Priapism and Sickle-Cell Anemia: Diagnosis and Nonsurgical Therapy

Journal of Sexual Medicine

Volumn 9, Issue 1, 2012, Pages 88-103

  Broderick, Gregory A ^a  

^a MAYO CLINIC   (United States)

Author keywords

Ischemic Priapism; Priapism; Prolonged Erection; Sickle Cell Anemia; Stuttering Priapism

Indexed keywords

ADRENALIN; ADRENERGIC RECEPTOR STIMULATING AGENT; AMFEBUTAMONE; BACLOFEN; BICALUTAMIDE; CHLORMADINONE; CHLORPROMAZINE; CLOZAPINE; CYCLIC GMP; EPHEDRINE; ETILEFRINE; FINASTERIDE; FLUOXETINE; FLUTAMIDE; HYDRALAZINE; HYDROXYZINE; KETOCONAZOLE; METARAMINOL; NORADRENALIN; PHENOTHIAZINE DERIVATIVE; PHENYLEPHRINE; PHOSPHODIESTERASE V; PRAZOSIN; PSEUDOEPHEDRINE; SILDENAFIL; TADALAFIL; TERBUTALINE; TESTOSTERONE; TRAZODONE; UNINDEXED DRUG;

ARTICLE; BRADYCARDIA; CLINICAL PRACTICE; CRYOTHERAPY; DECOMPRESSION; DISEASE ASSOCIATION; DIZZINESS; DRUG DOSE ESCALATION; EJACULATION; EMERGENCY CARE; ERECTILE DYSFUNCTION; FIRST AID; HEADACHE; HEART ARRHYTHMIA; HEMODIALYSIS; HUMAN; HYPERALIMENTATION; HYPERTENSION; ISCHEMIA; ISCHEMIC PRIAPISM; LEUKEMIA; MULTIPLE MYELOMA; NEEDLE BIOPSY; NONHUMAN; NONISCHEMIC PRIAPISM; PATHOGENESIS; PELVIS INJURY; PENILE FIBROSIS; PENIS INJURY; PERINEUM INJURY; PHENOTYPE; PRACTICE GUIDELINE; PRIAPISM; PRIORITY JOURNAL; RISK BENEFIT ANALYSIS; SICKLE CELL ANEMIA; STUTTERING PRIAPISM; SUBARACHNOID HEMORRHAGE; TACHYCARDIA; THALASSEMIA; URINE RETENTION; VEIN OCCLUSION;

EID: 84855358440     PISSN: 17436095     EISSN: 17436109     Source Type: Journal    
DOI: 10.1111/j.1743-6109.2011.02317.x     Document Type: Article

References (111)

