis sign) complementing physical examination. Mechanical compressive force was applied to the perineum of 1 boy, while the other received a watchful waiting program. All adults participated in an observation regimen except 1, who decided to undergo immediate embolization of the internal pudendal artery. RESULTS: Perineal compression led to the resolution of priapism in 1 child, while spontaneous resolution was noted in the other. An adult noticed spontaneous penile detumescence 3 to 4 months after trauma, which was attributable to site specific venous leakage and decreased, inflow in the contralateral cavernous artery. The patient underwent venous surgery and is on an intracavernous injection regimen. Successful embolization of the internal pudendal artery was performed immediately in 1 man and in the other 4 months after trauma due to social inconvenience. Adult patient 3 is still on the watchful waiting protocol (42 months), while the one who underwent cavernous artery ligation is receiving treatment for erectile dysfunction. CONCLUSIONS: Absent of long-term damaging effects of arterial priapism on erectile tissue combined with the possibility of spontaneous resolution or progressive concomitant hemodynamic abnormalities associated with blunt perineal trauma are suggestive of the introduction of an observation period in the management algorithm of high flow priapism. Such a period may help avoid unnecessary intervention and determine the impact of priapism on patient personal life. Perineal compression may be also added as part of the physical examination as a sign specifically indicative of arterial priapism.

Adult↗