Ultrasound guidance for real-time management of acute ischemic priapism.

Ischemic priapism is a relatively uncommon genitourinary condition that, if left untreated, can lead to permanent erectile dysfunction. Detumescence should ideally be attained within the first 36 h of onset to avoid irreversible fibrosis and necessary surgery. Opportunities to practice medical management of this condition are scarce, and the risk of iatrogenic injury of vessels, nerves, and urethra can be significant if performed blind. Visualizing these structures through ultrasonography can reduce the risk of injury and aid in the confirmation of drug delivery. This novel utilization of ultrasound guidance in active treatment can help improve physician confidence and success in managing this rare and urgent condition. To our knowledge, this is the first report of point-of-care ultrasound-guided penile nerve block used to manage pain associated with priapism. We present a 44-year-old male presenting with a painful erection lasting for eight hours. Penile doppler ultrasound was performed concurrent with medical management of priapism, with successful detumescence and discharge.

New Horizons in Emergency Department Management of Pediatric Sickle Cell Disease.

ABSTRACT: Sickle cell disease (SCD) is an important topic for emergency medicine audiences because complications of the disease account for a large proportion of hematologic emergencies that are seen in the emergency department each year. Early recognition and aggressive management of emergency complications of SCD can help to reduce the morbidity and mortality associated with this disease. Although the treatment recommendations for some complications of SCD are based on expert opinion, there has been advancement in the understanding of the pathogenesis of the disease and evidence regarding the treatment options available for managing acute complications. This continuing medical education article will provide a summary of the clinical manifestation and management of the most common acute complications of SCD: infection, vaso-occlusive episode, acute chest syndrome, splenic sequestration, stroke, and priapism.

Delayed placement of an inflatable penile prosthesis is associated with a high complication rate in men with a history of ischemic priapism.

BACKGROUND: Corporal fibrosis is known to result from prolonged priapism; however, the impact of the timing of penile prosthesis placement after priapism on complication rates is poorly understood. AIM: We sought to evaluate the impact of timing of inflatable penile prosthesis (IPP) placement on complications in men with a history of ischemic priapism. METHODS: We performed a multicenter, retrospective cohort study of patients with a history of priapism undergoing IPP placement by 10 experienced implantation surgeons. We defined early placement as ≤6 months from priapism to IPP. We identified a 1:1 propensity-matched group of men without a history of priapism and compared complication rates between men who had early placement, late placement, and no history of priapism. OUTCOMES: Our primary outcome was postoperative noninfectious complications, and secondary outcomes included intraoperative complications and postoperative infection. RESULTS: A total of 124 men were included in the study with a mean age of 50.3 ± 12.7 years. A total of 62 had a history of priapism and 62 were matched control subjects. The median duration of priapism was 37 (range, 3-168) hours and the median time from ischemic priapism to IPP placement was 15 months (range, 3 days to 23 years). Fifteen (24%) men underwent early (≤6 months) IPP placement at a median time of 2 months (range, 3 days to 6 months) following the ischemic priapism event. The remaining 47 (76%) underwent placement >6 months following priapism at a median time of 31.5 months (range, 7 months to 23 years). The complication rate in the delayed placement group was 40.5% compared with 0% in the early placement group and control group. Cylinder-related complications such as migration or leak accounted for 8 (57%) of 14 of the postoperative noninfectious complications. Full-sized cylinders were used in all patients who had a cylinder related complication. CLINICAL IMPLICATIONS: Priapism patients should be referred to prosthetic experts early to decrease complication rates in those needing an IPP. STRENGTHS AND LIMITATIONS: This is a multicenter study from experienced prosthetic urologists but is limited by the retrospective nature and small number of patients in the early placement group. CONCLUSION: IPP complication rates are high in men with a history of ischemic priapism, especially when implantation is delayed beyond 6 months.

Association between priapism and HIV disease and treatment.