1. Montague DK, Jarow J, Broderick GA, Dmochowsky RR, Heaton JP, Lue TF, Nehra A, Sharlip ID, American Urological Association Erectile Dysfunction Guideline Update Panel. Urological association guideline on the management of priapism. J Urol 2003;170:1318-24.
2. Broderick GA, Kadioglu A, Bivalacqua TJ, Ghanem H, Nehra A, Shamloul R. Priapism: Pathogenesis, epidemiology and management. J Sex Med 2010;7:476-500.
3. Spycher MA, Hauri D. The ultastructure of the erectile tissue in priapism. J Urol 1986;135:142-7.
4. Serjeant GR, de Ceulaer K, Maude GH. Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 1985;2:1274-6.
5. Tripe JW. Case of continued priapism. Lancet 1845;2:8.
6. Hinman F. Priapism: Report of case in a clinical study of the literature with reference to its pathogenesis and surgical treatments. Ann Surg 1914;60:689-716.
7. Hinman F Jr. Priapism: Reasons for failure of therapy. J Urol 1960;83:420-8.
8. Hauri D, Spycher M, Brühlmann W. Erection and priapism: A new physiopathological concept. Urol Int 1983;38:138-41.
9. Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 1980;140:1437-7.
10. Pohl J, Pott B, Kleinhans G. Priapism: A three-phase concept of management according to etiology and prognosis. Br J Urol 1986;58:113-8.
11. Lue TF. Physiology of penile erection and pathophysiology of erectile dysfunction and priapism. In: Walsh PC, Retik AB, Vaughan ED, Wein AJ, Kavoussi AR, Novick AC, Partin AW, Peters CA, eds. Campbell's urology. Philadelphia, PA: WB Saunders; 2002:1610-96.
12. Nelson JH, Winter CC. Priapism: Evolution of management in 48 patients in a 22 year series. J Urol 1977;117:455-8.
13. Tarry WF, Duckett JW, Snyder H. Urological complications of sickle cell disease in a pediatric population. J Urol 1987;138:592-4.
14. Adeyoju AB, Olujohungbe AB, Morris J, Yardumian A, Bareford D, Akenova A, Akinyanju O, Cinkotai K, O'Reilly PH. Priapism in sickle-cell disease; incidence, risk factors and complications-An international multicentre study. BJU Int 2002;90:898-902.
15. Aliyu ZY, Kato GJ, Taylor J, Babadoko A, Mamman AI, Gordeuk VR, Gladwin MT. Sickle cell disease and pulmonary hypertension in Africa: A global perspective and review of epidemiology, pathophysiology, and management. Am J Hematol 2008;83:63-70.
16. Morris CR, Kato GJ, Pojakovic M, Wang X, Blackwelder WC, Sachdev V, Hazen SL, Vichinsky EP, Morris SM Jr, Gladwin MT. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension and mortality in sickle cell disease. JAMA 2005;294:81-90.
17. Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequela of intravascular hemolysis and extracellular plasma hemoglobin: A novel mechanism of human disease. JAMA 2005;293:1652-62.
18. Kato GJ, Gladwin MT, Steinber MH. Deconstructing sickle cell disease: Reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007;21:37-47.
19. Kato GJ, McGowan V, Machado RF, Little JA, Taylor J, Morris CR, Nichols JS, Wang X, Poljakovic M, Morris SM, Gladwin MT. Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patient with sickle cell disease. Blood 2006;107:2279-85.
20. Merritt AL, Haiman C, Henderson SO. Myth: Blood transfusion is effective for sickle cell anemia-associated priapism. CJEM 2006;8:119-22.
21. Birnbaum BF, Pinzone JJ. Sickle cell trait and priapism: A case report and review of the literature. Cases J 2008;1:429.
22. Larocque MA, Cosgrove MD. Priapism: A review of 46 cases. J Urol 1974;112:770-2.
23. Broderick GA, Lue TF. Evaluation and nonsurgical management of erectile dysfunction and priapism. In: Walsh PC, Retik AB, Vaughan ED, Wein AJ, Kavoussi AR, Novick AC, Partin AW, Peters CA, eds. Campbell's urology. Philadelphia, PA: WB Saunders; 2002:1619-71.
