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Prevalence of priapism in children and adolescents with sickle cell anemia.

A questionnaire survey was conducted of patients with homozygous sickle cell anemia (Hb SS) and sickle cell beta(0)-thalassemia (Hb S-beta(0)) between 5 and 20 years of age to determine the prevalence and characteristics (number of episodes, timing, duration, cause, or precipitating event) of priapism. Ninety-eight male patients or their parents were surveyed by the same male investigator using a structured verbal interview, which was modified according to the age of the patient. Ninety-four patients had Hb SS and four Hb S-beta(0) thalassemia. Eleven (11%) patients were known to have experienced priapism previously. In response to the questionnaire, 16 of the remaining 87 (18%) patients reported having had priapism on one or more occasions. The actuarial probability of experiencing priapism by 20 years of age was 89% (+/- 9%). The mean age at the initial episode was 12 years, the mean number of episodes per patient was 15.7 (median, 1; range, 1-100), and the mean duration of an episode was 125 minutes. Episodes typically occurred around 4:00 am, and 75% of the patients surveyed had at least one episode starting during sleep or upon awakening from sleep. The prevalence of priapism in children and adolescents with SCA is much higher than previously described. Since early intervention and treatment may prevent irreversible penile fibrosis and impotence, patients and parents should be educated about this complication in advance of its occurrence.

Actuarial Analysis↗

[Diagnosis and therapeutic options for prolonged erection and priapism: up-date review].

OBJECTIVE: To review the pathophysiology, diagnostic methods and treatments of priapism, with special reference to alternative treatment options. METHODS: The different surgical options are presented and discussed, with special emphasis on the oral drugs and intracavernous therapy for priapism. The advantages and inconveniences of the different agents and surgical techniques are discussed. The publications in MEDLINE 1980-2000, our experience and the results of our studies previously reported in the literature were reviewed. RESULTS/CONCLUSIONS: Complete detumescence and recovery of normal arterial blood flow can be achieved in a majority of the cases by systematic and standardized management. Sedatives, alpha-adrenergic agents or oral ketamine hydrochlorate can be utilized. However, due to the importance of the time factor, intracavernosal therapy should be the priority for persistent erection, using alpha-adrenergic agonists or other alternatives, such as methylene blue, which do not have the well-recognized risks of the conventional agents. The etiology of the priapism should be clearly established by metabolic and hemodynamic studies, since treatment will be based on the underlying disorder. In veno-occlusive low flow priapism, surgical shunting should be performed if aspiration of intracavernosal blood and other treatments are not successful. Arterial embolization and surgical ligation should be performed for high flow persistent priapism.

Humans↗

[Low-flow priapism associated with systemic lupus erythematosus and nephrotic syndrome].

OBJECTIVE: To present a case of priapism associated with systemic lupus erythematosus and nephrotic syndrome. METHODS: A 29-year-old male patient with a history of multiple pathologies consulted at the emergency services with painful priapism of 12 hours' duration. The intracavernosal aspirated blood had a venous appearance. Among the risk factors analyzed, systemic lupus erythematosus and nephrotic syndrome are recognized to cause hypercoagulability, a condition that can lead to low flow priapism. RESULTS: There was no response to lavage-aspiration or intracorporeal administration of an alpha-adrenergic agonist. The high surgical risk of the patient advised against surgery. The priapism gradually diminished until complete remission on the sixth day. The patient has remained without spontaneous erection. CONCLUSION: Systemic lupus erythematosus and nephrotic syndrome can cause low flow priapism. The best treatment is by prevention with adequate antithrombotic prophylaxis.

Adult↗

Prepubertal high flow priapism: incidence, diagnosis and treatment.

