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Priapism: diagnosis and management.

Recent advances in the understanding of erectile physiology have improved the prompt diagnosis and treatment of priapism. During the initial assessment, the physician must distinguish between the two basic types of priapism--low and high flow--because their associated treatment and prognosis differ. To illustrate the diverse manifestation of priapism, we describe the management of four patients with a history of priapism due to varying causes. In addition, we propose an algorithm that provides a systematic and timely approach to treatment. Resumption of erectile function after a prolonged episode of priapism has traditionally been poor but has improved. Patients must be informed that the long-term sequelae of priapism can be avoided with prompt medical or surgical treatment.

Adolescent↗

[A report of two cases of priapism with metastatic penile tumor].

We report two cases of priapism with metastases to the penis. The first case was a 52-year old man, diagnosed as suffering from gastric cancer by endoscopic biopsy five years previously, but for whom no treatment was performed. He visited our office due to priapism with a duration of 11 days. Physical examination showed two palpable mass lesions on the glans. A glansocavernosum shunt (Winter shunt) was performed, but this was not effective. Radiotherapy was also ineffective. Pathological analysis revealed gastric cancer metastasis to the penis and this was diagnosed as the cause of the priapism. He died of respiratory failure on postoperation day 28. The second case was a 64-year old man with kidney cancer. Hemodialysis had been performed due to chronic renal failure for 20 years and visited our office due to priapism from which he had suffered for 30 days. Computed tomography (CT) demonstrated a left renal cell cancer and metastasized to the retroperitoneal lymph nodes. A Winter shunt was performed on the penis and then a cavernosospongiosum anastomosis was done. The priapism improved about 40%. Pathological analysis confirmed that the renal cell cancer had metastasized to the penis and this was concluded to be responsible for the priapism.

Carcinoma, Renal Cell↗

Cocaine associated priapism.

PURPOSE: Cocaine abuse is an ongoing epidemic in the United States. Priapism associated with cocaine use has been reported only twice in the urological literature. To our knowledge we report the first series of priapism associated with cocaine use and the first case associated with the use of this drug in its solid form, known as crack. MATERIALS AND METHODS: We retrospectively reviewed the presentation of 3 patients to our emergency department within the last year. Each patient presented with priapism and no identifiable predisposition other than the use of cocaine within 24 hours, as evident on positive urine toxicology. RESULTS: Each patient delayed seeking treatment, which added to the complexity of therapy. Intracavernosal aspiration and irrigations failed in all 3 cases. Cavernous spongiosal shunting failed in the first 2 cases. Cases 2 and 3 were complicated by the high flow variant of priapism. Case 1 ultimately required partial penectomy for infected, gangrenous, distal penile tissue. CONCLUSIONS: It appears that cocaine can be a cause of refractory priapism and treatment can be challenging. We suggest that urine toxicology screening be considered in such cases. The identification of underlying cocaine abuse is important in preventing priapism recurrence in these patients.

Adult↗

[High-flow priapism in the pediatric age: review].

OBJECTIVE: To review the literature on high flow priapism in children and analyze the etiology, pathophysiological mechanism, diagnostic tests, treatment and complications. METHODS: The literature is reviewed and an additional case of posttraumatic arterial priapism in an 11 year-old boy treated at our hospital is presented. RESULTS: 24 cases of high flow priapism in children were found; 18 had a history of genitoperineal trauma, 5 had sickle cell anemia and one child had Fabry's disease. Diagnosis was based on patient history, intracavernous blood gases and echo doppler findings. Fourteen patients with posttraumatic priapism, one patient with sickle cell anemia and the child with Fabry's disease were treated by pudendal arteriography with embolization. The immediate results and erectile function were good. CONCLUSIONS: Genitoperineal trauma is the most common cause of arterial priapism in children. High flow must be considered in children with sickle cell disease and priapism that do not respond to conventional treatment. Pudendal arteriography with embolization is the treatment of choice. Erectile function is recovered after treatment.

