- Email: [email protected]
Intracavernosal etilefrine self-injection therapy for recurrent priapism: One decade of follow-up
CASE REPORT
INTRACAVERNOSAL ETILEFRINE SELF-INJECTION THERAPY FOR RECURRENT PRIAPISM: ONE DECADE OF FOLLOW-UP CLAUDIO TELOKEN, EDUARDO PORTO RIBEIRO, MÁRIO CHAMMAS, JR, PATRICK E. TELOKEN, AND CARLOS ARY VARGAS SOUTO
ABSTRACT Recurrent idiopathic priapism is a rare condition that, if not properly treated, may lead to impaired quality of life and erectile dysfunction. Treatment can be achieved by prevention of priapism episodes with systemic therapy or by early intervention with intracavernosal self-injection of sympathomimetic agents. We describe a case of a young patient with recurrent idiopathic priapism who has used etilefrine self-injection for the past 10 years with good efficacy and libido and erectile function preservation. This report suggests that this approach may be safely indicated in selected cases, particularly when sexual function preservation is a major concern. UROLOGY 65: 1002.e22–1002.e23, 2005. © 2005 Elsevier Inc.
P
riapism is a pathologic condition of a persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation.1 Ischemic priapism is a urologic emergency that when not properly managed is associated with progressive fibrosis of the cavernosal tissues and erectile dysfunction.2 Stuttering (intermittent) priapism is a recurrent form of ischemic priapism in which unwanted painful erections occur repeatedly with intervening periods of detumescence.1 Although in children this condition is related to trauma and hemoglobinopathies such as sickle cell disease, in adults it is commonly idiopathic.1 Treatment has traditionally been composed of the administration of systemic therapy, such as sympathomimetic amines (oral etilefrine,3–5 terbutaline,6 baclofen7 and gabapentin8), antiandrogens,9,10 and luteinizing hormone-releasing hormone agonists11,12 or intracavernosal (IC) selfinjection of sympathomimetic amines.3,5,12,13 We report a case of stuttering priapism that has been successfully treated with IC etilefrine self-injection for the past 10 years. From the Division of Urology, Department of Surgery, Federal Foundation of Medical Sciences of Porto Alegre, Porto Alegre, Brazil Reprint requests: Claudio Teloken, M.D., Division of Urology, Department of Surgery, Federal Foundation of Medical Sciences of Porto Alegre, Porto Alegre, RS 90480-003, Brazil. E-mail: [email protected] Submitted: August 11, 2004, accepted (with revisions): December 1, 2004 © 2005 ELSEVIER INC. 1002.e22
ALL RIGHTS RESERVED
CASE REPORT A 27-year-old white man presented with a 1-year history of prolonged painful erections. These priapism episodes usually occurred after sexual intercourse, erotic stimulation, or even spontaneous nocturnal erections. It happened as often as three to four times a week, lasting from 30 minutes to 8 hours. His family and past medical history were unremarkable. He denied any use of illicit drugs or previous perineal or penile trauma. The physical examination and hematologic evaluation were normal, and selective pudendal arteriography excluded arteriovenous fistula. Preventive use of oral terbutaline 15 mg/ day was unsuccessful, and three times, emergent corporal drainage and irrigation with etilefrine 5 mg diluted in 500 mL plain saline was needed. The couple was not willing to accept any kind of treatment that could decrease his libido or jeopardize his erectile ability. IC self-injection of etilefrine 5 mg was then proposed, and, after a short training period, the patient was able to manage the priapism episodes. He administers the drug 1 hour after a spontaneous erection and repeats the injection every 15 minutes until detumescence is achieved. Since then, he has not needed to return to the emergency room for cavernous drainage. The penile shaft examination has been unremarkable. The patient has been sexually active and does not have erectile dysfunction. 0090-4295/05/$30.00 doi:10.1016/j.urology.2004.12.003