PURPOSE: We reviewed the literature of the last 40 years and report our experience with treating high flow priapism with fistula embolization in prepubertal boys. MATERIALS AND METHODS: Two boys had blunt perineal trauma and 1 had penile trauma (ages 6, 6 and 10 years). Painless priapism developed within 24 hours and lasted for 4 to 7 days before the patients presented to the hospital. Primary diagnosis was made on color Doppler ultrasound. When high flow priapism was diagnosed angiography of the internal iliac artery and embolization of the arteriocavernosal fistula were performed. Mean followup was 26 months. RESULTS: Color Doppler ultrasound revealed bilateral arteriocavernosal fistulas in 2 boys and a unilateral fistula in 1. Angiography showed fistulas of the branches of the internal pudendal artery in 2 patients and fistulas of the bulbourethral artery in 1. Microcoils were used in the bulbourethral artery and a gelatin sponge was used in other penile arteries. Complete detumescence with restored erectile function was achieved in all cases. CONCLUSIONS: High flow priapism in children can be diagnosed easily by typical clinical features combined with color Doppler ultrasound. In children with posttraumatic priapism embolization of the arteriocavernosal fistula is superior to surgical or medical procedures and should be the first line therapy. Embolization using microcoils for bulbourethral arteries and a gelatin sponge for other penile arteries has proved to be safe and successful therapy.

Age Factors↗

Persistent priapism and histological modifications of the erectile tissue. Two case reports.

Prolonged veno-occlusive priapism is associated with a high risk of fibrosis of the corpora and impotence. We present 2 cases of prolonged low-flow priapism who came under our observation more then 72 hours after the onset of priapism. The first case was a 51-years old man in which the aethiology of priapism was cauda equina compression by a L4-L5 discal haernia, not recovered after surgical decompression. The second case was a 23-years old man suffering of painful priapism lasting for more than 7 days due by abuse of cocaine, alcohol and psychopharmaceuticals. In both cases drainage and irrigation of the corpora followed by injection with an alpha-agonist agent has been insufficient. Detumescence has been obtained with shunt procedure and compressive bondage. The biopsy of the corpora cavernosa showed fibrosis.

Adult↗

A rare case of penile metastasis of testicular cancer presented with priapism.

Priapism is thought as a condition of penile erection that persists beyond or is unrelated to sexual stimulation. Commonly two different entities of priapism are known, one is low-flow priapism and the other is high-flow priapism. It is important to distinguish these two conditions for the subsequent different treatments. We report a rare case of an indistinguishable priapism caused by penile metastasis of testicular cancer.

Aged↗

[The treatment of priapism].

Priapism, that is a long lasting erection in absence of sexual desire and is sometimes painful, has represented a rare occurrence until few years ago; such occurrence has become much frequent since pharmaco-erection have been used. There are still many doubts about the etiopathogenesis of priapism, that anyway happens because of a long lasting pathological discrepancy between arterious flux to penis corpora cavernosa and venous down flowing. For to establish how and when we must treat priapism, it is very important to know the causes of it, so that we can use the more properly therapy for each case. It's possible to classify the therapies in the following ways: a) extracavernosa, b) located in the corpus cavernosum. These last ones are most used and include the punction and the drainage of the blood of the cavernous body and sometime the "shunt" spongio-cavernosum. The treatment must be effected without loss of time in those rare cases of spontaneous priapism. Indeed, according to our experience, it's not necessary to be extremely supporting for intervention for priapism post pharmaco-erection. In fact the wait of 8-10 hours reduces the number of invasive treatments, without danger for the integrity of cavernous tissue.

Anesthetics↗

Priapism in a stallion with generalized malignant melanoma.

