Standard Operating Procedures for Priapism

AimTo provide standard operating procedures for the diagnosis and management of priapism.MethodsReview of the literature.Main Outcome MeasuresReduction of priapism and preservation of erectile function.ResultsPriapism is a persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation. Priapism requires prompt evaluation and usually requires emergency management. There are two types of priapism: (i) ischemic (veno-occlusive or low flow), which is found in 95% of cases, and (ii) nonischemic (arterial or high flow). Stuttering (intermittent) priapism is a recurrent form of ischemic priapism. To initiate appropriate management, the physician must determine whether the priapism is ischemic or nonischemic. Necessary diagnostic steps are an accurate history, physical examination, and cavernous blood gas analysis and/or color duplex ultrasonography of the corpora cavernosa. Management of ischemic priapism should achieve resolution as promptly as possible. Initial treatment is therapeutic aspiration with or without irrigation of the corpora. If this fails, intracavernous injection of sympathomimetic drugs is the next step. Surgical shunts should be performed if nonsurgical treatment has failed. The initial management of nonischemic priapism should be observation. Selective arterial embolization is recommended for the management of nonischemic priapism in patients who request treatment. The goal of management for a patient with recurrent (stuttering) priapism is prevention of future episodes.ConclusionManagement of priapism has become increasingly successful as scientific understanding of the pathophysiology and molecular biology of priapism improves. The key to further success in the treatment of priapism is basic research of this uncommon but potentially devastating condition. Burnett AL and Sharlip ID. Standard operating procedures for priapism. J Sex Med **;**:**–**.     

Priapism: Pathogenesis,Epidemiology, and Management

IntroductionPriapism describes a persistent erection arising from dysfunction of mechanisms regulating penile tumescence, rigidity, and flaccidity. A correct diagnosis of priapism is a matter of urgency requiring identification of underlying hemodynamics.AimsTo define the types of priapism, address its pathogenesis and epidemiology, and develop an evidence-based guideline for effective management.MethodsSix experts from four countries developed a consensus document on priapism; this document was presented for peer review and debate in a public forum and revisions were made based on recommendations of chairpersons to the International Consultation on Sexual Medicine. This report focuses on guidelines written over the past decade and reviews the priapism literature from 2003 to 2009. Although the literature is predominantly case series, recent reports have more detailed methodology including duration of priapism, etiology of priapism, and erectile function outcomes.Main Outcome MeasuresConsensus recommendations were based on evidence-based literature, best medical practices, and bench research.ResultsBasic science supporting current concepts in the pathophysiology of priapism, and clinical research supporting the most effective treatment strategies are summarized in this review.ConclusionsPrompt diagnosis and appropriate management of priapism are necessary to spare patients ineffective interventions and maximize erectile function outcomes. Future research is needed to understand corporal smooth muscle pathology associated with genetic and acquired conditions resulting in ischemic priapism. Better understanding of molecular mechanisms involved in the pathogenesis of stuttering ischemic priapism will offer new avenues for medical intervention. Documenting erectile function outcomes based on duration of ischemic priapism, time to interventions, and types of interventions is needed to establish evidence-based guidance. In contrast, pathogenesis of nonischemic priapism is understood, and largely attributable to trauma. Better documentation of onset of high-flow priapism in relation to time of injury, and response to conservative management vs. angiogroaphic or surgical interventions is needed to establish evidence-based guidance. Broderick GA, Kadioglu A, Bivalacqua TJ, Ghanem H, Nehra A, and Shamloul R. Priapism: Pathogenesis, epidemiology and management.     

Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy

IntroductionPriapism describes a persistent erection lasting longer than 4 hours. Ischemic priapism and stuttering priapism are phenotypic manifestations of sickle‐cell disease (SCD).AimsTo define the types of priapism associated with SCD, to address pathogenesis, and to recommend best practices.SourcesLiterature review and published clinical guidelines.Summary of FindingsPriapism is a full or partial erection that persists more than 4 hours. There are three kinds of priapism: ischemic priapism (veno‐occlusive, low flow), stuttering priapism (recurrent ischemic priapism), and nonischemic priapism (arterial, high flow). Ischemic priapism is a pathologic phenotype of SCD. Ischemic priapism is a urologic emergency when untreated priapism results in corporal fibrosis and erectile dysfunction. The recommended treatment for ischemic priapism is decompression of the penis by needle aspiration and if needed, injection (or irrigation) with dilute sympathomimetic drugs. Stuttering priapism describes a pattern of recurring unwanted painful erections in men with SCD. Patients typically awaken with an erection that persists for several hours and becomes painful. The goals of managing stuttering ischemic priapism are: prevention of future episodes, preservation of erectile function, and balancing the risks vs. benefits of various treatment options. The current molecular hypothesis for stuttering priapism in SCD proposes that insufficient basal levels of phosphodiesterase type‐5 are available in the corpora to degrade cyclic guanosine monophosphate (cGMP). Nocturnal erections result from normal neuronal production and surges of cGMP. In the context of SCD stuttering priapism, these nocturnal surges in cGMP go unchecked, resulting in stuttering priapism.ConclusionsConsidering the embarrassing nature of the problem and the dire consequences to erectile function, it is important to inform patients, parents, and providers about the relationship of SCD to prolonged painful erections. Prompt diagnosis and appropriate medical management of priapism are necessary to spare patients surgical interventions and preserve erectile function. Broderick GA. Priapism and sickle‐cell anemia: Diagnosis and nonsurgical therapy. J Sex Med 2012;9:88–103.     

Clinical Outcomes of Periprocedural Antithrombotic Therapy in Ischemic Priapism Management

BackgroundPriapism 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.AimTo determine the short- and long-term outcomes of periprocedural ATT in the management of acute ischemic priapism.MethodsA 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.OutcomesATT use was the exposure of interest; outcome variables included priapism resolution, repeat episodes, long-term complications, and follow-up.Results70 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 ImplicationsOur 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 requiredStrengths & LimitationsThis 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.ConclusionIn 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.     

Priapism in sickle-cell disease: a hematologist's perspective

IntroductionPriapism is a familiar problem to hematologists, well known for its association with sickle‐cell disease (SCD). It also occurs in a variety of other hematological illnesses, nearly all forms of congenital hemolytic anemia, including other hemoglobinopathies and red blood cell membranopathies and enzymopathies.AimProvide urologists with a comprehensive review of priapism in SCD, with an emphasis on the perspective of a practicing hematologist.MethodsMedline searches through July 2010 were conducted using the terms priapism, erectile dysfunction, and sickle cell.Main Outcome MeasuresExpert opinion was based on review of the medical literature related to this subject matter.ResultsIn men with SCD, large epidemiological studies have linked the risk of priapism to clinical markers of the severity of intravascular hemolysis. Extracellular hemoglobin and arginase released during hemolysis has been implicated in reducing nitric oxide bioavailability, although the relevance of hemolysis to vascular dysfunction has been challenged by some scientists. Consistent with the role of impairment of the nitric oxide axis, mice genetically deficient in nitric oxide production have also been shown to develop priapic activity. Provocative new data indicate that hemolysis‐linked dysregulation of adenosine signaling in the penis contributes to priapism in sickle cell mice. Serious questions have arisen regarding the efficacy of mainstays of textbook dogma for treatment of acute severe priapism, including intravenous fluids, alkalinization, and exchange transfusion, and there is increasing acceptance for early aspiration and irrigation of the corpus cavernosum.ConclusionFor patients with sickle cell with recurrent priapism, there is very limited evidence for a medical prophylaxis role for hydroxyurea, etilefrine, pseudoephedrine, leuprolide, sildenafil, and other agents. Recent publications have highlighted nitric oxide and adenosine signal transduction pathways as worthy of additional research. Research and clinical management of sickle‐cell priapism is strengthened by multidisciplinary collaboration between hematologists and urologists. Kato GJ. Priapism in sickle‐cell disease: A hematologist's perspective. J Sex Med 2012;9:70–78.     