COMMENT Recurrent idiopathic priapism is a rare condition that, if not properly treated, may lead to impaired quality of life and erectile dysfunction. Treatment can be achieved by prevention of the priapism episodes with systemic therapy or by early intervention with IC self-injection of sympathomimetic agents.2 The use of antiandrogens or gonadotropin-releasing hormone agonists, which suppress nocturnal penile erections, can be effective in preventing priapism recurrence.10 However, these therapies may decrease the patient’s libido and have a contraceptive effect. For these reasons, alpha-adrenergic self-injection is considered a better option for sexually active young patients. Sympathomimetic amines have been used for the treatment of acute priapism attacks since 1986, shortly after the introduction of IC injection of papaverine and phentolamine for the diagnosis and treatment of erectile dysfunction.3,5,13,14 Self-injection protocols are well suited to allow fast management of the acute priapism episodes, decreasing the chance of corporeal damage.5 Etilefrine is an alpha1-selective agonist and, when used as an IC injection, has minimal cardiovascular adverse effects.5 Although its short-term efficacy and safety have been reported by several groups, a concern has been raised that with longer follow-up, side effects such as hypertension, erectile dysfunction, and scarring at the site of the injection may become apparent.3–5 The American Urological Association recently recommended that IC self-injection of sympathomimetic amines should not be considered preferred over systemic therapies, because priapism in such cases is being treated rather than prevented and the potential exists for adverse effects of inadvertent systemic administration of sympathomimetics.1 To our knowledge, this is the first report of such long-term follow-up of the early IC etilefrine self-injection approach, suggesting
UROLOGY 65 (5), 2005
that it may be safely indicated in selected cases, particularly when sexual function preservation is a major concern. REFERENCES 1. Montague DK, Jarow J, Broderick GA, et al: American Urological Association guideline on the management of priapism. J Urol 170: 1318 –1324, 2003. 2. Levine JF, Saenz de Tejada I, Payton TR, et al: Recurrent prolonged erections and priapism as a sequela of priapism: pathophysiology and management. J Urol 145: 764 –767, 1991. 3. Gbadoe AD, Atakouma Y, Kusiaku K, et al: Management of sickle cell priapism with etilefrine. Arch Dis Child 85: 52–53, 2001. 4. Okpala I, Westerdale N, Jegede T, et al: Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 118: 918 –921, 2002. 5. Virag R, Bachir D, Lee K, et al: Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine. Urology 47: 777–781, 1996. 6. Ahmed I, and Shaikh NA: Treatment of intermittent idiopathic priapism with oral terbutaline. Br J Urol 80: 341, 1997. 7. Rourke KF, Fischler AH, and Jordan GH: Treatment of recurrent idiopathic priapism with oral baclofen. J Urol 168: 2552–2553, 2002. 8. Perimenis P, Athanasopoulos A, Papathanasopoulos P, et al: Gabapentin in the management of the recurrent, refractory, idiopathic priapism. Int J Impot Res 16: 84 – 85, 2004. 9. Costabile RA: Successful treatment of stutter priapism with an antiandrogen. Tech Urol 4: 167–168, 1998. 10. Dahm P, Rao DS, and Donatucci CF: Antiandrogens in the treatment of priapism. Urology 59: 138, 2002. 11. Levine LA, and Guss SP: Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia-associated priapism. J Urol 150: 475– 477, 1993. 12. Steinberg J, and Eyre RC: Management of recurrent priapism with epinephrine self-injection and gonadotropinreleasing hormone analogue. J Urol 153: 152–153, 1995. 13. van Driel MF, Joosten EA, and Mensink HJ: Intracorporeal self-injection with epinephrine as treatment for idiopathic recurrent priapism. Eur Urol 17: 95–96, 1990. 14. Lue TF, Hellstrom WJ, McAninch JW, et al: Priapism: a refined approach to diagnosis and treatment. J Urol 136: 104 – 108, 1986.
