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.

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.

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.

Case Report: Priapism Following an Anterior Lumbar Interbody Fusion.

Priapism is a disorder where the penis without sexual stimulation maintains a prolonged rigid erection lasting 4 or more hours. There are two classifications of priapism, ischemic (low flow) or nonischemic high flow, and each have specific etiologies, diagnostic criteria, and management. This presented case study involved a 58-year-old male who experienced an ischemic priapism more than 24 hours after an anterior lumbar interbody fusion (ALIF). A flaccid penis was achieved after the patient received two 400 µg of phenylephrine HCL into the corpora cavernosum. Review of the literature suggests anesthetic medications given during the surgical procedure may have caused the priapism. Lessons that can be learned from this case study highlight that even though the nurse may not expect to see a priapism after an ALIF, the nurse must always be diligent and not become complacent with unexpected findings or assessments that may cause irreparable harm to the patient.

Therapeutic outcomes and analysis of Doppler findings in 25 patients with non-ischemic priapism.

Non-ischemic priapism (NiP) is painless partial tumescence caused by genital trauma and the formation of intracorporal arterio-venous fistula. This is a retrospective study of 25 men with NiP and reports the long-term erectile function and colour doppler ultrasound (CDUS) findings after treatment for NiP. Unstimulated CDUS was performed at diagnosis, 1 week and at last follow-up after treatment. CDUS traces were analysed: peak systolic velocity (PSV), end diastolic velocity (EDV), resistive index (RI) and mean velocity (MV) were calculated. Erectile function was assessed using the IIEF-EF questionnaire. At the last follow-up (median 24 months), 16 men had normal erectile function (64%): median IIEF-EF score 29 (IQR 28.5-30; σ2 2.78) and nine had erectile dysfunction (36%): median IIEF-EF score 17 (IQR 14-22; σ2 33.6). MV and EDV were statistically higher in those patients with erectile dysfunction at last follow-up compared to patients with normal erectile function: median MV 5.3 cm/s (IQR 2.4-10.5 cm/s; σ2 34) vs 2.95 cm/s (IQR 1.03-3.95; σ2 3.4) p < 0.002 and median EDV 4.0 cm/s (IQR 1.5-8.0; σ2 14.7) vs 0 cm/s (IQR 0-1.75; σ2 2.21) p < 0.004. Erectile dysfunction was observed in 36% of men treated for NiP and was associated with abnormal low resistance resting CDUS waveforms. Further investigation for persistent arteriovenous fistulation should be considered in these patients.

Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report. / Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso.

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.

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.

High flow priapism following the insertion of an inflatable penile prosthesis mimicking autoinflation: a case report.

Penile prosthesis implantation is a surgical option for erectile dysfunction when other treatments fail or the patient prefers implantation. Although penile prosthesis is generally considered safe and effective, various complications have been reported in the literature. High-flow priapism, resulting from an arteriovenous fistula between the cavernosal artery and the corpora cavernosa, is a rare complication after penile prosthesis implantation. Managing the condition as autoinflation may lead to unfortunate complications. A 54-year-old male patient underwent a penile prosthesis implantation due to erectile dysfunction lasting for 5 years. Doppler ultrasound revealed arterial insufficiency that was refractory to oral and intracavernosal treatments. A 3-piece inflatable penile prosthesis (Coloplast - Titan) was implanted through a midline penoscrotal incision without any complications. The patient reported uncontrolled tumescence after activating the device, which led us to suspect autoinflation. The final diagnosis was high-flow priapism due to an arteriovenous fistula in the cavernosal artery. The patient was given an antiandrogenic medication and the prosthesis was deflated for 3 months. The fistula closed without any additional intervention. High-flow priapism is a rare but potential complication of penile prosthesis implantation. Careful evaluation and management of patients' symptoms are necessary for diagnosing and treating this condition. This case highlights the importance of considering high-flow priapism as a potential cause of uncontrolled tumescence after penile prosthesis implantation and the possibility of successful non-surgical management.

Erectile response profiles of men using PDE5 inhibitors combined with intracavernosal injections as part of a penile rehabilitation program after radical prostatectomy.