New-onset priapism associated with ingestion of terazosin in an otherwise healthy man

IntroductionPriapism has been reported as a rare effect of the commonly used alpha 1-antagonists through direct inhibition of the sympathetic input necessary for detumescence. Although previously reported as an adverse event in a patient with spinal cord injury, to the best of our knowledge, terazosin-induced priapism in an otherwise healthy man has not been previously described.AimWe describe an otherwise healthy man with lower urinary symptoms who developed priapism after ingestion of the commonly prescribed alpha-blocker terazosin.ResultsThe priapism resolved after a combination of cavernosal aspiration and alpha-agonist administration.ConclusionPriapism is an extremely rare side effect of alpha-blocker therapy and has previously been described in association with other alpha-blockers, as well as with terazosin, in a spinal cord-injured patient. We report a case of priapism specifically associated with terazosin prescribed for lower urinary tract symptoms in an otherwise healthy man. Sadeghi-Nejad H, and Jackson I. New-onset priapism associated with ingestion of terazosin in an otherwise healthy man.     

Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study

IntroductionPriapism is the persistent and painful erection of the penis and is a common sickle cell disease (SCD) complication.AimThe goal of this study was to characterize clinical and genetic factors associated with priapism within a large multi-center SCD cohort in Brazil.MethodsCases with priapism were compared to SCD type-matched controls within defined age strata to identify clinical outcomes associated with priapism. Whole blood single nucleotide polymorphism genotyping was performed using a customized array, and a genome-wide association study (GWAS) was conducted to identify single nucleotide polymorphisms associated with priapism.Main Outcome MeasureOf the 1,314 male patients in the cohort, 188 experienced priapism (14.3%).ResultsPriapism was more common among older patients (P = .006) and more severe SCD genotypes such as homozygous SS (P < .0001). In the genotype- and age-matched analyses, associations with priapism were found for pulmonary hypertension (P = .05) and avascular necrosis (P = .01). The GWAS suggested replication of a previously reported candidate gene association of priapism for the gene transforming growth factor beta receptor 3 (TGFBR3) (P = 2 × 10^?4).Clinical ImplicationsOlder patients with more severe genotypes are at higher risk of priapism, and there is a lack of consensus on standard treatment strategies for priapism in SCD.Strengths & LimitationsThis study characterizes SCD patients with any history of priapism from a large multi-center cohort. Replication of the GWAS in an independent cohort is required to validate the results.ConclusionThese findings extend the understanding of risk factors associated with priapism in SCD and identify genetic markers to be investigated in future studies to further elucidate priapism pathophysiology.Ozahata M, Page GP, Guo Y, et al. Clinical and Genetic Predictors of Priapism in Sickle Cell Disease: Results from the Recipient Epidemiology and Donor Evaluation Study III Brazil Cohort Study. J Sex Med 2019;16:1988–1999.     

Priapism as a Result of Chronic Myeloid Leukemia: Case Report,Pathology, and Review of the Literature

