Is urgent surgical management necessary for priapism in pediatric patients with hematologic conditions?

Patel, Sagar; Reddy, Amit; Dai, Michelle; Passoni, Niccolò; Khera, Mohit; Koh, Chester J.

doi:10.1016/j.jpurol.2022.06.028

Cited by 2 publications

(1 citation statement)

References 17 publications

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“…Ischemic priapism of prolonged duration (usually longer than 48 hours) is associated with a higher risk of erectile dysfunction (ED), corporal fibrosis with penile deformity, penile gangrene, and psychological sequelae regardless of adequate treatment [2]. Whereas the management of prolonged ischemic priapism in adults follows well-established guidelines, consensus on such guidelines is yet to be established for priapism in children [2,4].…”

Section: Introductionmentioning

confidence: 99%

Prolonged ischemic priapism in an adolescent with sickle cell anemia: Challenges of management

Aja Obasi,

Sunday Omebe

2024

J Case Rep Images Surg

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Introduction: Persistent penile erection lasting more than 4 hours and unrelated to sexual interest or stimulation is called priapism. Priapism is uncommon in children. Of the three widely accepted types: ischemic, non-ischemic, and stuttering priapism; ischemic priapism is the predominant type seen in children. A common cause of ischemic priapism in children is sickle cell disease. Ischemic priapism of prolonged duration is associated with a higher risk of complications especially irreversible loss of erectile function. It is a difficult clinical condition to manage and there are no established guidelines for its management in children. Adequate surgical treatment does not guarantee against the risk of devastating longterm sequelae. Case Report: We report a case of a 12-year-old boy with sickle cell anemia who presented after 48 hours of sustained painful penile erection. He required multiple surgical shunting procedures including distal corporoglanular shunt with tunneling of both corpora cavernosa with Hegar’s dilator to achieve detumescence. The management challenges encountered are highlighted. Conclusion: A potential role for anticoagulation and the use of low-dose hydroxyurea in the prevention of recurrent ischemic priapism in children with sickle cell anemia are advocated.

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Section: Introductionmentioning

confidence: 99%

Prolonged ischemic priapism in an adolescent with sickle cell anemia: Challenges of management

Aja Obasi,

Sunday Omebe

2024

J Case Rep Images Surg

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Priapism at Diagnosis of Pediatric Chronic Myeloid Leukemia: Data Derived from a Large Cohort of Children and Teenagers and a Narrative Review on Priapism Management

Suttorp

Sembill

Kałwak

et al. 2023

JCM

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Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1/100,000) typically presenting with leucocyte counts >100,000/µL. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16/491 (3.2%) boys (median age 13.5 years, range 4–18) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months (n = 1), over 6 weeks (n = 1), over 1–2 weeks (n = 2), over several days (n = 2), or 24 h (n = 1), while the remaining 4 boys reported continuous erection lasting over 11–12 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000/µL, range 236,700–899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids ± heparin (n = 2), penile puncture (n = 5) ± injection of sympathomimetics (n = 4) ± intracavernous shunt operation (n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19–25) responded to a questionnaire. All had maintained full erectile function; however, 5/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted <12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients’ lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions ± cytotoxic chemotherapy.

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Is urgent surgical management necessary for priapism in pediatric patients with hematologic conditions?

Cited by 2 publications

References 17 publications

Prolonged ischemic priapism in an adolescent with sickle cell anemia: Challenges of management

Prolonged ischemic priapism in an adolescent with sickle cell anemia: Challenges of management

Priapism at Diagnosis of Pediatric Chronic Myeloid Leukemia: Data Derived from a Large Cohort of Children and Teenagers and a Narrative Review on Priapism Management

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