Priapism stuttering

Clinical features Priapism is defined as a painful and unwanted erection, which is classified as stuttering (less than 3 hours), often multiple prolonged (more than 3 hours). Up to 89% of males with SCD will have at least one episode by age 20. The mean age of the initial episode is 12 years of age. Repeated episodes can cause fibrosis and impotence. 

Signs and symptoms Prepubertal children often present with stuttering priapism. Older males can present with prolonged episodes that last for days and should be managed as medical emergencies. Urinary obstruction can occur in severe cases. 

Harmon JD, Ginsberg PC, Nachmann MM, Manzarbeita C, Harkaway RC. Stuttering priapism in a hver transplant patient with toxic levels of FK506. Urology 1999 54(2) 366. 

The etiology of priapism has been traditionally divided into primary or idiopatic and secondary to some other condition or disease process. However, in accordance with Pryor (2004), for the purposes of clinical management, it is appropriate to distinguish between high-flow, low-flow, and recurrent or stuttering priapism. 

Serjeant GR, de Ceulaer K, Maude GH (1985) Stilboestrol and stuttering priapism in homozygous sickle-cell disease. Lancet 2 1274-1276... 

Recently, recurrent or stuttering priapism has been described as a poorly understood condition that may present clinically with low-flow or, more frequently, with high-flow episodes, alternatively (Pautler and Brock 2001 Pryor et al. 2004). 

A variety of clinical situations can result in diffuse cavernosal flbrotic changes. Low-flow priapism is a well-known cause of cavernosal tissue fibrosis with resulting venous occlusive deficiency. It is commonly accepted that prompt treatment is mandatory because recovery of erectile function becomes increasingly unlikely over time (El-Bahnasawy et al. 2002). Cavernosal tissue damage leading to fibrotic changes can occasionally occur also in patients with stuttering priapism and with arterial priapism (Hakim et al. 1996 Bertolotto et al. 2003). 

Deficiencies of anticoagulant proteins can increase the risk of skin necrosis due to warfarin. In a 19-year-old man with protein C deficiency, recurrent (stuttering) priapism attributed to warfarin was complicated by skin necrosis, presumably because of paradoxical hypercoagulability [30" ]. Activated protein C concentrate can be used to treat such cases [31" ]. In another case, skin necrosis occurred on the pinna of an 82-year-old man with protein S deficiency after warfarin therapy for 2 weeks [32 ]. 

Abu Sham a RA, Kufri FH, Yassin IH. Stuttering priapism complicating warfarin therapy in a patient with protein C deficiency. Int J Lab Hematol 2008 30(4) 339-43. 

---