PEYRONIE DISEASE, PRIAPISM, THROMBOSIS
Peyronie disease (PD), also known as induratio penis plastica, is a benign, poorly understood condition that is associated with penile deformity. It occurs mainly in men 50 to 60 years of age, although it can occur at any age. The condition includes penile curvature or a plaque, penile pain, and erectile dysfunction. Among these, penile curvature is the most common deformity. Interestingly, PD is also associated with Dupuytren contracture of the palma fascia of the hand.
The penis may bend in any direction, although an upward bend is most common. Usually, the deformity is only evident during erection. Less commonly, a “waist” or “hourglass” defect may exist in which one segment of the penis is narrower than the surrounding areas. Importantly, PD is a separate entity from chordee, which is a congenital penile curvature observed in newborns and is not associated with plaques or pain. A ﬁrm, ﬂat, benign nodule or plaque may be felt on the penis and may contribute to curvature. The plaque is located within the tunica albuginea, the tough ﬁbrous covering of the corpora cavernosal bodies. The plaque may accumulate calcium and become bone-like. Associated penile pain is most severe during erection but may be present at rest. Pain is often the ﬁrst sign and occurs before noticeable bending. Bending occurs toward the side with the plaque. In most cases, the pain will resolve with time although plaques and curvature may persist. About 50% of men who present with PD also have erectile dysfunction.
The process by which penile plaques develop is unknown. The leading theory is that minor trauma (often unnoticed) from penile buckling during sex shears layers of the tunica albuginea and disrupts small blood vessels. Bleeding and trauma induce the release of proinﬂammatory agents such as transforming growth factor-β (TGF-beta) and ﬁbrin. The inability to drain these inﬂammatory mediators away from the injury leads to prolonged inﬂammation and ﬁbrosis. In 15% of patients, PD will resolve with time. In persistent cases, empirical medical treatments include antioxidants, anti-inﬂammatory agents, and penile stretching devices. Surgical cures are routine with either penile plication (straightening) procedures or plaque excision and grafting procedures and may involve penile pros-thesis implantation.
Priapism is a prolonged and often painful penile erection lasting more than 4 hours and not related to sexual desire or stimulation. The word is derived from the Roman god Priapus, a deity renowned for his erect penis. Priapism can affect boys and men at any age.
There are two types of priapism, ischemic and nonischemic. Ischemic, low-ﬂow, or venoocclusive priapism occurs when there is no penile blood ﬂow. With obstruction to ﬂow, trapped blood increases pressure and the penile shaft becomes very hard and painful. Nonischemic priapism, also known as high ﬂow priapism, is rare and occurs with excessive blood ﬂow through the penis as a result of arterial rupture within the erectile tissue, most commonly from blunt injury to the groin or pelvis. In nonischemic priapism, the penis is enlarged but not as rigid as a normal erection and there is usually less pain. It is critical to distinguish these two forms of priapism, as ischemic priapism is a medical emergency that can permanently injure the penis and lead to erectile dysfunction. This generally occurs after 48 hours of unwanted erection as thrombosis within the cavernous spaces causes ﬁbrosis and permanent loss of function.
Priapism can be idiopathic or secondary in nature. Drugs associated with priapism include papaverine, phentolamine, prostaglandin (when given for erectile dysfunction), trazodone, propranolol, hydralazine, thioridazine, antidepressants, and cocaine. Medical conditions associated with priapism include spinal cord injury, leukemia, gout, sickle cell anemia, and advanced pelvic and metastatic cancer. Treatment is directed at relieving the erection with corporal irrigation to remove blood clots, intracorporal injection of α-agonist drugs to contract arteries, and occasionally surgical shunts to restore venous outﬂow. It is also important to ﬁnd and treat the root cause of ischemic priapism with intra venous ﬂuids, pain medication, oxygen, radiation, or chemotherapy.