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A pathophysiology-based approach to the management of early priapism.

ABSTRACT: Priapism is a rare condition that involves persistent penile erection for greater than 4 h. Distinct variants exist, each with unique characteristics. Ischemic priapism is a painful medical emergency that may occur as a result of veno-occlusion leading to hypoxia and tissue death. Recurrent bouts of ischemic priapism, or stuttering priapism, require treatment for individual attacks as well as long-term prevention. Non-ischemic priapism is associated with trauma and may be managed conservatively. Recent advances into the pathophysiology of priapism have allowed the development of treatment algorithms that specifically target the mechanisms involved. In this review, we outline the basics of smooth muscle contraction and describe how derangement of these pathways results in priapism. A pathophysiological approach to the treatment of priapism is proposed with duration-based algorithms presented to assist in management.

SUBMITTER: Kovac JR

PROVIDER: S-EPMC3739130 | biostudies-literature | 2013 Jan

REPOSITORIES: biostudies-literature

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A pathophysiology-based approach to the management of early priapism.

Kovac Jason R JR Mak Siu K SK Garcia Maurice M MM Lue Tom F TF

Asian journal of andrology 20121203 1

Priapism is a rare condition that involves persistent penile erection for greater than 4 h. Distinct variants exist, each with unique characteristics. Ischemic priapism is a painful medical emergency that may occur as a result of veno-occlusion leading to hypoxia and tissue death. Recurrent bouts of ischemic priapism, or stuttering priapism, require treatment for individual attacks as well as long-term prevention. Non-ischemic priapism is associated with trauma and may be managed conservatively. ...[more]

PMID: 23202699

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Project description:Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4-18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months (n = 1), over 6 weeks (n = 1), over 1-2 weeks (n = 2), over several days (n = 2), or 24 h (n = 1), while the remaining 4 boys reported continuous erection lasting over 11-12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700-899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin (n = 2), penile puncture (n = 5) ± injection of sympathomimetics (n = 4) ± intracavernous shunt operation (n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19-25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients' lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy.

Project description:Penile shunting is the standard of care in management of ischemic priapism refractory (IPR) to non-surgical interventions. Due to high rates of impotence, corporal fibrosis, and loss of penile length, recent literature suggests these patients benefit from immediate penile prosthesis (PP) placement. An IRB-exempt anonymous electronic survey of the 2168 members of the International Society for Sexual Medicine (ISSM) was conducted. The survey included demographic information, confidence, and experience-related management of IPR. The aim was to evaluate current practice patterns in management of IPR and to investigate the role of immediate PP implantation in the management of prolonged (>36 h) IPR. The survey response rate was 11.6% (n = 251). Most respondents were urologists (173), from the USA (49.1%), and had completed a fellowship in male sexual medicine, men's health, reconstruction, or andrology (71.1%). The majority (91.3%) see at least one case of prolonged priapism (>36 h) that requires surgical management yearly. When looking at volume in training and after, our respondents had a significantly higher experience with penile prostheses (over 70%, > = 10) as compared with shunts (less than 40%, > = 10). Overall, 70.9% of respondents felt more comfortable with a malleable PP than a shunt. However, penile shunts are still preferred as the first line of surgical management by ~80% of respondents as compared with 12.7% who instead prefer a PP. We also found that under 40% of respondents currently use penile MRI or corporal biopsies in their management of prolonged assessment. This is the first study to assess current clinical practices in management of IPR globally. As in any anonymous self-reported survey-based research, recall and sampling bias are an inherent limitation. Penile shunting for IPR continues to be the preferred treatment despite emerging data. Respondents performed PP surgery routinely and feel more confident placing PP than performing penile shunting procedures.

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A pathophysiology-based approach to the management of early priapism.

Publications

A pathophysiology-based approach to the management of early priapism.

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