Priapism as an initial manifestation in an adult patient with chronic myeloid leukemia in Japan: case report and review of the literature.

We report the first documented Japanese case in the English literature of chronic myeloid leukemia (CML) in which priapism was the presenting symptom. Priapism, a rare manifestation in CML patients, is particularly uncommon in Japan. This can be attributed to the high quality of medical services and proactive health strategies implemented by the Japanese government. These strategies include recommending regular blood tests for company employees aged 35 and above, thereby facilitating early detection of CML. Hence, it is crucial to consider CML when examining any patient presenting with priapism, particularly among those who have not undergone regular medical check-ups.

Interventional radiology: Diagnosis and treatment of post-traumatic nonischemic priapism: A case report.

Priapism is defined as a form of erectile dysfunction characterized by a prolonged and involuntary penile erection, either partial or complete, occurring without sexual stimulation and lasting for more than 4 hours. Its incidence is estimated to be 0.5-0.9 cases per 100,000 people per year. The most frequent form is ischemic priapism, results from paralysis of the cavernous smooth muscles, which are unable to contract, leading to the stagnation of hypoxic blood within the sinusoidal spaces. Characterized by a painful rigid and sustainable erection. Non-ischemic priapism constitutes a rare entity, unlike the former, this type is typically painless. It is caused by an excessive influx of blood into the penis without a concomitant increase in outgoing blood flow. Blunt trauma is the most commonly reported etiology. And finally, recurrent priapism is characterized by recurrent episodes of prolonged erection and can be challenging to treat, often requiring long-term management to prevent recurrences. We report a case of high-flow priapism in a 10-year old child, secondary to a cavernous arterial fistula following a straddle injury during sports activity. It was suspected clinically and confirmed by ultrasound-Doppler, then successfully treated radiologically with highly selective embolization, with very satisfactory postoperative outcomes.

Artificial Intelligence Modeling and Priapism.

PURPOSE OF REVIEW: This narrative review aims to outline the current available evidence, challenges, and future perspectives of Artificial Intelligence (AI) in the diagnosis and management of priapism, a condition marked by prolonged and often painful erections that presents unique diagnostic and therapeutic challenges. RECENT FINDINGS: Recent advancements in AI offer promising solutions to face the challenges in diagnosing and treating priapism. AI models have demonstrated the potential to predict the need for surgical intervention and improve diagnostic accuracy. The integration of AI models into medical decision-making for priapism can also predict long-term consequences. AI is currently being implemented in urology to enhance diagnostics and treatment work-up for various conditions, including priapism. Traditional diagnostic approaches rely heavily on assessments based on history, leading to potential delays in treatment with possible long-term sequelae. To date, the role of AI in the management of priapism is understudied, yet to achieve dependable and effective models that can reliably assist physicians in making decisions regarding both diagnostic and treatment strategies.

Potential efficacy of crizanlizumab in treating priapism in sickle cell disease: A case report.

This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.

Penile prosthesis in priapism: a systematic review of outcomes and complications.

Background: Priapism is a rare condition characterized by persistent erection of the penis that lasts more than 4 hours in the absence of sexual stimulation and is associated with significant morbidity and complications, including erectile dysfunction and penile fibrosis. Surgical management of priapism can be extremely challenging. We herein provide a comprehensive review that aims to evaluate the role of penile prosthesis (PP) implantation in the management of priapism. Methods: A systematic literature search was performed using the following databases: PubMed, Embase, and Scopus to identify studies that evaluated the effectiveness of PP implantation in treating priapism and the long-term complications, outcomes, and patients' satisfaction rate. Results: Out of 717 English-language studies published between 2002 and 2022, 17 were chosen for this review. Majority of patients had a malleable PP (MPP) implant, either early or delayed after the priapism episode. Early placement (EP) of PP is widely defined between studies ranging from less than 72 hours, within 1 week, and within 3 weeks. Most common causes of priapism were sickle cell anemia (SCA), medication-induced, and idiopathic. Studies show a higher satisfaction rate ranging between 80% and 100%, with sexual intercourse achievement ranging between 64.2% and 100%. Based on the GRADE system, included studies rated as very low quality of evidence. Commonly reported complications that arise after PP procedures, include device infection, erosion, curvature, and mechanical malfunction, such as auto-inflation. Conclusions: PP can be an effective treatment option for priapism, particularly in cases of ischemic priapism lasting more than 36 hours or recurrent priapism that is medically refractory. However, due to the very low quality of evidence, larger, well-designed studies are warranted where long-term outcomes, patients' satisfaction, and complications following priapism-related PP implantation are measured as endpoints.

Severe penile necrotizing infection with subsequent glandular gangrene after distal corporal aspiration and irrigation procedure for ischemic priapism.

