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.

Effects of ketamine on penile tissues in an experimental priapism model in rats.

BACKGROUND: This study aimed to evaluate the histopathological and biochemical effects of ketamine on penile tissues following ischemia-reperfusion injury induced by priapism. METHODS: Twenty-four male rats were randomized into three groups. Group 1 served as the control group. Group 2 underwent the priapism model to induce ischemia-reperfusion injury. Group 3, the treatment group, experienced a similar ischemia-reperfusion model as Group 2; additionally, 50 mg/kg of ketamine was administered intraperitoneally just before reperfusion. Blood biochemical analyses and penile histopathological evaluations were performed. RESULTS: In Group 3, significant improvements were observed in all histopathological scores, including desquamation, edema, inflammation, and vasocongestion compared to Group 2 (p<0.001). Blood biochemical analyses showed that the malondialdehyde (MDA) levels were recorded as 10 in Group 2, with a significant decrease in Group 3 (p=0.013). Similarly, proinflammatory cytokine levels, including interleukin-1 beta (IL-1ß), interleukin-6 (IL-6), and tumor necrosis factor-alpha (TNF-α), were found to be suppressed in Group 3 compared to Group 2 (p=0.003, p=0.022, and p=0.028, respectively). Antioxidant enzyme activities, such as glutathione peroxidase (GSH-Px) and superoxide dismutase (SOD), were higher in Group 3 compared to Group 2 (p=0.016 and p=0.024, respec-tively). CONCLUSION: Ketamine is an effective anesthetic agent in alleviating the effects of penile ischemia-reperfusion injury.

Successful Endovascular Microembolization for Post-Traumatic High-Flow Priapism: A Case Report.

BACKGROUND High-flow (non-ischemic) priapism is a rare urological condition usually related to blind trauma to the penis or perineum causing an arterial-lacunar fistula. It can be treated conservatively, but in some cases when conservative treatment fails, the interventional approach is indicated. In the past, only surgical treatment was available, which was associated with a significant risk of complications. Endovascular techniques use a novel approach and offer clinical benefits for the patient. CASE REPORT A 51-year-old man was admitted to the hospital after referral from the urology department with high-flow priapism related to blunt trauma. Angio-computed tomography showed extravasation of contrast medium to the corpus cavernosum, and angiography revealed a fistula between the distal segment of the left internal pudendal artery and corpora cavernosa. A successful endovascular microembolization of the arterial-lacunar fistula with the use of microcoils was performed. The postprocedural period was uneventful and the patient was discharged. Despite incomplete angiographic follow-up at 6 months, the initial symptoms were fully resolved with the absence of any erectile dysfunction and no recurrence of priapism occurred. CONCLUSIONS Post-traumatic high-flow priapism can be safely and effectively treated by endovascular means. Microembolization has proven to be successful and beneficial to preserve sexual functions.

[Priapism]. / Priapismus.

Priapism is defined as penile erection lasting more than four hours that is unrelated to sexual arousal. Priapism is classified based on the oxygenation of the penile tissue into ischemic and non-ischemic subtypes. As the most common form, ischemic priapism is usually associated with pain and carries a significant risk of permanent loss of erectile function; thus, rapid intervention is necessary. Initial therapy consists of corporal aspiration and injection of sympathomimetic agents. If detumescence is not achieved, a cavernosal shunt is necessary. Non-ischemic priapism is less common than the ischemic type and is usually the result of perineal trauma. In this subtype, there is usually no pain and treatment is initially conservative. Recurrent (stuttering) priapism is a variant of the ischemic subtype, but is self-limiting and usually occurs during sleep with a duration of less than three to four hours. In the case of prolonged erection, therapy is analogous to that of the ischemic subtype.

Delineating patient errors in an intracavernosal injection program.

