Priapism
Low-flow priapism
Priapism is an abnormally persistent, usually painful erection of the penis. It must be defined as either a low-flow or a high-flow type.
Presentation
Symptoms vary depending on the type of priapism that has occurred. The following is a list of symptoms explaining both type of priapism.
Ischemic (low flow) priapism
- Involuntary erection that lasts for 4 hours or even longer
- Pain during erection
- The penis is tender to touch and is rigid
- Several bouts of involuntary erection – a condition referred to as stuttering priapism [6]
Non-ischemic (high flow) priapism
- Involuntary erection that lasts for at least 4 hours
- The penis is erect but not painful
Immune System
Massive Splenomegaly
- Chronic myeloid leukemia (CML) is a chronic myeloproliferative disorder that usually presents with high white blood cell counts and massive splenomegaly. Priapism is a rare manifestation of CML and is mostly due to hyperleukocytosis.[ncbi.nlm.nih.gov]
Entire Body System
Leg Swelling
- His postoperative course was uneventful, and his priapism as well as the scrotal and leg swelling improved. He was discharged home on full anticoagulation.[ncbi.nlm.nih.gov]
Lower Extremity Pain
- We report on a 42-year-old male who presented with priapism, severe scrotal swelling, and left lower extremity pain and swelling. Initial management of priapism failed, and he was noted to have both cavernosal and glandular venous obstruction.[ncbi.nlm.nih.gov]
Epilepsy
- Panayiotopoulos syndrome is an idiopathic epilepsy syndrome presenting with a large variety of autonomic symptoms. The mechanism of autonomic symptoms is still not well understood.[ncbi.nlm.nih.gov]
Developmental Disabilities
- Author information 1 Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, GA, United States of America. 2 The James Buchanan Brady Urological Institute, Johns[ncbi.nlm.nih.gov]
Gastrointestinal
Abdominal Cramps
- We present a case of a 3-year-old boy who was bitten by a black widow and presented with abdominal cramping and priapism.[ncbi.nlm.nih.gov]
Musculoskeletal
Leg Cramp
- We report a case of a previously healthy 2-year-old boy who presented with severe irritability, leg cramps, and stomach ache.[ncbi.nlm.nih.gov]
Neurologic
Behavior Problem
- Risperidone is an atypical antipsychotic widely prescribed for the treatment of behavior problems in children with autism spectrum disorder. It seems associated with priapism in children.[ncbi.nlm.nih.gov]
Urogenital
Painful Erection
- It is seen in patients with sickle cell anemia, advanced malignancy, spinal trauma; and certain drug treatments Persistent and usually painful erection that lasts for at least four hours, in the absence of physical or psychological stimulation.[icd9data.com]
- Indian J Pharmacol 2013;45:629-30 » Introduction Priapism is defined as a prolonged and persistent, painful erection of the penis without sexual stimulation or arousal. 2 Risperidone/sodium valproate Reactions Weekly. 2014; 1484(1): 30[doi.org]
- A 12-year-old male, with sickle cell disease, presented with 72 hours of painful erection. The patient failed conservative measures including aspiration, injection of phenylephrine, as well as distal shunt procedure.[ncbi.nlm.nih.gov]
- Priapism is the prolonged, painful erection of penile tissue not accompanied by sexual arousal. Priapism has been established as a rare adverse drug reaction to drugs such as antipsychotics, psychostimulants, antidepressants, and mood stabilizers.[ncbi.nlm.nih.gov]
Penile Pain
- A 42-year-old man presented to the emergency room with penile pain and partial erection. Examination revealed partial erection and palpable space-occupying lesion of the corpus cavernosum without lymphadenopathy. Malignant workup was negative.[ncbi.nlm.nih.gov]
- In both cases, the erection and penile pain were immediately diminished, and corporal blood flow measured by color Doppler ultrasonography was restored after the surgery. There was no urethral injury or perforation of the tunica albuginea.[ncbi.nlm.nih.gov]
- He appears uncomfortable from penile pain but is nontoxic.[pedsinreview.aappublications.org]
- Signs and symptoms include: Erection lasting more than four hours or unrelated to sexual interest or stimulation Rigid penile shaft, but the tip of penis (glans) is soft Progressive penile pain Recurrent or stuttering priapism, a form of ischemic priapism[mayoclinic.org]
Dysuria
- Abstract A 51-year-old man presented at our department 2 days after the onset of a painful mass in the perineum and dysuria.[ncbi.nlm.nih.gov]
- Review of systems should seek symptoms suggesting a cause, including dysuria ( UTIs ), urinary hesitancy or frequency ( prostate cancer ), fever and night sweats ( leukemia ), and lower-extremity weakness (spinal cord pathology).[msdmanuals.com]
- He is not sexually active and denies penile discharge or dysuria. In addition, he cannot recall any recent trauma or injury to his penis, scrotum, or perineum. Vitals are notable for a HR of 102.[emdocs.net]
Workup
In the preliminary phase, the individual will be asked about the length of the penile erection and frequency of the condition. Following this, a thorough physical examination will be carried out to understand the cause behind the development of priapism. In addition to this, the following methods will be carried out to diagnose the condition [7]:
- Blood gas measurement: In this blood is drawn from the penis to be analyzed for the type of gases present in the sample. Such a type of test determines the type of priapism present.
