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.

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

Recurrent priapism is a rare and poorly known entity. It is defined by recurrent episodes of painful erections that last less than four hours. The etiology is similar to that of ischemic priapism. Episodes lasting more than four hours require immediate intervention to prevent penile fibrosis and subsequent erectile dysfunction. A 42-year-old male with no significant chronic-degenerative history was referred to our medical center from his second-level medical unit after a 56-hour history of ischemic priapism with the persistence of tumescence despite medical and surgical treatment. Upon interrogation, the patient reported stuttering (recurrent) episodes of painful erections lasting approximately three to four hours, not associated with sexual activity or arousal, in the past two years, with spontaneous resolution. He denied the use of psychotropics or drugs for erectile dysfunction. As a palliative measure, a left saphenous-cavernous (Grayhack) bypass was performed, with a 90% decrease in tumescence and total resolution of pain during the first 12 hours. There is little information and treatment recommendations for patients with recurrent priapism, and even less for patients who are refractory to conventional medical and surgical treatment. Recurrent or stuttering priapism is a condition with a low incidence and a pathophysiology compatible with low-flow priapism. It is difficult to treat and has a poor prognosis in terms of erectile function. Likewise, it is mostly associated with the use of psychotropic drugs such as cocaine and marijuana, medications for erectile dysfunction such as phosphodiesterase inhibitors, prostaglandin E1 analogues, and hematological malignancies such as sickle cell anemia and multiple myeloma. The aim of this article is to share our experience with a patient refractory to multiple medical and surgical treatments.

Stuttering Priapism in a Dog-First Report.

A 5-year-old recently castrated male Doberman dog presented for prolonged erection of one week's duration with associated pain and dysuria. This was the fourth episode within a year. Each episode was associated with an unusual event, which was stressful for the dog. Castration performed two months prior to the final episode did not prevent recurrence. Due to tissue necrosis, penile amputation and urethrostomy had to be performed. The dog recovered fully. Prolonged erection that persists beyond or that is unrelated to sexual stimulation is called "priapism". This term refers to the Greek god Priapus, a god of fertility, memorialized in sculptures for his giant phallus. In humans, depending on the mechanism involved, priapism is classified as nonischemic or ischemic. Because prognosis and treatment are different, priapism must be determined to be nonischemic or ischemic. Nonischemic priapism is a rare condition observed when an increase in penile arterial blood flow overwhelms the capacity of venous drainage; it is often associated with penile trauma, and does not require medical intervention. Ischemic priapism is associated with decreased venous return. In humans, ischemic priapism accounts for 95% of cases, the majority of which are idiopathic. Ischemic priapism is a urological emergency; simple conservative measures such as aspiration of blood from the corpora cavernosa and intracavernosal injection of an adrenergic agent are often successful. Stuttering priapism, also called recurrent or intermittent priapism, is a particular form of ischemic priapism reported in humans that is characterized by repetitive episodes of prolonged erections. Management consists of treating each new episode as an episode of acute ischemic priapism, and preventing recurrence with oral medications such as dutasteride and/or baclofen, gabapentin, or tadalafil. To the authors' knowledge, this case is the first report of stuttering priapism in a dog.

Stuttering priapism in a pediatric patient with pheochromocytoma-induced thrombocytosis.

Priapism is an erection of more than 4 h without sexual stimulation. Ischemic priapism may lead to irreversible erectile dysfunction after a long-lasting period. Stuttering priapism is characterized by a pattern of recurrence that may progress to an unrelenting ischemic crisis, which is a urological emergency. Few reports have revealed that priapism is associated with essential thrombocythemia. The reactive thrombocytosis is uncommonly manifested by pheochromocytoma and rarely causes thrombotic events even if the platelet count is extremely high. We presented priapism related to reactive severe thrombocytosis in a 12-year-old male with pheochromocytoma. The cornerstone of care was prompt medical and surgical intervention by a multidisciplinary team approach to save life and preserve erectile function.

