Although rare in pediatrics, ischemic priapism carries significant risk of erectile dysfunction if not treated promptly. We report a case of idiopathic ischemic priapism in a male aged 14 years, refractory to traditional therapies, including aspiration/irrigation, phenylephrine injection, and distal shunt. However, the priapism resolved after intracavernosal injections of tissue plasminogen activator (tPA) with preservation of normal erectile function. To our knowledge, this is the first reported case of intracavernosal tPA for treatment of pediatric priapism. tPA may be a useful management option for recalcitrant ischemic priapism in pediatric patients and may prevent devastating outcomes such as lifelong erectile dysfunction.
Priapism is defined as a persistent erection lasting >4 hours in the absence of sexual stimulation. In childhood, 65% of episodes occur in association with sickle cell disease. Less common causes include leukemia, trauma, and medications, and ∼10% of cases are idiopathic.1 Priapism has an estimated incidence rate of 1.5 per 100 000 person-years across males of all ages.2 Although priapism is uncommon in childhood, 1 survey of pediatric patients with sickle cell disease reported that 27.5% admitted to at least 1 episode of priapism.3 Given the paucity of data regarding treatment of pediatric priapism, most management in children is on the basis of adult practices. Priapism can be classified as either ischemic or nonischemic (also known as “high flow” priapism and most commonly caused by trauma) on the basis of cavernosal blood gas values. Additionally, the erection is typically painful in cases of ischemia. This is a crucial distinction, because ischemic priapism requires emergent intervention, whereas high-flow, nonischemic priapism can usually be managed conservatively. This is because prolonged ischemia induces later fibrosis in erectile tissues that can result in permanent erectile dysfunction. Treatment approaches progress in a stepwise manner from conservative to more invasive, from corporal aspiration and lavage, injection of sympathomimetics, to various surgical shunts and implantation of penile prosthesis as a last resort.1,4 We report a case of idiopathic ischemic priapism in a male aged 14 that was refractory to traditional therapies but resolved after treatment with intracavernosal injections of tissue plasminogen activator (tPA).
Case History
A 14-year-old White male with history of asthma, environmental allergies, and attention-deficit/hyperactivity disorder treated with methylphenidate presented to the emergency department with a painful erection present for 28 hours. Presentation was delayed given his reluctance to reveal symptoms to caregivers. He denied penile trauma, drug use, or previous similar episodes. There was no family history of hemoglobinopathy or blood disorders. His mother had history of deep venous thrombosis, with reported negative workup for underlying constitutional coagulation disorder. The deep venous thrombosis was attributed to a combination of oral contraceptives and smoking. The patient and his parent gave informed consent for his case to be published.
Complete blood count and urine drug screen were unremarkable. The patient did not have known history of coronavirus disease 2019 infection and nasopharyngeal polymerase chain reaction was negative. On exam, the patient was Tanner stage 4 with fully rigid phallus and tender corporal bodies suggestive of ischemic priapism. Corporal blood gas demonstrated ischemic priapism, prompting bedside aspiration/irrigation with injection of phenylephrine (interventions outlined in Fig 1). Given no improvement in penile rigidity after 45 minutes, he was taken emergently to the operating room. Epidural anesthesia (EA) was induced with ropivacaine, without improvement in the erection. A 10-French Foley catheter was inserted. A modified Winter’s shunt was performed using an 18-gauge needle inserted through the glans into each corpora in multiple places until adequate cavernoglandular communication was achieved, resulting in complete detumescence.
However, the priapism recurred in the postanesthesia care unit when EA was still effective. Although EA has been used to treat priapism, it has also been reported to induce priapism, though this is quite rare.5 Repeat aspiration/irrigation was performed without detumescence. After sedation in the postanesthesia care unit and with continued EA, a larger distal shunt was created utilizing a No. 11 blade scalpel. The patient was admitted to the PICU for monitoring and procedural sedation. Aspiration/irrigation and phenylephrine injection on postoperative day (POD) 1 was minimally successful. Penile duplex ultrasound demonstrated inflow of corporal arterial blood, but no flow within the deep dorsal vein (Fig 2). Al-Ghorab (larger distal shunt) or proximal shunting was discussed with the family, but given the prolonged duration of priapism and the high risk of erectile dysfunction with these interventions, the family declined. Hematology was consulted, and the patient was initiated on therapeutic anticoagulation with infusion of unfractionated heparin after removal of his epidural catheter. Because of the high risk of organ-damaging ischemia with the prolonged priapism, we proceeded with intracorporal injection of tPA (6 mg/2.5 mL saline per side), similar to the procedure described by Rutchik et al in an adult patient, the only previous reported case of this technique.6 Subsequent corporal blood gas (∼21 hours later) showed improved oxygenation. In the setting of multiple procedures requiring aspiration of blood from the penis and prolonged oozing from shunt and aspiration sites while anticoagulated, the patient required transfusion of a total of 6 units packed red blood cells over a 4-day period. The heparin infusion was stopped, and he remained hemodynamically stable. Between frequent physical examinations, a compressive dressing was applied to the penis to minimize bleeding.
