Priapism in Sickle Cell Disease
Section snippets
Physiology
The penis contains three vascular chambers: the paired lateral corpora cavernosa and a corpus spongiosum surrounding the urethra. Erection occurs when increased blood volume in the cavernosa and spongiosum is constrained by the tunica albuginea, leading to mechanical elongation of the penis. An erection is normally maintained by the partial obstruction of venous drainage [5]. Priapism occurs when there is either unregulated arterial inflow or persistent obstruction of venous outflow [2].
Epidemiology and natural history
Historically, only 5% to 10% of male patients of any age with sickle cell disease were said to have had priapism [6], [12], [13], [14]. A retrospective review of adult patients now indicates that at least 30% to 45% of patients have a history of this complication [15], [16]. An interview survey of sequential male patients aged 5 to 20 years from Dallas who had sickle cell anemia demonstrated a prevalence of 27.5% by 15 years of age. The calculated actuarial probability of experiencing priapism
Management of acute episodes
Physical examination revealing a tumescent phallus with a history of the unwanted painful erection lasting 30 minutes or more is adequate for diagnosis of priapism. When priapism lasts 4 hours or more, the potential for permanent ischemic injury is present, and intervention is warranted [3]. A suggested approach to the patient presenting for medical attention during an episode of priapism is presented in Box 1.
Numerous therapeutic modalities have been used for treatment of priapism associated
Strategies for the secondary prevention of priapism
For patients who have frequently recurrent stuttering spells or several prolonged episodes requiring repeated aspiration and irrigation, alternative strategies to prevent episodes should be considered. Treatment with hydroxyurea has been suggested by some authors to prevent priapism [45], [46], although others found it ineffective [47]. A controlled trial of the efficacy of hydroxyurea treatment in the prevention of priapism has never been reported. However, this agent, which increases total
Summary
Priapism is, for most patients, an unpredictable, recurrent event associated with devastating consequences for erectile function and quality of life. It occurs in a significant number of males who have all forms of sickle cell disease. Patients should be educated about priapism and currently available treatment options; this education should focus on the need to report recurrent events and to present for care within 4 hours of onset during a prolonged episode. Hematologists, as the physicians
References (53)
- et al.
Hemodynamics of penile erection
Urol Clin North Am
(1988) - et al.
Multisystem damage associated with tricorporal priapism in sickle cell disease
Am J Med
(1993) - et al.
Priapism
Hematol Oncol Clin North Am
(1996) - et al.
Impotence following childhood priapism
J Urol
(1990) - et al.
Priapism in association with sickle hemoglobinopathies in children
J Pediatr
(1975) - et al.
Urological complications of sickle cell disease in a pediatric population
J Urol
(1987) - et al.
Priapism in children with sickle cell disease, part 2
J Urol
(1995) - et al.
Priapism associated with the sickle cell hemoglobinopathies: prevalence, natural history and sequelae
J Urol
(1991) - et al.
Priapism as a complication of sickle cell disease
J Urol
(1991) - et al.
Priapism associated with sickle cell hemoglobinopathy in children: long-term effects on potency
J Urol
(1996)