Is urgent surgical management necessary for priapism in pediatric patients with hematologic conditions?

INTRODUCTION: Acute priapism is usually considered a medical emergency that warrants prompt urologic evaluation and treatment. Efforts have been made to determine the optimal management strategy for pediatric priapism. OBJECTIVE: The aim of this study is to assess differences in conservative, minimally-invasive, and operative management of acute priapism in the pediatric population. STUDY DESIGN: A retrospective study of pediatric patients with acute priapism from 2015 to 2021 at a single tertiary care children's hospital was conducted. Conservative, minimally-invasive, and operative approaches for the priapism episodes during these hospital encounters were analyzed. RESULTS: Thirty-nine patients were identified with a total of 61 cases of acute pediatric priapism were evaluated in the study period. Eight-three percent of patients were African-Americans, and 72% of patients had a history of sickle cell disease. Oxygen therapy (P = 0.001) and hydration with intravenous fluids (P = 0.00318) were more commonly utilized for hematologic-associated cases compared to other etiologies. For priapism episodes of hematologic etiology, 18 (40.0%) and 18 (40.0%) patients received phenylephrine injection and aspiration/irrigation (e.g., minimally-invasive therapy), respectively, while for the other causes of priapism, three (18.8%) and four (25.0%) received phenylephrine injection and aspiration/irrigation (e.g., minimally-invasive), respectively. Conservative and minimally-invasive treatment resulted in complete resolution of priapism in 27 (60%) and 16 (35.5%) patients with hematologic-associated priapism while 12 (75%) and 1 (6.3%) patients with other etiologies had resolution of priapism with conservative and minimally-invasive treatment, respectively. One patient received shunting in the hematologic group while two patients received shunting in the non-hematologic group (P = 0.1031). DISCUSSION: Hematologic disorders are the most common causes of priapism in children and adolescents. An overwhelming majority of priapism events in the pediatric population can be managed with conservative therapies including oxygenation and intravenous hydration as well as minimally-invasive procedures such as corporal aspiration, irrigation and/or injections. The utilization of corporal shunting, anesthesia, and hospital resources is infrequently necessary for pediatric priapism episodes. CONCLUSION: While urgent surgical management is often performed in the adult population, a minimally-invasive management strategy can be implemented in the pediatric population where an extended period of conservative management that avoids operative management and general anesthesia is effective.

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.

Rare Disorders of Painful Erection: A Cohort Study of the Investigation and Management of Stuttering Priapism and Sleep-Related Painful Erection.

BACKGROUND: A chief complaint of men with stuttering priapism (SP) and sleep-related painful erections (SRPE) is bothersome nocturnal erections that wake them up and result in poor sleep and daytime tiredness. SP and SRPE are rare entities that have similarities in their clinical features, but that require different treatment approaches. AIM: The aim of this study was to describe the clinical features, investigations, and effective management options for men with SP and SRPE. METHODS: Retrospective cohort study of 133 men with bothersome nocturnal painful erections that attended a tertiary andrology unit between 2004 and 2018. These men were divided into 3 groups. Group 1 (n = 62) contains men with sickle cell SP; group 2 (n = 40) has men with non-sickle cell SP and group 3 (n = 31) contains men with SRPE. OUTCOME: To determine the effectiveness of medical and surgical treatments for men with SP and SRPE. RESULTS: Hydroxyurea and automated exchange transfusion were the most effective treatment options in the sickle cell SP group. Hormonal manipulation and α-agonist therapies were effective in both SP cohorts (groups 1 and 2). Baclofen was the most effective therapy in men with SRPE. For men who failed medical management, implantation of a penile prosthesis resulted in complete resolution of the symptoms in men with SP (groups 1 and 2). Surgical management (penile prosthesis implantation and embolization) did not improve the patients' symptoms in the SRPE group. CLINICAL IMPLICATIONS: This study differentiates between sickle cell SP, non-sickle cell SP, and SRPE and describes effective treatment options for each group. STRENGTHS & LIMITATIONS: This is the largest cohort study for both SP and SRPE, respectively. Limitations include its retrospective nature and single-center experience. CONCLUSION: Managing men in these 3 groups differently and in accordance with the proposed treatment pathway provides a more structured approach to the management of these rare conditions. Johnson M, McNeillis S, Chiriaco G, et al. Rare Disorders of Painful Erection: A Cohort Study of the Investigation and Management of Stuttering Priapism and Sleep-Related Painful Erection. J Sex Med 2021;18:376-384.