BACKGROUND: Priapism, a urologic emergency, has known associations with certain medical conditions. Many cases are idiopathic, suggesting an opportunity to identify novel risk factors. AIM: We sought to identify medical conditions and pharmaceutical treatments that are associated with priapism using data-mining techniques. METHODS: Using deidentified data in a large insurance claims database, we identified all men (age ≥20 years) with a diagnosis of priapism from 2003 to 2020 and matched them to cohorts of men with other diseases of male genitalia: erectile dysfunction, Peyronie disease, and premature ejaculation. All medical diagnoses and prescriptions used prior to first disease diagnosis were examined. Predictors were selected by random forest, and conditional multivariate logistic regressions were applied to assess the risks of each predictor. OUTCOMES: We identified novel relationships of HIV and some HIV treatments with priapism and confirmed existing associations. RESULTS: An overall 10 459 men with priapism were identified and matched 1:1 to the 3 control groups. After multivariable adjustment, men with priapism had high associations of hereditary anemias (odds ratio [OR], 3.99; 95% CI, 2.73-5.82), use of vasodilating agents (OR, 2.45; 95% CI, 2.01-2.98), use of HIV medications (OR, 1.95; 95% CI, 1.36-2.79), and use of antipsychotic medications (OR, 1.90; 95% CI, 1.52-2.38) as compared with erectile dysfunction controls. Similar patterns were noted when compared with premature ejaculation and Peyronie disease controls. CLINICAL IMPLICATIONS: HIV and its treatment are associated with priapism, which may affect patient counseling. STRENGTHS AND LIMITATIONS: To our knowledge, this is the first study to identify risk factors for priapism utilizing machine learning. All men in our series were commercially insured, which limits the generalizability of our findings. CONCLUSION: Using data-mining techniques, we confirmed existing associations with priapism (eg, hemolytic anemias, antipsychotics) and identified novel relationships (eg, HIV disease and treatment).

Erectile response profiles of men using PDE5 inhibitors combined with intracavernosal injections as part of a penile rehabilitation program after radical prostatectomy.

BACKGROUND: Phosphodiesterase type 5 (PDE5) inhibitor labeling states that these agents should not be used in conjunction with other erectogenic medications for fear of priapism occurring. AIM: We explored the risk of priapism and prolonged erections in men in our post-radical prostatectomy (RP) penile injection program who were using regular PDE5 inhibitor and intracavernosal injections (ICIs) as part of their rehabilitation program. METHODS: The study cohort included men on penile injection therapy who (1) were taking tadalafil 5 mg daily or taking sildenafil 25 mg on noninjection days, (2) had an RP, (3) were using their respective PDE5 inhibitor regularly at the time of penile injection training, and (4) complied with the program instructions regarding penile injection use. Demographics, comorbidity details, PDE5 inhibitor dose and utilization, and injection dose and utilization data were collected. All patients underwent in-office injection training and used trimix (papaverine/phentolamine/prostaglandin E1) as the intracavernosal medication. OUTCOMES: Priapism was defined as a patient self-reported penetration hardness erection ≥4 hours in duration, while prolonged erection was defined as a penetration hardness erection lasting ≥2 hours. RESULTS: A total of 112 tadalafil users and 364 sildenafil users were compared. Mean age and duration post-RP were 62 ± 14 years and 5.2 ± 12 months, respectively, and there was no difference between tadalafil and sildenafil groups. The mean trimix dose was tadalafil 24 ± 24 units and sildenafil 31 ± 37 units (P < .05). Priapism occurred in 2 (1.7%) of 112 tadalafil users and 5 (1.4%) of 364 sildenafil users (P = .47). Excluding those men experiencing priapism on any occasion, those with any reported penetration hardness erection lasting ≥2 hours were 7 (6.3%) of 112 tadalafil users and 12 (3.3%) of 364 sildenafil users (P < .01). A total of 53% of these prolonged erections occurred within the first 6 injections at home (no difference between tadalafil and sildenafil groups). CLINICAL IMPLICATIONS: We emphasize the need for continued monitoring and education on proper injection techniques to minimize the risk of adverse events in ICI and PDE5 inhibitor combination therapy. STRENGTHS & LIMITATIONS: This study has a relatively large patient population with a considerable follow-up time. Additionally, the rigorous training, education, and monitoring of the participants, as well as the use of formal definitions for priapism and prolonged erections, enhances the accuracy and reliability of the results. However, there are some limitations, such as social desirability, confounding factors, and recall bias. CONCLUSION: There is no significant difference in the incidence of priapism in an ICI program in which men combine ICI with tadalafil or sildenafil. However, tadalafil patients had a higher rate of prolonged erections, which was found to occur mostly early during the titration phase.