IntroductionPriapism is rare‐presenting feature in male patients with chronic myeloid leukemia (CML). Several hypotheses for pathogenesis have been described. Management has been controversial; some authors described resolution following priapism‐specific interventions, and others recommended addition of CML‐specific therapy or even CML‐specific therapy alone.AimIn this report, we describe presentation and management of a man with refractory priapism that was the first presenting manifestation of CML. We also report, for the first time, the pathology sections of the sinusoidal tissue in such cases. Literature is reviewed for similar cases and their outcome.MethodsA 21‐year‐old male patient presented with painful priapism that started 6 days earlier and failed aspiration–irrigation. CBC revealed marked leucocytosis. Oncology care diagnosed CML, and treatment with Imatinib was commenced with prior semen cryopreservation. Following remission, a penile prosthesis was implanted, assisted by optical corporotomy. Sinusoidal tissue biopsy was stained by hematoxylin/eosin (H&E) and CD34.Main Outcome MeasuresPathology sections of cavernous tissue following CML‐induced priapism.ResultsThe penile implant survived without complications. H&E examination of the sinusoidal tissue biopsy revealed leukemic infiltration associated with vascular endothelial damage. CD34 staining showed the mixed picture of leukemic infiltrates, intact vascular endothelium with lumena showing leukemic cells, alternating with destroyed vessels, and no vascular lumena and ruminants of endothelial cells.ConclusionPriapism can be the first manifestation of previously undetected CML. The pathological picture of sinusoidal tissue in such cases is presented. In the case at hand, a complete blood picture was helpful in early diagnosis of CML and early initiation of targeted chemotherapy along with the corporal irrigation/aspiration or shunt surgery. It is therefore recommended to have a CBC examined at presentation of any case of ischemic priapism of unknown etiology, early initiation of CML therapy along with aspiration/irrigation, preferably cryopreserving a semen sample before CML therapy. Shaeer OKZM, Shaeer KZM, AbdelRahman IFS, El‐Haddad MS, and Selim OM. Priapism as a result of chronic myeloid leukemia: Case report, pathology, and review of the literature. J Sex Med 2015;12:827–834.     

Valsalva Maneuver-induced Priapism: A Hidden Culprit

IntroductionPriapism is defined as an abnormal prolonged penile erection without sexual interest and failure to subside despite orgasm. The disorder is enigmatic yet devastating because of its elusive etiology, irreversible erectile tissue damage, and resultant erectile dysfunction. A wide variety of provocative factors have been implicated in different types of priapism; however, myelopathy-related ischemic priapism induced by the Valsalva maneuver in the context of spinal extradural arachnoid cyst has never been described.AimTo report a case with spinal extradural arachnoid cyst heralded by Valsalva maneuver-induced priapism and review the mechanistic basis for acute myelopathy-related priapism.MethodsThe case report profiled a 42-year-old Chinese man presenting with ischemic priapism following in-flight Valsalva maneuver for unblocking the ears during descent. Magnetic resonance imaging unveiled the hidden culprit behind myelopathy-related priapism, as demonstrated by acute spinal cord compression from a giant extradural arachnoid cyst.ResultsThe symptoms subsided rapidly after treatment with ice packing, analgesics, and corporal irrigation with diluted epinephrine. However, surgical removal of the extradural arachnoid cyst failed to achieve a complete recovery of neurological deficits. After 1 year of follow-ups, he still experienced a mild weakness and hypesthesia of the right leg but no further episodes of priapism or sexual dysfunction.ConclusionsMyelopathy-related priapism potentiated by the Valsalva maneuver can be easily overlooked without heightened vigilance, leading to poor therapeutic response and prognosis. The indolent nature of spinal extradural arachnoid cyst should be reinforced and better outcomes can only be achieved through expeditious diagnosis and management. Chen WL, Tsai WC, and Tsao YT. Valsalva maneuver-induced priapism: A hidden culprit. J Sex Med **;**:**–**.     

