What is priapism?
Priapism is an erection that is not related to sexual arousal, lasts for hours and does not go down by itself. It is rare in children, but its most common form is an emergency: without timely treatment it can cause permanent damage to the tissue of the penis.
Morning erections and short erections are normal. They go away on their own and do not hurt.
Types
- Ischemic priapism: the most common form (about 95%). Blood gets trapped in the penis and oxygen levels fall. The erection is rigid and painful, while the head of the penis stays soft. This is an emergency.
- Stuttering priapism: short, painful erections keep coming back and go away on their own. These episodes often come before a severe ischemic priapism, so always tell the doctor about them.
- Non-ischemic priapism: usually develops 3 hours to 7 days after a blow to the penis, the perineum (the area between the legs) or the pelvis. The erection is not fully rigid. It is not an emergency, but a doctor needs to examine him.
- Priapism in a newborn: very rare, and it usually settles on its own.
Causes
The most common cause of priapism in children is sickle cell disease: about 65% of cases. Other causes include leukemia, injury and some medicines. In about 1 in 10 children no cause is found.
In boys with sickle cell disease the first episode happens at about 15 years of age on average, but 1 in 4 cases occurs before puberty.
What can we do at home?
If an erection will not go down, ask your child to pee, walk around and drink water. You can give pain relief suitable for his age.
If these measures have not helped within 2 hours, do not wait: go straight to the emergency department. If the pain is very severe or he has sickle cell disease, go sooner.
Diagnosis
The doctor asks when the erection started and about pain, previous episodes, medicines, blood disorders and injuries. A Doppler ultrasound of the penis shows blood flow and helps identify the type of priapism. Blood tests are done, including a test for sickle cell disease. If needed, blood is taken from the penis to check its oxygen level.
Treatment
- Ischemic priapism is treated urgently. Treatment starts with the gentlest method and moves to the next step if needed. In children these procedures are usually done under anesthesia.
- In sickle cell disease the pediatric urologist works together with a pediatric hematologist. The goal is both to stop the episode and to prevent new ones.
- Non-ischemic priapism and priapism in newborns are first managed with observation and simple measures.
The sooner the erection goes down, the better the outlook for the future function of the penis. So do not feel embarrassed and do not lose time.
When to seek urgent care
If any of the following is present, do not wait for an appointment or for a reply from the doctor: call your local emergency number or go to the nearest emergency department right away.
- A painful, rigid erection that has not gone down within 2 hours despite simple measures
- An erection that will not go down in a child with sickle cell disease or leukemia
- The erection is not going down and the pain is getting worse
Call your local emergency number (112 in Europe, 911 in North America)
Note for pediatricians and family doctors
Priapism is a full or partial erection lasting ≥4 hours and unrelated to sexual stimulation; it is rare in children. Causes: sickle cell disease (about 65%), leukemia (10%), trauma (10%), idiopathic (10%), drugs (5%). In sickle cell disease the mean age at first episode is about 15 years, and 25% present before puberty. Ischemic (low-flow) priapism is the most common form (95%): painful, with rigid corpora cavernosa and a soft glans and spongiosum; because of the risk of irreversible damage it is an emergency. Stuttering priapism (recurrent, self-limiting episodes) often precedes an ischemic episode. Non-ischemic (high-flow) priapism usually follows penile, perineal or pelvic trauma by 3 hours to 7 days and is not an emergency; neonatal priapism is very rare, is not associated with sickle cell disease, has polycythemia as the most common identifiable cause, and follows a benign course. EAU recommendations (all strong): Doppler ultrasound in all patients; full blood count and hemoglobinopathy screen in ischemic priapism; a multidisciplinary approach with pediatric hematology for sickle cell-associated priapism; a stepwise approach to ischemic priapism starting with the least invasive therapy; initial conservative management of neonatal and non-ischemic priapism. When ischemia is suspected, a corporal blood gas (pO2 < 30, pCO2 > 60, pH < 7.25) is informative. If conservative measures (walking, voiding, fluids, analgesia) fail for 2 hours or more, urgent care is needed.
What happens at the visit?
- A child with suspected priapism is seen without delay; we ask when the erection started, about previous episodes, medicines and any injury.
- A Doppler ultrasound of the penis and blood tests are done, and if needed a blood sample is taken from the penis to check oxygen levels.
- Ischemic priapism is treated step by step under anesthesia, starting with the gentlest method; in sickle cell disease decisions are made together with a pediatric hematologist.
Frequently asked questions
My son has erections in the morning. Is that priapism?
No. Morning erections and short erections are normal; they go away on their own and do not hurt. Priapism is an erection that lasts for hours, is often painful and does not go down by itself.
My child has sickle cell disease. What should I do?
If an erection is painful and does not go down, go straight to the emergency department and tell them your child has sickle cell disease. If short, repeated episodes happen, tell his hematologist and pediatric urologist: together they make a plan to prevent further episodes.
An erection started a few days after a blow and will not go down. Is this urgent too?
This may be non-ischemic priapism after an injury. It is not as urgent as the ischemic type, but have a pediatric urologist see him the same day: only an examination can tell the types apart.
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Sources
This information is for educational purposes and does not replace medical advice. Decisions about your child’s treatment are made with the doctor after an examination.
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