Analysis of the Causes of Newborn Priapism: A Retrospective Clinical Study.

Priapism is a rare condition in the newborn. The aim of this study was to investigate the demographic, etiologic and clinical features of neonatal priapism. We retrospectively analysed the data of 11 patients diagnosed with neonatal priapism in the neonatal intensive care unit between 2000 and 2019. Priapism was defined as an erection in the neonatal period, lasting more than 4 hours. Etiological examinations revealed polycythemia in one (9.09%) patient, D-dimer elevation in three patients, and heterozygous methyltetrahydrofolate 667 gene mutations in one patient. Other patients were considered idiopathic. Detumescence was achieved in all 11 (100%) patients during the follow-up period. The median hospitalization duration was 6 (IQR [4, 8]; range, 2-9) days. The median follow-up duration was 38 (IQR [30, 42]; range, 13-94) months for patients followed-up in our hospital after discharge. Neonatal priapism is a rare condition. Successful treatment results can be achieved with conservative methods. Data acquired from our study showed that diseases with a tendency to hypercoagulation belong to the etiology by damaging penile microcirculation and make the response to conservative treatment more challenging.

[Non-ischemic priapism (post-traumatic arteriovenous fistula of the right cavernous body) in the background of Landusy-Dejerine myopathy].

Landouzy-Dejerine myopathy (Facial-shoulder-shoulder myodystrophy) is a disease which causes weakness of the muscles of the shoulder girdle, back and hip muscles, which predisposes patients to an increased risk of injury and disability. The article presents a clinical observation of non-ischemic priapism, which developed as a result of perineal trauma with the formation of a fistula of the right cavernous body in a patient against the background of facial shoulder-shoulder myodystrophy. Methods for the differential diagnosis of this condition are also discussed.

The Diagnosis and Management of Recurrent Ischemic Priapism, Priapism in Sickle Cell Patients, and Non-Ischemic Priapism: An AUA/SMSNA Guideline.

PURPOSE: Priapism is a persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation and results in a prolonged and uncontrolled erection. Given its time-dependent and progressive nature, priapism is a situation that both urologists and emergency medicine practitioners must be familiar with and comfortable managing. METHODOLOGY: A comprehensive search of the literature on acute ischemic priapism and non-ischemic priapism (NIP) was performed by Emergency Care Research Institute for articles published between January 1, 1960 and May 1, 2020. A search of the literature on NIP, recurrent priapism, prolonged erection following intracavernosal vasoactive medication, and priapism in patients with sickle cell disease was conducted by Pacific Northwest Evidence-based Practice Center for articles published between 1946 and February 19, 2021. Searches identified 4117 potentially relevant articles, and 3437 of these were excluded at the title or abstract level for not meeting inclusion criteria. Full texts for the remaining 680 articles were ordered, and ultimately 203 unique articles were included in the report. RESULTS: This Guideline provides a clinical framework for the treatment (non-surgical and surgical) of NIP, recurrent ischemic priapism, and priapism in patients with sickle cell disease. The treatment of patients with a prolonged erection following intracavernosal vasoactive medication is also included. The AUA guideline on the diagnosis of priapism and the treatment of acute ischemic priapism was published in 2021. CONCLUSIONS: All patients with priapism should be evaluated emergently to identify the sub-type of priapism (acute ischemic versus non-ischemic) and those with an acute ischemic event should be provided early intervention when indicated. NIP is not an emergency and treatment must be based on patient objectives, available resources, and clinician experience. Management of recurrent ischemic priapism requires treatment of acute episodes and a focus on future prevention of an acute ischemic event. Sickle cell disease patients presenting with an acute ischemic priapism event should initially be managed with a focus on urologic relief of the erection; standard sickle cell assessment and interventions should be considered concurrent with urologic intervention. Treatment protocols for a prolonged, iatrogenic erection must be differentiated from protocols for true priapism.

