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A comprehensive review and clinical guideline to priapism in children, "Beyond the Abstract," by James F. Donaldson

BERKELEY, CA (UroToday.com) - Boys' erections, prolonged or otherwise, are a taboo, and are likely to remain so. Boys (especially those with only a single mother to turn to), and even their parents, may therefore avoid seeking a medical consultation for priapism. Thus, the prevalence of paediatric priapism is difficult to estimate, but it appears to be rare, although clearly remains higher in populations with higher proportions of Afro-Caribbean ethnicities, in whom sickle cell disease is prevalent.

Paucity of evidence
Paediatric urologists may see just a handful of cases in their career, particularly those treating predominantly Caucasian populations. Researching paediatric priapism is therefore clearly extremely difficult and thus there is a striking paucity of evidence, which our review article highlights.[1] An evidence-based approach is therefore difficult to adopt, with many principles from adult priapism assumed by necessity. Both European Association of Urology (EAU)[2] and American Urology Association (AUA)[3] guidelines on the management of priapism, written predominantly by adult urologists, consider a few aspects which will differ for children. However, in my view, these guidelines fail to provide a comprehensive guideline for the management of priapism in children. The continuing emergence of both andrology and paediatric urology as sub-specialities (and corroboration therein) will hopefully improve the evidence base.

Anaesthesia
The management of paediatric priapism is therefore controversial. Whilst aspiration may be performed without anaesthesia in adults, recent EAU guidelines suggest "oral conscious sedation" for children. This would be combined with a local anaesthetic penile block and is supported by reports in children where this is presumably routine practice in some centres.[4] However, many British paediatric urologists would advocate general anaesthesia for painful procedures in children, particularly those involving genitals, given the potential psycho-sexual sequelae (which may significantly complicate treating subsequent erectile dysfunction in adulthood).[5]

General anaesthesia may not be without consequence, particularly during a sickle cell crisis or where paediatric anaesthetic expertise is not readily on hand. Evidence from adults suggests outcomes are worse with longer delays until treatment in ischaemic priapism. These factors must all be balanced, no doubt presenting a difficult decision in some centres/circumstances. In the majority of children, however, I would favour general anaesthesia.

Sympathomimetic choice
Phenylephrine is widely accepted to be the agent of choice in adults; and both EAU and AUA guidelines recommend "a lower concentration or volume... for children."[6, 7] However, this recommendation is based on panel consensus alone in both guidelines, without citing evidence. Our paper summarises the evidence for different sympathomimetics in children of different ages: the safety/dosage of intracorporal phenylephrine in children < 11years has not been widely reported.[8] However, side effects are presumably seldom encountered in children, where excessive dosages or injection after the erection has subsided are avoided. We found very few reports of significant side effects in children. However, it should be remembered that intracorporal use of any sympathomimetics is off-licence, even in adults.

Social acceptability and inexperience
The acceptability of neonatal/high-flow priapism to clinicians, parents and children will vary in different societies. The acceptability of trialling conservative/mechanical management approaches will therefore vary. Further, inexperience with penile Doppler & embolisation, even in quaternary paediatric centres, may complicate management decisions. The 2014 EAU guidelines state that a "specialist paediatric vascular radiology department" is required to facilitate successful embolisation.[9]

Finally, the EAU guidelines suggests considering penile prosthesis insertion acutely for all patients with refractory priapism of > 36hours duration.[9] The probability of subsequent erectile dysfunction is more difficult to predict in children, with some surprising reports of normal potency returning even after 72 hours of ischaemic priapism -- although validated questionnaires are not widely used.[10] Consideration to penile prosthesis insertion in the acute setting in children will therefore largely not be appropriate, regardless of the child's physical or psycho-sexual developmental stage.[11]

References:

  1. Donaldson JF, Rees RW, Steinbrecher HA. Priapism in children: a comprehensive review and clinical guideline. J Pediatr Urol. 2014 Feb;10(1):11-24.
  2. Salonia A, Eardley I, Giuliano F, Hatzichristou D4, Moncada I, Vardi Y, Wespes E, Hatzimouratidis K. European Association of Urology guidelines on priapism. Eur Urol. 2014;65(2):480-9.
  3. Montague DK, Jarow J, Broderick GA, Dmochowski RR, Heaton JP, Lue TF, et al. American urological association guideline on the management of priapism. J Urol 2003; 170:1318e24.
  4. Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 2000;95:78e82.
  5. Hegarty PK. Commentary to 'Priapism in children: a comprehensive review and clinical guideline'. J Pediatr Urol. 2014 Feb;10(1):24-5.
  6. Montague DK, Jarow J, Broderick GA, Dmochowski RR, Heaton JP, Lue TF, et al. American urological association guideline on the management of priapism. J Urol 2003; 170:1318e24.
  7. Salonia A, Eardley I, Giuliano F, Hatzichristou D4, Moncada I, Vardi Y, Wespes E, Hatzimouratidis K. European Association of Urology guidelines on priapism. Eur Urol. 2014;65(2):480-9.
  8. Donaldson JF, Rees RW, Steinbrecher HA.Priapism in children: a comprehensive review and clinical guideline. J Pediatr Urol. 2014 Feb;10(1):11-24.
  9. Salonia A, Eardley I, Giuliano F, Hatzichristou D4, Moncada I, Vardi Y, Wespes E, Hatzimouratidis K. European Association of Urology guidelines on priapism. Eur Urol. 2014;65(2):480-9.
  10. Donaldson JF, Rees RW, Steinbrecher HA.Priapism in children: a comprehensive review and clinical guideline. J Pediatr Urol. 2014 Feb;10(1):11-24.
  11. Donaldson JF, Rees RW, Steinbrecher HA. Response to Commentary to 'Priapism in children: a comprehensive review and clinical guideline'. J Pediatr Urol. 2014 Feb;10(1):25.

Written by:
James F. Donaldson as part of Beyond the Abstract on UroToday.com. This initiative offers a method of publishing for the professional urology community. Authors are given an opportunity to expand on the circumstances, limitations etc... of their research by referencing the published abstract.

Department of Paediatric Urology, University Hospital Southampton NHS Foundation Trust, Southampton, Hampshire, UK

Priapism in children: A comprehensive review and clinical guideline - Abstract

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