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A Priapism is a painful, persistent, abnormal erection without sexual desire or arousal. It occurs most often in boys aged 5-10 years and in men aged 20-50 years. Pathophysiology The penis consists of three erectile tissue: two corpora cavernosa and one corpus spongiosum. An erection is caused by smooth muscle relaxation and increased arterial blood flow into the corpora cavernosa, resulting in swelling and stiffness. Ischemic priapism Most cases of priapism can be attributed to the failure of a Detumesenz and usually arise due to prevented venous outflow (d. E. “Low-Flow”), also called ischemic priapism known. occur after 4 hours ischämiebedingt to severe pain. If it lasts longer (> 4 hours) can lead to Corpus fibrosis with subsequent erectile dysfunction or even Penisnekrose and gangrene priapism. Under stuttering priapism (h d.. “High flow”) is defined as a recurrent form of ischemic priapism with repeated episodes and in the meantime occurring DetumeszenzNichtischämischer priapism less likely to encounter priapism due to unregulated arterial inflow on, usually as a result of arterial fistula after trauma. Ischemic priapism is not painful and does not lead to necrosis. Below there are often erectile dysfunction. Etiology In adults, the most common cause (see Table: Causes of priapism) Drug therapy for erectile dysfunction In children, the most common causes are hematologic diseases (eg, sickle cell anemia, leukemia rare.) Frequently priapism is idiopathic and may occur repeatedly. Causes of priapism cause suspicious findings diagnostic approach medications for erectile dysfunction: alprostadil (injected intraurethral) papaverine (injected) phentolamine (injected) phosphodiesterase-5 (PDE-5) inhibitors (Avanafil, sildenafil, tadalafil, vardenafil) Painful ischemic priapism in men with prior treatment of erectile dysfunction Clinical evaluation recreational drugs amphetamines (stimulants) Cocaine Painful ischemic priapism and psychomotor agitation and anxiety Clinical evaluation Sometimes toxicological screening Other drugs: ?-blockers (prazosin, tamsulosin, terazosin) anticoagulants (warfarin) antihypertensive drugs (nifedipine) * antipsychotics (risperidone, haloperidol, clozapine, quetiapine, trazodone, chlorpromazine) corticosteroids antidiabetics (tolbutamide) lithium methaqualone Painful ischemic priapism in men who are Clinical evaluation due to other medical disorder treatment Hematological diseases: leukemia lymphoma, sickle cell disease (rare, sickle cell anemia) thalassemia Young men, often of African origin or from the Mediterranean blood hemoglobin electrophoresis Locally advanced prostate cancer Any metastatic disease men> 50 with previous increasing symptoms of bladder outlet obstruction Prostate Specific Antigen test CT spinal stenosis or spinal cord compression Continuous epidural infusions concurrent weakness of lower extremities CT or MRI of the spinal cord trauma (the arterial inflow to unregulated or arterial fistula leads) Non-Ischemic, painless permanent erection in men with recent onset trauma duplex sonography of the penile angiography MRI Rare causes cerebrospinal diseases (e.g.. As syphilis, tumor) genital infection and inflammation (eg. As prostatitis, urethritis, cystitis), especially if complications occur due to bladder stones pelvic hematoma or cell carcinoma pelvic venous thrombosis Complete parenteral nutrition Various Various * All atypical antipsychotics can cause priapism. Assessment priapism requires urgent treatment to prevent chronic complications (especially erectile dysfunction). Diagnosis and treatment should be carried out simultaneously. History The history of the present illness, the duration of erection should be ascertained whether partial or total stiffness and pain exist and have occurred whether recently or in the past trauma. For history, the question is one of accompanying medications and patients should be asked directly about the use of recreational drugs and medicines for the treatment of erectile dysfunction. In reviewing the organ systems should be sought for causative symptoms such. B. dysuria (UTI), urinary retention or urinary urgency (prostate cancer), fever and night sweats (leukemia), and lower limb weakness (spinal cord pathology). With a history of known disorders should be recognized that see with priapism associated (see Table: Causes of priapism), particularly hematological diseases. Patients should after the occurrence of hemoglobinopathies (sickle cell anemia or thalassemia) in the family questioned werden.Körperliche investigation Focused