Successful Management of Psychotropics Induced Stuttering Priapism with Pseudoephedrine in a Patient with Schizophrenia.

Stuttering Priapism is a recurrent, persistent penile erection in the absence of sexual desire due to altered genital hemodynamics, affecting the arterial component (high flow, non-ischemic) or the veno-occlusive mechanism (low flow, ischemic). Both typical and atypical antipsychotics increase the risk for priapism with greater implications in typicals than atypicals. Prompt recognition and treatment are important as 40% to 50% of patients with stuttering priapism may develop an erectile dysfunction if left untreated. There are several case reports in the literature about the association between psychotropic agents and priapism. However, there are no reports of successfully treating stuttering priapism using pseudoephedrine (sudafed) in the adult population. Here we present successful management of psychotropics induced stuttering priapism with pseudoephedrine in a male patient with schizophrenia.

Advances in the understanding of priapism.

Priapism, a persistent penile erection lasting longer than 4 hours and unrelated to sexual activity, is one of the most common emergencies treated by urologists. Priapism can be categorized as ischemic, recurrent ischemic (stuttering), and non-ischemic. Advances in understanding the pathophysiology of various types of priapism have led to targeted management strategies. This review aims to provide an up-to-date picture of the pathophysiology and management of priapism. A search of Medline and PubMed for relevant publications using the term "priapism" was performed. In addition to the "classical" articles, emphasis was placed on publications from January 2013 to September 2016 to evaluate the most recent literature available. Though advances in both basic and clinical research continue and effective treatment options are available, methods for the prevention of priapism continue to be elusive.

[Priapism: Current diagnostics and therapy]. / Priapismus : Aktuelle Diagnostik und Therapie.

Priapism is characterized by involuntary persistent penile erection after or independent of sexual stimulation. The diagnostic clarification, including patient history, physical findings, duplex ultrasonography and analysis of blood gases is decisive for the underlying pathophysiology and the appropriate therapeutic procedure. Non-hypoxic and non-acidotic blood gas parameters enable a conservative approach, hypoxic, hypercarbic and acidotic parameters may lead to fibrosis of the corpora cavernosa and, in turn, to a loss of penile function. Low-flow or ischemic (veno-occlusive) priapism is an emergency situation and can lead to irreversible erectile dysfunction within 4 h. Treatment consists of blood aspiration and possibly intracavernosal injection of sympathomimetic drugs. A distal shunt is necessary in the case of treatment failure (in rare cases a proximal shunt). Management of recurrent priapism (stuttering) includes self-injection of sympathomimetic drugs and preventive long-term administration of erection inhibitory and erection promoting substances. This concept still needs to be validated. High-flow or non-ischemic priapism does not necessitate immediate treatment measures and should be kept under observation. In cases of a detectable fistula selective artery embolization is often a successful option.

Semi-rigid penile prosthesis as a salvage management of idiopathic ischemic stuttering priapism.

INTRODUCTION: Priapism is the persistent erection resulting from dysfunction of the mechanisms that regulate penile swelling, stiffness, and sagging. It is a full or partial erection that persists for a period more than 4 hours beyond sexual stimulation and/or orgasm or is unrelated to sexual stimulation. Ischemic priapism should be managed in a step-by-step fashion. OBJECTIVE: To demonstrate step-by-step management of stuttering refractory ischemic priapism. We report a case of stuttering refractory ischemic priapism. Moreover, we reviewed different approaches to priapism management in the literature. CASE PRESENTATION: A 53-year-old male presented with a painful erection of 29 hours' duration, probably caused by consumption of alcohol. The penile blood gas showed a pH of 7.08, PCO2 of 75 mmHg and PO2 of 39 mmHg. Aspiration was followed by irrigation of an α-adrenergic, Winter and T-shunt operations were preformed, and finally a semi-rigid penile prosthesis was implanted to overcome the refractory stuttering ischemic priapism. CONCLUSION: In case of stuttering refractory ischemic priapism, immediate implantation of a penile prosthesis is a simple and effective procedure that manages both the acute episode and the inevitable erectile dysfunction that would otherwise occur, while preserving penile length.