24. Junemann KP, Persson-Junemann C, Alken P. Pathophysiology of erectile dysfunction. Semin Urol 1990;8:80-93.
25. Porst H. The rational for prostaglandin E1 in erectile failure: A survey of worldwide experience. J Urol 1996;155:802-15.
26. Morales A, Gingell C, Collins M, Wicker PA, Osterloh IH. Clinical safety of oral sildenafil citrate (VIAGRA) in the treatment of erectile dysfunction. Int J Impot Res 1998;10:69-73.
27. Montorsi F, Verheyden B, Meuleman E, Jünemann KP, Moncada I, Valiquette L, Casabé A, Pacheco C, Denne J, Knight J, Segal S, Watkins VS. Long-term safety and tolerability of tadalafil in the treatment of erectile dysfunction. Eur Urol 2004;45:339-44.
28. McVary KT, Roehrborn CG, Kaminetsky JC, Auerbach SM, Wachs B, Young JM, Esler A, Sides GD, Denes BS. Tadalafil relieves lower urinary tract symptoms secondary to benign prostatic hyperplasia. J Urol 2007;177:1401-7.
29. Aoyagi T, Hayakawa K, Miyaji K, Ishikawa H, Hata M. Sildenafil induced priapism. Bull Tokyo Dent Coll 1999;40:215-7.
30. Sur RL, Kane CJ. Sildenafil citrate-associated priapism. Urology 2000;55:950.
31. Kassim AA, Fabry ME, Nagel RL. Acute priapism associated with the use of sildenafil in a patient with sickle cell trait. Letter to the editor. Blood 2000;95:1878-9.
32. Goldmeier D. Prolonged erection produced by dihydrocodeine and sildenafil. BMJ 2002;524:1555.
33. McMahon CG. Priapism associated with concurrent use of phosphodiesterase inhibitor drugs and intracavernous injection therapy. Int J Impot Res 2003;15:383-4.
34. Wilt TJ, Fink HA. Is antidepressant plus sildenafil a recipe for priapism? Postgrad Med 2004;116:11-2.
35. Galatti L, Fioraavanti A, Salvo F, Polimeni G, Giustini SE. Interaction between tadalafil and itraconazole. Ann Pharmacother 2005;39:200.
36. Kumar R, Jindal L, Seth A. Priapism following oral sildenafil abuse. Natl Med J India 2005;18:49.
37. King SH, Hallock M, Strote J, Wessells H. Tadalafil associated priapism. Urology 2005;66:432e15-8.
38. Wills B, Albinson C, Wahl M, Clifton J. Sildenafil citrate ingestion and prolonged priapism and tachycardia in a pediatric patient. Clin Toxicol 2007;45:798-800.
39. Chow K, Payne S. The pharmacological management of intermittent priapismic states. BJU Int 2008;102:1515-21.
40. de Jesus LE, Dekermacher S. Priapism in children: Review of pathophysiology and treatment. J Pediatr (Rio J) 2009;85:194-200.
41. Witt MA, Goldstein I, Saenz de Tejada I, Greenfield A, Krane RJ. Traumatic laceration of intracavernosal arteries: The pathophysiology of nonischemic, high flow arterial priapism. J Urol 1990;143:129-32.
42. Dubocq FM, Tefilli MV, Grignon DJ, Ponte JE, Dhabuwala CB. High flow malignant priapism with isolated metastasis to the corpora cavernosa. Urology 1998;51:324-6.
43. Brock G, Breza J, Lue TF, Tanagho EA. High flow priapism: A spectrum of disease. J Urol 1993;150:969-71.
44. Burgu B, Talas H, Erdeve O, Karagol BS, Fitoz S, Soygur TY. Approach to newborn priapism: A rare entity. J Pediatr Urol 2007;3:509-11.
45. Liguori G, Garaffa G, Trombetta C, Capone M, Bertolotto M, Pozzi-Mucelli F, Belgrano E. High-flow priapism secondary to Nesbit operation: Management by percutaneous embolization and colour Doppler guided compression. Int J Impot Res 2005;17:304-6.
46. Wolf JS, Lue TF. High flow priapism and glans hypervascularization following deep dorsal vein arterialization for vasculogenic impotence. Urol Int 1992;49:227-9.
47. McMahon CG. High flow priapism due to an arterial-sinusoidal fistula complicating initial veno-occlusive priapism. Int J Impot Res 2002;14:195-6.