In vitro and in vivo animal models in priapism research

IntroductionPriapism is an enigmatic yet devastating clinical phenomenon. In the last two decades, the use of various animal models to study this disorder has dramatically advanced our understanding of this mysterious disorder.AimThis report reviews various animal models used to study ischemic priapism and informs basic science researchers the broad view of priapism research.MethodsRetrospective review of pertinent literature from the last two decades via PubMed search using the keywords “ischemic priapism” and “priapism model.”Main Outcome MeasuresFindings on the animal models used in ischemic priapism research and its advantages and limitations.ResultsIn vitro and in vivo animal models varying from dogs, cats, rabbits, rats to mice were used in priapism research. In vitro models included: (i) corpora cavernosa smooth muscle (CCSM) strip in organ bath; (ii) corporal tissue binding assay; (iii) CCSM cell culture under hypoxia/anoxia. In vivo models could be categorized as: (i) pharmacologically induced by corpus cavernosum medicine injection; (ii) ventilation induced by tidal volume control; (iii) mechanical induced by a constrictor band placed around the base of the penis combined with induced erection; (iv) genetic engineered by intracorporal gene transfer, transgenic, or gene knock‐out.ConclusionsThe ischemic priapism animal models are shifting from pharmaceutically or mechanically induced to genetically engineered. The knowledge generated by those models is enhancing our understanding and management of this clinical challenge. Dong Q, Deng S, Wang R, and Yuan J. In vitro and in vivo animal models in priapism research.     

Conversion of Low‐Flow to High‐Flow Priapism: A Case Report and Review (CME)

Lutz A, LaCour S, and Hellstrom W. Conversion of low‐flow to high‐ flow priapism: A case report and review. J Sex Med 2012;9:951–954Priapism is defined as an erection lasting greater than 4 hours. It is grouped into three subtypes: ischemic (low flow), nonischemic (high flow), and stuttering priapism. We present an interesting case and review of the conversion from a low‐flow to a high‐flow priapism. This conversion has rarely been reported. It has resulted from treatment of low‐flow priapism with minimally invasive procedures including injection therapy as well as more invasive distal and proximal shunt procedures. Penile Doppler ultrasound and angiogram are particularly important to the clinician in the diagnosis and treatment of this rare entity.     

Nationwide Emergency Department Visits for Priapism in the United States

IntroductionThe epidemiology of priapism is not well characterized. A small number of studies based on inpatient data or small population samples have estimated the incidence to range from 0.34 to 1.5 cases per 100,000 males.AimTo estimate the current epidemiology and impact on resource utilization of priapism in the United States (US).Main Outcome MeasuresRate of emergency department encounters for priapism in the US.MethodsEmergency department (ED) visits for priapism were analyzed using discharge data from the Nationwide Emergency Department Sample (NEDS), Healthcare Cost and Utilization Project (HCUP). Priapism encounters were identified by ICD9 code. Priapism encounters were analyzed for patient and hospital characteristics, associated diagnoses, and hospital charge. Established weighting in the sample was used to calculate nationwide estimates.ResultsA total of 8,738 ED encounters for priapism were identified between 2006 and 2009 in the NEDS. This translated to an estimated 39,964 encounters out of a total of 496,195,793 ED visits, or 8.05 per 100,000 ED visits (95% confidence interval [CI] 7.59–8.51). 21.1% of patients had a concurrent diagnosis of sickle cell disease (SCD). 72.1% of all patients were discharged home from the ED, while only 49.6% of patients with SCD were discharged home. A concurrent diagnosis of SCD was associated with an odds ratio (OR) of 3.84 (95% CI 3.65–4.05) for admission to the hospital when controlling for age, region, hospital and payer type. The mean hospital charge was $1,778 per encounter if discharged home and $41,909 per encounter if admitted. The estimated mean total annual charge for priapism was $123,860,432 with 86.8% of charges attributed to inpatient admissions.ConclusionsOur estimate of the rate of ED visits for priapism was significantly higher than prior estimates with a SCD concurrence rate lower than previously estimated. Stein DM, Flum AS, Cashy J, Zhao LC, and McVary KT. Nationwide emergency department visits for priapism in the United States. J Sex Med 2013;10:2418–2422.     