BACKGROUND: Intracavernosal injection therapy (ICI) is a well-established therapeutic strategy for men with erectile dysfunction. Complications are often related to patient error when performong ICI. AIM: The objective of this study was to examine patient errors in an established patient training program for performing ICI and identify factors that could predict major errors. METHODS: Patients enrolled in our ICI program are trained on technical aspects, and dose titration is begun. Patients are given explicit instructions during training, both verbally and in written form. Records were reviewed for men using ICI for ≥6 months. Multivariable analysis was used to define predictors of major errors. OUTCOMES: Errors were listed as minor (zero-response injection, penile bruising, expired medication) and major (errors potentially leading to priapism: dose self-titration, double injecting). RESULTS: Overall, 1368 patients met the inclusion criteria and were included in the analysis. The mean patient age was 66 ± 22 (range 29-91) years. Regarding education, 41% of patients had graduate-level education, 48% had college education, and 11% high school education. Mean follow-up was 3.2 ± 7.6 (range 0.5-12) years. The agents used were trimix (62%), bimix (35%), papaverine (2%), and prostaglandin E1 monotherapy (1%). At least 1 error occurred during self-administration in 42% of patients during their time in the program. Errors included zero response to medication due to technical error (8% of patients), penile bruising (34%), use of an expired bottle (18%), self-titration (5%), and double injecting (4% of patients); 12% of men committed ≥1 error during their time in the program. On multivariable analysis, independent predictors of the occurrence of a major error included: young age, graduate-level education, and <12 months of injection use. CLINICAL IMPLICATIONS: To the best of our knowledge, this is the first reported study to investigate ICI errors and risk factors. The identification of factors predictive of major errors allows for more tailored and intensive training in this subset of patients. STRENGTHS AND LIMITATIONS: Strengths of this study include a large patient population (1386 men) with a considerable follow-up time. Additionally, the rigorous training, education, and monitoring of the participants, as well as the use of formal definitions, enhances the accuracy and reliability of the results. Despite the strengths of the study, recall bias may be a limitation concern. CONCLUSION: The majority of patients were error free, and the majority of the errors were minor in nature. Major errors occurred in <10% of patients. Younger age, graduate-level education, and less experience with ICI were independent predictors of major errors.

Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso / Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

Early vs delayed insertion of penile prosthesis in patients with refractory priapism: a systematic review and meta-analysis.

INTRODUCTION: Refractory priapism, characterized by persistent and prolonged painful erections despite initial treatment maneuvers, can significantly impair erectile function secondary to ischemia-induced corporal tissue fibrosis. These patients will likely require subsequent penile prosthesis (PP) surgery to regain sexual activity, yet consensus regarding the optimal timing of implantation remains lacking. OBJECTIVES: To evaluate and compare the clinical outcomes associated with early vs delayed PP implantation in individuals with priapism-induced erectile dysfunction (ED). METHODS: We included studies that focused on refractory priapism leading to ED and its management with PP implantation. We assessed cohort study bias with a risk-of-bias tool and case series bias with the modified Newcastle-Ottawa Scale. Pooled odds ratios (ORs) were calculated by a fixed-effect model. RESULTS: We included 9 studies, comprising 4 cohort studies and 5 case series, involving a total of 278 patients. Total complications were higher in the delayed group (OR, 4.16; 95% CI, 2.77-6.26). Fibrosis was significantly more pronounced in the delayed group (OR, 118.18; 95% CI, 20.06-696.32). The odds of erosion, infections, and penile injury did not show statistically significant differences between the groups (OR, 2.52 [95% CI, 0.67-9.49], 0.89 [0.38-2.10], 1.83 [0.79-4.26], respectively). Patients' satisfaction resulted in a pooled OR of 0.15 (95% CI, 0.04-0.49) in favor of the early PP insertion group. CONCLUSION: The results from this study favor an early approach to ED (within 30 days) following ischemic priapism. However, it is important to consider patients' preferences, values, and psychological factors to make an informed decision.

Erección prolongada dolorosa / Prolonged painful erection

El priapismo es una erección peneana prolongada y dolorosa, que ocurre sin estímulo sexual previo. Existen dos tipos principales, el priapismo de alto flujo y el priapismo de bajo flujo. Aunque en la mayoría de las ocasiones la causa subyacente será desconocida, puede ser la primera manifestación de una enfermedad grave. En el paciente pediátrico con una erección prolongada se debe diferenciar entre la erección peneana recurrente y los distintos tipos de priapismo, puesto que cada entidad requiere un manejo concreto e implica un pronóstico diferente. (AU)

Priapism is a prolonged and painful penile erection, which occurs without prior sexual stimulation. There are two main types, high-flow priapism and low-flow priapism. Although on most occasions the underlying cause will be unknown, it may be the first manifestation of serious disease. In the pediatric patient with prolonged erection we must differentiate between recurrent penile erection and the different types of priapism since each entity requires a specific management and implies a different prognosis. (AU)

What Is Priapism?

This JAMA Patient Page describes the diagnosis, prevention, and treatment of priapism.