48. Rodriguez J, Cuadrado JM, Frances A, Franco E. High flow priapism as a complication of veno-occlusive priapism. Two case reports. Int J Impot Res 2006;18:215-7.
49. Bertolotto M, Ciampalini S, Martingano P, Mucelli FP. High flow priapism complicating ischemic priapism following iatrogenic laceration of the dorsal artery during a Winter procedure. J Clin Ultrasound 2009;37:61-4.
50. Seftel A, Haas CA, Brown SL, Herbener TE, Sands M, Lipuma J. High flow priapism complicating veno-occlusive priapism: Pathophysiology of recurrent idiopathic priapism? J Urol 1998;159:1300-1.
51. Wallace CJ, Hoag N, Pommerville PJ, Huk ME. Recurrent idiopathic high-flow priapism treated with selective arterial embolization after repeated initial treatments for low-flow priapism. Can Urol Assoc J 2009;3:60-3.
52. Cruz Guerra NA, Pozo Mengual B, Perales Cespedes MP, Allona Almagro A, Saenz Medine J, Tarroc Blanco A. Metachronous recurrence of idiopathic high flow priapism. Arch Esp Urol 2004;57:82-4.
53. Ricciardi R, Bhatt GM, Cynamon J, Bakal CW, Melman A. Delayed high-flow priapism: Pathophysiology and management. J Urol 1993;149:119-21.
54. Leal J, Walker D, Egan EA. Idiopathic priapism in the newborn. J Urol 1978;120:376.
55. Shapiro SR. Idiopathic priapism in the newborn. J Urol 1979;121:838.
56. Amlie RN, Borgeois B, Huxtable R. Priapism in preterm infant. Urology 1977;9:558-9.
57. Walker JR, Casale AJ. Prolonged penile erection in the newborn. Urology 1997;50:796-9.
58. Meijer B, Bakker HHR. Management of priapism in the newborn. Urology 2003;61:224.
59. Pietras JR, Cromie WJ, Duckett JW. Ketamine as a detumescence agent during hypospadias repair. J Urol 1979;121:654.
60. Hatzichristou D, Salpiggidis G, Hatzimouratidis K, Apostolidis A, Tzortzis V, Bekos A, Saripoulos D. Management strategy for arterial priapism: Therapeutic dilemmas. J Urol 2002;168:2074-7.
61. Broderick GA, Gordon D, Hypolite J, Levin RM. Anoxia and corporal smooth muscle dysfunction: A model for ischemic priapism. J Urol 1994;151:259-62.
62. Broderick GA, Harkaway R. Pharmacologic erection: Time-dependent changes in the corporal environment. Int J Impot Res 1994;6:9-16.
63. Muneer A, Cellek S, Dogan A, Kell PD, Ralph DJ, Minhas S. Investigation of cavernosal smooth muscle dysfunction in low flow priapism using an in vitro model. Int J Impot Res 2005;17:10-8.
64. Saenz de Tejada I, Kim NN, Daley JT, Royai R, Hypolite J, Broderick GA, Garcia-Diaz F, Levin R. Acidosis impairs rabbit trabecular smooth muscle contractility. J Urol 1997;157:722-6.
65. Munarriz R, Park K, Huang YH, Saenz de Tejada I, Moreland RB, Goldstein I, Traish AM. Reperfusion of ischemic corporal tissue: Physiologic and biochemical changes in an animal model of ischemic priapism. Urology 2003;62:760-4.
66. Jin YC, Gam SC, Jung JH, Hyun JS, Chang KC, Hyun JS. Expression and activity of heme oxygenase-1 in artificially induced low-flow priapism in rat penile tissues. J Sex Med 2008;5:1876-82.
67. Moreland RB, Traish A, McMillin MA, Smith B, Goldstein I, Saenz de Tejada I. PGE1 suppresses the induction of collagen synthesis by transforming growth factor-beta 1 in human corpus cavernosum smooth muscle. J Urol 1995;153:826-34.
68. Bivalacqua TJ, Diner EK, Novak TE, Vohra Y, Sikka SC, Champion HC, Kadowitz PJ, Hellstrom WJ. A rat model of Peyronie's disease associated with a decrease in erectile activity and an increase in inducible nitric oxide synthase protein expression. J Urol 2000;163:1992-8.
69. Jeong WI, Do SH, Yun HS, Song BJ, Kim SJ, Kwak WJ, Yoo SE, Park HY, Jeong KS. Hypoxia potentiates transforming growth factor-beta expression of hepatocyte during the cirrhotic condition in rat liver. Liver Int 2004;24:658-68.
