Madelung disease with postoperative priapism and multiple venous thromboses: case report and literature review.

Madelung disease is an uncommon metabolic disorder of uncertain pathogenesis, distinguished by the symmetric accumulation of nonencapsulated adipose tissue within the subcutaneous layer of the neck, abdomen, thighs, and other anatomical regions. This condition has been tightly connected with comorbidities including diabetes, dyslipidemia, hyperuricemia, hypothyroidism, and adrenal dysfunction, as well as sensory, motor, and autonomic polyneuropathy. The prevalence of Madelung disease is conspicuously higher in Mediterranean and Eastern European, with a distinct scarcity within the Asian population. Surgical interventions involving lipectomy and liposuction represent the foremost and most efficacious treatment approach. Herein, we present a case encompassing type II Madelung disease featuring bilateral thighs adipose tissue accumulation. The patient exhibited unexplained priapism alongside multiple venous thrombosis during four surgical interventions. The infrequent manifestation of postoperative hypercoagulability in patients of Madelung disease merits broad attention, owing to the potentiality for extensive venous thrombosis and consequential severe outcomes such as pulmonary embolism or cerebral infarction arising from thrombus dislodgment. Building upon this clinical scenario, we systematically documented the clinical manifestations and disease progression in this patient, meticulously analyzed the causes of complications, and proposed targeted preventive measures. Additionally, we conducted a comprehensive review of the relevant literature to summarize the clinical and epidemiological features of Madelung disease and to elucidate its mechanisms. This study will provide a valuable reference for future clinical treatments and mitigate perioperative complications of Madelung disease.

Priapism Presentations in a Saudi Arabian Emergency Department: A Retrospective Study at a Tertiary Care Hospital.

OBJECTIVES: To examine the distribution, clinical characteristics, and management of priapism in a Saudi Arabian tertiary care setting to provide a regional perspective. SUBJECTS AND METHODS: This retrospective chart review included 29 male patients presenting with priapism at a tertiary care hospital in Riyadh, Saudi Arabia, from January 2011 to June 2023. Data were collected on patient demographics, clinical presentation, treatment modalities, and outcomes. RESULTS: The study found recurrent episodes of priapism in many patients, with a significant number associated with hematological diseases, notably sickle cell disease. Most treatments involved non-surgical methods. A notable finding was the correlation between the duration of priapism episodes and the likelihood of hospital admissions, suggesting that prolonged episodes often required more extensive medical attention. CONCLUSIONS: Priapism often presents as a chronic and recurrent condition requiring personalized management strategies. This study emphasizes the importance of recognizing regional occurrence patterns to enhance the management of priapism and suggests a need for further research in regions where this condition is less common.

Treatment of obstructive sleep apnea relieves stuttering priapism in patient with sickle cell disease: a case report.

Background: Stuttering priapism is recurrent, self-limited episodes of sustained penile erection and is common in patients with sickle cell disease (SCD). Prevention of stuttering priapism is important to avoid progression to episodes of ischemic priapism which can cause erectile dysfunction. Priapism has been shown to be associated with increased nocturnal hypoxemia in patients with SCD. Case Description: A 43-year-old male with nocturnal episodes of stuttering priapism that was refractory to treatment with multiple medications was found to have obstructive sleep apnea (OSA). Following treatment of this condition with a continuous positive airway pressure (CPAP), the patient had immediate symptom relief and has had three months without an episode of priapism. Conclusions: OSA should be considered as an underlying cause of nocturnal stuttering priapism in patients with SCD, particularly in patients who present with stuttering priapism later in life or patients who present strictly with nocturnal episodes. Appropriate management of OSA can significantly decrease the incidence of stuttering priapism in patients with SCD.

Contemporary management of ischemic priapism: A 12-year population-based analysis from a large US database.