BACKGROUND: Phosphodiesterase type 5 (PDE5) inhibitor labeling states that these agents should not be used in conjunction with other erectogenic medications for fear of priapism occurring. AIM: We explored the risk of priapism and prolonged erections in men in our post-radical prostatectomy (RP) penile injection program who were using regular PDE5 inhibitor and intracavernosal injections (ICIs) as part of their rehabilitation program. METHODS: The study cohort included men on penile injection therapy who (1) were taking tadalafil 5 mg daily or taking sildenafil 25 mg on noninjection days, (2) had an RP, (3) were using their respective PDE5 inhibitor regularly at the time of penile injection training, and (4) complied with the program instructions regarding penile injection use. Demographics, comorbidity details, PDE5 inhibitor dose and utilization, and injection dose and utilization data were collected. All patients underwent in-office injection training and used trimix (papaverine/phentolamine/prostaglandin E1) as the intracavernosal medication. OUTCOMES: Priapism was defined as a patient self-reported penetration hardness erection ≥4 hours in duration, while prolonged erection was defined as a penetration hardness erection lasting ≥2 hours. RESULTS: A total of 112 tadalafil users and 364 sildenafil users were compared. Mean age and duration post-RP were 62 ± 14 years and 5.2 ± 12 months, respectively, and there was no difference between tadalafil and sildenafil groups. The mean trimix dose was tadalafil 24 ± 24 units and sildenafil 31 ± 37 units (P < .05). Priapism occurred in 2 (1.7%) of 112 tadalafil users and 5 (1.4%) of 364 sildenafil users (P = .47). Excluding those men experiencing priapism on any occasion, those with any reported penetration hardness erection lasting ≥2 hours were 7 (6.3%) of 112 tadalafil users and 12 (3.3%) of 364 sildenafil users (P < .01). A total of 53% of these prolonged erections occurred within the first 6 injections at home (no difference between tadalafil and sildenafil groups). CLINICAL IMPLICATIONS: We emphasize the need for continued monitoring and education on proper injection techniques to minimize the risk of adverse events in ICI and PDE5 inhibitor combination therapy. STRENGTHS & LIMITATIONS: This study has a relatively large patient population with a considerable follow-up time. Additionally, the rigorous training, education, and monitoring of the participants, as well as the use of formal definitions for priapism and prolonged erections, enhances the accuracy and reliability of the results. However, there are some limitations, such as social desirability, confounding factors, and recall bias. CONCLUSION: There is no significant difference in the incidence of priapism in an ICI program in which men combine ICI with tadalafil or sildenafil. However, tadalafil patients had a higher rate of prolonged erections, which was found to occur mostly early during the titration phase.

Hydroxyurea does not reverse functional alterations of the nitric oxide-cGMP pathway associated with priapism phenotype in corpus cavernosum from sickle cell mouse.

Sickle cell disease (SCD) is a genetic disorder that has been associated with priapism. The role of hydroxyurea, a common SCD therapy, in influencing the nitric oxide (NO)-cGMP pathway and its effect on priapism is unclear. To investigate the effect of hydroxyurea treatment on smooth muscle relaxation of corpus cavernosum induced by stimulation of the NO-cGMP pathway in SCD transgenic mice and endothelial NO synthase gene-deficient (eNOS-/-) mice, which are used as model of priapism associated with the low bioavailability of endothelial NO. Four-month-old wild-type (WT, C57BL/6), SCD transgenic, and eNOS-/- male mice were treated with hydroxyurea (100 mg/Kg/day) or its vehicle (saline) daily for three weeks via intraperitoneal injections. Concentration-response curves for acetylcholine (ACh), sodium nitroprusside (SNP), and electrical field stimulation (EFS) were generated using strips of mice corpus cavernosum. The SCD mice demonstrated an amplified CC relaxation response triggered by ACh, EFS, and SNP. The corpus cavernosum relaxation responses to SNP and EFS were found to be heightened in the eNOS-/- group. However, the hydroxyurea treatment did not alter these escalated relaxation responses to ACh, EFS, and SNP in the corpus cavernosum of the SCD group, nor the relaxation responses to EFS and SNP in the eNOS-/- group. In conclusion, hydroxyurea is not effective in treating priapism associated with SCD. It is likely that excess plasma hemoglobin and reactive oxygen species, which are reported in SCD, are reacting with NO before it binds to GCs in the smooth muscle of the corpus cavernosum, thus preventing the restoration of baseline NO/cGMP levels. Furthermore, the downregulation of eNOS in the penis may impair the pharmacological action of hydroxyurea at the endothelial level in SCD mice. This study emphasize the urgency for exploring alternative therapeutic avenues for priapism in SCD that are not hindered by high plasma hemoglobin and ROS levels.

[Non-ischemic priapism (post-traumatic arteriovenous fistula of the right cavernous body) in the background of Landusy-Dejerine myopathy].

Landouzy-Dejerine myopathy (Facial-shoulder-shoulder myodystrophy) is a disease which causes weakness of the muscles of the shoulder girdle, back and hip muscles, which predisposes patients to an increased risk of injury and disability. The article presents a clinical observation of non-ischemic priapism, which developed as a result of perineal trauma with the formation of a fistula of the right cavernous body in a patient against the background of facial shoulder-shoulder myodystrophy. Methods for the differential diagnosis of this condition are also discussed.