70. Bivalacqua TJ, Musicki B, Hsu LL, Gladwin MT, Burnett AL, Champion HC. Establishment of a transgenic sickle cell mouse model to study the pathophysiology of priapism. J Sex Med 2009;6:2494-504.
71. Champion HC, Bivalacqua TF, Takimoto E, Kass DA, Burnett AL. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 2005;102:1661-6.
72. Bivalacqua TJ, Burnett AL. Priapism: New concepts in the pathophysiology and new treatment strategies. Curr Urol Rep 2006;7:497-502.
73. Lin G, Xin ZC, Lue TF, Lin CS. Up and down-regulation of phosphodiesterase-5 as related to tachyphylaxis and priapism. J Urol 2003;170:S15-9.
74. Burnett AL, Bivalacqua TJ. Priapism: Current principles and practice. Urol Clin North Am 2007;34:631-42.
75. Burnett AL. Priapism. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA. Campbell-Walsh Urology. Philadelphia, PA: WB Saunders; 2007:839-49.
76. Kirkham A, Illing RO, Minahas S, Allen C. MR imaging of nonmalignant penile lesions. Radiographics 2008;28:837-53.
77. Ralph DJ, Garaffa G, Muneer A, Freeman A, Rees R, Christopher AN, Minhas S. The immediate insertion of a penile prosthesis for acute ischaemic priapism. Eur Urol 2009;56:1033-8.
78. Lowe FC, Jarow JP. Placebo-controlled study of oral terbutaline in management of prostaglandin E1 induced prolonged erections. J Urol 1993;42:51-3.
79. Ateyah A, Rahman El-Nashar A, Zohdy W, Arafa M, Saad El-Den H. Intracavernosal irrigation by cold saline as a simple method of treating iatrogenic prolonged erection. J Sex Med 2005;2:248-53.
80. Muneer A, Minhas S, Freeman A, Kumar P, Ralph DJ. Investigating the effects of high-dose phenylephrine in the management of prolonged ischaemic priapism. J Sex Med 2008;5:2152-9.
81. Munnarriz R, Wen CC, McAuley I, Goldstein I, Traish A, Kim N. Management of ischemic priapism with high-does intracavernosal phenylephrine: From bench to bedside. J Sex Med 2006;3:918-22.
82. Pryor J, Akkus E, Alter G, Jordan G, Lebret T, Levine L, Mulhall J, Perovic S, Ralph D, Stackl W. Priapism. J Sex Med 2004;1:116-20.
83. Davila HH, Parker J, Webster JC, Lockhart JL, Carrion RE. Subarachnoid hemorrhage as complication of phenylephrine injection for the treatment of ischemic priapism in sickle cell disease patient. J Sex Med 2008;5:1025-8.
84. Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin North Am 2005;19:917-28.
85. Mantadakis E, Ewalt DH, Cavender JD, Rogers ZR, Buchanan GR. Outpatient penile aspiration and epinephrine irrigation for young patients with sickle cell anemia and prolonged priapism. Blood 2000;95:78-82.
86. Teloken C, Ribeiro EP, Chammas M, Teloken PE, Souto CA. Intracavernosal etilefrine self-injection therapy for recurrent priapism. Urology 2005;65:1002.
87. Virag R, Buchi D, Lee K, Galacteros E. Preventive treatment of sickle cell disease with oral and self-administered intracavernous injection of etilefrine. Urology 1996;47:777-81.
88. Okpala I, Westerdale N, Jegede T, Cheung B. Etilefrine for the prevention of priapism in adult sickle cell disease. Br J Haematol 2002;118:918-21.
89. Gbadoe AD, Assimadi JK, Segbena YA. Short period of administration of diethylstilbestrol in stuttering priapism in sickle cell anemia. Am J Hematol 2002;69:297-8.
90. Chinegwundoh FI, Anie KA. Treatments for priapism in boys and men with sickle cell disease. Cochrane Database Syst Rev 2004;18:CD004198.
91. Shamloul R, el Nashaar A. Idiopathic stuttering priapism treated successfully with low-dose ethinyl estradiol: A single case report. J Sex Med 2005;2:732-4.