BACKGROUND: Priapism is a urological condition characterized by a persistent erection. The management varies based on its subclassifications. Despite established clinical guidelines for ischemic priapism, there is a lack of large-scale research focused on patient characteristics and management strategies. OBJECTIVES: To analyze the contemporary management of ischemic priapism in the US, exploring patient demographics and clinical characteristics, as well as predictors of erectile dysfunction (ED) and penile prosthesis implantation (PPI). MATERIALS AND METHODS: We performed a retrospective analysis of the PearlDiver Mariner database, reviewing records from 2010-2021. Adult males diagnosed with ischemic priapism were included. Data analysis covered demographic, clinical variables, and management strategies. Predictors of de novo ED and PPI were evaluated using multivariable logistic regression analysis. RESULTS: Of 36,120 patients, most (93%) received only medical management, and a minority underwent surgical interventions (penile shunt surgery [PSS], PPI or both). Medical management was typically effective, as 67.08% of the patients in this group experienced only one episode of priapism. However, de novo ED occurred in 16.57% of these patients. The majority of patients undergoing PPI had an inflatable prosthesis (81%). Older age (odds ratio, OR 1.02), the presence of metabolic diseases (OR 1.39), neurogenic disorders (OR 1.72), solid pelvic malignancies (OR 1.09), and multiple episodes of priapism were identified as significant predictors of de novo ED (all p < 0.05). Similarly, age (OR 1.03), the presence of metabolic diseases (OR 1.23), solid pelvic malignancies (OR 1.99), and multiple episodes of priapism were associated with higher likelihood of PPI (all p < 0.05). CONCLUSION: Most cases of ischemic priapism are managed with the medical therapy. Less than 3% of patients with ischemic priapism receive PPI, and when this occurs an inflatable prosthesis is favored. Age, specific comorbidities, and multiple episodes of priapism appear to be significant predictors of ED and PPI.

Sildenafil-induced priapism in a dog : an unusual case report.

BACKGROUND: Priapism is defined as erection that lasts for more than 4 h without sexual stimulation. There are various causes of priapism, but there are no reports of sildenafil-induced priapism in dogs. In human medicine, there were no pre-marketing reports of priapism caused by sildenafil, but post-marketing surveillance has shown that it is rare. In cases of pulmonary hypertension in dogs, sildenafil is the first-line drug of choice for symptomatic relief. CASE PRESENTATION: An 11-year-old neutered male Maltese dog that presented with tachypnea and cough was diagnosed with myxomatous mitral valve disease, American College of Veterinary Internal Medicine (ACVIM) stage C, and was treated medically. Eighteen months after the diagnosis, severe pulmonary hypertension occurred due to left heart disease. At 20 months postdiagnosis, pleural effusion occurred, and sildenafil (2 mg/kg twice daily) was added to the existing treatment. Two weeks later, the dyspnea recurred, confirming pleural fluid recurrence, and sildenafil was increased to 2 mg/kg thrice daily. One day later, the patient developed persistent erections and penile pain. Penile amputation and urethrostomy were recommended but were refused; therefore, analgesia and palliative care were provided. The patient died of acute dyspnea 22 months after the first presentation, with no specific priapism recurrence at the time of death. CONCLUSION: To the best of our knowledge, this is the first report of sildenafil-induced priapism in a dog with pulmonary hypertension.

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.

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.

Treatment of Refractory Idiopathic Ischemic Priapism: A Case Report and Literature Review.

Priapism is a medical condition characterized by a prolonged period of penile rigidity in the absence of external sexual stimulation. Three broad categories exist for this condition: ischemic (low venous flow), nonischemic (high arterial flow), and recurrent (stuttering). Ischemic priapism is a urological emergency necessitating immediate medical attention. This literature aims to highlight the importance of prompt workup and treatment of ischemic priapism in order to prevent irreversible damage to the penis, such as erectile dysfunction and impotence. This case report presents a 35-year-old patient who developed refractory ischemic priapism in the absence of an underlying causative agent. Fortunately, through pharmacological and surgical interventions, the patient was successfully treated with complete resolution of his symptoms.

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.

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.

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.

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.

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.

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.

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)

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.

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.