Emergency department management of patients with sickle cell disease.

Sickle cell disease is a chronic hematologic disease that affects over 100,000 people in the United States. Many of these patients will present to the emergency department seeking treatment for an acute complication. Vaso-occlusive crisis, the most common recurring complication, can be difficult to manage because of the stigma patients face surrounding management of their pain. Patients with sickle cell disease presenting with conditions such as pain, infection, respiratory distress, stroke, or priapism must be given special consideration, as management can differ from that of the general population. This review evaluates the current guidelines and literature on acute complications related to sickle cell disease to dispel misconceptions about seemingly harmless interventions and provide clarification on those that are more controversial. Novel treatments that may have future impact on the management of patients with sickle cell disease are also reviewed.

[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.

What Is Priapism?

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

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.

Priapism in children: an experience of the modified Winter procedure.

BACKGROUND: Priapism in children is a rare disease, which seldom presents during the pediatric surgery practice. It is, however, a surgical and urological emergency. Early diagnosis and prompt management can prevent the devastating sequelae of this potentially fatal condition. MATERIALS AND METHODS: A prospective study was conducted between March 1st, 2007 and February 28th, 2019 at the Department of Pediatric Surgery, Khyber Teaching hospital, Peshawar. All the patients between 3 and 15 years of age, with the diagnosis of priapism, were enrolled in the study with ethical approval. RESULTS: A total of ten patients were enrolled in the study period from March 1st, 2007 to February 28th, 2019. The age ranged between 3 and 15 years and the mean age of presentation was 8 years. The mean duration of symptoms was 4 h. The mean hospital stay was 4 days. The modified Winter procedure by creating a corporoglanular shunt was performed in all cases. Successful detumescence was achieved in eight patients, while two patients needed further detumescence and manual evacuation. Symptomatic relief was achieved in all the children. CONCLUSION: Priparism in children is a rare urological emergency that can lead to permanent erectile dysfunction if prompt medical intervention is not done. The modified Winter procedure technically is a less invasive procedure to achieve satisfactory clinical outcome in terms of achieving good erectile functions.

Diagnosis and Management of Priapism.

This JAMA Clinical Guidelines Synopsis summarizes the 2022 American Urological Association/Sexual Medicine Society of North America guidelines on diagnosis and management of priapism.

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

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

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.

Management of Priapism in the Emergency Department.

Patients presenting to the emergency department with priapism require immediate evaluation and treatment. Priapism is a urological emergency that carries the risk of erectile dysfunction if not managed in a timely manner. Therefore, it is important for providers to be able to identify and manage these patients emergently. Priapism has various causes, and knowing the difference between high-flow and low-flow priapism will help determine the appropriate patient management. Although the prevalence of priapism is thought to be low, there have been increasing reports over the years linked to new drugs used for the treatment of erectile dysfunction (Roghmann et al., 2013). For this reason, it is imperative that providers understand the etiology behind the different causes of priapism. Using a case of drug-induced priapism as an exemplar, this article discusses the epidemiology, etiology, and management of priapism conditions.

Treatment dilemmas: strategies for priapism, chronic leg ulcer disease, and pulmonary hypertension in sickle cell disease.

Sickle cell disease is a disorder characterized by chronic hemolytic anemia and multiorgan disease complications. Although vaso-occlusive episodes, acute chest syndrome, and neurovascular disease frequently result in complication and have well-documented guidelines for management, the management of chronic hemolytic and vascular-related complications, such as priapism, leg ulcers, and pulmonary hypertension, is not as well recognized despite their increasing reported prevalence and association with morbidity and mortality. This chapter therefore reviews the current updates on diagnosis and management of priapism, leg ulcers, and pulmonary hypertension.

Management of Priapism: 2021 Update.

Priapism is defined as a persistent penile erection lasting more than 4 hours. Priapism is a rare condition but when present it requires prompt evaluation and definitive diagnosis. Priapism has 2 pathophysiologic subtypes: ischemic and nonischemic. Ischemic priapism accounts for a majority of cases reported. Ischemic priapism is a urologic emergency and requires intervention to alleviate pain and prevent irreversible damage to erectile tissues. This article highlights current guidelines and the contemporary literature on priapism.

Acute Ischemic Priapism: An AUA/SMSNA Guideline.

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

Characteristics and Long Term Follow up of Men Who Suffer Ischemic Priapism Secondary to Recreational Use of Intracavernosal Injectable Medications.

OBJECTIVE: To better understand patient experience, risk factors, culture, and ED outcomes surrounding recreational ICI use that led to ischemic priapism. METHODS: After IRB approval, men presenting for ischemic priapism secondary to recreational ICI use from January 2010 to December 2018 were contacted by mail and then via telephone. Standardized questions were asked of all study participants on the topics of erectile function (IIEF-5), sexual practices, and at-risk behavior at the time of priapism. Qualitative data analysis was performed using grounded theory methodology. RESULTS: 14 men age 24-59 were successfully recruited. All men described themselves as men having sex with men (MSM) and one (7.1%) as having both male and female sexual partners. Average follow up IIEF-5 among participants was 13 (SD 4.0). Eleven men (78.6 %) described illicit drug use at the time of priapism. Qualitative data analysis yielded several preliminary themes: concomitant drug use, naivety, peer pressure, and delay in seeking treatment. Men frequently reported illicit drug use in group sex scenarios and ICI use under pressure to perform sexually or to counteract effects of illicit substances. CONCLUSIONS: Recreational ICI in this cohort was part of a lifestyle of risky behavior. Methamphetamine use and group sex encounters strongly motivate recreational ICI use. Substance abuse centers may offer an entry point into this population for counseling and primary prevention.