Abdominal Injuries↗

Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism.

The optimal management of prolonged priapism for patients with sickle cell anemia (SCA) has not been established. We prospectively studied in an outpatient setting the efficacy and safety of a procedure that employs aspiration of blood from the corpora cavernosa and irrigation with a dilute epinephrine solution under local anesthesia to relieve priapism in young patients with SCA. If hydration and analgesics failed to produce detumescence or if priapism had lasted >4 hours, the protocol was activated in the emergency room or clinic. Fifteen patients with homozygous SCA (Hb SS) were treated on 39 occasions; 10 patients were treated once, 1 patient twice, 2 patients 3 times, 1 patient 6 times, and 1 patient 15 times. Median age of patients at first treatment was 14.3 years (range, 3.9-18.3 years). The procedure was successful in producing immediate detumescence on 37 of 39 occasions (95% efficacy, 95% confidence intervals (CI): 81%-99%). No serious immediate or long-term side effects were observed. None of the patients who demonstrated detumescence required hospitalization. The 2 patients whose priapism persisted after aspiration and irrigation presented with episodes lasting >24 hours. All evaluable patients whose priapism resolved after aspiration and irrigation self-reported normal erectile function at a median of 40 months (range, 3-58 months) after the last procedure. Thus, aspiration of the corpora cavernosa followed by irrigation with dilute epinephrine is effective in producing immediate and sustained detumescence and should be the initial therapy employed for patients with SCA and prolonged priapism. (Blood, 2000; 95:78-82)

Adolescent↗

[Sickle cell disease priapism: treatment with intracavernous injections of etilefrine].

Intracavernous injection (ICI) of adrenergic agonists has recently been proposed for treatment of priapism associated with sickle cell hemoglobinopathy and appears to be effective in cases less than 28 hours old. The purpose of this study was to confirm the usefulness of this technique in a large series of patients. From January 1996 through September 1998, 19 patients with sickle cell disease were treated by ICI of etilefrine for one or several episodes of low-flow priapism lasting between 2 hours and 15 days. A total of 72 consecutive ICI were performed alone in patients with priapism less than 6 hours old or in combination with prior drainage in patients with priapism more than 6 hours old. Results depended mainly on the delay to treatment. Detumescence was achieved in all 10 cases treated within 29 hours as opposed to only 2 of 9 cases treated beyond 29 hours. This finding is in agreement with experimental findings demonstrating histological evidence of necrosis of endothelial cells and cavernous smooth muscle fibers after 24 hours. However based on our experience showing that good results with preservation of erectile function can be achieved after delays longer than 40 hours (42 hours and 5 days in two of our patients), we recommend that treatment by ICI be tried before surgery in sickle cell anemia patients even with long-standing priapism. Untoward side effects including intense pain in the penis and retrosternal pain were observed in 4 patients. ICI of adrenergic agonists is one of the best treatment currently available for management of priapism in patients with sickle cell disease. Surgery should be used only after failure of ICI.

Adolescent↗

[Priapism: decisional algorithm, our experience, and role of sildenafil in sexual rehabilitation].

OBJECTIVE: We report our experience in the management of priapism. MATERIALS AND METHODS: In a 2-year period we observed 7 patients of whom 4 presented with low flow and 3 with high flow priapism. RESULTS: In 2 of the patients with ischemic priapism, simple blood aspiration from the corpora allowed for a quick detumescence, while in the other 2 cases a derivative intervention (1 spongio cavernous and 1 glans cavernous) had to be performed. In all the 3 patients with high flow priapism we performed a superselective arteriography that obtained the visualisation of the arteriovenous fistula. These patients restarted their sexual activity after about three months. At six months a patient with low flow priapism restored sexual activity due to sildenafil 50 mg. CONCLUSION: The importance of distinguishing low and high flow priapism was confirmed.

Adult↗

[Priapism in the course of generalized atopic dermatitis].