Successful Treatment of SCD-Related Priapism With Crizanlizumab: A Case Series.

Priapism, an unwanted, painful, prolonged erection that is unrelated to sexual stimulation, is a common complication of sickle cell disease (SCD). Priapic events in SCD are stuttering, meaning they occur repeatedly with intervening periods of detumescence. Without health care intervention, repeated episodes can lead to erectile dysfunction. There are limited treatment options for SCD-related priapism and no approved targeted therapies. Crizanlizumab is a monoclonal antibody that binds to P-selectin and is used to reduce the frequency of vaso-occlusive crises in patients with SCD. Here, we report the cases of 3 patients with SCD-related priapism who were treated with crizanlizumab. All patients were African American men who experienced numerous priapic episodes that interfered with their daily lives. Upon treatment with crizanlizumab, priapic events were reduced in all 3 patients. These successful cases suggest a potential role for crizanlizumab in the prevention of SCD-related priapism.

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.

Delayed placement of an inflatable penile prosthesis is associated with a high complication rate in men with a history of ischemic priapism.

BACKGROUND: Corporal fibrosis is known to result from prolonged priapism; however, the impact of the timing of penile prosthesis placement after priapism on complication rates is poorly understood. AIM: We sought to evaluate the impact of timing of inflatable penile prosthesis (IPP) placement on complications in men with a history of ischemic priapism. METHODS: We performed a multicenter, retrospective cohort study of patients with a history of priapism undergoing IPP placement by 10 experienced implantation surgeons. We defined early placement as ≤6 months from priapism to IPP. We identified a 1:1 propensity-matched group of men without a history of priapism and compared complication rates between men who had early placement, late placement, and no history of priapism. OUTCOMES: Our primary outcome was postoperative noninfectious complications, and secondary outcomes included intraoperative complications and postoperative infection. RESULTS: A total of 124 men were included in the study with a mean age of 50.3 ± 12.7 years. A total of 62 had a history of priapism and 62 were matched control subjects. The median duration of priapism was 37 (range, 3-168) hours and the median time from ischemic priapism to IPP placement was 15 months (range, 3 days to 23 years). Fifteen (24%) men underwent early (≤6 months) IPP placement at a median time of 2 months (range, 3 days to 6 months) following the ischemic priapism event. The remaining 47 (76%) underwent placement >6 months following priapism at a median time of 31.5 months (range, 7 months to 23 years). The complication rate in the delayed placement group was 40.5% compared with 0% in the early placement group and control group. Cylinder-related complications such as migration or leak accounted for 8 (57%) of 14 of the postoperative noninfectious complications. Full-sized cylinders were used in all patients who had a cylinder related complication. CLINICAL IMPLICATIONS: Priapism patients should be referred to prosthetic experts early to decrease complication rates in those needing an IPP. STRENGTHS AND LIMITATIONS: This is a multicenter study from experienced prosthetic urologists but is limited by the retrospective nature and small number of patients in the early placement group. CONCLUSION: IPP complication rates are high in men with a history of ischemic priapism, especially when implantation is delayed beyond 6 months.

Evaluation of knowledge of priapism in sickle cell patients in Senegal.

PURPOSE: Erectile dysfunction is a dreadful complication of priapism especially with delay in diagnosis and management. The lack of awareness of priapism as a vaso-occlusive complication of sickle cell disease (SCD) is more concerning. The objective of this study was to evaluate the burden of priapism in adult Senegalese males adults with sickle cell disease. METHODS: A cross-sectional study was carried out amongst consecutive consenting males aged 18 years and older with SCD presenting to the in Haematology Department of the National Blood Transfusion Centre (Dakar, Senegal). All participants completed a questionnaire detailing knowledge on the definition of priapism, its association with SCD, consequences of untreated priapism and treatment options. RESULTS: A total of 219 participants completed the questionnaire. The mean age of the respondents was 27.1 years with a range of (18-54). Of the respondents, 78.5% (n = 172) did not have any knowledge of the term "priapism". After the term was explained, 38.4% (n = 84) thought that there may be a risk of developing priapism given the diagnosis of sickle cell disease. Among the participants, 41.5% (n = 91) reported having a history of priapism. Among all patients who experienced priapism, 36.3% (n = 33) did not seek medical attention with episodes of priapism. It was found that 48.4% (n = 106) of the participants thought there may be a risk of irreversible complications associated with priapism and a corresponding proportion, 42% (n = 92) thought this risk was time dependent. 36.5% (n = 80) of patients believed priapism could lead to erectile dysfunction. CONCLUSION: Priapism is a common complication of sickle cell disease in Senegalese adults which is not well known by sickle cell patients. The health authorities must undertake efforts to raise awareness of priapism as a complication amongst sickle cell patients.