On POD 2, the patient’s priapism improved. On repeat aspiration/irrigation, blood was easily drawn back from the proximal corpora, but not distally, likely reflecting persistent clotted blood occluding the distal corpora. The same dose of tPA was again injected, focusing on the distal corpora. By POD 5, both corporal bodies remained soft with no bleeding. The patient was transferred out of the PICU, had no further priapism recurrence, and was discharged on POD 7 with aspirin 325 mg daily for 1 week.
Extensive hypercoagulability workup (during the initial presentation with repeat testing of nongenetic markers 2 months later) was not suggestive of underlying blood clotting disorder that would explain the recalcitrant priapism. Testing included complete blood count with smear and differential analysis, prothrombin time, partial thromboplastin time, antithrombin III activity, protein C and S activity, protein S free antigen, lupus anticoagulant, lipoprotein A, cardiolipin immunoglobulin G, β-2-glycoprotein immunoglobulin G and immunoglobulin M, von Willebrand panel, Factor V Leiden mutation analysis, and serum homocysteine level. The patient’s newborn screen was reviewed and negative for hemoglobinopathies, including sickle cell trait.
Within 1 week of discharge, the patient reported nonpainful erections. After 2 weeks, his erections had returned to normal entirely. Penile Doppler ultrasound 3 months later was normal (Fig 2), and he reported continued excellent erectile function.
Discussion
Priapism is defined as a persistent erection lasting >4 hours in the absence of sexual stimulation. Although rare in pediatric patients, ischemic priapism carries a significant risk of permanent erectile dysfunction if not treated promptly.7 In this case, after nonoperative measures including epidural anesthesia were not successful,4,8–10 we proceeded with a modified Winter’s shunt.11 This technique uses a smaller (18-gauge) needle for creation of fistulous communication between the corpora cavernosa and glans rather than a biopsy needle used in a traditional Winter’s shunt. The smaller fistula is thought to reduce the risk of future erectile dysfunction secondary to persistent venous leak, particularly given the disproportionately large size of tissue cored out in a child compared with an adult. In Raveenthiran’s experience using this procedure in 7 patients, only 1 had immediate recurrence.11 Notably, the duration of priapism at presentation in this cohort (range 5–20 hours) was shorter than the 28 hours reported by our patient. Given that priapism recurred immediately after the modified Winter’s shunt, we performed a distal shunt creation using an 11-blade scalpel without success. A proximal shunt could also have been considered, although these carry higher rates of complications and erectile dysfunction.1
Tissue plasminogen activator is a thrombolytic agent most commonly used for treatment of acute ischemic stroke. It is a serine protease that stimulates fibrinolysis by catalyzing the conversion of plasminogen to plasmin. Though thrombosis is not traditionally considered a significant contributor to ischemic priapism, success has been reported with 1 previous case of thrombolytic therapy for priapism unresponsive to conventional therapies.6,12,13 Rutchik et al described using tPA to reverse risperidone-induced priapism in a 35-year-old that was refractory to corporal irrigation, phenylephrine, and Al-Ghorab shunt. However, by 15 minutes after intracavernosal injection of 15 mg of tPA, 80% detumescence had occurred.6 Subsequent bleeding from the previous shunt site was managed with a figure-of-eight suture and pressure dressing. Erectile function outcomes were not reported.
Our case was particularly challenging given the prolonged duration of erection before presentation and the subsequent failure of multiple traditional therapies, including aspiration/irrigation, phenylephrine injection, and distal shunt. The patient’s course was significant for a weeklong hospitalization (including PICU care), transfusion of 6 U packed red blood cells, and numerous procedures: multiple rounds of aspiration/irrigation, intracavernosal phenylephrine, distal shunt creation, and intracavernosal tPA injection. We believe that tPA coupled with aggressive bedside aspiration/irrigation helped resolve clot formed because of prolonged priapism, relieving the ischemia. Though the patient was shunted, we used injection of tPA in the bilateral corpora cavernosa to maximize the shunt’s action. Although in theory, tPA could be effective when combined with aspiration/irrigation and in absence of a shunt, at this point, we would not use tPA before standard priapism treatments (ie, distal shunt) given more experience with these time-tested procedures and increased risk of bleeding with tPA, as evidenced by the patient’s high transfusion requirement. Within hours after the first injection, the corporal bodies became less rigid and cavernosal blood gases improved. Despite the prolonged ischemic priapism and multiple procedures, the patient had return of normal erections within days after discharge. He confirmed normal erectile function at follow-up 3 months later.
Conclusions
We conclude that tPA may be a useful additional management option for prolonged ischemic priapism in pediatric patients and should be considered in recalcitrant cases that do not respond to conventional therapies. In priapism refractory to distal shunt creation, early tPA injection might prevent the need for proximal shunting and minimize the risk of subsequent erectile dysfunction. Future studies and long-term follow-up will help delineate the appropriate role for this therapy.
Dr Otto was affiliated with the University of Wisconsin at the time of the case and is currently affiliated with the Center for Cancer and Blood Disorders at Phoenix Children’s Hospital in Phoenix, Arizona.
Dr Sherrer conceptualized the report and drafted the initial manuscript; Drs Otto, Srinivasan, Grinde, and Farhat conceptualized the report; and all authors critically reviewed and revised the manuscript, approved the final manuscript as submitted, and agree to be accountable for all aspects of the work.
FUNDING: No external funding.
CONFLICT OF INTEREST DISCLAIMER: The authors have indicated that there are no potential conflicts of interest to disclose.
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