​Kembara Xtra - Medicine - Priapism

​Kembara Xtra - Medicine - Priapism  
Penile (or less frequently clitoral) erection that lasts longer than four hours without being caused by sexual stimulation or arousal is classified as:
95 percent of cases are ischemic (low-flow, veno-occlusive). It is connected to corpora cavernosa ischemia.
- Nonischemic (high-flow, arterial): less prevalent, frequently painless, associated with earlier trauma, does not require immediate treatment. Malignant priapism is rare and most frequently results from penile metastases associated to primary bladder, prostatic, rectosigmoid, and renal cancers. It affects the reproductive and vascular systems. Recurrent ischemic ("stuttering") priapism is episodic, short-lived, and may not require intervention.  Neurophysiologic, sexual, and psychosocial dysfunction Child Safety Considerations
Sickle cell disease (SCD) is the most frequent cause in children, accounting for 63% of cases. Leukemia, idiopathic, penile trauma (such as following circumcision), and illicit drugs (up to 35% of cases) are less frequent etiologies that tend to develop more frequently in adolescence.

Epidemiology Incidence: 5.3 per 100,000 males annually; Age: Since 2008, there has been a trend in favor of men in their 40s. Men over the age of 40 have a twofold increase in incidence (2.9 vs. 1.5/100,000 person-years).
 RACE: 61.1% African Americans (6.3% Hispanic), 30% Caucasians (correlated with frequency of SCD),

Pathophysiology and Etiology 

 Physiology and anatomy:
- The penis is made up of three longitudinally oriented corpora: the glans penis, which is formed by a single ventral corpus spongiosum that surrounds the two dorsolaterally paired corpora cavernosa responsible for penile erection.
- The penis is typically supplied by the penile artery, which is a branch of the internal pudendal artery, which is a branch of the internal iliac artery. It splits into three branches: the cavernosal artery, which supplies the corpus spongiosum with blood, the bulbar artery, which supplies the dorsal artery.
- The cavernosal arterioles' smooth muscles relax during an erection, causing a high-volume inflow to the sinusoids that compresses the departing venules. The corpora cavernosa's volume significantly expands as a result.
- The sympathetic nervous system predominates during the flaccid resting state. Nitric oxide produced by the parasympathetic nervous system is what causes penile tumescence and erection. The phosphodiesterase type 5A (PDE5A) pathway, which produces cyclic guanosine monophosphate (cGMP), is used to relax smooth muscle. Reduced venous outflow causes an increase in intracavernosal pressure in ischemic priapism. This causes erection, a compartment syndrome, reduced arterial inflow, blood stasis, local hypoxia, and acidosis.
If priapism lasts longer than 24 hours, it may lead to penile tissue necrosis and fibrosis. Dysregulation of the NO/cGMP, RhoA/Rho kinase, and opiorphin signaling pathways as well as excessive adenosine signaling are hypothesized to be pathophysiology factors that contribute to poor smooth muscle relaxation and reduced venous outflow.
In nonischemic priapism, a prolonged, painless, partially stiff erection is the result of increased arterial flow without a corresponding decrease in venous outflow.
 Root Causes
Priapism that is ischemic Idiopathic: around 50% of cases
Hematologic dyscrasias include factor V Leiden mutation, SCD, thalassemia, leukemia, multiple myeloma, fat emboli after hyperalimentation, and hemodialysis.  Toxin-mediated illnesses include malaria, rabies, scorpion stings, and urinary tract infections.
 Nephrolithiasis, amyloidosis, Fabry disease, gout are examples of metabolic illnesses. Syphilis, spinal cord injuries, cauda equina syndrome, autonomic neuropathy, lumbar disc herniations, spinal stenosis, cerebrovascular accidents, brain tumors, and spinal anesthesia are examples of neurogenic illnesses. Neoplasms include those of the penis, urethra, bladder, prostate, kidney, and rectum.
Vasoactive erectile dysfunction medications, such as papaverine, phentolamine, and prostaglandin E1/alprostadil; and antagonists of the adrenergic receptors, such as prazosin, terazosin, doxazosin, and tamsulosin.  Antipsychotics and antidepressants (such as phenothiazines, chlorpromazine, bupropion, fluoxetine, sertraline, lithium, clozapine, risperidone, and olanzapine)  Antihypertensives, such as propranolol, guanethidine, and hydralazine
 Hormones, such as testosterone and gonadotropin-releasing hormone
 Antidepressants (hydroxyzine)
 Blood thinners (heparin, warfarin)
 Drugs used for recreation, such as alcohol, marijuana, and cocaine
Nonischemic Pruritus (2) • Penile or perineal trauma that causes a fistula between the corpus cavernosum and the cavernous artery.
 Serious spinal cord damage
 Ischemic priapism therapy

Risk Elements 
A lifetime risk of ischemic priapism is 29–42% in those with SCD.
Dehydration linked to SCD or a characteristic Previous history of priapism

Prevention 

 Prevent dehydration (in cases of SCD).
Avoid or minimize the use of medicines that cause the condition.
Avoid causing genital harm.