Surgical management of ischemic priapism

IntroductionSurgery is a mainstay in the management of ischemic priapism. The surgical armamentarium for this condition has recently been expanded with the introduction of several innovative procedures.AimTo review surgical procedures offered in the treatment of ischemic priapism and present a rational framework for their use.MethodsMedline searches through July 2010 were conducted using the terms priapism, surgery, shunt, and prosthesis.Main Outcome MeasureExpert opinion was based on review of the medical literature related to this subject matter.ResultsA host of surgical procedures exist to address the genital complications of both acute presentations of ischemic priapism and its non‐acute pathologic sequelae, which include penile deformities and erectile dysfunction. For the former, the intervention is used principally in an emergent context with the intention to relieve the acute pathologic effects of the condition and preserve erectile function. For the latter, the intervention is aimed generally toward restoring anatomic normalcy and the functional ability to perform sexual intercourse. A rational framework for surgical management, based on the circumstances of the clinical presentation, is described.ConclusionsThe surgical management for ischemic priapism has evolved with the application of a host of surgical procedures. These procedures address acute and non‐acute genital complications of the condition and are intended to retain or restore sexual ability effectively and safely. They can be applied using a rational clinical management framework. Burnett AL. Surgical management of ischemic priapism. J Sex Med 2012;9:114–120.     

Adenosine Deaminase Enzyme Therapy Prevents and Reverses the Heightened Cavernosal Relaxation in Priapism

IntroductionPriapism featured with painful prolonged penile erection is dangerous and commonly seen in sickle cell disease (SCD). The preventive approaches or effective treatment options for the disorder are limited because of poor understanding of its pathogenesis. Recent studies have revealed a novel role of excess adenosine in priapism caused by heightened cavernosal relaxation, and therefore present an intriguing mechanism-based therapeutic possibility.AimThe aim of this study was to determine the therapeutic effects of adenosine deaminase (ADA) enzyme therapy to lower adenosine in priapism.MethodsBoth ADA-deficient mice and SCD transgenic (Tg) mice display priapism caused by excessive adenosine. Thus, we used these two distinct lines of mouse models of priapism as our investigative tools. Specifically, we treated both of these mice with different dosages of polyethylene glycol–modified ADA (PEG–ADA) to reduce adenosine levels in vivo. At the end points of the experiments, we evaluated the therapeutic effects of PEG–ADA treatment by measuring adenosine levels and monitoring the cavernosal relaxation.Main Outcome MeasuresAdenosine levels in penile tissues were measured by high-performance liquid chromatography, and cavernosal relaxation was quantified by electrical field stimulation (EFS)-induced corporal cavernosal strip (CCS) assays.ResultsWe found that lowering adenosine levels in penile tissues by PEG–ADA treatment from birth in ADA-deficient mice prevented the increased EFS-induced CCS relaxation associated with priapism. Intriguingly, in both ADA-deficient mice and SCD Tg mice with established priapism, we found that normalization of adenosine levels in penile tissues by PEG–ADA treatment relieved the heightened EFS-induced cavernosal relaxation in priapism.ConclusionsOur studies have identified that PEG–ADA is a novel, safe, and mechanism-based drug to prevent and correct excess adenosine-mediated increased cavernosal relaxation seen in two independent priapic animal models, and suggested its therapeutic possibility in men suffering from priapism. Wen J, Jiang X, Dai Y, Zhang Y, Tang Y, Sun H, Mi T, Kellems RE, Blackburn MR, and Xia Y. Adenosine deaminase enzyme therapy prevents and reverses the heightened cavernosal relaxation in priapism.     