- Blood tests would be done to detect the presence of any underlying disease conditions such as sickle cell anemia or leukemia.
- Color duplex ultrasound examination will be carried out to detect presence of tumor or any other abnormality inside the penis. This test would also provide insight into the type of priapism present.
- Toxicology test would help in revealing whether drug abuse is the cause of priapism.
EEG
Irregular Generalized Spike-and-Slow-Waves
- An outpatient EEG 6 months later showed abundant multifocal, predominantly posterior, as well as irregular generalized spike-and-slow-wave discharges on normal background consistent with the diagnosis of Panayiotopoulos syndrome.[ncbi.nlm.nih.gov]
Other ECG Findings
Ischemic Changes
- He was noticed to have developed ischemic changes of the distal part of the penile skin which progressed to gangrene of the distal part of the penis on the 4th day post intervention.[doi.org]
Treatment
Treatment of priapism is geared towards relieving the erection and preserving the erectile function. If treatment is initiated within 4 to 6 hours of erection, then medications should be enough to correct the condition. In addition, the following methods are employed to treat priapism [8]:
- Ice packs: Application of ice packs to the penis and neighboring areas may help relieve the condition.
- Surgical ligation is employed when there is arterial rupture. In this process, the artery is tied in order to restore the blood supply.
- Shunt: In this a shunt is surgically inserted to divert the blood flow in order to restore the normal blood circulation in the penis [9] [10].
- Needle aspiration: In this method, a needle is inserted into the penis to drain out the blood in order to correct the erection.
Prognosis
The prognosis of the condition can be favorable only if prompt treatment is initiated. Failure to do so can result in development of permanent damage to the penis. When priapism continues for more than 24 hours then erectile dysfunction can set in permanently [5].
Etiology
In many cases, the exact cause of priapism remains a mystery. However individuals with sickle cell anemia are known to fall easy prey to this condition. Sickle cell anemia is a heredity blood disorder in which the abnormally shaped red blood cells inhibit blood flow to the penis giving rise to priapism [2].
In addition to sickle cell anemia, other type of blood disease such as leukemia is also known to play a role in causation of the disease. Research has also pointed towards the fact that certain drugs commonly used for treating sexual dysfunction, depression and psychotic disorder can also lead to development of priapism.
There have been instances that have reported injury to be one of the major causes of non-ischemic priapism. Other causes of priapism include injury to the spinal cord, blood clots or poisonous venom.
Epidemiology
Priapism is an uncommon condition accounting for 1.5 cases per 100,000 individuals-years globally. A rise in the rate of incidence of priapism has been noted for men aged 40 years and above. It has been estimated that in this older population the incidence increased to 2.9 cases per 100,000 individuals-years [3]. The condition can strike males at any age, but is more common in boys below 10 years and in men above 20 years.