Necrotizing penile infection following ischemic priapism takedown is a rare complication. A 60-year-old man presented with 1-week history of green and pink purulent discharge, penile swelling, and inability to void. Computed tomography demonstrated gas and fluid accumulation within the corporal bodies, suggestive for bilateral penile necrotizing corporal infection with possibility of intra-corporal abscesses. The patient underwent extensive serial surgical debridement of the penile and corporal tissues which was complicated with poly-microbial tissue cultures, growing Candida albicans and Staphylococcus epidermidis. Despite maximal penile drainage, glans penis gangrene was developed to level of mid penile shaft necessitating a partial penectomy.

A Case of Fournier's Gangrene in a Patient With Malignant Priapism.

Fournier's gangrene is a rare and potentially life-threatening type of necrotizing fasciitis that affects the genital and perineal regions. Malignant priapism is a rare and serious medical condition characterized by persistent and painful erection of the penis that is not associated with sexual arousal or stimulation. We present a case of a 77-year-old man with concurrent Fournier's gangrene and malignant priapism. He first underwent surgical debridement to remove necrotic tissue and aspiration of blood from the corpora cavernosa. Then a palliative penectomy was performed. The patient succumbed to severe sepsis and died after 14 days of hospitalization.

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.

European Association of Urology/European Society for Paediatric Urology Guidelines on Paediatric Urology: Summary of the 2024 Updates.

BACKGROUND AND OBJECTIVE: We present an overview of the 2024 updates for the European Association of Urology (EAU)/European Society for Paediatric Urology (ESPU) guidelines on paediatric urology to offer evidence-based standards for perioperative management, minimally invasive surgery (MIS), hydrocele, congenital lower urinary tract obstruction (CLUTO), trauma/emergencies, and fertility preservation. METHODS: A broad literature search was performed for each condition. Recommendations were developed and rated as strong or weak on the basis of the quality of the evidence, the benefit/harm ratio, and potential patient preferences. KEY FINDINGS AND LIMITATIONS: Recommendations for perioperative management include points related to fasting, premedication, antibiotic prophylaxis, pain control, and thromboprophylaxis in patients requiring general anaesthesia. MIS use is increasing in paediatric urology, with no major differences observed among different MIS approaches. For hydrocele, observation is the initial approach recommended. For persistent cases, treatment varies according to the type of hydrocele. CLUTO cases should be managed in tertiary centres with multidisciplinary expertise in prenatal and postnatal management. Neonatal valve ablation remains the mainstay of treatment, but associated bladder dysfunction requires continuous treatment. Among urological traumas and emergencies, renal trauma is still an important cause of morbidity and mortality. Conservative management has become the standard approach in haemodynamically stable children. Ischaemic priapism is a medical emergency and requires stepwise management. Initial management of nonischaemic priapism is conservative. Fertility preservation in prepubertal children and adolescents has become an increasingly relevant issue owing to the ever-increasing number of cancer survivors receiving gonadotoxic therapies. A major limitation is the scarcity of relevant literature. CONCLUSIONS AND CLINICAL IMPLICATIONS: This summary of the 2024 EAU/ESPU guidelines provides updated guidance for evidence-based management of some paediatric urological conditions. PATIENT SUMMARY: We provide a summary of the updated European Association of Urology/European Society for Paediatric Urology guidelines on paediatric urology. There are recommendations on steps to take before and immediately after surgery, management of hydrocele, congenital lower urinary tract obstruction, and urological trauma/emergencies, as well as preservation of fertility. Recommendations are based on a comprehensive review of recent studies.

Partial Segmental Thrombosis of the Corpus Cavernosum Associated With Inappropriate Sildenafil Use.

The case presented is of a 39-year-old male with severe right groin pain and perineal pain the morning after sexual intercourse with the use of sildenafil without a diagnosis of erectile dysfunction. Partial segmental thrombosis of the corpus cavernosum (PSTCC) was diagnosed using magnetic resonance imaging and treated with direct oral anticoagulation without complications. Sildenafil use has been noted as an inciting factor for PSTCC in only two other cases of less than 60 cases reported in the literature and has even been used successfully as a component of therapeutic management of PSTCC in another previous case.

Sleep-related painful erections treated with sodium oxybate.

A 39-year-old male with a medical history significant for migraine, psoriatic arthritis, postural orthostatic tachycardia syndrome, vitamin D deficiency, and hypoglycemia presented with a 2-year history of sleep-related painful erections. Because the reported prevalence is low, there is limited understanding of the possible etiologies of the disorder and few published clinical data on treatment algorithms. Thus, he had tried multiple therapies. Baclofen was effective but not tolerated. Pelvic physiotherapy and tadalafil were ineffective. Imipramine, clonazepam, vitamin B, iron, and selenium provided minimal benefit. Opiates were initially effective but lost efficacy after 2-3 weeks. Finally, he was started on sodium oxybate after fully counseling the patient on the potential side effects of the treatment and consenting the patient for off-label use. This has effectively treated his sleep-related painful erections. Sodium oxybate is a novel therapy for and a possible new treatment for this rare and challenging disorder that merits further study. CITATION: Chaudhary HS, Zeidman E, Punjani N, Tashman Y. Sleep-related painful erections treated with sodium oxybate. J Clin Sleep Med. 2024;20(7):1209-1211.