Clinical Practice Patterns for Surgical Shunts and Penile Prosthesis Placement in Men With Priapism: A Retrospective Large Claims Database Analysis.

INTRODUCTION: The objective of this study was to assess the rates of surgical shunting and prosthesis placement for acute ischemic priapism using a large multi-institutional claims database. METHODS: A US claims database network (TriNetX Diamond Network) was queried from 2010 to 2020. We constructed a cohort of men ages ≥ 16 years who (1) had a diagnosis of priapism and (2) underwent an irrigation of the corpora cavernosa for priapism. We assessed the number of men who then had a surgical penile shunt or penile prosthesis placement. Demographics, time to surgical procedure, and order of procedures were collected. RESULTS: A total of 6392 men were identified with the diagnosis of priapism and the procedure of corpora cavernosal irrigation. Of these men, 693 (11%) proceeded to surgical shunt. One hundred forty-four men (2%) underwent initial penile prosthesis placement. Of the men undergoing initial penile prosthesis, only 17 of 144 (12%) cases occurred within the first month of corpora cavernosal irrigation. Finally, when assessing choice of initial shunts vs initial penile prosthesis before and after 2015, overall rates of initial shunt (10.0% vs 8.5%, P < .0001) and initial prosthesis (3.1% vs 2.1%, P < .0001) were lower after 2015 when compared with rates prior to 2015. CONCLUSIONS: In this US claims-based analysis of men presenting with ischemic priapism and treated with initial irrigation, a small percentage (11%) of men went on to receive surgical shunting, and only 2% received an initial prosthesis. Men receiving initial prostheses were more likely to have more comorbidities, and overall surgical management of priapism has decreased over time.

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.

Cocaine-related ischemic priapism. Systematic review and presentation of a single center series.

INTRODUCTION: Ischemic priapism is a medical emergency that, if not treated, could lead to permanent erectile dysfunction. The association between cocaine and priapism is well-known; however, data on patient characteristics, treatment, and outcomes is missing. This work aimed to answer the research question: What are the characteristics, management strategies, and erectile prognosis of patients consuming cocaine and presenting with priapism? METHODS: We conducted a systematic review according to PRISMA guidelines and described our case series. RESULTS: Eight studies were selected for qualitative synthesis, presenting information on ten patients. In our case series, we showed information regarding four patients. From the systematic review, the mean presentation time was 42.6 h, and the mean number of procedures to solve priapism was 2,4; in our case series was 42.75 h and 2, respectively. CONCLUSION: Cocaine-related priapism might present with a delayed diagnosis, need more procedures to be managed, and have a worse prognosis. More extensive and prospective studies are required.

Images: Sleep-related painful erection with concomitant hypnic headache.

Sleep-related painful erection (SRPE) is a parasomnia defined by the repetition of painful erections during rapid eye movement (REM) sleep. Hypnic headache (HH) is a primary headache occurring exclusively at night, often during REM sleep. We report the observation of a 33-year-old man with simultaneous SRPE and HH. Physical examination was normal. Comprehensive urological and endocrine explorations excluded other organic differential diagnoses. Polysomnography revealed several awakenings in REM, due to SRPE and concurrent HH. Medication by baclofen at bedtime seemed to have resulted in a decrease in SRPE episodes, confirmed by polysomnography, but at the cost of excessive daytime sleepiness, and was discontinued by the patient. Caffeine intake at bedtime was proposed, but the patient was reluctant because he was concerned about worsening insomnia. At 9-month follow-up, the patient had accepted his medical condition and was coping with both SRPE and HH. He felt reassured and wished no "overmedicalization." To our knowledge, the coexistence of both conditions has not yet been reported, yet their frequencies might be underestimated. We hypothesize a common underlying pathophysiology with a possible dysfunction of the vascular control and/or the autonomic nervous system and that could involve the hypothalamus. Somnologists should be aware of SRPE, potentially overlapping with HHs. SRPE should be considered in case of sleep-maintenance insomnia. Patient reassurance seems to be central in the care process of SRPE. CITATION: Moreau A, Monnier L, Medde A, Bourgin P, Ruppert E. Images: sleep-related painful erection with concomitant hypnic headache. J Clin Sleep Med. 2024;20(5):837-839.

[Priapism associated to zuclopentixol and the choice for an alternative]. / Priapisme bij gebruik van zuclopentixol en de keuze voor een alternatief.