BACKGROUND: Priapism is a pathologically prolonged and painful penile erection, not resulting in ejaculation. Causes include certain oral medication, perineal trauma, thrombo-embolic process or primary priapism. We report a case of priapism secondary to diffuse general atopic dermatitis. CASE REPORT: An 11 year-old child, with atopic dermatitis, presented over the last 36 hours an irreduced painful penile erection. Other than priapism, he presented numerous scratched lesions on the body and the penis having developped over the last 2 months (treated by daily topical corticosteroid application) and consistent with atopic dermatitis exacerbation. Due to the urological emergency, a puncture of the corpora cavernosa was made, followed by an injection of etilephrine in the penis. Complete and definitive detumescence was obtained. Application of betamethasone healed the cutaneous lesions. The child was treated with ciclosporine for his atopic dermatitis with good results at 6 months. DISCUSSION: In our case, hypothesis of myeloid leukemia, sickle-cell disease and essential thrombocythemia were turned down. The young child did not exhibit any recent perineal trauma but the scratched lesions can be considered as microtraumas. The formation of an inflammatory oedema obstructed venous drainage of the penis and provoked priapism. We can not rule out the responsibility of topical corticosteroids in the formation of the priapism in this patient. Indeed, it was the only drug therapy prescribed, and since introduction of ciclosporine, there has been no relapse.

Anesthesia, General↗

[Management of priapism in patients with sickle-cell anaemia].

Sickle-cell anaemia is an autosomal recessive hereditary haemoglobinopathy. Patients with sickle-cell anaemia present a high risk of priapism. At least 40% of sickle-cell patients report episodes of priapism. Priapism in patients with sickle-cell tend to start during childhood and rapidly threaten the erectile prognosis. Apart from specific treatment of sickle-cell anaemia, the urologist must be able to recognize forms of intermittent priapism and promote medical prevention. The urologist must also be able to distinguish low-flow priapism from the rarer high-flow priapism, as the treatments differ. Treatment strategies are increasingly well defined, with an increasingly limited place for surgery.

Anemia, Sickle Cell↗

Priapism and impotence in homozygous sickle cell disease.

A questionnaire study of Jamaican patients with homozygous sickle cell (SS) disease indicated a 42% prevalence of priapism, with a median age at onset of 21 years. Two predominantly different patterns of priapism were recorded: short "stuttering" episodes lasting less than three hours, with normal consequent sexual function, and severe prolonged attacks (generally more than 24 hours) commonly followed by impotence. Stuttering episodes were frequently a prodrome to a major attack. Over one fourth of those who had suffered priapism had some degree of impotence. Hematologic analysis indicated that priapism was significantly associated with low hemoglobin F levels and high platelet counts. Patients with severe attacks of priapism had lower hemoglobin F levels and reticulocyte count, and a higher mean corpuscular volume, than patients with only stuttering episodes. Priapism and impotence contribute to the morbidity of SS disease more frequently than previously recognized.

Adolescent↗

Priapism - etiology, pathophysiology and management.

The understanding of erectile physiology has improved the prompt diagnosis and treatment of priapism. Priapism is defined as prolonged and persistent erection of the penis without sexual stimulation and failure to subside despite orgasm. Numerous etiologies of this condition are considered. Among others a disturbed detumescence mechanism, which may due to excess release of contractile neurotransmitters, obstruction of draining venules, malfunction of the intrinsic detumescence mechanism or prolonged relaxation of intracavernosal smooth muscle are postulated. Treatment of priapism varies from a conservative medical to a drastic surgical approach. Two main types of priapism; veno-occlusive low flow (ischemic) and arterial high flow (non-ischemic), must be distinguished to choose the correct treatment option for each type. Patient history, physical examination, penile hemodynamics and corporeal metabolic blood quality provides distinction between a static or dynamic pathology. Priapism can be treated effectively with intracavernous vasoconstrictive agents or surgical shunting. Alternative options, such as intracavernous injection of methylene blue (MB) or selective penile arterial embolization (SPEA), for the management of high and low flow priapism are described and a survey on current treatment modalities is given.