1002.e23
INTRACAVERNOSAL ETILEFRINE SELF-INJECTION THERAPY FOR RECURRENT PRIAPISM: ONE DECADE OF FOLLOW-UP CLAUDIO TELOKEN, EDUARDO PORTO RIBEIRO, MÁRIO CHAMMAS, JR, PATRICK E. TELOKEN, AND CARLOS ARY VARGAS SOUTO
ABSTRACT Recurrent idiopathic priapism is a rare condition that, if not properly treated, may lead to impaired quality of life and erectile dysfunction. Treatment can be achieved by prevention of priapism episodes with systemic therapy or by early intervention with intracavernosal self-injection of sympathomimetic agents. We describe a case of a young patient with recurrent idiopathic priapism who has used etilefrine self-injection for the past 10 years with good efficacy and libido and erectile function preservation. This report suggests that this approach may be safely indicated in selected cases, particularly when sexual function preservation is a major concern. UROLOGY 65: 1002.e22–1002.e23, 2005. © 2005 Elsevier Inc.
P
riapism is a pathologic condition of a persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation.1 Ischemic priapism is a urologic emergency that when not properly managed is associated with progressive fibrosis of the cavernosal tissues and erectile dysfunction.2 Stuttering (intermittent) priapism is a recurrent form of ischemic priapism in which unwanted painful erections occur repeatedly with intervening periods of detumescence.1 Although in children this condition is related to trauma and hemoglobinopathies such as sickle cell disease, in adults it is commonly idiopathic.1 Treatment has traditionally been composed of the administration of systemic therapy, such as sympathomimetic amines (oral etilefrine,3–5 terbutaline,6 baclofen7 and gabapentin8), antiandrogens,9,10 and luteinizing hormone-releasing hormone agonists11,12 or intracavernosal (IC) selfinjection of sympathomimetic amines.3,5,12,13 We report a case of stuttering priapism that has been successfully treated with IC etilefrine self-injection for the past 10 years. From the Division of Urology, Department of Surgery, Federal Foundation of Medical Sciences of Porto Alegre, Porto Alegre, Brazil Reprint requests: Claudio Teloken, M.D., Division of Urology, Department of Surgery, Federal Foundation of Medical Sciences of Porto Alegre, Porto Alegre, RS 90480-003, Brazil. E-mail: [email protected] Submitted: August 11, 2004, accepted (with revisions): December 1, 2004 © 2005 ELSEVIER INC. 1002.e22
ALL RIGHTS RESERVED
CASE REPORT A 27-year-old white man presented with a 1-year history of prolonged painful erections. These priapism episodes usually occurred after sexual intercourse, erotic stimulation, or even spontaneous nocturnal erections. It happened as often as three to four times a week, lasting from 30 minutes to 8 hours. His family and past medical history were unremarkable. He denied any use of illicit drugs or previous perineal or penile trauma. The physical examination and hematologic evaluation were normal, and selective pudendal arteriography excluded arteriovenous fistula. Preventive use of oral terbutaline 15 mg/ day was unsuccessful, and three times, emergent corporal drainage and irrigation with etilefrine 5 mg diluted in 500 mL plain saline was needed. The couple was not willing to accept any kind of treatment that could decrease his libido or jeopardize his erectile ability. IC self-injection of etilefrine 5 mg was then proposed, and, after a short training period, the patient was able to manage the priapism episodes. He administers the drug 1 hour after a spontaneous erection and repeats the injection every 15 minutes until detumescence is achieved. Since then, he has not needed to return to the emergency room for cavernous drainage. The penile shaft examination has been unremarkable. The patient has been sexually active and does not have erectile dysfunction. 0090-4295/05/$30.00 doi:10.1016/j.urology.2004.12.003