92. Levine LA, Guss SP. Gonadotropin releasing hormone analogues in the treatment of sickle cell anemia-associated priapism. J Urol 1993;150:475-7.
93. Steinberg J, Eyre RC. Management of recurrent priapism with epinephrine self-injection and gonadotropin-releasing hormone analogue. J Urol 1995;153:152-3.
94. Bitran D, Miller SA, McQuade DB, Leipheimer RE, Sachs BD. Inhibition of sexual reflexes by lumbosacral injection of a GABA-B agonist in the male rat. Pharmacol Biochem Behav 1988;31:657-66.
95. Costabile RA. Successful treatment of stutter priapism with an antiandrogen. Tech Urol 1998;4:167-8.
96. Dahm P, Rao DS, Donatucci CF. Antiandrogens in the treatment of priapism. Urology 2002;59:138.
97. Yamashita N, Hisasue S, Kato R, Masumori N, Takahashi A, Itoh N, Tsukamoto T. Idiopathic stuttering priapism: Recovery of detumescence mechanism with temporal use of antiandrogen. Urology 2004;63:1182-4.
98. Rachid-Filho D, Cavalcanti AG, Favorito A, Costa WS, Sampaio FJB. Treatment of recurrent priapism in sickle cell anemia with finasteride: A new approach. Urology 2009;74:1054-7.
99. Abern MR, Levine LA. Ketoconazole and prednisone to prevent recurrent ischemic priapism. J Urol 2009;188:1401-6.
100. Paredes RG, Agmo A. The GABA-B antagonist CGP 35348 inhibits the effects of baclofen on sexual behavior and motor coordination. Brain Res Bull 1995;36:495-7.
101. Vaidyanathan S, Watt JW, Singh G, Hughes PL, Selmi F, Oo T, Soni BM, Sett P. Management of recurrent priapism in a cervical spinal cord injury patient with oral baclofen therapy. Spinal Cord 2004;42:134-5.
102. Denys P, Mane M, Azouvi P, Chartier-Kastler E, Thiebaut JB, Bussel B. Side effects of chronic intrathecal baclofen on erection and ejaculation in patients with spinal cord lesions. Arch Phys Med Rehabil 1998;79:494-6.
103. Rourke KF, Fischler AH, Jordan GH. Treatment of recurrent idiopathic priapism with oral baclofen. J Urol 2002;168:2552-3.
104. D'Aleo G, Rifici C, Kofler M, Saltuari L, Bramanti P. Favorable response to intrathecal, but not oral, baclofen of priapism in a patient with spinal cord injury. Spine 2009;34:E127-9.
105. Bialecki ES, Bridges KR. Sildenafil relieves priapism in patients with sickle cell disease. Am J Med 2002;113:252.
106. Burnett AL, Bivalacqua TJ, Champion HC, Musicki B. Feasibility of the use of phosphodiesterase type 5 inhibitors in a pharmacologic prevention program for recurrent priapism. J Sex Med 2006;3:1077-84.
107. Burnett AL, Bivalacqua TJ, Champion HC, Musicki B. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism. Urology 2006;67:1043-8.
108. Tzortzis V, Mitrakas L, Gravas S, Mamoulakis C, Meissner A, Kyriakou D, Melekos MD. Oral phosphodiesterase type 5 inhibitors alleviate recurrent priapism complicating thalassemia intermedia: A case report. J Sex Med 2009;6:2068-71.
109. Pryor JP, Hehir M. The management of priapism. Br J Urol 1982;54:751-4.
110. Bennett N, Mulhall J. Sickle cell disease status and outcomes of African American men presenting with priapism. J Sex Med 2008;5:1244-50.
111. Montorsi F, Adaikan G, Becher E, Giuliano F, Khoury S, Lue TF, Sharlip I, Althof SE, Andersson KE, Brock G, Broderick G, Burnett A, Buvat J, Dean J, Donatucci C, Eardley I, Fugl-Meyer KS, Goldstein I, Hackett G, Hatzichristou D, Hellstrom W, Incrocci L, Jackson G, Kadioglu A, Levine L, Lewis RW, Maggi M, McCabe M, McMahon CG, Montague D, Montorsi P, Mulhall J, Pfaus J, Porst H, Ralph D, Rosen R, Rowland D, Sadeghi-Nejad H, Shabsigh R, Stief C, Vardi Y, Wallen K, Wasserman M. Summary of the recommendations on sexual dysfunctions in men. J Sex Med 2010;7:3572-88.