A 29-year-old man developed priapism following the (re)administration of zuclopentixol. In the previous days, a significant amount of alcohol was consumed, presumably in combination with amphetamine and cannabis. Priapism is a rare but serious side effect of various psychoactive medications and recreational drugs, leading to permanent loss of erectile function if not treated in time. In this case the side effect was discovered in a late stage, at which curative treatment was no longer viable. A clear guideline for choosing an alternative antipsychotic agent is currently lacking, but an antipsychotic with low alfa-adrenergic affinity seems preferable. To prevent erectile disfunction following priapism, awareness of its severity is essential, for both doctor and patient.

Nitric Oxide Resistance in Priapism Associated with Sickle Cell Disease: Mechanisms, Therapeutic Challenges, and Future Directions.

Patients with sickle cell disease (SCD) display priapism, a prolonged penile erection in the absence of sexual arousal. The current pharmacological treatments for SCD-associated priapism are limited and focused on acute interventions rather than prevention. Thus, there is an urgent need for new drug targets and preventive pharmacological therapies for this condition. This review focuses on the molecular mechanisms linked to the dysfunction of the NO-cyclic guanosine monophosphate (cGMP)-phosphodiesterase type 5 (PDE5) pathway implicated in SCD-associated priapism. In murine models of SCD, reduced nitric oxide (NO)-cGMP bioavailability in the corpus cavernosum is associated with elevated plasma hemoglobin levels, increased reactive oxygen species levels that inactive NO, and testosterone deficiency that leads to endothelial nitric oxide synthase downregulation. We discuss the consequences of the reduced cGMP-dependent PDE5 activity in response to these molecular changes, highlighting it as the primary pathophysiological mechanism leading to excessive corpus cavernosum relaxation, culminating in priapism. We also further discuss the impact of intravascular hemolysis on therapeutic approaches, present current pharmacological strategies targeting the NO-cGMP-PDE5 pathway in the penis, and identify potential pharmacological targets for future priapism therapies. In men with SCD and priapism, PDE5 inhibitor therapy and testosterone replacement have shown promising results. Recent preclinical research reported the beneficial effect of treatment with haptoglobin and NO donors. SIGNIFICANCE STATEMENT: This review discusses the molecular changes that reduce NO-cGMP bioavailability in the penis in SCD and highlights pharmacological targets and therapeutic strategies for the treatment of priapism, including PDE5 inhibitors, hormonal modulators, NO donors, hydroxyurea, soluble guanylate cyclase stimulators, haptoglobin, hemopexin, and antioxidants.

Case Report: Priapism Following an Anterior Lumbar Interbody Fusion.

Priapism is a disorder where the penis without sexual stimulation maintains a prolonged rigid erection lasting 4 or more hours. There are two classifications of priapism, ischemic (low flow) or nonischemic high flow, and each have specific etiologies, diagnostic criteria, and management. This presented case study involved a 58-year-old male who experienced an ischemic priapism more than 24 hours after an anterior lumbar interbody fusion (ALIF). A flaccid penis was achieved after the patient received two 400 µg of phenylephrine HCL into the corpora cavernosum. Review of the literature suggests anesthetic medications given during the surgical procedure may have caused the priapism. Lessons that can be learned from this case study highlight that even though the nurse may not expect to see a priapism after an ALIF, the nurse must always be diligent and not become complacent with unexpected findings or assessments that may cause irreparable harm to the patient.

Priapism in a Young Adult With Sickle Cell Disease.

This article presents a case study focusing on priapism in a patient with sickle cell disease, with repeated emergency department (ED) visits and hospitalizations. The patient was successfully identified and treated by the ED nurse practitioner (NP) with aspiration of the corpus cavernosum. Priapism is a persistent penile erection that continues for an extended time. There is some argument about what that length of time is, but generally, the consensus is more than 4 hr beyond sexual stimulation or unrelated to sexual stimulation or sexual interest (Bivalacqua et al., 2022). Priapism is a fairly common but underrecognized complication of sickle cell disease. It represents a urological emergency in which timely diagnosis and appropriate treatment are vital to preserving penile tissue and sexual function. The diagnosis is made clinically with a comprehensive history, physical examination, and appropriate laboratory test values. Initial management can be conservative with hydration and analgesics or, if necessary, more invasive with needle aspiration to promote detumescence. Permanent tissue damage or erectile dysfunction can result if priapism is unrecognized, untreated, or not treated immediately. The NP plays an integral role in treating and preventing permanent damage. Patient education should focus on instructions for preventing priapism and managing episodes at home.