Journal Article↗

[One case report of clitoral priapism and literature review].

OBJECTIVE: To investigate the clinical characteristics of female clitoral priapism. METHODS: A 29-year-old case of painful priapism of the clitoris lasting 60 hours was reported. The etiology, diagnosis and treatment of clitoral priapism were discussed and the literature reviewed. RESULTS: The patient was cured by intracavernous administration of adrenaline. CONCLUSION: Clitoral priapism is extraordinarily rare. It is primarily due to oral antidepressant drugs or pelvic malignant neoplasm which leads to tissue infiltration of the clitoral veins and obstruction of the clitoral corporeal blood flow. The efficient therapeutic approach to clitoral priapism is intracavernous administration of alpha-agonists similar to the treatment of penile priapism.

Adult↗

Treatment of "high-flow" priapism with superselective transcatheter embolization: a useful alternative to surgery.

PURPOSE: To review the efficacy of treatment of high-flow priapism with superselective transcatheter embolization. METHODS: Over a 2-year period, we reviewed five patients who were treated for traumatic high-flow priapism with superselective embolization. All patients underwent diagnostic angiography that demonstrated a communication between the cavernosal artery and the corpora cavernosa. Each identified fistula was embolized using gel-foam, blood clot, microcoils, or a combination of these. RESULTS: All five patients had successful detumesence of priapism postprocedure. There was normal recurrence of early morning erection with successful detumescence for each patient within a 2- to 4-week period. Two of five patients (40%) presented with recurrence of priapism at 6 months and required a repeat embolization procedure. At 1 year, four of the five patients (80%) had normal erectile function. CONCLUSION: Superselective transcatheter embolization is a useful therapeutic modality in the treatment of high-flow priapism. It provides equivalent rates of detumesence when compared to surgical techniques, and appears to have a greater success in preserving erectile function.

Adult↗

Priapism: new concepts in the pathophysiology and new treatment strategies.

Priapism is defined as prolonged and persistent erection of the penis without sexual stimulation. This is a poorly understood disease process, and we have little information on the etiology and pathophysiology of this erectile disorder. Two main types of priapism exist: low-flow (ischemic) and high-flow (nonischemic) priapism. When the physician first diagnoses which type of priapism exists, distinguishing the type of priapic event is paramount in order to choose the correct treatment options. Until recently, we had not sufficiently understood the pathogenesis of this erectile disorder and therefore, could not effectively manage its pathologic consequences of erectile tissue damage and erectile dysfunction. In this review, the proposed pathogenesis of ischemic priapism is reviewed, and a survey regarding novel treatment modalities is given.

Humans↗

Priapism in sickle cell disease.

Priapism, an unwanted painful erection of the penis, is a little discussed but common complication of sickle cell disease. What is known about the prevalence of priapism, efficacy of management approaches, and outcome is drawn primarily from retrospective and single-center reports. Priapism occurs in two patterns: prolonged and stuttering (ie, recurrent brief episodes that resolve spontaneously). If priapism persists for 4 hours or more without detumescence, the patient is at risk for irreversible ischemic penile injury, which may terminate in fibrosis and impotence. Large multicenter studies examining the epidemiology and current treatments and well-organized trials of novel therapies are urgently needed for patients who have sickle cell disease and priapism.

Anemia, Sickle Cell↗

Intracavernous papaverine/phentolamine-induced priapism can be accurately predicted with color Doppler ultrasonography.