Management of Recurrent Ischemic Priapism 2014: A Complex Condition with Devastating Consequences.

INTRODUCTION: The management of recurrent ischemic priapism (RIP) is not clearly defined. Given the rarity of this condition, most treatment options are supported at best by level 3 or 4 evidence. AIM: In this article, we review the current literature regarding the pathophysiology and management of RIP and discuss the risks and benefits associated with each option, which includes ketoconazole (KTZ), 5-α-reductase inhibitors and other hormonal therapies, phosphodiesterase type 5 (PDE5) inhibitors, intracavernosal sympathomimetic injection, oral sympathomimetic agents, and other investigational therapies. METHODS: A comprehensive literature review was performed regarding the management options for RIP. MAIN OUTCOME MEASURE: To examine the pathophysiology of RIP and evaluate the treatment options. RESULTS: Multiple agents have been investigated to manage RIP. KTZ, finasteride, anti-androgens, gonadotropin-releasing hormone agonists, and estrogen have been shown to be effective in several reports, though some of these agents may have significant hormonal side effects. PDE5 inhibitors currently appear to be well tolerated in this patient population, though evidence of its efficacy is limited. Intracavernosal α-agonist therapy may be used to treat episodes of priapism after they occur. Very limited data suggest terbutaline, oral α-agonists, digoxin, hydroxyurea, and gabapentin may have a role in RIP management. CONCLUSIONS: An ideal management strategy for RIP should focus on prevention of priapic episodes using an effective, well-tolerated, cost-effective medication. We currently have several proposed options, with varying efficacy rates and side effect profiles. While significant advancements in our understanding and management of stuttering priapism have been made within the past few years, clearly continuing research and clinical studies are needed to guide our management of this disease process. Hoeh MP and Levine LA. Management of recurrent ischemic priapism 2014: A complex condition with devastating consequences. Sex Med Rev 2015;3:24-35.

Penile Doppler ultrasound in men with stuttering priapism and sickle cell disease -- a labile baseline diastolic velocity is a characteristic finding.

INTRODUCTION: Stuttering priapism (SP) is seen in sickle cell disease (SCD) and characterized by short-lived painful erections. Imbalanced vascular tone is the postulated cause and this may be reflected in changes in baseline penile blood flow as measured using penile Doppler ultrasound (PDU). AIM: The aim of this study was to investigate the baseline penile blood flow characteristics in men with SCD and SP, by comparing with men without SP. METHODS: PDU findings were retrospectively analyzed in 100 men during flaccid state. Nine men had SP (age range 20-40 years), 4 had Peyronie's disease (PD) (35-48 years), 67 men had erectile dysfunction (16-67 years), and 20 men had normal erectile function (18-42 years). MAIN OUTCOME MEASURES: The variables measured were peak systolic and end-diastolic velocities, and the Doppler velocity waveform. Values in men with SP were compared with those in the other groups. RESULTS: Median systolic and diastolic velocity was significantly higher in men with SP (systolic/diastolic velocity was 26/4 cm/second in men with SP vs. 13/0 cm/second, 14/0 cm/second, and 16/0 cm/second in men with PD, ED, and normal erectile function, respectively; P=0.0001). Men with SP had a characteristic low peripheral resistance (PR) waveform with fluctuating velocities; the diastolic velocity was consistently positive (2-7 cm/second) and fluctuated between +2 and +8 cm/second. In comparison, the other 91 men had high PR waveform and consistently negative diastolic velocity (range 0 to -2 cm/second). CONCLUSIONS: Men with SP had a unique baseline Doppler ultrasound waveform, with a low PR waveform and an elevated, variable cavernosal artery velocity. We propose that this may be the sonographic manifestation of a reduced, fluctuating smooth muscle tone and that PDU may have a role for diagnosis and therapeutic monitoring of SP.

