MANAGEMENT AND THERAPY OF SICKLE CELL DISEASE

NIH Publication No. 95-2117, Revised December 1995 (Third Edition) National Institutes of Health, National Heart, Lung, and Blood Institute

Chapter 19-Priapism

Priapism is a persistent, painful penile erection. Three clinical categories are discussed in this chapter, including recurrent acute priapism, acute prolonged priapism, and chronic priapism.

RECURRENT ACUTE PRIAPISM

This is the most common form of priapism in which the patient gets short attacks that subside spontaneously. The patient usually copes with this problem and may not seek medical advice. The natural history is variable. Most patients will continue to get attacks throughout their lives and will maintain some degree of potency. Others will eventually become impotent. A small group will develop an acute attack that will not subside without active and aggressive treatment in a timely fashion.

ACUTE PROLONGED PRIAPISM

The painful erection that does not subside after several hours is an emergency and should be treated aggressively. If untreated, the attack will last for several weeks, resulting in total impotence. Even with aggressive treatment, preservation of potency may be compromised. The presenting features are persistent erection, severe pain, and tenderness of the penile shaft. Radionuclear penile scan may show a very low flow, and penile blood gas may have a pO2 of less than 5.

CHRONIC PRIAPISM

This is a rare form of presentation that may follow an acute episode or may arise de novo. The penis is semierect and there is no pain. Clinically, this results in a progressive increase in the size of the penis, and penile scan shows good blood flow. Penile ultrasound shows dilated deep dorsal arteries in both corporeal bodies, and the patient has difficulty in getting a complete erection. Occasionally these patients may develop an acute episode. Suddenly for no apparent reason the penis becomes rigid and painful. These episodes may affect only a part of the penile shaft.

PATHOPHYSIOLOGY

The specific mechanisms that precipitate attacks of acute priapism in patients with sickle cell disease are unknown. Acute attacks often start during sleep, occasionally following sexual activity, but frequently no identifiable event is noted. An unusually full bladder is commonly associated with the onset of nocturnal attacks. Some episodes appear to be triggered or exacerbated by dehydration. Some patients with priapism have preservation of flow to the glans and spongiosal tissue. Venous outflow from the corpora cavernosa is not always totally occluded as had been earlier postulated. These facts establish the rationale for transfusing normal red cells because these cells can be expected to gradually reach areas of poor circulation. Alternatively some patients may have tricorporal priapism, which is more severe. Presumably, a viscous mass of deoxygenated and damaged red cells develops that further impairs or occludes outflow. Edema and inflammation can result. Evacuation of this material can delay irreversible damage to the corpora cavernosa. Hemoglobin electrophoresis of penile blood in transfused patients occasionally shows that no Hb A has entered the corpora cavernosa. These observations establish the rationale for aspiration and irrigation and for the temporary corpora spongiosum shunt procedure currently in use (see Winter procedure below).

GENERAL MANAGEMENT

The goals of management are to assist with emptying the corpora cavernosa, relieve the patient's pain, and, if possible, prevent impotence. As with other inflammatory events in sickle cell disease, fever and leukocytosis can occur in the absence of infection. Nevertheless, infectious etiologies should be ruled out. Recurrent Acute Priapism Patients often manage this condition at home. Emptying the bladder, warm baths, and exercise are all recommended techniques. The patient should be encouraged to empty the bladder frequently once the attack has begun. At the same time, however, the patient should be encouraged to increase oral intake of fluid to offset any possible contribution of systemic dehydration. If the episode does not resolve in 3 hours, the patient should seek medical attention.

Acute Prolonged Priapism

Possible etiologies for this pattern should be sought. These include infection (particularly of the prostate), recent trauma, medications with autonomic effects, alcohol excess, marijuana use, or sexual activity. Initial treatment of acute, prolonged priapism should include rigorous intravenous hydration, parenteral narcotics to relieve pain, and if necessary, insertion of a Foley catheter to promote bladder emptying. In addition, preparations should be made to reduce the percentage of sickle cells to less than 30 percent while maintaining the hematocrit below 35 percent. This objective can most effectively be accomplished by exchange transfusion.

Patients who are likely to respond to transfusion regimens begin detumescence within 1 or 2 days, although complete detumescence may take weeks to months. There are reports of cerebrovascular hemorrhage in children following exchange transfusions for priapism. Concepts in the surgical treatment of severe priapism in sickle cell disease are currently changing toward a simpler procedure and much earlier intervention. Opinion is not yet uniform, but recent studies suggest that if detumescence has not begun within 24 hours following completion of transfusion therapy, a significant response to transfusion alone is unlikely. In this case, a penile aspiration should be tried first; if this is not successful, a Winter procedure (spongiosum-cavernosum shunt) or a Hashmat shunt are recommended under local anesthesia in adults or, preferably, general anesthesia in children. A saphenous shunt is no longer recommended. Despite interventions, impotence remains a frequent complication of priapism. For incapacitating recurrent priapism, a trial of chronic transfusions for 3 to 6 months is often successful.

MANAGEMENT OF IMPOTENCE

Although the patient is not suffering with pain, erectile function is often absent or impaired. Management of impotence by implantation of a prosthesis has been performed by some urologists. Other patients, however, are able to adjust to the altered sexual function, usually with the support of an accommodating partner, because ejaculation, orgasm, and fertility remain intact. Surgical intervention is rarely needed in prepubertal boys.

BIBLIOGRAPHY

Bertram RA, Webster GD, Carson CC. Priapism: etiology, treatment, and results in series of 35 presentations. Urology 1985;26:229-32.

Emond AM, Holman R, Hayes RJ, Serjeant GR. Priapism and impotence in homozygous sickle cell disease. Arch Intern Med 1980;140(11):143-47.

Hamre MR, Harmon EP, Kirkpatrick DV, et al. Priapism as a complication of sickle cell disease. J Urol 1991;145:1-5.

Hashmat AI, Das S. The penis. Philadelphia: Lea and Febiger, 1993.

Hashmat AI, Samanthi R, Singh I, Macchia R. 99m Tc penile scan: an investigative modality in priapism. Urol Radiol 1989;11:58-60.

Noe HN, Wilimas J, Jerkins GR. Surgical management of priapism in children with sickle cell anemia. J Urol 1981;126(6):770-1.

Rackoff WR, Ohene-Frempong K, Month S, Scott JP, Neahring B, Cohen AR. Neurologic events after partial exchange transfusion for priapism in sickle cell disease. J Pediatr 1992;120:882-5.

Sharpsteen JR Jr, Powars D, Johnson C, Rogers ZR, Williams WD, Posch RJ. Multisystem damage associated with tricorporal priapism in sickle cell disease. Am J Med 1993;94:289-95.

Winter CC. Priapism cured by creation of fistule between glans penis and corpora cavernosa. J Urol 1980;423:595-6.

Send mail to aplatt@emory.edu with questions or comments about this web site.
Copyright © 1997 Sickle Cell Information Center
Last modified: September 07, 1997