OBJECTIVES: To determine the value of penile duplex ultrasonography in predicting the occurrence of intracavernous drug-induced ischemic priapism directly after the rigid erection phase. METHODS: We evaluated 250 men with erectile dysfunction by color Doppler ultrasonography after the diagnostic injection of papaverine hydrochloride and phentolamine mesylate. Cavernosal artery waveforms were recorded, and peak systolic velocity (PSV) and end diastolic velocity (EDV) of the recorded waveforms were measured. In cases of priapism, color Doppler ultrasonography findings were evaluated to identify any finding that would predict priapism. RESULTS: In all, 41 patients experienced painful rigid erection for more than 1 hour after injection. All patients were divided into two groups. Group I included 16 patients who achieved full erection lasting less than 6 hours. Group II included 25 patients who achieved full erection lasting more than 6 hours. All patients of group I had PSV less than 61 cm/s and EDV of 0 to 2 cm/s. On the other hand, all patients of group II had PSV greater than 66 cm/s and EDV of 0 to 1 cm/s. These findings were accurate predictors of persistent erection requiring intervention in all 25 patients of group II and in none of the group I patients (100% specificity and sensitivity). CONCLUSIONS: Our results show that during the first hour of penile color Doppler ultrasound study, PSV of 66 cm/s or more together with EDV of 0 cm/s is a reliable predictive factor for priapism. This finding is reliable enough that treatment for priapism should be initiated without further delay.

Adult↗

Arterial priapism: diagnosis, treatment and long-term followup.

We report on the long-term followup of 7 patients 11 to 50 years old treated for arterial priapism following perineal or penile trauma with arteriographic evidence of contrast medium extravasating from a lacerated cavernous artery into surrounding erectile tissue lacunae (an arterial-lacunar fistula). All patients underwent medical record review and completed a mailed questionnaire. The priapism erections were described as devoid of pain or tenderness, incompletely but constantly rigid and able to increase rigidity with sexual stimulation. Bright red corporeal aspirates were observed in all cases. Color flow Doppler ultrasound findings of focal areas of high flow turbulence correlated with diagnostic arteriography (correlation coefficient 1.00). Initial treatment by mechanical or pharmacological means was unsuccessful when performed. Superselective transcatheter embolization of the ipsilateral common penile artery resolved the priapism in all cases. The interval from onset to resolution of priapism was 4 to 126 days. Full erectile function return was delayed from 2 weeks to 5 months, most likely from resolving clot lysis. Full erection quality was restored in 6 of 7 patients with persistent function and restored frequency of intercourse at 6 to 67 months. Reestablished cavernous artery flow in previously embolized arteries was demonstrated on followup ultrasonography. Surgical treatment was not required in any case. We conclude that arterial priapism occurs in the absence of neurogenic-mediated relaxation, and is sustained by high oxygen tension and shear stress associated with the cavernous artery laceration. Embolization therapy offers effective management of the pathophysiology with high preservation of premorbid erectile function.

Adolescent↗

Priapism associated with the sickle cell hemoglobinopathies: prevalence, natural history and sequelae.

To determine the prevalence and characteristics of priapism associated with sickle cell hemoglobinopathic conditions we interviewed and examined 52 men with sickle cell anemia, 10 with sickle C disease, 3 with sickle B(+)-thalassemia and 19 with sickle cell trait. Of the men 20 with sickle cell anemia (38%) and 1 with sickle B(+)-thalassemia (33%) reported past priapism attacks. Average patient age at onset in the sickle cell anemia patients was 19 years (range 8 to 30 years). Four men had had only 1 attack. The average number of attacks per year in the remaining patients ranged from 1 to 52. Of the patients who reported multiple attacks the average duration of a typical episode and the duration of the longest self-limiting episode was 1.6 and 7.0 hours, respectively. Six patients had experienced 1 attack that persisted for longer than 24 hours. There were no significant differences between the clinical and hematological parameters of the sickle cell anemia patients who did and did not experience priapism or of the priapism patients who had and had not experienced episodes lasting greater than 24 hours. Six patients had been hospitalized for priapism and 4 were treated with a shunting procedure. Of the latter patients 2 were impotent but there was no significant alteration in the sexual function of the other study participants.

Adolescent↗