A Rare Cause of Autoinflation after Penile Prosthesis Insertion: Case Series and Systematic Review.

BACKGROUND: In 2016, we reported the first case of high flow priapism and arteriocavernosal fistula caused by penile prosthesis insertion that mimicked device autoinflation. AIM: To raise awareness amongst implanters, we describe further cases from our institution and perform a systematic review of the literature to understand the rarity of this phenomenon. METHODS: Patient demographics, management and outcomes were extracted retrospectively. A systematic search of the EMBASE, PubMed and PubMed Central libraries for studies reporting arteriocavernosal fistula mimicking autoinflation since 1946 was performed. OUTCOMES: To identify and report all known cases of high flow priapism and arteriocavernosal fistula presenting as autoinflation of an inflatable penile prosthesis. RESULTS: Four patients in total (median age 56, range 46-60 years) were identified. Catastrophic bleeding (1.8L) occurred during revision surgery for presumed autoinflation in Patient 1 and subsequent ultrasound (US) confirmed a fistula which was embolized. Patient 2 redeveloped autoinflation following revision surgery. Ultrasound confirmed high flow priapism from an arteriocavernosal fistula. Patient 3 underwent penile magnetic resonance imaging (MRI) to investigate autoinflation and residual penile curvature. MRI showed a tumescent penis despite a deflated device and the fistula was embolized successfully. Patient 4 with sleep-related painful erections did not improve following insertion of penile prosthesis. Doppler US identified 2 fistulae that was embolized but with no resolution of symptoms. Subsequent embolization of both common penile arteries were done to control his symptoms. No other publications apart from the published abstract from 2016 reporting patient 1 was found. CLINICAL IMPLICATIONS: If considered prior to revision surgery, the fistula can be managed safely by minimally invasive percutaneous angioembolisation avoiding surgery which can potentially be associated with significant complications. STRENGTHS AND LIMITATIONS: The rarity of this phenomenon was supported by a systematic review. Our study however does present the findings from a small number of patients. CONCLUSION: Damage to the cavernosal artery during inflatable penile prosthesis insertion can create an arteriocavernosal fistula that mimics autoinflation, leading to catastrophic intra-operative bleeding or unnecessary surgery. Lee WG, Satchi M, Skrodzka M, et al. A Rare Cause of Autoinflation after Penile Prosthesis Insertion: Case Series and Systematic Review. J Sex Med 2022;19:879-886.

Priapism caused by partial deficiency of tetrahydrobiopterin through hypofunction of the sympathetic neurons in sepiapterin reductase gene-disrupted mice.

6R-L-erythro-5,6,7,8-tetrahydrobiopterin (BH4) is an essential cofactor for aromatic L-amino acid hydroxylases, including tyrosine hydroxylase (TH), alkylglycerol monooxygenase, and three types of nitric oxide (NO) synthases (NOS). Sepiapterin reductase (SPR) catalyzes the third step of BH4 biosynthesis. SPR gene-disrupted (Spr-/- ) mice exhibit a dystonic posture, low body weight, hyperphenylalaninemia, and unstable hypertension with endothelial dysfunction. In this study, we found that Spr-/- mice suffered from a high incidence of severe priapism. Their erections persisted for months. The biopterin, BH4, and norepinephrine contents, and TH protein levels in the penile tissue of Spr-/- mice without and with priapism were significantly reduced compared to those of Spr+/+ mice. In contrast, their neural NOS (nNOS) protein levels were increased, and the cyclic guanosine monophosphate (cGMP) levels were remarkably elevated in the penises of Spr-/- mice with priapism. The symptoms were relieved by repeated administration of BH4. The biopterin, BH4, and norepinephrine contents were increased in penile homogenates from BH4-supplemented Spr-/- mice, and the TH protein levels tended to increase, and their nitrite plus nitrate levels were significantly lower than those of vehicle-treated Spr-/- mice and were approximately the same as vehicle- and BH4-supplemented Spr+/+ mice. Thus, we deduced that the priapism of Spr-/- mice is primarily caused by hypofunction of the sympathetic neurons due to cofactor depletion and the loss of TH protein and, further, dysregulation of the NO/cGMP signaling pathway, which would be caused by disinhibition of nNOS-containing neurons and/or abnormal catabolism of cyclic nucleotides is suggested.

