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.

Malignancy: A Rare, Important and Poorly Understood Cause of Priapism.

INTRODUCTION: Priapism is rare yet has the potential to inflict significant suffering on patients, often with lasting consequences such as erectile dysfunction, corporal muscle necrosis, and a loss of sexual function. Although rare, it is a pathology that has received little focus in the literature, particularly that caused by malignancy, and it is in this form that the long-term prognosis becomes particularly poor. AIM: This review looks at malignant priapism in detail moving from the etiology and pathogenesis through investigations and management to provide an up-to-date picture. METHODS: In so doing, more than 30 articles are reviewed and examined from databases such as PubMed. Significant cases are provided as examples to provide a comprehensive review of a topic that receives little attention but can cause significant patient morbidity. MAIN OUTCOME MEASURE: The main outcome measure was the use of aspiration, sympathomimetics, and surgery as the main treatment modalities and how each one is used with regard to both the underlying etiology of the priapism and also the prognosis. We look at the need for treatment and how that relates to quality of life and erectile function thereafter. RESULTS: Solid tumor invasion-both primary and secondary-and hematologic malignancies represent the key etiologies of malignant priapism and aggressive treatment is needed. Recovery of erectile function can occur if intracavernosal phenylephrine is quickly administered or distal shunts are placed; however, the prognosis is often poor, and subsequent chemotherapy treatment is often required. CONCLUSION: The importance of a clear history and examination cannot be understated, and although the prognosis is often poor, this review hopes to give clinicians better understanding to be able to recognize malignancy as a potential cause of priapism. Ralph O, Shroff N, Johnson MJ, et al. Malignancy: A Rare, Important and Poorly Understood Cause of Priapism. J Sex Med 2021; 9:312-319.

The surgical management of ischaemic priapism.

Priapism is a urological emergency that is defined as a prolonged penile erection lasting more than 4 h, remaining despite orgasm and in the absence of sexual stimulation. Without prompt and complete detumescence, time-dependent changes occur to the smooth muscle of the corpus cavernosa that can result in permanent erectile dysfunction and penile deformity (curvature, shortening and loss of girth). The diagnosis is confirmed with a hypoxic and acidotic blood sample from the corpus cavernosa. The trapped blood inside the corpus cavernosa is aspirated and can be irrigated with 0.9% normal saline. Intracavernosal injection of a sympathomimetic agent is used to cause smooth muscle contraction if the previous measures fail. Failure or recurrence of priapism following these conservative measures is an indication for surgical management. Shunt procedures that create a connection with the corpus cavernosa and a neighbouring structure are often used first line. Multiple shunt procedures have been described and these are summarised in this article. Distal shunt procedures are the most commonly used as they are easier to perform and seem to have at least comparable detumescence and potency rates. Refractory or prolonged (>48 h) ischaemic priapism maybe an indication of immediate placement of a penile prosthesis.