Cases reported "Priapism"

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1/9. Arterial embolization in the treatment of post-traumatic priapism.

    priapism is a prolonged penile erection not associated with sexual arousal. Two types of priapism have been described: the more common one is the "veno-occlusive" priapism and can be frequently observed as the consequence of an intracavernosal injection of vaso-active drugs for the treatment of erectile dysfunction. The less common type of priapism is known as "high flow" priapism and usually follows perineal or direct penile trauma. The clinical presentation in case of high flow priapism is quite typical: hystory of recent penile or perineal trauma followed, by the onset of a painless, incomplete and constant erection of the penis. A color-flow Doppler sonogram should be performed as first diagnostic step: this examination allows to identify the presence of patent cavernous arteries and prominent venous drainage with focal area of high flow turbulence along the pathway of one or both the cavernous arteries. An arterial blood sample taken from the corpora will confirm the diagnosis. At first, conservative therapeutical attempts can be suggested, with mechanical external compression of the perineum, the use of ice packs, corporeal aspiration and irrigation with saline. Besides, intracorporeal administration of alpha-agonists and methylene blue should be performed. Unfortunately, these conservative measures often result unsuccessful, and more invasive approaches must be considered. The radiological superselective transcatheter embolization of the proximal artery supplying arterial-lacunar fistula should be the present treatment of choice in these cases of high-flow priapism refractory to conservative and medical treatments. The first successful management of high flow priapism by selective arterial embolization was reported by Wear and coworkers in 1977. Autologous clots and gelatine sponge have been extensively used and become very popular as the embolic agent. More recently, platinum microcoils have been proposed with the aim to achieve more precise and selective embolization. In our single-case-experience on the treatment of high flow priapism by arterial embolization, we used the recently introduced tungsten microcoils. At the time of the follow-up, 2 months later, patient reported satisfactory intercourse with an approximately 75% of penile rigidity. By comparison with microsurgical ligature of the damaged vessel, selective embolization is, at least theoretically, a less invasive procedure, particularly with reference to the trauma caused to the erectile tissue. High-flow priapism is a fairly rare urological pathology which does not require immediate and emergency treatment (as is the case, instead, with venous-occlusive priapism), since the risk of post-ischaemic fibrosis is excluded thanks to the fact that oxygen is supplied to the cavernous tissue. Once the diagnosis has been established with certainty, therefore, the specialist has the necessary time at disposal to arrange for the most appropriate therapeutic steps. When, as is frequently the case, conservative measures prove ineffective, the current treatment of choice for cases of fistula of the cavernous artery would appear to be superselective embolization of the artery, provided same can be performed at specialized centres and by experienced personnel.
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2/9. Behcet's disease presenting as deep venous thrombosis and priapism.

    Behcet's disease (BD) is a multi-system inflammatory disorder which may involve the vascular system. Currently, it is general practice to use the International Study Group (ISG) criteria for the diagnosis of BD. However, even though vascular involvement may be seen in one-fourth to one-half of BD patients, and occasionally is the presenting and only manifestation of BD, large vessel disease is not included among the ISG criteria. In this report we describe a patient who had deep venous thrombosis and priapism, but who does not fulfill ISG criteria for the diagnosis of BD.
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3/9. Juvenile posttraumatic high-flow priapism: current management dilemmas.

    High-flow priapism results from disruption of the intercavernosal artery resulting in an arteriocavernosal fistula and is rarely encountered in the pediatric and adolescent population. Clinically it manifests as a painless, prolonged erection after perineal trauma. Treatment has ranged from expectant management to open surgical exploration with vessel ligation. Internal pudendal arteriogram and superselective embolization with autologous blood clot has emerged as a safe and effective treatment modality in the young male population. Here the authors present 3 patients with high-flow priapism and discuss management of this rare clinical entity.
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4/9. Neurologic events after partial exchange transfusion for priapism in sickle cell disease.

    We describe six boys with homozygous sickle cell disease, aged 7 to 13 years, in whom acute, severe neurologic abnormalities developed 1 to 11 days after partial exchange transfusion was performed to treat priapism that was unresponsive to more conservative therapy. Hemoglobin levels were 10.5 to 13.4 gm/dl (mean 12.1 gm/dl), and hemoglobin S levels were 18% to 33% (mean 27%) before the onset of neurologic complications. Severe headache was the initial finding in five patients, four of whom had increased intracranial pressure and three of whom required tracheal intubation and hyperventilation. Four patients had seizures; three had focal neurologic deficits for more than 24 hours. Cerebral arteriography demonstrated vascular abnormalities, including irregularity, stenosis, and complete occlusion of vessels. patients treated with regular erythrocyte transfusions had no recurrence of neurologic signs or symptoms when hemoglobin S levels were kept at 30% to 50%. The occurrence of serious neurologic complications after partial exchange transfusion in patients with homozygous sickle cell disease from three centers indicates the possibility of a causal relationship between the events. Early and thorough investigation of neurologic symptoms, especially severe headache, is warranted in this clinical setting.
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5/9. Thrombosis and priapism in a patient with Henoch-Schonlein purpura.