Psychometric Impact of Priapism on Lives of Adolescents and Adults With Sickle Cell Anemia: A Sequential Independent Mixed-Methods Design.

Despite priapism being one of the most frequent complications of sickle cell anemia (SCA) in male individuals, little has been reported about the impact of priapism in this population. The authors used a sequential independent mixed-methods design, which used both international multicenter focus group discussions (n=35) and a quantitative patient-reported outcome measure (n=131) to determine the impact of priapism on men with SCA in Nigeria and the United States. The authors analyzed data from focus groups using an iterative inductive-deductive approach. Comparison of the Priapism Impact Profile data was done using the Kruskal-Wallis H test. Our result showed that priapism, across cultures, is associated with shame and embarrassment. These emotions interfere with timely clinical and family communication about priapism symptoms and complications. Participants were dissatisfied with the quality of care at emergency facilities. The quality of life and physical wellness of men with SCA-related priapism were significantly different for the 3 groups: (1) priapism condition getting better, (2) priapism condition getting worse, and (3) priapism condition remain the same (P=0.002 and P=0.019, respectively). Psychological, sexual, and physical wellbeing are all adversely affected by priapism. Evidence-based methods are necessary for adequate medical, educational, and psychological treatment for recurrent priapism.

Review of Ischemic and Non-ischemic Priapism.

PURPOSE OF REVIEW: Priapism is a rare condition that has different presentations, etiologies, pathophysiology, and treatment algorithms. It can be associated with significant patient distress and sexual dysfunction. We aim to examine the most up-to-date literature and guidelines in the management of this condition. RECENT FINDINGS: Priapism is a challenging condition to manage for urologists, since the etiology is often multi-factorial and the suggested treatment algorithms are based on small studies and expert anecdotal experience, perhaps due to the rarity of the disorder. Ischemic priapism of less than 24 h can be managed non-surgically in most cases with excellent results. Ischemic priapism of more than 36 h is frequently associated with permanent erectile dysfunction. Management of prolonged priapism with penile shunting still may result in poor erectile function, so penile prosthesis can be discussed in these scenarios.

IgG4-related prostatitis manifesting as urinary obstruction in a 28-year-old male.

BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a systemic lymphoproliferative disorder characterized by elevated serum IgG4 levels and tumefactive lesions that can involve nearly every organ system. Involvement of the prostate is rare but has been reported in limited cases. CASE PRESENTATION: A 28-year-old man of Asian descent with a history of sinusitis and priapism presented to hospital with rigors and voiding symptoms. He was diagnosed with IgG4-RD one month prior to presentation, following pathological analysis of a submandibular mass that demonstrated chronic sclerosing sialadenitis. On presentation, white blood cell count, C-reactive protein, and prostate serum antigen levels were all within normal limits. Examination was notable for a large, firm prostate, and a foley catheter was inserted. Contrast CT of the abdomen was unremarkable. Further workup revealed elevated serum IgG4 levels (9.22 g/L) and he was subsequently started on prednisone 35 mg daily. Imaging to screen for systemic IgG4-RD involvement demonstrated paravertebral soft tissue involvement and he was given rituximab 1000 mg IV × 2 doses. MRI revealed diffuse prostatitis. Five days after starting prednisone and one day after his first dose of rituximab, he successfully passed trial of void and was discharged home. CONCLUSIONS: IgG4-related prostatitis is a rare and underrecognized manifestation of IgG4-RD. Our case highlights the need to consider IgG4-related prostatitis as an etiology of urinary obstruction in young individuals. Resolution of symptoms following treatment with steroids may be diagnostic of IgG4-related prostatitis, and may potentially avoid the need for invasive diagnostic procedures such as prostate biopsy.

Getting the diagnosis of priapism wrong; when not to aspirate.