A Thoroughbred stallion developed priapism that was unresponsive to medical treatment and lavage of the corpus cavernosum penis with heparinized 0.9% NaCl solution. Three weeks after onset of priapism, the penis was firm and noncompliant, and penile pain sensation and ability to retract the penis were lost. Ultrasonography confirmed thrombosis of the corpus cavernosum penis. The stallion was euthanatized because of poor prognosis for return to breeding soundness. Necropsy revealed enlargement of numerous lymph nodes. The dorsal penile nerves were demyelinated distal to the crura of the penis. A diagnosis of generalized malignant melanoma was made; however, neither metastasis to the vertebral canal nor compression of spinal nerve roots as they exited the vertebral foramen was found. Priapism is a persistent erection without sexual arousal and is initially unassociated with penile paralysis, but if prolonged, leads to irreversible venous occlusion where collecting veins join the cavernous spaces. Damage to the dorsal penile nerves may explain the long-term penile paralysis and loss of sensation that accompanied priapism in this stallion. Priapism unassociated with the use of phenothiazine-derivative tranquilizers is uncommon in horses.

Animals↗

Psychotropic medication and priapism: a comprehensive review.

Numerous reports have emphasized the association between priapism and the ingestion of psychotropic medication. Clinicians are becoming increasingly aware of this association and its subsequent severe morbidity. Review of the literature reveals that medications possessing alpha-adrenergic blocking properties are most frequently associated with priapism. These medications include trazodone, several antipsychotics, and the antihypertensive agent, prazosin. Awareness of these associations and an appreciation of potentially serious consequences of this disorder may assist clinicians in choosing psychotropic agents that minimize the risk of developing priapism. It is essential that patients who are to receive psychotropic medications be forewarned about priapism. In addition, patients should be questioned concerning prior occurrence of prolonged erections, since a past history of delayed detumescence is present in approximately 50% of subsequent cases of priapism.

Adolescent↗

[Therapy of priapism (author's transl)].

Due to the poor knowledge concerning etiology and pathophysiology of priapism the treatment necessarily remains largely symptomatic. There are two main therapeutic aims: 1. The penile detumescence in order to relief the often severe local pain and 2. the preservation of the compromised erectile potency. To compare different therapeutic principles a follow-up of our own relatively large series of 55 cases of priapism ((1962-1980) is presented. Conservation of potency was the criterion of therapeutic success. In idiopathic priapism 50% of the patients kept their potency after shunt-operations, whereas in priapism of known origin ("secondary priapism" due to ileofemoral thrombosis, leukemia, hemodialysis) after different treatment methods (thrombolytic pharmacotherapy, shunt-operations) the result nearly always was a loss of potency. Considering the lower technical expense and the lower rate of complications today the transglandular cavernosum-spongiosum (cavernoglandular) shunt (Ebbehøj-Winter) is to be preferred to the cavernosum-saphenous (Grayhack) and cavernosum-spongiosum-shunt (Quackels).

Adolescent↗

[Priapism in childhood (author's transl)].

Priapism is a very rare problem in childhood. Although sickle cell disease and leukemia may produce persistent erection, juvenile priapism is most commonly idiopathic. The physiology and pathology of erection are reviewed, and the pathomechanisms of diseases which may produce priapism are described. If red cell sickling and leukemia are excluded, immediate surgical intervention by either corporosaphenous or corporospongiosum shunts is recommended for resolution of priapism and preservation of potency. A case of juvenile priapism is presented in which HCG medication as underlying factor is discussed.

Age Factors↗

Effects of priapism lasting 24 hours or longer caused by intracavernosal injection of vasoactive drugs.

Twenty-four patients, treated after > or = 24 h of priapism caused by the injection of vasoactive drugs, were examined. The aetiology of impotence was psychogenic in > 50% of the patients (13/24). Most priapisms that lasted < 36 h could be treated successfully by puncture and alpha-adrenergic drugs without any fibrosis of the corpora cavernosa, and most patients continued to receive vasoactive agents for the treatment of impotence; but this approach was no longer adequate after priapism had lasted > = or 36 h when glandulocavernosal shunts were needed to achieve permanent detumescence. After > 48 h of priapism all the patients developed fibrosis of the corpora cavernosa and none of them was able to continue with the injections of vasoactive drugs. In addition to fibrosis of the penis, one fulminant infection, one case of urethral damage, one permanent shunt and two cases of transient hypertonia with ECG changes were seen. In order to save time, glandulocavernosal shunts should be performed without delay once priapism has lasted > 36 h.

