Fatal Erection

During a shift I had a 3 year old child present. His Mum said he had been unable to pass any urine and kept saying he needed the toilet but every time she took him nothing happened. She also noticed he had an erection. She thought it had been erect for about 1–2 hours as far as she knew, which she felt was unusual.

On examination the boy still had an erection and appeared to be tender in his lower abdomen. His bladder scan showed 300 mls. He had a past medical history of recieving chemotherapy for Neurofibromatosis which is a type of phakomatosis or syndrome with neurological and cutaneous manifestations, causing benign tumours of the nerves and growths in other parts of the body including the skin.

I suspected he had a Priapism. Priapism is a prolongued erection of the penis. The full or partial erection can continue for several hours and is not always related to sexual stimulation.

There are 2 types ischemic and non-ischemic.

Ischemic or ‘low flow’ Priapism is the result of blood not being able to leave the penis. Blood is trapped because it cannot flow out from the veins in the penis or there is an issue with the contraction of smooth muscles within the erectile tissue of the penis. This is the most common type and requires immediate medical attention to prevent necrosis, which can begin within 3 hours of the onset.

Another type of ischemic Priapism is ‘stuttering Priapism’ or recurrent Priapism. This is an uncommon condition that involves prolongued erections and often includes episodes of ischemic priapism. Much more common in sickle cell disease and can begin in childhood.

Signs and symptoms include:

1. Erection lasting more than 4 hours or unrelated to sexual interest or stimulation.

2. Rigid penile shaft, but the tip of the penis is soft.

3. Progressively worsening penile pain.

It occurs more commonly in certain groups of people including sickle cell disease.

Non-ischemic priapism, also known as ‘high-flow’ priapism, occurs when blood flow through the arterties of the penis is not working properly. However, the penile tissues continue to recieve oxygen . This is often caused by trauma.

Signs and symptoms include:

1. Erection lasting more than 4 hours or unrelated to sexual activity or stimulation.

2. Erect but not fully rigid penile shaft.

3. Usually not painful.

The underlying cause of priapism often is left unknown but several conditions may play a role:

1. Sickle cell disease

2. Leukemia

3. Thalassemia

4. Multiple myeloma

5. Other blood diseases

6. Side effects of medications including anti-depressants, alpha blockers, medications used to treat anxiety or psychotic disorders, anticoagulants including warfarin and heparin, hormones such as testosterone, ADHD medications.

7. Alcohol and substance abuse

8. Spider bites, scorpion sting or other toxic infections

9. Metabolic disorders such as gout or amyloidosis

10. Neurogenic disorders, such as a spinal cord injury or syphilis

11. Cancers involving the penis.

Ischemic priapism can cause serious complications. If an erection lasts more than 4 hours this lack of oxygen will begin to destroy tissues in the penis and cause chronic erectile dysfunction.

Diagnostic tests include:

Penile blood gas measurement: You sample blood from the penis. If the blood is black it suggests it is deprived of oxygen and most likely ischemic. If it is bright red then it is non-ischemic.

Blood tests to look for blood disorders or signs of infection or toxins.

Ultrasound doppler scan to check the flow of blood in arteries and veins.

Treatment of Ischemic Priapism:

Urgent Urology review is essential.

1. Good pain control

2. Aspiration decompression-excess blood is drained from the penis.

3. Medications such as phenylepherine may be injected into the penis. This drug constricts blood vessels that carry blood into the penis.

4. Surgery to re-route blood flow or drainage of blood from the penis in theatre.

Treatment of non-ischemic Priapism:

Urgent Urology review is essential.

1. Non-ischemic Priapsim usually goes away with no treatment and because the risk is much lower a watch and wait approach is usually taken.

2. Ice packs and pressure on the perineum might help end the erection.

As the child had a history of Neurofibromatosis I suspected this could be the cause. He was sent to the local Emergency department for Urgent Urological review. Thankfully, by the time he was seen the erection had resided, he managed to empty his bladder and no treatment was required. He was referred back to his GP for further investigations to investigate the cause.