Priapism in children: a comprehensive review and clinical guideline

  • James F. Donaldson
    Correspondence
    Corresponding author. Tel.: +44 2380 777 222; fax: +44 2380 795 230.
    Affiliations
    Department of Paediatric Urology, University Hospital Southampton NHS Foundation Trust, Tremona Road, Southampton, Hampshire SO16 6YD, UK
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +44 2380 777 222; fax: +44 2380 795 230.
    Rowland W. Rees
    Footnotes
    1 Tel.: +44 2380 777 222; fax: +44 2380 795 230.
    Affiliations
    Department of Urological Surgery, University Hospital Southampton NHS Foundation Trust, Tremona Road, Southampton, Hampshire SO16 6YD, UK
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +44 2380 777 222; fax: +44 2380 795 230.
    Henrik A. Steinbrecher
    Footnotes
    1 Tel.: +44 2380 777 222; fax: +44 2380 795 230.
    Affiliations
    Department of Paediatric Urology, University Hospital Southampton NHS Foundation Trust, Tremona Road, Southampton, Hampshire SO16 6YD, UK
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +44 2380 777 222; fax: +44 2380 795 230.
Published:October 16, 2013DOI:https://doi.org/10.1016/j.jpurol.2013.07.024

      Abstract

      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.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of Pediatric Urology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

      1. The World Health Organisation collaborating centres for classification of diseases. International classification of diseases, international statistical classification of diseases and related health problems 10th revision. 2010 (Retrieved March 2013 from:)
        • Broderick G.A.
        • Kadioglu A.
        • Bivalacqua T.J.
        • Ghanem H.
        • Nehra A.
        • Shamloul R.
        Priapism: pathogenesis, epidemiology, and management.
        J Sex Med. 2010; 7: 476-500
        • Jesus L.E.
        • Dekermacher S.
        Priapism in children: review of pathophysiology and treatment.
        J Pediatr (Rio J). 2009; 85: 194-200
        • Cherian J.
        • Rao A.R.
        • Thwaini A.
        • Kapasi F.
        • Shergill I.S.
        • Samman R.
        Medical and surgical management of priapism.
        Postgrad Med J. 2006; 82: 89-94
        • Dust N.
        • Daboval T.
        • Guerra L.
        Evaluation and management of priapism in a newborn: a case report and review of the literature.
        Paediatr Child Health. 2011; 16: 6-8
        • Montorsi F.
        • Basson R.
        • Adaikan G.
        • Becher E.
        • Clayton A.
        • Giuliano F.
        • et al.
        Sexual medicine: sexual dysfunction in men and women; the third international consultation on sexual medicine.
        International Consultation on Urological Diseases. Health Publication Ltd, France2010 (Retrieved March 2013 from:)
        • Emond A.M.
        • Holman R.
        • Hayes R.J.
        • Serjeant G.R.
        Priapism and impotence in homozygous sickle cell disease.
        Arch Intern Med. 1980; 140: 1434-1437
        • Chow K.
        • Payne S.
        The pharmacological management of intermittent priapismic states.
        BJU Int. 2008; 102: 1515-1521
        • Mockford K.
        • Weston M.
        • Subramaniam R.
        Management of high-flow priapism in paediatric patients: a case report and review of the literature.
        J Pediatr Urol. 2007; 3: 404-412
        • Aktoz T.
        • Tepeler A.
        • Gündoğdu E.O.
        • Ozkuvanci U.
        • Müslümanoğlu A.Y.
        Priapism in the newborn: management and review of literature.
        Andrologia. 2011; 43: 65-67
        • Kulmala R.V.
        • Lehtonen T.A.
        • Tammela T.L.
        Priapism, its incidence and seasonal distribution in Finland.
        Scand J Urol Nephrol. 1995; 29: 93-96
        • Eland I.A.
        • van der Lei J.
        • Stricker B.H.
        • Sturkenboom M.J.
        Incidence of priapism in the general population.
        Urology. 2001; 57: 970-972
        • Burnett A.L.
        • Bivalacqua T.J.
        Priapism: current principles and practice.
        Urol Clin North Am. 2007; 34: 631-642
        • Adeyoju A.B.
        • Olujohungbe A.B.
        • Morris J.
        • Yardumian A.
        • Bareford D.
