Priapism in Children

Priapism in ChildrenThe vast majority of the time, caring for patients, particularly children, is extremely rewarding and life affirming. We are among the few who are privileged to make a true positive impact on a person’s life. There are times, however, when the specifics of an encounter can make the job less than appealing, although, important nonetheless. One specific condition that may lead to a less than enjoyable shift is priapism. Why? Well, the procedure is not one people typically thank you for doing afterwards, even if successful. In addition, like Fever of Unknown Origin, it may also lead to a tough conversation about oncologic conditions. Let’s take a minute to review Priapism in Children:

 

Priapism: Basic

  • Priapism is prolonged penile erection without sexual stimulation. [McGrath, 2011]
  • Priapism is caused by an imbalance of penile blood inflow and outflow.
  • Priapism is classified into two categories:
    • Low-Flow / Ischemic
      • Most common type
        • Usually related to poor venous blood flow out of the penis
        • TRUE Emergency
          • Essentially compartment syndrome of the penis
          • Little or no intracorporal blood flow (hence “low-flow”)
      • Characteristics: [Hazra, 2013]
        • Painful
        • Rigid penis
        • Blood sample results:
          • Low pH (< 7.25)
          • Hypercarbia (> 60 mmHg; some references say > 90)
          • Hypoxia (< 30 mmHg)
    • High-Flow / Non-ischemic
      • Usually related to trauma
      • Increased arterial inflow overwhelms venous outflow. [Hazra, 2013]
      • Less worrisome acutely as there is continued arterial blood flow
      • Characteristics: [Hazra, 2013]
        • Can be painless
        • Penis is not completely rigid
        • Blood sample results:
          • Normal pH (> 7.40)
          • Normal CO2 (< 40 mmHg)
          • Normal O2 levels (> 90 mmHg)
          • BRIGHT RED Oxygenated Blood
    • 3rd type Described as “Recurring” or “Stuttering” [Park, 2015]
      • Variant of ischemic priapism
      • More likely in patients with sickle cell disease.
  • Prolonged or recurrent priapism is associated with: [McGrath, 2011]
    • Sexual dysfunction
    • Cosmetic abnormalities (from fibrosis)

 

Priapism: Considerations for Children

  • Low-Flow Priapism [Jacobs, 2015; Hazra, 2013]
    • Sickle Cell Disease
      • Most common discovered cause (>60%) in children [Park, 2015]
    • Leukemia
      • May be the first manifestation of a leukemia, although rare [Hazra, 2013]
        • Accounts for ~15% of priapism cases in children
        • Chronic myeloid leukemia is most common oncologic cause
        • Acute myeloblastic and lymphoblastic leukemia also noted as causes
      • Likely related to hyper-leukocytosis leading to slow flow from leukemic cell aggregation.
      • Can also lead to central nerve system infiltration and subsequent sacral nerve dysfunction.
    • Adverse Drug Reactions
      • Antidepressants [Park, 2015]
        • Trazodone
        • SSRIs
        • TCAs
      • Antipsychotics
        • Risperidone
        • Phenothiazines
      • Anticoagulants
      • Antihypertensives
      • Illicit substances (ex, cocaine, marijuana)
    • Hypercoagulable States
      • Mycoplasma pneumoniae infection has been reported [Jacobs, 2015]
  • High-Flow Priapism [Jacobs, 2015; Hazra, 2013]
    • Severe, blunt, perineal Trauma, but… may also be less “noticeable” trauma [De Rose, 2017]
      • Can present in delayed fashion (24-48 hours after trauma)
      • Often initiated by sexual stimulation.
    • Recent procedure may injure dorsal vessels. [Granieri,2016]
  • Delays in seeking care may occur and lead to increased complication risk:
    • Embarrassment may cause child to avoid informing anyone
    • High-Flow priapism may be less painful, so more easily avoided.

 