Adult↗

[Risperidone-induced priapism].

Priapism or pathological, prolonged erection, is a rare adverse effect sometimes associated with psychotropic medication and is due to alpha adrenergic receptor blockade. Priapism in a 44-year-old mental patient receiving risperidone is described. Urinary retention was relieved by catheterization, followed by saline irrigations. Priapism was relieved when a hematoma was evacuated from his penis. This may be the first description of priapism following risperidone alone, since a previous report included a patient receiving risperidone together with 2 other psychotropic medications. Priapism should be looked for in men receiving risperidone, a novel antipsychotic medication.

Adult↗

[High-flow priapism: a rare, easily treatable disorder with excellent prognosis].

Four patients, men aged 33, 37, 37 and 12 years, were examined because of priapism following trauma. In all four high-flow priapism was diagnosed, based on intracavernous blood gas analysis and selective angiography. One of the men aged 37 was subsequently found to be suffering from low-flow priapism caused by chronic myeloid leukaemia (the blood gas analysis had been performed after decompression of the cavernous body). Two other patients were treated by selective internal pudendal artery embolisation. In the fourth, who developed a vascular spasm at angiography, embolisation was not performed: he recovered spontaneously. In contrast to high-flow priapism, low-flow priapism is an urological emergency for diagnosis and treatment to prevent permanent impotence.

Adult↗

A case of priapism with ruptured intracranial aneurysm.

A man of 35 years, who had had three attacks of subarachnoid hemorrhage in the previous 3 years, was admitted to hospital with complaints of headache and priapism. There had been intermittent priapism with abnormal acceleration of sexual desire since the first attack, and erection of the penis had persisted with intolerable pain after the last attack of subarachnoid hemorrhage. A carotid angiogram revealed an aneurysm at the junction of the left internal carotid and posterior communicating arteries. Clipping of the aneurysmal neck was successfully performed. However, priapism continued for 22 days after the operation and resulted in sexual impotence. The neurological problems of priapism are discussed with special reference to a hypothalamic lesion caused by the ruptured intracranial aneurysm in this report.

Adult↗

Cavernospongiosum shunt in management of priapism: is it a reliable method?

The efficiency of cavernospongiosum shunt in the management of priapism was evaluated in 10 patients. Technical details such as the "Z" perineal incision and the opening followed by closure of the contralateral corpus cavernosum, at the time of corpora evacuation, can improve the local exposure and permits a more complete drainage of the corpus cavernosum, but did not improve our results when they were compared with other techniques for treatment of priapism. Penile flaccidity and preservation of sexual potency occurred in 6 (60%) and 5 (50%) patients, respectively, and no surgical complications were observed. Immediate penile flaccidity and regaining of physiologic erection were, however, not complete even in the successfully treated patients. The cavernospongiosum shunt does not seem to represent the definitive form of treating priapism. New knowledge about mechanisms involved in priapism has to be obtained, in order to improve the approach and the prognosis of this disabling condition.

Adolescent↗

Pharmacological priapism: comparison of trazodone- and papaverine-associated cases.

Priapism occurred in ten patients undergoing treatment of depression with trazodone or impotence with papaverine. Trazodone-related priapism uniformly required surgical procedures and resulted in impotence in two of three cases. In contrast, papaverine-associated priapism was successfully managed by aspiration and all seven patients continued to respond to intracorporal treatment. Iatrogenic priapism is an important complication of therapy with vasoactive drugs.

Depressive Disorder↗

[History of high-flow priapism: 1960-2005].

High-flow priapism caused by a pathological arterial influx to the cavernous bodies was first described by F.B. Burt in 1960. The pathophysiological differentiation of high- and low-flow priapism was developed in 1983. The development of diagnostic tools for differentiation of different forms of priapism and the progress in the therapy of high-flow priapism from arterial ligation to supraselective embolization is presented.

Europe↗