        • Akenova A.
        • et al.
        Priapism in sickle-cell disease; incidence, risk factors and complications–an international multicentre study.
        BJU Int. 2002; 90: 898-902
        • Mantadakis E.
        • Cavender J.D.
        • Rogers Z.R.
        • Ewalt D.H.
        • Buchanan G.R.
        Prevalence of priapism in children and adolescents with sickle cell anemia.
        J Pediatr Hematol Oncol. 1999; 21: 518-522
        • Merlob P.
        • Livne P.M.
        Incidence, possible causes and follow up of idiopathic prolonged penile erection in the newborn.
        J Urol. 1989; 141: 1410-1412
        • Dean R.C.
        • Lue T.F.
        Physiology of penile erection and pathophysiology of erectile dysfunction.
        Urol Clin North Am. 2005; 32: 379-395
        • Lue T.F.
        Male sexual dysfunction.
        in: McAninch J.W. Lue T.F. Smith & Tanagho's general urology. 18th ed. The McGraw Hill Companies Inc, 2013: 596-616
        • Donaldson J.F.
        • Davis N.
        • Davies J.H.
        • Rees R.W.
        • Steinbrecher H.A.
        Priapism in teenage boys following depot testosterone.
        J Pediatr Endocrinol Metab. 2012; 25: 1173-1176
        • Broderick G.A.
        Priapism and sickle-cell anemia: diagnosis and nonsurgical therapy.
        J Sex Med. 2012; 9: 88-103
        • Birnbaum B.F.
        • Pinzone J.J.
        Sickle cell trait and priapism: a case report and review of the literature.
        Cases J. 2008; 1: 429
        • Castagnetti M.
        • Sainati L.
        • Giona F.
        • Varotto S.
        • Carli M.
        • Rigamonti W.
        Conservative management of priapism secondary to leukemia.
        Pediatr Blood Cancer. 2008; 51: 420-423
        • Morrison B.F.
        • Burnett A.L.
        Priapism in hematological and coagulative disorders: an update.
        Nat Rev Urol. 2011; 8: 223-230
        • Yuan J.
        • Desouza R.
        • Westney O.L.
        • Wang R.
        Insights of priapism mechanism and rationale treatment for recurrent priapism.
        Asian J Androl. 2008; 100: 88-101
        • Walker S.
        • Hanna B.D.
        • Meluskey H.
        • Ramsey E.
        • Dubey S.
        Sildenafil use in pediatric pulmonary hypertension: minimal risk.
        in: Pulmonary hypertension professional network symposium. Pulmonary Hypertension Association, USASeptember 2011 (Abstract retrieved April 2013 from:)
        • Succu S.
        • Mascia M.S.
        • Melis T.
        • Sanna F.
        • Boi A.
        • Melis M.R.
        • et al.
        Morphine reduces penile erection induced by the cannabinoid receptor antagonist SR 141617A in male rats: role of paraventricular glutamic acid and nitric oxide.
        Neurosci Lett. 2006; 404: 1-5
        • Maples B.L.
        • Hagemann T.M.
        Treatment of priapism in pediatric patients with sickle cell disease.
        Am J Health Syst Pharm. 2004; 61: 355-363
        • Corbetta J.P.
        • Durán V.
        • Burek C.
        • Sager C.
        • Weller S.
        • Paz E.
        • et al.
        High flow priapism: diagnosis and treatment in pediatric population.
        Pediatr Surg Int. 2011; 27: 1217-1221
        • Bastuba M.D.
        • Saenz de Tejada I.
        • Dinlenc C.Z.
        • Sarazen A.
        • Krane R.J.
        • Goldstein I.
        Arterial priapism: diagnosis, treatment and long-term followup.
        J Urol. 1994; 151: 1231-1237
        • National Institute for Health and Clinical Excellence
        Sedation in children and young people: sedation for diagnostic and therapeutic procedures in children and young people.
        (Clinical Guideline (CG112))2010 (Retrieved March 2013 from:)
        • American Academy of Pediatrics
        Timing of elective surgery on the genitalia of male children with particular reference to the risks, benefits, and psychological effects of surgery and anesthesia.
        Pediatrics. 1996; 97: 590-594
        • Marchant W.A.
        • Walker I.
        Anaesthetic management of the child with sickle cell disease.