SCD (42.9%) or sickle cell trait (2.5%) are associated conditions.  Substance abuse (7.9%)
Leukemia, G6PD deficiency, and neoplasm


Perineal or penile trauma (blunt force or needle injury), difficult urination while erectional, history of any hematologic abnormalities (such as SCD or trait), cardiovascular disease, medications, and recreational drug use are all indicators of priapism.

clinical assessment 
A physical exam should often include the following: - A thorough examination of the penile, scrotum, and perineum to look for signs of damage, gangrene (rare), or prosthetic - A lymph node and abdominal exam to rule out any underlying problems
 The following observations on examination should be made: - Ischemic (or stuttering) priapism, which is characterized by a fully erected, painful, or tender penis, hard corpora cavernosa, and flaccid corpora spongiosum and glans.
- Nonischemic priapism: The glans and corpora spongiosum are flaccid, the penis is slightly erect but not painful or tender, and the corpora cavernosa are semirigid and nontender.

Laboratory Results 
Coagulation profile, CBC with reticulocyte count, sickling hemoglobin (Hgb) solubility test, Hgb electrophoresis, urine toxicology, and CBC with reticulocyte count
To differentiate between ischemia and nonischemic priapism, a corporal blood gas (CBG) should be taken.
While ischemia priapism raises some concerns, nonischemic blood gas analysis is identical to normal arterial blood. pCO2 >60 mm Hg, pH 7.25, and pO2 30 mm Hg.
To distinguish between ischemic (no blood flow in the cavernosal arteries) and nonischemic (high blood flow) priapism, a color Doppler ultrasound (CDUS) may be required.
Penile arteriography can be used to detect arterial cavernous fistula or nonischemic pseudoaneurysms.
Penile MRI can be used to assess candidates for rapid implantation of a penile prosthesis and cases that have lasted longer than 48 to 72 hours.

Management Depending on the kind:
- Urology consultation and urgent therapy are necessary for ischemic priapism. Initial conservative treatment should consist of ice, pain relief, promoting ejaculation, and intense activity between 4 and 24 hours (4).
Using a large-bore needle, aspirate the cavernosa (success rate: 30%). Injecting phenylephrine into the cavernosa up to 1,500 times (with blood pressure monitoring). Shunt procedures are considered if this doesn't work.
priapism that is not ischemic
 Site-specific compression, icing, and observation; the causal fistula may close on its own.
If nonischemic priapism does not go away, stuttering priapism may develop after arteriography and selective embolization.
The initial objective is to stop a recurrence. However, there is a chance that the incident will turn into ischemic priapism if it lasts too long.
 The use of ejaculation, exercise, icing, and/or a cold shower as a first-line treatment.
 Treat acute episodes as ischemic patients by aspirating and then injecting: Phenylephrine: 200 g every three to five minutes, up to a maximum of 1 mg in an hour; Etilefrine: 2.5 mg diluted in one to two mL of saline; Adrenaline: up to five doses of 2 mL of 1/100,000 solution given over a 20-minute period; Methylene blue: 50 to 100 mg
Treat the underlying problem in all situations, but especially in SCD cases (for instance, SCD does not postpone intracavernous therapy).
Blood transfusions are an option, especially severe SCD instances, but alternative therapies should be looked into and tested out first.

General Actions 
In SCD instances, IV hydration, supplementary oxygen, partial exchange, or repeated transfusions to lower the percentage of sickle cells to 50% are recommended.


Medication: If necessary, analgesics (such as opioids) for pain; while waiting for an intravenous injection, oral pseudoephedrine or terbutaline may be used. Initial Line
First-line treatment involves injecting phenylephrine intravenously.

Next Line
Etilefrine (intracavernous), the second-most popular sympathomimetic, is used to treat ischemic priapism.
- Methylene blue (intracavernous) is a cGMP inhibitor that can be used to treat pharmacologically caused priapism.
- Phenylephrine has a lower success rate than epinephrine (intracavernous), but it has more adverse cardiovascular consequences.
Stuttering priapism: - Daily oral medicine use to prevent recurrence:
Pseudoephedrine and etilefrine act as adrenergic agonists. Antiandrogens, 5'-reductase inhibitors, and ketoconazole act as androgen suppressants. PDE5 inhibitors include sildenafil, and digoxin or terbutaline can regulate smooth muscle.
Others include hydroxyurea, baclofen, and gabapentin.


Referral 

All suspected priapism instances should be seen by a urologist.

Surgical Procedures Following medicinal therapy for ischemic priapism, the following surgical procedures are available: - Shunts for the distal cavernoglandula
Open: Al-Ghorab or Burnett Percutaneous: Ebbehoj, Winter, or T Shunt
- Venous shunts - Proximal cavernoglandular shunts - Open: Quackles or Sacher
Barry (deep dorsal vein shunt) and Grayhack (saphenous vein shunt)
- Immediate implantation of a penile prosthesis: Early surgery prevents cavernosus fibrosis, which provides the opportunity to maintain penile size and prevent penile curvature owing to cavernosal fibrosis. Relative indications include corporal smooth muscle necrosis, failure of intracavernous therapies, MRI/biopsy, or failure of intracavernous therapies.
● Surgical alternatives for nonischemic priapism include the following: - As a last resort, surgical ligation, selective embolization with autologous blood clot, absorbable (gel foam or sponge) or nonabsorbable materials (coils or acrylic glue).

Patient Follow-Up Monitoring
There must be diligent urological follow-up.

Prognosis 
Complete detumescence may take several weeks due to edema, even with excellent treatment for a prolonged priapism.
In ischemic priapism, impotence from irreversible corporal fibrosis is likely, and the likelihood is up to 90% if the priapism lasts longer than 24 hours.


Erectile dysfunction  is the complication.

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