Pathophysiology
Under normal conditions, penis erection occurs in response to sexual stimulation. Due to stimulation, the blood vessels relax and expand increasing the blood flow to the penis. Such sequence of events causes the penis to erect. When the stimulation ends, the blood flows out and the penis returns to its normal (flaccid) state [4].
When some kind of disruption occurs in these normal events, priapism develops. Ischemic (low flow) priapism is the most common type and occurs when the blood gets trapped in the penis and is unable to flow out. Such an event does not allow the penis to return to its normal state.
Non-ischemic (high flow) priapism is pretty uncommon compared to the former type. It occurs when an artery gets ruptured that prevents the normal circulation of blood within the penis.
Prevention
Several steps can be followed to prevent recurrent attacks of priapism. If the cause of the underlying condition is known then appropriate treatment should be initiated to treat the condition. In case, drugs were the cause then attempts should be made to change the medications.
Men are also advised against use of recreational drugs and alcohol which have a known association with priapism episodes. The practice of self-injection of phenylephrine should be discouraged as it can trigger development of priapism.
Summary
Priapism is characterized by penile erection that occurs without any kind of sexual stimulation. It is a painful condition requiring prompt treatment. Such condition occurs in young boys aged 5 to 10 years or in adults between 20 to 50 years of age. Priapism can be either ischemic or non-ischemic in nature. The condition can last for as long as 4 hours or even more. Priapism requires immediate medical intervention in order to avoid onset of irreversible conditions. Failure to treat the condition can call for development of erectile dysfunction [1].
Patient Information
Definition
Priapism is prolonged erection of the penis without any physical or psychological stimulation. It is an extremely painful condition that should receive prompt treatment. Priapism can affect males of all age groups, but is more common in young boys and adult males.
Cause
Underlying disease conditions such as sickle cell anemia and leukemia are known to be major contributory factors. In addition, trauma or injury to the genital can also cause development of priapism. Individuals in the habit of using illegal drugs or alcohol are also susceptible. In many cases, it has been noticed that prescription drugs that have been advised for treatment of various disease conditions also predisposes males to develop priapism.
Symptoms
Symptoms of priapism include painful penile erection for 4 hours or more. The penis become rigid and may be tender to touch.
Diagnosis
Diagnosis is made through blood gas measurement in which blood that is trapped in the penis is tested for certain gases which confirms the presence of priapism. In addition, blood tests and ultrasound for detecting the presence of various disease conditions are also done.
Treatment
Treatment of priapism is done through medications and surgical intervention. If the duration of erection is less than 4 hours then medications alone help rectify the condition.
References
Article
- Quan D, Ruha AM. Priapism associated with Latrodectus mactans envenomation. Am J Emerg Med. Jul 2009;27(6):759.e1-2.
- Pryor J, Akkus E, Alter G, et al. Priapism. J Sex Med 2004; 1:116.
- Eland IA, van der Lei J, Stricker BH, Sturkenboom MJ. Incidence of priapism in the general population.Urology. May 2001;57(5):970-2.
- Burnett AL, Bivalacqua TJ. Priapism: current principles and practice. Urol Clin North Am 2007; 34:631.
- Dubin J, Davis JE. Penile emergencies. Emerg Med Clin North Am. Aug 2011;29(3):485-99.
- Muneer A, Minhas S, Arya M, Ralph DJ. Stuttering priapism--a review of the therapeutic options. Int J Clin Pract 2008; 62:1265.
- Bassett J, Rajfer J. Diagnostic and therapeutic options for the management of ischemic and nonischemic priapism. Rev Urol. Winter 2010;12(1):56-63.
- Montague DK, Jarow J, Broderick GA, et al. American Urological Association guideline on the management of priapism. J Urol 2003; 170:1318.
- Mains E, Aboumarzouk O, Ahmad S, El-Mokadem I, Nabi G. A minimally invasive temporary cavernoso-saphenous shunt in the management of priapism after failed conservative treatment. Minim Invasive Ther Allied Technol. Dec 5 2011
- Brant WO, Garcia MM, Bella AJ, et al. T-shaped shunt and intracavernous tunneling for prolonged ischemic priapism. J Urol 2009; 181:1699.