Priapism in children: a comprehensive review and clinical guideline.

OBJECTIVE: 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. PATIENTS: Male patients aged ≤ 18 years. RESULTS: 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%). CONCLUSIONS: 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.

Priapism in teenage boys following depot testosterone.

Priapism is rare in children and may result in erectile dysfunction and sexual aversion behaviours. Testosterone therapy is commonly regarded as safe in children and is widely used in constitutional delay of growth and puberty, hypogonadism, hypospadias and micropenis. We report two cases of priapism in teenage boys with constitutional delay of growth and puberty after a change in the formulation of depot testosterone. One case required surgical intervention and the other was preceded by stuttering priapism. These cases illustrate the importance of patient and/or parent counselling before testosterone administration and consideration of lower doses in at-risk patients.

The utilization of stents in the management of primary obstructive megaureters requiring intervention before 1 year of age.

OBJECTIVE: To analyse the long-term outcome of 16 infants with primary obstructive megaureter managed by (endoscopic or open) stenting over a 10-year period. METHODS: A retrospective case-note and imaging review was performed between 1997 and 2007. Data are presented as medians (range) and were compared using the Mann-Whitney test. RESULTS: Sixteen infants with 19 obstructed megaureters were stented at a median age of 24 weeks, for a median of 6 months. One-third of stents were inserted endoscopically. Complications (stent migration, stone formation or infection) occurred in 31.6%. One infant was excluded due to a co-existent pelviureteric junction obstruction. Drainage improved in 10 ureters (56%) following stent removal. Of the remaining renal units, 6 were reimplanted, and 2 underwent a nephrectomy due to deterioration in renal function following stent removal. Resolution of the vesicoureteric junction obstruction occurred in 4 of 6 infants following endoscopic stent insertion, and in 6 of 12 infants following open stent insertion (P=0.60). There was no difference in the length of time that ureters were stented in the resolved (7.5 (6-15) months) versus the reimplanted (6.0 (1-18) months) group (P=0.13). CONCLUSIONS: Of obstructive megaureters managed by stenting for a median of 6 months, 56% did not require further surgery. However, morbidity occurred in one third of patients, and function deteriorated after removal of stent in two patients who later required a nephrectomy.