Priapism in children: a comprehensive review and clinical guideline

We review the English literature between 1980 and 2013 and summarize the clinical classification, aetiology, physiology, and pathophysiology of paediatric priapism. We propose a clinical guideline for the management of priapism in children.

Male patients aged ≤ 18 years.

Priapism, a prolonged penile erection lasting >4 h, is a rare condition in childhood. There are 3 widely accepted types of priapism: 1) ischaemic priapism, the commonest type seen in children; 2) stuttering priapism, recurrent, self-limiting prolonged erections; and 3) non-ischaemic priapism, rare in children, usually due to trauma. Neonatal priapism has also been described. Ischaemic priapism is a urological emergency causing fibrosis of the corpora cavernosa, subsequent erectile dysfunction and penile disfigurement. The commonest causes of priapism in children are sickle cell disease (65%), leukaemia (10%), trauma (10%), idiopathic (10%), and pharmacologically induced (5%).

Priapism in children must be assessed urgently. Rapid resolution of ischaemic priapism prevents permanent cavernosal structural damage and is associated with improved prognosis for potency later in life. Stuttering priapism requires careful counselling for episodic management. Chronic prophylaxis may be obtained using α-adrenergic sympathomimetics, phosphodiesterase type 5 inhibitors and, in sickle cell disease, hydroxyurea. Non-ischaemic and neonatal priapism may generally be treated less urgently.