Managing Reproduction Emergencies in the Field: Part 1: Injuries in Stallions; Injury of the External Portion of the Reproductive Tract and Gestational Conditions in the Mare.

Selected emergency conditions of male and female reproductive tracts in horses are described, including injuries affecting the external genitalia of male horses and emergent conditions arising during gestation in mares. Conditions affecting male horses are discussed in the context of breeding stallions, but kicks or other mechanisms of trauma in the groin can also affect geldings. Priapism, paraphimosis, trauma to the scrotum and testicles, and penile injury are discussed. In mares, traumatic vestibular injury, placentitis, hydropsic conditions, prepubic tendon and abdominal wall compromise, and uterine torsion are included. Clinical recognition of the problem, diagnostic procedures, and treatments are summarized.

Veno-Occlusive Priapism in COVID-19 Disease.

Infection by COVID-19, being a respiratory disease caused by SARS-CoV-2, can predispose to arterial and venous thrombotic disease, in response to excessive inflammation, platelet activation, endothelial dysfunction, and venous stasis. During the COVID-19 pandemic period, the technological and resource availability for the care of these patients with thrombotic disease is critical, marking a factor of morbidity and poor prognosis in these cases. We describe a case of priapism in a patient with COVID-19, during the course of systemic inflammatory response syndrome and respiratory distress syndrome with a procoagulant state, seeking to relate the pathophysiological factors of ischemic priapism in patients with infection with SARS-Cov-2.

Priapism and Sickle Cell Disease: Special Considerations in Etiology, Management, and Prevention.

Sickle cell disease (SCD) is an inherited medical condition where sickled red blood cells cause vaso-occlusive crisis. One major complication of SCD is priapism, defined as an erection of the penis lasting over four hours beyond sexual stimulation or orgasm. SCD priapism is caused by sickled erythrocytes obstructing venous outflow and can lead to permanent erectile dysfunction. This article reviews the pathology, physiology, and management of SCD priapism, including potential novel therapeutic agents.

Priapism in COVID-19: A thromboembolic complication.

SARS-CoV-2 (COVID-19) infection is frequently associated with thromboembolic complications. In this case report, we describe the diagnosis and management of priapism as a thromboembolic complication of severe COVID-19.

Priapism, a symptom of claudication of the cauda equina in spinal stenosis.

Priapism is defined as a persistent penile erection in the absence of sexual arousal. This symptom has been documented in patients with spinal stenosis although it is considered a rare finding. The European Association of Urology guidelines on priapism [Salonia et al., 2014] list cauda equina syndrome and spinal stenosis as causative factors for ischemic priapism although the literature describing this phenomenon appears sparse. Priapism can be a rare symptom of lumbar spine stenosis/transient cauda equina compression. This presentation is complex and believed to be a parasympathetic mediated autonomic disorder. This article discusses the relationship between spinal stenosis, cauda equina syndrome and priapism using available literature. Greater awareness of this clinical finding may help clinicians in their clinical decision making. In patients with suspected cauda equina syndrome, subjective enquiry regarding the symptom priapism may add to the patients overall clinical picture.

[When a urological emergency indicates an internal medical crisis : Priapism as the first clinical manifestation of leukemia]. / Wenn ein urologischer Notfall auf eine internistische Krise hinweist : Priapismus als klinische Erstmanifestation einer Leukämie.

Priapism as a sign of a severe hematological disease is a rare event, which has to be considered as both a urological and a hematological emergency that requires immediate treatment. This article describes a clinical case of priapism as the first clinical manifestation of a hitherto undiagnosed chronic myeloid leukemia (CML) and discusses the results of a literature review on this topic.

Pretreatment screening and counseling on prolonged erections for patients prescribed trazodone.

PURPOSE: We examined whether patients are appropriately screened for previous prolonged erections or priapism and counseled about trazodone complications, specifically prolonged erections and priapism, prior to trazodone treatment. MATERIALS AND METHODS: We identified patients under the age of 50 on trazodone as of February 27, 2019 at the VA New Jersey Health Care System. Patients were asked about information provided to them prior to medication initiation, occurrence of prolonged erections/priapism, and reporting rate of side effects. RESULTS: Two hundred and twenty nine out of five hundred and twenty four male patients agreed to participate in the study. Forty three out of two hundred and twenty nine of patients were informed about the side effects of prolonged erections and 37/229 of patients were informed of risk of priapism prior to treatment. Only 17/229 of patients were asked if they had had any episodes of prolonged erection or priapism in the past. Eighteen patients developed prolonged erection while taking trazodone. Only 5/18 patients who had developed prolonged erections informed their physicians. CONCLUSIONS: Only a fraction of patients were properly screened for previous prolonged erections or priapism and properly informed about the side effects of trazodone. Urologist should better educate trazodone prescribers, such as family medicine and psychiatric colleagues, regarding the side effects of trazodone. It is imperative that prescribing physicians appropriately screen and educate patients prior to trazodone initiation and instruct patients to report any treatment side effects to avoid potential long-term adverse outcomes.