    Henoch-Schonlein purpura (HSP) belongs to the category of systemic small-vessel vasculitis. Although long-term outcome is generally good, serious complications may occur. Thrombosis and priapism have been reported only as extremely rare complications of HSP. We describe a 37-year-old man who developed recurrent thrombotic events shortly after he had been diagnosed as having HSP. Although he had additional risk factors for thrombosis, such as prothrombin G20210A mutation and use of celecoxib before the last episode, temporal relation of the thrombotic attacks to the onset of HSP suggest that the disease itself may lead to a prothrombotic state. This case is the first adult HSP patient with priapism, which probably developed secondary to thrombosis of the dorsal penile vein.
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6/9. Locally aggressive granular cell tumor causing priapism of the crus of the clitoris. A light and ultrastructural study, with observations concerning the pathogenesis of fibrosis of the corpus cavernosum in priapism.

    A case of focal priapism of the clitoris caused by a microscopic granular cell tumor (GCT) is described. This neoplasm is considered locally aggressive because it invades the lumens of peripheral cavernous sinuses of the crus of the clitoris. Caverns adjacent to those invaded by tumor exhibit stasis, telangiectasia, and necrosis of the smooth muscle of the trabecular wall. These alterations lead to telescoping collapse and compression of the cavernous spaces and culminate in fibrosis. Ultrastructurally, replicated basal lamina is found surrounding clusters of granular cells. We suspect that the multilayered lamina, in addition to being produced by tumor cells, is derived from the trabecular endothelium surrounding the caverns invaded by the GCT. The replication of the basal lamina may be provoked by cycles of injury and repair to these vessels caused by repeated episodes of prolonged vascular stasis. A peculiar large vein with perforating branches was observed in the center of the cavernous spaces of the crus. This vein is not found in normal crura and, therefore, represents a morphologic adaptation created to drain the cavernous spaces.
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7/9. Treatment of priapism by transcatheter embolization of internal pudendal arteries.

    priapism can be successfully treated by unilateral or bilateral percutaneous transcatheter occlusion of the internal pudendal arteries. Occlusion should be reversible in order to avoid impotence. Embolization with autologous clot satisfies this requirement because of the clot lysis and consequent vessel recanalization. Three cases are extensively described which demonstrate the feasibility of the method and the ability to preserve sexual potency. The rationale for using interventional angiography in priapism is also discussed.
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8/9. Conservative management of high-flow priapism.

    High-flow priapism most frequently occurs as a result of penile trauma with subsequent unchecked arterial flow into the corpora cavernosa. An improved understanding of its underlying pathophysiology has allowed a more selective therapeutic approach, usually with preservation of potency. We report on 4 cases of traumatic high-flow priapism seen over a 5-year period. The patients were managed successfully with conservative treatment consisting of observation after arteriography or percutaneous duplex ultrasound-guided autologous clot embolization where tortuous vessels prevented selective embolization. No patient required surgical repair and hospitalization was brief (0 to 2 days). The use of color duplex ultrasonography provided a noninvasive modality for establishing the site of the injury and allowed serial follow-up evaluation. When conservative measures failed, the color duplex ultrasound study confirmed the presence of the ruptured cavernous artery and allowed percutaneous autologous clot embolization in 1 patient. Long-term follow-up (1 to 4 years) of these patients revealed a return to their preinjury state of erectile function.
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9/9. Delayed high flow priapism: pathophysiology and management.

    Two cases of the management and pathophysiology of high flow arterial priapism are presented. Both cases were post-traumatic with delayed onset of priapism and both had angiographically diagnosed arteriocorporeal fistulas. Case 1 was managed with selective cavernous artery ligation and case 2 resolved spontaneously, both with excellent return of premorbid levels of erectile function. We propose that the pathophysiological mechanism involves injury to the intracavernous artery, causing ischemic necrosis. After a delay the arterial segment blows out, leading to unregulated blood flow into the corpus cavernosum. Management with surgical ligation is highly selective and nondisruptive to unaffected vessels, offering precise control of the bleeding vessel.
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