Priapism is defined as a full or partial erection lasting greater than 4 h due to a fault in the normal detumescence mechanism of the penis. We describe the case of a confused 73-year-old gentleman presenting with painless priapism, a 2 cm non-tethered lesion in the scrotum and a vague palpable pelvic mass. On the presumption that this was a case of high-flow non-ischaemic priapism secondary to pelvic malignancy, the urology registrar attempted corporal body aspiration. Clear fluid was aspirated and the penis became flaccid instantly. A CT scan performed to determine the presence of a pelvic mass, revealed a penile prosthesis and artificial reservoir. While iatrogenic penile prosthesis malfunctions are well established in the literature, a case managed as an acute priapism is yet to be reported. This case teaches us the importance of taking an adequate medical history and clinical examination prior to formulating a diagnosis and administering treatment.

Point-of-Care Ultrasound Diagnosis of High Flow Priapism.

BACKGROUND: Priapism can be categorized as low flow or high flow. Low flow priapism is a compartment syndrome and requires immediate treatment to avoid long-term ischemic damage. Alternatively, high flow priapism is not an emergent condition and can be managed as an outpatient. The diagnosis has traditionally been made via cavernosal blood gas analysis; however, this is painful and can cause iatrogenic harm. CASE REPORT: We present a case of high flow priapism whereby point-of-care ultrasound (POCUS) identified the presence of pulsatile cavernosal arterial flow to confirm the diagnosis. This is the first case report in the emergency medicine literature to highlight the utility of POCUS in the diagnosis and management of high flow priapism. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Low flow priapism accounts for 95% of cases and is a urologic emergency requiring immediate invasive intervention. High flow priapism is much less common and does not require emergent management. While blood gas analysis can differentiate between high and low flow priapism, POCUS is a rapid, noninvasive, accurate diagnostic means to identify the presence or absence of cavernosal pulsatile arterial flow.

[A Case of Successful Selective Arterial Embolization for Traumatic Priapism].

The first-line treatment for arterial (traumatic) priapism is follow-up, but no recommended duration has been established. We report a case of traumatic priapism that did not improve after one year of follow-up and was cured by arterial embolization. The patient was a 21-year-old male with a urethral injury caused by traffic trauma, and a urethral catheter was placed under fluoroscopic guidance. Magnetic resonance imaging (T2-weighted image) showed a low-signal area in the right penile corpus cavernosum. The urethral catheter was removed 1 month after the injury, but the erection persisted, and the patient was referred to our department 8 months after the injury. Contrast-enhanced computed tomography (CT) revealed enhancement effect of the right penile corpus cavernosum, which was diagnosed as traumatic priapism, and selective arterial embolization was performed 1 year after the injury. Angiography revealed an extravascular leak from the right patent ductus arteriosus into the cavernous sinus of the penis, and a gelatin sponge (Serescue®ï¸Ž) was injected as an embolization material into the distal portion of the right patent ductus arteriosus. Immediately after the operation, the penis became fully erect, but gradually softened. One month after embolization, priapism improved, and 6 months after embolization, contrast-enhanced CT confirmed the disappearance of the enhancement effect of the right corpus cavernosum. There has been no relapse of symptoms for 10 months after embolization. Selective arterial embolization for traumatic priapism is considered to be a useful treatment even after a certain period of follow-up.

[Sexuality assessment of homozygous adult sickle cell patients with a history of priapism in Senegal]. / Évaluation de la sexualité des drépanocytaires adultes homozygotes ayant des antécédents de priapisme au Sénégal.

OBJECTIVES: The objective of this study was to evaluate the sexuality of SS sickle cell patients with a history of priapism. METHODS: This was a case-control study of adult SS sickle cell patients. The occurrence of priapism as well as the nature of the priapism had been investigated. The patients were subdivided into three groups: Group 1 (no priapism), Group 2 (intermittent priapism) and Group 3 (acute priapism). The patients' sexuality was studied using the IIEF-15 questionnaire. RESULTS: We interviewed 191 SS sickle cell patients. The mean age was 27.1±7.1 years. Priapism was observed in 43.5 %. Only 77 patients were eligible for the IIEF15 questionnaire. Groups 1 and 2 performed significantly better than group 3 on erectile function (EF) and orgasmic function (OF) scores. There was no significant difference in the EF and OF scores between groups 1 and 2. No significant difference was observed between the three groups for the scores of sexual desire (SD), intercourse satisfaction (IS), and overall satisfaction (OS). The impairment of erectile function in group 2 was related to the age of the first episode of priapism and the last episode. The impairment of erectile function in group 3 was related to the duration of evolution (P<0.05). CONCLUSION: This study shows that priapism is responsible for impaired erectile function in SS adult sickle cell patients. A program to prevent intermittent episodes of priapism should be put in place.