New insights into the pathophysiology of sickle cell disease-associated priapism

IntroductionPriapism is defined as an erectile disorder, in which erection persists uncontrollably without sexual purpose. The precise mechanisms involved in the development of sickle cell disease‐associated priapism are ill defined.AimTo summarize the recent developments that increase our understanding of the molecular mechanisms of priapism.MethodsThis article reviews the literature (Medline search 2000–2010) that relates the key molecular signaling pathways that contribute to the development of priapism associated with sickle‐cell disease. It focuses on basic science investigations using multiple animal models.Main Outcome MeasuresThe reader will be informed of the most current research regarding the role of endothelial nitric oxide synthase, phosphodiesterase type 5 (PDE5), adenosine, RhoA/Rho‐kinase (ROCK), and opiorphins in the pathophysiology of priapism.ResultsNew concepts in the field of priapism research suggest that priapism often results from altered vascular homeostatic actions in the penis and is associated with deficient erection control mechanisms on a molecular level. A leading proposal in this regard is the notion of aberrant signaling of the endothelium‐derived nitric oxide and PDE5 signal transduction pathway in the penis. Additionally, dysfunctional regulatory control of signal transduction systems which interact with this pathway such as adenosine and RhoA/Rho‐kinase may contribute to the development of priapism. Recent investigations of opiorphins also demonstrate a role in regulating corporal smooth muscle tone and thereby dysregulation of erection physiology in priapism. These advances have paved the way for understanding this disorder as having a molecular pathogenesis.ConclusionsAs the science underlying priapism further emerges, increasingly effective therapeutics for sickle cell disease‐associated priapism is certain to follow. Bivalacqua TJ, Musicki B, Kutlu O, and Burnett AL. New insights into the pathophysiology of sickle cell disease‐associated priapism. J Sex Med 2012;9:79–87.     

Potential risks of chronic sildenafil use for priapism in sickle cell disease

IntroductionPriapism is a common concern in sickle cell disease. With a high frequency of recurrences and serious long‐term sequela, a preventative, rather than traditionally reactive approach, needs to be taken in these patients. Reports have shown successful use of sildenafil as a prophylactic treatment but have failed to address adverse outcomes, including vasoocclusive pain crises, of chronic sildenafil therapy in sickle cell patients.AimsWe wish to draw attention to the potential adverse outcomes of this therapy on the overall state of the patient's disease for consideration in future studies.MethodsWe used sildenafil in a patient suffering from almost daily attacks of priapism.ResultsSildenafil was successful in decreasing the frequency of priapism; however, our patient experienced an increased frequency of vasoocclusive crises, something not previously addressed.ConclusionFuture studies of sildenafil use in sickle cell disease need to assess the global state of the disease, not just the frequency of priapism. Lane A and Deveras R. Potential risks of chronic sildenafil use for priapism in sickle cell disease. J Sex Med 2011;8:3193–3195.     

Emergency Management of Priapism in the United Kingdom: A Survey of Current Practice

Background

Despite its importance, current practice in the emergency management of priapism in the United Kingdom is unknown.

Corporal “Snake” Maneuver: Corporoglanular Shunt Surgical Modification for Ischemic Priapism

IntroductionCurrent surgical shunting procedures for major ischemic priapism do not always effectively resolve acute presentations of this disorder.AimTo evaluate a modification of the Al-Ghorab distal penile corporoglanular shunt surgery for ischemic priapism.MethodsThree previously potent men (48, 43, 40 years of age) presented with major ischemic priapism episodes (5, 2, and 6 days in duration, respectively), which were refractory to clinical management including sympathomimetic intracavernosal treatments, intracorporal aspiration and saline irrigation, and penile shunt surgery attempts. We offered a surgical technique for facilitating corporal blood evacuation by retrograde insertion of a cavernosal dilator through the excised tunical windows of the distal corpora cavernosa after transglanular incision.Main Outcome MeasuresClinical evaluation of priapism resolution and erection recovery.ResultsAll men achieved successful resolution of priapism, with meaningful erection recovery assessable in one man.ConclusionsThe modified Al-Ghorab corporoglanular shunt surgery appears to offer an advantageous management approach to resolve ischemic priapism, particularly for cases refractory to first-line management. Burnett AL, and Pierorazio PM. Corporal “snake” maneuver: Corporoglanular shunt surgical modification for ischemic priapism. J Sex Med **;**:**–**.