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.

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.

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.

Ischemic priapism caused by self intracavernous injection of tadalafil.

Introduction: We present a case of ischemic priapism caused by self intracavernous injection of tadalafil. Case presentation: A 77-year-old man developed priapism due to self-injection of tadalafil into the corpus cavernosum. He presented to our hospital 2 days after the development of priapism and severe penile pain. The blood gas analysis of the corpus cavernosum revealed ischemic priapism. At first, we performed percutaneous distal shunt (T-shunt) and cavernosal irrigation, resulting in slight improvement of penile tumescence. Several hours later, penile tumescence and severe pain reappeared. Bilateral proximal (corpora-spongiosal) shunt was performed under anesthesia again. Penile tumescence was slowly and gradually relieved. His erectile function was declined. Conclusion: We experienced a case of priapism due to self intracavernous administration of tadalafil who needed a proximal shunt to relieve the severe penile pain. This case report may serve as a warning for physicians and patients not to use phosphodiesterase 5 inhibitor inappropriately.

Endovascular intervention with high-flow priapism: A case report.

High-flow priapism is a rare condition in the form of priapism unrelated to sexual stimulation. The disease causes a decline in quality of life and has the risk of causing long-term erectile dysfunction if not treated promptly. We report a case of a 48-year-old male patient with prolonged high-flow erection after trauma who received endovascular intervention with n-butyl-2 cyanoacrylate biological glue. Results after 40 days of intervention and use of sildenafil 25 mg/day, the patient has an erection and has normal sexual activity. Cavernous arteriovenous fistula causing priapism with high flow is a rare condition. Computed tomography helps supplement information about feeding vessels as well as accompanying injuries for comprehensive assessment before treatment. Currently, there are many treatment methods, but transcatheter arterial embolization is modality of choice for achieve clinical efficacy and can safely and flexibly reduce the risk of secondary erectile dysfunction.

Refractory Ischemic Priapism Following Hemodialysis.

Priapism, characterized by prolonged and painful penile erection, is a rare urological emergency with diverse etiologies. We present a case of refractory ischemic priapism following hemodialysis in a 57-year-old male with a history of type II diabetes mellitus, hypertension, and end-stage renal disease. Despite standard conservative management, the patient's condition persisted, necessitating penile distal shunting through an intracorporeal dilatation plus Al-Ghorab corporoglandular shunt. Blood gas analysis of corpus cavernosum blood revealed severe acidosis and hypoxemia, emphasizing the systemic impact of ischemic priapism. The patient's history of erythropoietin injections and the administration of heparin during dialysis emerged as potential contributors to priapism. We discuss the complex interplay between erythropoietin, coagulation cascade, and heparin in the context of priapism development. The case underscores the need for further research to understand the specific mechanisms contributing to priapism in patients undergoing hemodialysis.

Priapism as an Unusual Symptom of T-cell Acute Lymphoblastic Leukemia in a Pediatric Case.

Acute lymphoblastic leukemia (ALL) in pediatric patients typically presents with recognizable symptoms such as fever, pallor, and bone pain. However, atypical manifestations can complicate the diagnostic landscape. We present a unique case of a seven-year-old male with T-cell ALL whose presenting symptom was priapism. This case underscores the need for heightened awareness among healthcare professionals regarding the diverse clinical presentations of leukemia, emphasizing the importance of a multidisciplinary team approach for comprehensive evaluation and management. Our seven-year-old patient presented with priapism. A comprehensive diagnostic workup, including complete blood counts and subsequent bone marrow examination, led to the diagnosis of T-cell ALL. Given the rare presentation, a multidisciplinary team consisting of pediatric oncologists/hematologists, urologists, and other relevant specialists collaborated to formulate a tailored treatment plan. The patient received an intensified chemotherapy regimen, resulting in the resolution of priapism and hematologic improvement. Priapism as an initial presentation of T-cell ALL in a pediatric patient is an exceptional occurrence, necessitating a specialized and collaborative approach to diagnosis and management. This case report highlights the importance of interdisciplinary coordination involving pediatric oncologists and urologists in addressing the unique challenges posed by atypical leukemia presentations. The rarity of this manifestation emphasizes the need for further research to elucidate the underlying mechanisms and establish optimal management strategies for similar cases.

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)