        Paediatr Anaesth. 2003; 13: 473-489
        • Mantadakis E.
        • Ewalt D.H.
        • Cavender J.D.
        • Rogers Z.R.
        • Buchanan G.R.
        Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism.
        Blood. 2000; 95: 78-82
        • Hatch D.A.
        Preventing hematomas during artificial erection.
        Urol Clin North Am. 1990; 17: 17
        • Montague D.K.
        • Jarow J.
        • Broderick G.A.
        • Dmochowski R.R.
        • Heaton J.P.
        • Lue T.F.
        • et al.
        American urological association guideline on the management of priapism.
        J Urol. 2003; 170: 1318-1324
        • Molina L.
        • Bejany D.
        • Lynne C.M.
        • Politano V.A.
        Diluted epinephrine solution for the treatment of priapism.
        J Urol. 1989; 141: 1127-1128
        • Halls J.E.
        • Patel D.V.
        • Walkden M.
        • Patel U.
        Priapism: pathophysiology and the role of the radiologist.
        Br J Radiol. 2012; 85: 79-85
        • Bertolotto M.
        • Quaia E.
        • Mucelli F.P.
        • Ciampalini S.
        • Forgács B.
        • Gattuccio I.
        Color Doppler imaging of posttraumatic priapism before and after selective embolization.
        Radiographics. 2003; 23: 495-503
        • Shah A.
        • Parashar K.
        • Chandran H.
        Paediatric priapism – treatment conundrum.
        Pediatr Surg Int. 2004; 20: 806-808
        • Segal R.L.
        • Readal N.
        • Pierorazio P.M.
        • Burnett A.L.
        • Bivalacqua T.J.
        Corporal Burnett “Snake” surgical maneuver for the treatment of ischemic priapism: long-term followup.
        J Urol. 2013; 189: 1025-1029
        • Ralph D.J.
        • Garaffa G.
        • Muneer A.
        • Freeman A.
        • Rees R.
        • Christopher A.N.
        The immediate insertion of a penile prosthesis for acute ischaemic priapism.
        Eur Urol. 2009; 56: 1033-1038
        • Herschorn S.
        • Ordorica R.C.
        Penile prosthesis insertion with corporeal reconstruction with synthetic vascular graft material.
        J Urol. 1995; 154: 80-84
        • Siegel J.F.
        • Rich M.A.
        • Brock W.A.
        Association of sickle cell disease, priapism, exchange transfusion and neurological events: ASPEN syndrome.
        J Urol. 1993; 150: 1480-1482
        • Chinegwundoh F.
        • Anie K.A.
        Treatments for priapism in boys and men with sickle cell disease.
        Cochrane Database Syst Rev. 2004; 4: CD004198
        • Muneer A.
        • Minhas S.
        • Arya M.
        • Ralph D.J.
        Stuttering priapism – a review of the therapeutic options.
        Int J Clin Pract. 2008; 62: 1265-1270
        • Ware R.E.
        How I use hydroxyurea to treat young patients with sickle cell anemia.
        Blood. 2010; 115: 5300-5311
        • Steinberg M.H.
        • McCarthy W.F.
        • Castro O.
        • Ballas S.K.
        • Armstrong F.D.
        • Smith W.
        • et al.
        The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: a 17.5 year follow-up.
        Am J Hematol. 2010; 85: 403-408
        • Moreira D.M.
        • Pimentel M.
        • da Silva Moreira B.F.
        • Stein A.C.
        • Koff W.J.
        Recurrent priapism in the young patient treated with baclofen.
        J Pediatr Urol. 2006; 2: 590-591
        • Sneed R.C.
        • Stover S.L.
        Undiagnosed spinal cord injuries in brain-injured children.
        Am J Dis Child. 1988; 142: 965-967
        • Imamoglu A.
        • Bakirtas H.
        • Conkbayir I.
        • Tuygun C.
        • Sarici H.
        An alternative non-invasive approach for the treatment of high flow priapism in a child: duplex ultrasound-guided compression.
        J Pediatr Surg. 2006; 41: 446-448
        • Bivalacqua T.J.
        • Burnett A.L.
        Priapism: new concepts in the pathophysiology and new treatment strategies.
        Curr Urol Rep. 2006; 7: 497-502