Priapism in paediatric patients with sickle cell disease - a report of three cases at the University of Port Harcourt Teaching Hospital

Abstract

Background: Priapism is an abnormal persistent erection of the penis which may be painful or not painful. Approximately two-thirds of all paediatric patients who have priapism also have sickle cell disease (SCD). The aim of this study is to review all the cases of priapism admitted into the paediatric emergency room of the University of Port Harcourt Teaching Hospital (UPTH) over a 5-year period and to determine the prevalence, presentation, precipitating factors and management of cases seen.
Method: The admission register in the Children\'s emergency ward was reviewed from January 2001 to December 2005. All the cases of sickle cell disease were extracted. The folders of the patients with priapism were studied and data analysed.
Results: Of the 185 SCD cases, three (1.6%) had priapism. They were adolescents aged 17years, 11years and 10 years 9months respectively. Two patients had never attended a sickle cell clinic, never been on routine drugs nor received advice on oral liberal fluids intake. One patient had stuttering priapism, 24hours before the event. Two of them had glanulo-cavernous shunts, with early detumescence on Day 3 and 4 respectively, while the patient who was managed conservatively had detumescence after 12 days.
Conclusion: Priapism is an uncommon presentation of SCD in our environment. With prolonged duration, priapism has severe sequelae of impotence and erectile dysfunction, thus, its presentation and management should be known by patients and parents or care givers. Surgical intervention is recommended as it is more effective and results in shorter hospital stay.


Keywords: Priapism, Sickle cell disease, Glanulo-cavernous shunt, Conservative management

Port Harcourt Medical Journal Vol. 2 (1) 2007: pp. 74-77