Barry shunt for treatment of a 76-hour stuttering priapism without subsequent erectile dysfunction.

INTRODUCTION: This paper reports treatment of a 76-hour low-flow priapism with a shunting procedure that was first described by Barry in 1976. We were able to observe the preservation of erectile function despite the long period of ischemia. A review of the literature shows that there are few reports of erectile function recovery after a priapism of similar duration. MATERIALS AND METHODS: A 42-year-old patient presented with a 76-hour priapism, probably caused by consumption of alcohol and illegal drugs. A Barry Shunt procedure was performed. The erectile function of the patient was assessed by means of International Index of Erectile Function score over a follow-up period of 30 months. Moreover, we reviewed different surgical options for treatment of priapism in the literature. RESULTS: Partial return of erection without sexual arousal occurred on two occasions during the 10-day hospitalization, but was treated by manipulation of the penis, ie, by milking the tumescence into the shunt. After 3 months, the shunt was still palpable as a subcutaneous swelling. Six months postoperatively, the residual swelling had disappeared. The International Index of Erectile Function score was of 21 without phosphodiesterase type 5 inhibitors after a follow-up of 2.5 years. CONCLUSION: Barry shunt is an effective alternative surgical option for the treatment of low-flow priapism. In the case of our patient, it was also effective after a 76-hour-lasting priapism.

Prevention of recurrent ischemic priapism with ketoconazole: evolution of a treatment protocol and patient outcomes.

INTRODUCTION: The management of recurrent ischemic priapism (RIP) is not clearly defined. Ketoconazole (KTZ) is used to treat RIP and produces a temporary hypogonadal state to suppress sleep-related erections (SREs), which often evolve into episodes of ischemic priapism in this population. AIM: We review our experience to prevent RIP using KTZ and present our outcomes using a decreased dose regimen. METHODS: A retrospective chart review and phone survey of 17 patients with RIP was performed. KTZ inhibits adrenal and gonadal testosterone production with a half-life of 8 hours. By suppressing testosterone levels, SREs are interrupted. We compared our previous protocol of three times daily (TID) KTZ dosing with prednisone for 6 months with our current regimen of initiating KTZ 200 mg TID with prednisone 5 mg daily for 2 weeks and then tapering to KTZ 200 mg nightly for 6 months. MAIN OUTCOME MEASURES: The primary outcome was the prevention of RIP using KTZ. Secondary outcomes included side effects secondary to KTZ use and patient satisfaction. RESULTS: All men experienced daily or almost daily episodes of prolonged, painful erections prior to starting KTZ. The mean number of emergency room (ER) visits per patient prior to starting KTZ was 6.5. No patient required an ER visit for RIP while on KTZ. Sixteen of 17 patients (94%) had complete resolution of priapism while on KTZ with effects noted immediately after starting therapy and no reported sexual side effects attributed to KTZ. One man stopped therapy after 4 days because of nausea/vomiting. Fourteen of 16 men eventually discontinued KTZ after a median duration of 7 months. Twenty-nine percent reported no recurrent priapic episodes after discontinuing. A total of 78.6% had partial or complete resolution of symptoms persisting after KTZ was discontinued with a mean post-treatment follow-up of 36.7 months. CONCLUSION: No reliable effective preventative therapy has been identified for RIP. In our relatively sizable single-center experience, KTZ appears to be a reasonably effective, safe, and inexpensive treatment to prevent RIP while preserving sexual function. We now recommend our tapered dose regimen listed above. After 6 months, we recommend stopping the medication as we have found a majority of patients will not need to resume nightly KTZ.