Priapism: A Review of Children with Sickle Cell Disease in Port Harcourt, Nigeria
DOI:
https://doi.org/10.12974/2312-5411.2015.02.01.6Keywords:
Sickle cell diseases, priapism, treatment.Abstract
Background: Priapism is a complication of sickle cell disease (SCD) that if left untreated results in irreversible fibrosis and impotency. The aim of this study was to determine the prevalence, pattern, steady state laboratory parameters of children with priapism and their treatment.
Methods: A retrospective study of children with sickle cell disease who were diagnosed with priapism at the Sickle Cell Clinic and the Emergency Ward of the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt, Nigeria, from 1st August 2010 to 31st July 2015. Demographic as well as steady-state clinical data were extracted from the patients' medical records. Laboratory parameters were aged matched with children (HbSS) without priapism in steady state. Data analysed included age, sex, steady-state packed cell volume, leucocyte and platelet count; and treatment.
Results: A total of 345 folders of children with SCD (342, HbSS; 3HbSC) were retrieved during the period under review. Five children were found with diagnosis of priapism. The mean age was 8.0±4.6 (range 3-15years). The mean packed cell volume (PCV), white blood cell count (WBC) and platelet counts were 22±2.7; 8±2.9 and 179.4±25.7 respectively. The mean PCV of children with priapism was significantly (P<0.05) higher than the control. Fever 4(80%), dehydration 2(40%), rigorous physical exercise 1(20%) and emotional disturbances 1(20%) were associated risks factors. Stuttering pattern was the commonest 3(60%). Most 4(80%) of them were managed conservatively with irrigation of saline and adrenaline. None of them had exchange blood transfusion. Two(40%) of them had shunt after intumescence of 5 and10 days respectively. Only 1(20%) had recurrence. None had impotency.
Conclusion: The prevalence of priapism among patients with SCD is low (1.5%) in Port Harcourt. High steady state haematocrit values were significantly associated with priapism. Fever and dehydrated were the commonest pre-morbid conditions associated with priapism. The treatment options for all types of priapism were initially conservative but surgical therapy must be available when applicable to circumvent erectile dysfunction.
References
Rees DC, Williams TN and Gladwin MT. Sickle-cell disease. Lancet 2010; 376(9757): 2018-31. http://dx.doi.org/10.1016/S0140-6736(10)61029-X
Makani J, Cox SE, Soka D, Komba AN, Oruo J, Mwamtemi H, et al. Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PloS one 2011; 6(2): e14699. http://dx.doi.org/10.1371/journal.pone.0014699
CDC. Global health Nigeria: Sickle Cell Disease Atlanta, USA: Centers for Disease Prevention and Control; 2012
[updated2012; cited 2014 19, June]. Available from: http: //www.cdc.gov/globalhealth/countries/nigeria/what/scd.htm.
Eland IA, van der Lei J, Stricker BH and Sturkenboom MJ. Incidence of priapism in the general population. Urology 2001; 57: 970-972. http://dx.doi.org/10.1016/S0090-4295(01)00941-4
Diggs LW and Ching RE. Pathology of sickle cell anemia. Southern Med J 1934; 27: 839-845. http://dx.doi.org/10.1097/00007611-193410000-00003
Adedeji MO, Onuora VC and Ukoli FA. Haematological parameters associated with priapism in Nigerian patients with homozygous sickle cell disease. J Trop Med Hyg 1988; 91: 157-159.
Badmus TA, Adediran IA, Adesunkanmi AR and Katung IA. Priapism in south western Nigeria. East Afr Med J 2003; 80: 518-524.
Gbadoe AD, Dogba A, Segbena AY, et al. Priapism in sickle cell anemia in Togo: prevalence and knowledge of this complication. Hemoglobin 2001; 25: 355-361. http://dx.doi.org/10.1081/HEM-100107871
Mantadakis E, Cavender JD, Rogers ZR, Ewalt DH and Buchanan GR. Prevalence of priapism in children and adolescents with sickle cell anemia. J Pediatr Hematol Oncol 1999; 21: 518-522. http://dx.doi.org/10.1097/00043426-199911000-00013
Maples BL and Hagemann TM. Treatment of priapism in pediatric patients with sickle cell disease. Am J Health Syst Pharm 2004; 15; 61(4): 355-63.
Burnett AL Priapism. In: Wein AJ, ed. Campbell-Walsh Urology. 9thed. Philadelphia. Pa: Saunders Elsevier 2007; chap 26.
Rogers ZR. Priapism in sickle cell disease. Hematol Oncol Clin North Am 2005; 19: 917-28. http://dx.doi.org/10.1016/j.hoc.2005.08.003
Otaigbe BE and Yaguo-Ide LE. Priapism in paediatric patients with sickle cell disease- a report of three cases at University of Port Harcourt Teaching Hospital. Port Harcourt Medical Journal2007; 2(1): 74-77. http://dx.doi.org/10.4314/phmedj.v2i1.38898
Fowler JE, Koshy M, Strub M and Chinn SK. Priapism associated with sickle cell haemoglobinopathies. Prevalence, natural history and sequelae. J Urol 1991; 145: 65-8.
Adeyoju AB, Olujohungbe AB, Morris J, et al. Priapism in sickle-cell disease: incidence, risk factors and complications - an international multicentre study. BJU Int 2002; 90: 898-902. http://dx.doi.org/10.1046/j.1464-410X.2002.03022.x
Furtado PS, Costa MP, Ribeiro do Prado Valladares F, Oliveira da Silva L, Lordêlo M, Lyra I, et al. The prevalence of priapism in children and adolescents with sickle cell disease in Brazil. Int J Hematol 2012; 95(6): 648-51. http://dx.doi.org/10.1007/s12185-012-1083-0
Nolan VG, Wyszynski DF, Farrer LA, et al. Hemolysisassociated priapism in sickle cell disease. Blood 2005; 106: 3264-3267. http://dx.doi.org/10.1182/blood-2005-04-1594
Ahmed SG, Ibrahim UA and Hassan AW. Hematological parameters in sickle cell anemia patients with and without priapism. Ann Saudi Med 2006; 26: 439-443.
Lawani J, Aken'Ova YA and Shittu OB. Priapism: an appraisal of surgical treatment. Sci 1999; 28(1-2): 21-3.
Saad ST, Lajolo C, Gilli S, et al. Follow‐up of sickle cell disease patients with priapism treated by hydroxyurea. Am J Hematol 2004; 77: 45-49. http://dx.doi.org/10.1002/ajh.20142
Permenis P, Athanasopoulos A, Papathanasopoulos P, et al. Gabapentin in the management of the recurrent, refractory, idiopathic priapism. Int J Impot Res 2004; 16: 84-85. http://dx.doi.org/10.1038/sj.ijir.3901165
Lowe FC and Jarow JP. Placebo‐controlled study of oral terbutaline and pseudoephedrine in management of prostaglandin E1‐induced prolonged erections. Urology 1993; 4: 251-53. http://dx.doi.org/10.1016/0090-4295(93)90338-b
Govier FE, Jonsson E and Kramer‐Levien D. Oral terbutaline for the treatment of priapism. J Urol 1994; 151: 878-879.
Martinez‐Portillo FJ, Fernandez Arancibia MI, et al. Methylene blue: an effective therapeutic alternative for priapism induced by intracavernous injection of vasoactive agents. Arch Esp Urol 2002; 55: 303-308.
Cherian J, Rao AR, Thwaini A, Kapasi F, Shergill IS and Samman R. Medical and surgical management of priapism. Postgrad Med J 2006; 82(964): 89-94. http://dx.doi.org/10.1136/pgmj.2005.037291