Regimented Phosphodiesterase Type 5 Inhibitor Use Reduces Emergency Department Visits for Recurrent Ischemic Priapism.

PURPOSE: We evaluated the real-world effectiveness of regimented phosphodiesterase type 5 inhibitor dosing on recurrent ischemic priapism outcomes using emergency department visits as a proxy for therapeutic control of the disorder. MATERIALS AND METHODS: We performed a retrospective chart review of patients with recurrent ischemic priapism who were started on regimented phosphodiesterase type 5 inhibitor therapy from May 2006 to January 2020. We compared the number of emergency department visits per month during a 6-month period before treatment, during treatment and after treatment discontinuation. We extracted and categorized priapism outcomes such as priapism frequency and duration. RESULTS: Of 216 patients identified with all cause priapism 114 were diagnosed with recurrent ischemic priapism and 42 were initiated on regimented phosphodiesterase type 5 inhibitor therapy. Treatment effectiveness was analyzed for 24 evaluable patients. Priapism etiology was idiopathic in 12 patients (50%), sickle cell disease in 11 (46%) and drug-induced in 1 (4%). The median length of regimented phosphodiesterase type 5 inhibitor use was 3 months (IQR 2-7). Treatment decreased emergency department visits per month by 4.4-fold (p <0.001), priapism duration tiers (p <0.001) and priapism frequency tiers (p <0.001). Of 24 patients 22 (92%) reported improvement in priapism outcomes, 9 of whom reported resolution of recurrent ischemic priapism episodes. A subgroup analysis of 17 patients with recurrent ischemic priapism relapse after treatment discontinuation showed a significant increase in priapism duration (p <0.001) and frequency (p <0.001) but no significant change in emergency department visits per month (p=0.91). CONCLUSIONS: Regimented phosphodiesterase type 5 inhibitor therapy was an impactful treatment in managing recurrent ischemic priapism according to objective and subjective parameters. This study provides further support for the use of regimented phosphodiesterase type 5 inhibitor dosing as a preventive strategy for recurrent ischemic priapism.

Acute Ischemic Priapism: An AUA/SMSNA Guideline.

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

Priapism in patients with hemolytic disorders: a nationwide retrospective cohort study.

Priapism is a persistent, painful erection, which can lead to permanent penile damage and reduced quality of life. Patients with sickle cell disease have an increased risk of priapism which has been related to chronic hemolysis. This study investigates the prevalence of priapism in all major hereditary and acquired forms of hemolytic disorders. Patients with hemolytic disorders were identified in the nationwide Danish Hemolysis Cohort. Each patient was age-sex-matched with 50 comparisons from the general population without hemolysis. We identified the episodes of hospital-registered priapism events for both patients with hemolysis disorders and comparisons in the Danish National Patient Register between 1977 and 2016. We identified 4181 male patients with hemolytic disorders and 205,994 male comparisons, with 2,294,027 person-years of total observation time. Totally, 101 episodes of priapism occurred during follow-up period. Six episodes of priapism were recorded in three patients with a hemolytic disorder, all affected by sickle cell disease. Two of these patients had verified genotype HbSS. The incidence rate for first priapism in sickle cell disease was 432.8 per 100,000 person-years [95% CI: 139.6; 1341.8] versus 0.84 per 100,000 person-years [95% CI 0.54; 1.32] in comparisons. Using a large nationwide cohort, we found that only sickle cell disease is associated with priapism among patients with hemolytic disorders. The incidence rate of priapism in patients with sickle cell disease was lower than previously reported.