genital examination should be performed to assess the degree of stiffness and sensitivity and determine if the glans and corpus spongiosum are also affected. In penile or perineal trauma and signs of infection, inflammation or Gangrenous changes should be taken. When complete physical examination should be looked taken on psychomotor agitation and in the investigation of the head and neck should after pupil dilation associated with stimulants related. Abdomen and suprapubic area should be scanned to rule out lesions or splenomegaly and a digital rectal exam should be performed to detect an enlarged prostate or other illnesses. A neurological examination is useful to determine signs of lower limb weakness or paresthesia of the saddle blocks that könnten.Warnzeichen indicate spinal pathology The following findings are of particular importance: Pain priapism in a child recently suffered trauma fever and night sweats interpretation of findings In most cases, the history shows a previous drug treatment for erectile dysfunction, as well as illegal drug use or a past sickle cell disease or anemia; In these cases, no further tests are performed. In patients with ischemic priapism full stiffness is determined with pain and tenderness of the corpus cavernosa on physical examination typically where glans and corpus spongiosum are not affected. In contrast, an ischemic priapism runs painless and insensitive, and the penis is partially or completely erigiert.Tests If the cause is not clear, one moment lymphoma, UTI and other causes of hemoglobinopathies leukemia. Blood count urinalysis and urine culture hemoglobin electrophoresis at dunkeläutigen men and women of Mediterranean origin Many doctors also conduct a drug screening, intracavernous BGA tests and duplex sonography. A duplex scanning of the penis in men will show ischemic priapism little or no cavernosal blood flow and in men with nonischemic priapism a normal to high cavernosal blood flow. Duplex ultrasound can also show anatomical abnormalities, such as cavernous arterial fistula or pseudoaneurysm that point usually on nonischemic priapism. Occasionally an MRI with contrast is useful to show arteriovenous fistulas or aneurysms. Even with therapy known etiology, treatment is often difficult and sometimes unsuccessful. Whenever possible, patients should be referred to an emergency room; Patients should preferably be examined and treated urgently by a urologist. Other diseases are treated. For example, a priapism often disappears when the sickle cell anemia is treated. The methods by which a priapism is treated alone, depend on the type. Ischemic priapism Treatment should start immediately, usually with aspiration of blood from the base of one of the two corpora cavernosa using a syringe nichtheparinisierten, often with a saline solution and intracavernous injection of ?-receptor agonist phenylephrine. In Phenylephrininjektionen 1 ml of 1% phenylephrine (10 mg / ml) was added to 19 ml of 0.9% saline solution to 500 ug / ml to manufacture; 100-500 micrograms (0.2-1 ml) is injected every 5-10 minutes until there is an improvement or a total dose of 1000 micrograms administered. From aspiration or injection dorsal line or infiltration anesthesia is done. If these measures are not successful, or if the priapism has already lasted for> 48 h (and thus it is unlikely that these measures are successful), a surgical shunt between the corpus cavernosum and glans penis or corpus sponiosum and another vein can be placed . “Stuttering” priapism “Stuttering” priapism is treated in the acute stage, as well as other forms of ischemic priapism. There is a report on a number of cases that were caused by sickle cell anemia, and have responded to a single oral dose of sildenafil. To treatments that are helpful to prevent relapses of “Stuttering” priapism include anti-androgen therapy with gonadotropin-releasing hormone agonists, estrogen, bicalutamide, flutamide, phosphodiesterase-5 inhibitors and ketoconazole. The aim of the antiandrogen therapy is to reduce plasma testosterone mirror to <10%. Digoxin, terbutaline, gabapentin and hydroxyurea were also with some success eingesetzt.Ischämischer priapism The conservative therapy (eg ice packs and analgesics.) Is usually successful; if not, be selective embolization or surgery empfohlen.Therapierefraktärer priapism If other treatments are unsuccessful, a penile prosthesis can be placed. Conclusion priapism requires urgent investigation and treatment. Drugs (prescription and recreational drugs) and sickle cell anemia are the most common causes. Acute treatment with ?-agonists, puncture, or both.