COMMENT Recurrent idiopathic priapism is a rare condition that, if not properly treated, may lead to impaired quality of life and erectile dysfunction. Treatment can be achieved by prevention of the priapism episodes with systemic therapy or by early intervention with IC self-injection of sympathomimetic agents.2 The use of antiandrogens or gonadotropin-releasing hormone agonists, which suppress nocturnal penile erections, can be effective in preventing priapism recurrence.10 However, these therapies may decrease the patient’s libido and have a contraceptive effect. For these reasons, alpha-adrenergic self-injection is considered a better option for sexually active young patients. Sympathomimetic amines have been used for the treatment of acute priapism attacks since 1986, shortly after the introduction of IC injection of papaverine and phentolamine for the diagnosis and treatment of erectile dysfunction.3,5,13,14 Self-injection protocols are well suited to allow fast management of the acute priapism episodes, decreasing the chance of corporeal damage.5 Etilefrine is an alpha1-selective agonist and, when used as an IC injection, has minimal cardiovascular adverse effects.5 Although its short-term efficacy and safety have been reported by several groups, a concern has been raised that with longer follow-up, side effects such as hypertension, erectile dysfunction, and scarring at the site of the injection may become apparent.3–5 The American Urological Association recently recommended that IC self-injection of sympathomimetic amines should not be considered preferred over systemic therapies, because priapism in such cases is being treated rather than prevented and the potential exists for adverse effects of inadvertent systemic administration of sympathomimetics.1 To our knowledge, this is the first report of such long-term follow-up of the early IC etilefrine self-injection approach, suggesting
UROLOGY 65 (5), 2005
that it may be safely indicated in selected cases, particularly when sexual function preservation is a major concern. REFERENCES 1. Montague DK, Jarow J, Broderick GA, et al: American Urological Association guideline on the management of priapism. J Urol 170: 1318 –1324, 2003. 2. Levine JF, Saenz de Tejada I, Payton TR, et al: Recurrent prolonged erections and priapism as a sequela of priapism: pathophysiology and management. J Urol 145: 764 –767, 1991. 3. Gbadoe AD, Atakouma Y, Kusiaku K, et al: Management of sickle cell priapism with etilefrine. Arch Dis Child 85: 52–53, 2001. 4. Okpala I, Westerdale N, Jegede T, et al: Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 118: 918 –921, 2002. 5. Virag R, Bachir D, Lee K, et al: Preventive treatment of priapism in sickle cell disease with oral and self-administered intracavernous injection of etilefrine. Urology 47: 777–781, 1996. 6. Ahmed I, and Shaikh NA: Treatment of intermittent idiopathic priapism with oral terbutaline. Br J Urol 80: 341, 1997. 7. Rourke KF, Fischler AH, and Jordan GH: Treatment of recurrent idiopathic priapism with oral baclofen. J Urol 168: 2552–2553, 2002. 8. Perimenis P, Athanasopoulos A, Papathanasopoulos P, et al: Gabapentin in the management of the recurrent, refractory, idiopathic priapism. Int J Impot Res 16: 84 – 85, 2004. 9. Costabile RA: Successful treatment of stutter priapism with an antiandrogen. Tech Urol 4: 167–168, 1998. 10. Dahm P, Rao DS, and Donatucci CF: Antiandrogens in the treatment of priapism. Urology 59: 138, 2002. 11. Levine LA, and Guss SP: Gonadotropin-releasing hormone analogues in the treatment of sickle cell anemia-associated priapism. J Urol 150: 475– 477, 1993. 12. Steinberg J, and Eyre RC: Management of recurrent priapism with epinephrine self-injection and gonadotropinreleasing hormone analogue. J Urol 153: 152–153, 1995. 13. van Driel MF, Joosten EA, and Mensink HJ: Intracorporeal self-injection with epinephrine as treatment for idiopathic recurrent priapism. Eur Urol 17: 95–96, 1990. 14. Lue TF, Hellstrom WJ, McAninch JW, et al: Priapism: a refined approach to diagnosis and treatment. J Urol 136: 104 – 108, 1986.
1002.e23