Priapism: Management

  • History and Physical (goes without saying)
    • New meds?
    • History of Sickle Cell Disease
    • Unexplained fevers or weight loss?
    • Prior episodes of priapism
  • Labs:
    • CBC with differential
      • Especially for Sickle Cell disease or if concern for leukemia
    • Corporal Blood Gas Sample
      • A blood aspirated from the corpus cavernous can be placed in a heparinized syringe and sent for analysis. [McGrath, 2011]
      • Helps discern high-flow from low-flow.
      • High-Flow (non-ischemic) priapism generally resolves spontaneously and rarely requires intervention. [McGrath, 2011]
  • Pain management:
    • Consider dorsal penile nerve block
    • Parenteral analgesics and sedatives may be needed
      • Interestingly, some reports of Ketamine being effective at relieving low-flow priapism [Park, 2015]
        • May be property of the medication, or may be effect of dissociative sedation.
  • Treat possible underlying etiology
    • ex: Sickle Cell Disease may benefit from:
      • Hydration
      • Blood Transfusion (to increase portion of Adult vs Sickle Hemoglobin)
      • Plasmapheresis
  • Corpus Cavernous Aspiration
    • Some (~30%) will only require reduction of pressure from simple aspiration. [McGrath, 2011]
    • Send the sample for blood gas testing
    • Use 19 to 21 gauge needle / butterfly needle or angiocatheter
    • (See EMin5 on YouTube)
  • Instillation of alpha sympathomimetic
    • Phenylephrine is a pure alpha agent
    • Instill 1 mL of dilute phenylephrine (100-500 micrograms per milliliter of normal saline) into the corpus cavernous. [McGrath, 2011]
    • Smaller volumes may need to be used for younger children.
  • Call Some Friends
    • Even if you are successful at resolving the priapism.
    • Consult Urology
      • May require surgical options (ex, Shunts) if unsuccessful.
    • Consult Hematology for those with Sickle Cell Disease or concern for oncologic process.

 

Moral of the Morsel

  • History is important! It may help you discern the cause as well as the type of priapism.
  • Pain control is also important! Consider ketamine … like always!
  • It isn’t all due to sickle cell disease. Is it Leukemia?

 

References

De Rose AF1, Paraboschi I2, Mantica G1,3, Szpytko A3, Ackermann H3, De Caro G4, Terrone C1, Mattioli G2. Cycling Trauma as a Cause of Arterial Priapism in Children and Teenagers. Rev Urol. 2017;19(4):273-277. PMID: 29472833. [PubMed] [Read by QxMD]

Kuwano AY1, Cavalcante A1, Costa-Matos A1, Spanholi EF1, Mascarenhas de Souza FM1. Management in Neonatal Priapism: Case and Review. Urol Case Rep. 2017 Jul 18;14:48-49. PMID: 28752068. [PubMed] [Read by QxMD]

Wang HH1, Herbst KW2, Rothman JA3, Shah NR4, Wiener JS5, Routh JC6. Trends in Sickle Cell Disease-related Priapism in U.S. Children’s Hospitals. Urology. 2016 Mar;89:118-22. PMID: 26674747. [PubMed] [Read by QxMD]

Granieri MA1, Fantony JJ1, Routh JC1. High Flow Priapism in a Pediatric Patient after Circumcision with Dorsal Penile Nerve Block. Case Rep Pediatr. 2016;2016:6976439. PMID: 27648333. [PubMed] [Read by QxMD]

Park DB1, Hayden GE. Ketamine Saves the Day: Priapism in a Pediatric Psychiatric Patient. Pediatr Emerg Care. 2015 Jul;31(7):508-10. PMID: 26148100. [PubMed] [Read by QxMD]

Jacobs M1, Lo MD, Lendvay TS. Painless pediatric priapism and cough. Pediatr Emerg Care. 2015 Jan;31(1):36-8. PMID: 25285386. [PubMed] [Read by QxMD]

Armstrong WR1, Grimsby GM2, Jacobs MA3. Pediatric Priapism Secondary to Psychotherapeutic Medications. Urology. 2015 Aug;86(2):376-8. PMID: 26199163. [PubMed] [Read by QxMD]

Sekerci CA1, Akbal C1, Sener TE1, Sahan A1, Sahin B1, Baltacioglu F2, Simsek F1. Resistant pediatric priapism: A real challenge for the urologist. Can Urol Assoc J. 2015 Jul-Aug;9(7-8):E562-4. PMID: 26609335. [PubMed] [Read by QxMD]

Eiland LS1, Bell EA2, Erramouspe J3. Priapism associated with the use of stimulant medications and atomoxetine for attention-deficit/hyperactivity disorder in children. Ann Pharmacother. 2014 Oct;48(10):1350-5. PMID: 24982313. [PubMed] [Read by QxMD]

Hazra SP1, Priyadarshi V1, Gogoi D1, Sharma PK1, Pal DK1, Chakraborty SC1. Pediatric priapism: a rare first manifestation of leukemia. APSP J Case Rep. 2013 Oct 18;4(3):39. PMID: 24381835. [PubMed] [Read by QxMD]

McGrath NA1, Howell JM, Davis JE. Pediatric genitourinary emergencies. Emerg Med Clin North Am. 2011 Aug;29(3):655-66. PMID: 21782080. [PubMed] [Read by QxMD]

Sean M. Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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