PRIAPISM Management
IN BOYS with Cavernosaphenous
MARTIN I. RESNICK, JAMES
M. HOLLAND,
LOWELL
Shunt
M.D. M.D.
R. KING, M.D.
JOHN T. GRAYHACK,
M.D.
From the Department of Urology, Northwestern University Medical School, and Division of Urology, Children’s Memorial Hospital, Chicago, and Evanston Hospital, Evanston, Illinois
ABSTRACT - Two children with priapism are presented; one case was secondary to trauma and the other was idiopathic in nature. Both boys were effectively treated by bilateral corporosaphenous shunts. The mechanism of normal erection and the pathophysiology of priapism are discussed.
,Priapism is an abnormally prolonged, frequently painful erection of the penis unaccompanied by sexual desire. Typically the corpora cavernosa are distended and firm; however, the glans penis and corpus spongiosum remain flaccid. If left untreated, impotence results because of the eventual fibrosis of the cavernosus septa. Treatment prior to the introduction of the shunting procedures in the 1960s had been varied and generally not uniformly effective. In the early nineteenth century Callaway’ described the use of leeches, enemas, emetics, and cold solutions. However, when all failed, he recommended incision and drainage of the corporus cavernosum which led to detumescence but was associated with impotence. McKay and Colston in 1928’ reported a favorable response with aspiration and irrigation of the corpora cavernosa. Other recommended forms of treatment have been the use of intravenous fibrinolytic therapy,3 anticoagulant therapy,4 hypotensive agents5 ligation of the pudendal arteries6 and use of estrogenic’ and adrenocortical’ hormones. Following the introduction of the corporosaphenous shunt9 and the spongiocavernosum shunt in 1964 a more uniformly acceptable form of treatment had been made available.lO When performed in
492
the appropriate patient, these procedures frequently provide a satisfactory result. Shunting procedures for priapism in children are rarely indicated because the majority of patients have sustained erections secondary to either leukemia or sickle cell disease that are best treated by correction of the hematologic abnormality. We recently encountered 2 children with priapism, one secondary to trauma and the other idiopathic in nature, who were effectively treated by bilateral corporosaphenous bypass. Their histories are reported herein. Case Reports Case 1 A thirteen-year-old white boy was transferred to Children’s Memorial Hospital with a history of priapism for forty-eight hours. He had two previous episodes.at one and one-half years and three weeks prior to admission that began when he had the desire to urinate but did not because it was not convenient at the time. After the onset of priapism, however, he was unable to urinate. Both episodes responded satisfactorily to conservative therapy, such as urethral catheterization, sedation, and application of ice packs. The
UROLOGY
/ APRIL 1975 / VOLUME
V, NUMBER4
present episode began in the same manner as previous ones had but was not associated with urinary retention. Physical examination was unremarkable except for a fully erect penis without turgidity of the corpus spongiosum and glans penis. The urinary bladder was not palpable. Complete blood count, urinalysis, blood urea nitrogen, serum creatinine, and hemoglobin electrophoresis were all within normal limits. Ketamine anesthesia was administered because of the reported benefit of this agent in the treatment of penile turgescence. l1 Priapism persisted, and spinal anesthesia was used which was also ineffective in reducing the erection. The corpora were then irrigated with a dilute heparin solution until all the dark, nonclotted blood was removed and typical arterial blood appeared. The erection was not reduced, and it was elected to perform bilateral corporosaphenous shunts. Following surgery the erection gradually subsided, and at the time of discharge six days postoperatively the penis had become flaccid. Postoperatively the patient complained of weakness of the urinary stream, and a retrograde urethrogram performed one month following surgery revealed a stricture of the bulbous urethra. The patient underwent a successful Johanson two-stage urethroplasty. Eight months after the bypass procedure he had not experienced any recurrent episodes of priapism; however, spontaneous erections have occurred. Case 2 A six-year-old white boy was admitted to Evanston Hospital with a prolonged penile erection. Two days previously he fell from a stepladder and sustained a straddle injury on the side of a chair following which a persistent nonpainful erection was noted. He did not have difficulty voiding. On physical examination a superficial ecchymosis was noted over the penoscrotal junction. The penis was nontender and approximately 80 per cent of being fully erect. The corpora were not apparently ruptured or thrombosed. The glans penis and corpus spongiosum were not turgid. Complete blood count, urinalysis, blood urea nitrogen, serum creatinine and hemoglobin electrophoresis were within normal limits. The patient was placed on bedrest, ice packs and scrotum, were applied to the penis, and slight improvement was noted. perineum, Prostatic massage had no effect. He was sent home with the belief that this traumatic partial priapism would gradually subside with conservative therapy. However, when he was seen one
UROLOGY
/ APRIL 1975 / VOLUME
V, NUMBER4
week after injury the penis appeared slightly more erect, and at seventeen days following injury the penis was again at its initial state of an 80 per cent erection. Surgical intervention was elected at this time. Irrigation of the corpora cavernosa with a dilute heparin solution was successful in reducing the priapism slightly, but when the irrigating needles were removed the erection returned despite firm compression. Therefore, a bilateral corporosaphenous shunt was performed. In three to four hours following surgery the penis was 30 to 40 per cent erect, and at the time of discharge one week following surgery the penis was flaccid. At six months follow-up, the patient had not had any recurrence, however, he has not been noted to experience any spontaneous erections. Comment A discussion of the pathophysiology of priapism requires a review of the mechanism of normal erection and detumescence. Stieve in 193012 first described the muscular pillars or cushions lying longitudinally within the small arteries and veins of the penis. In 1952 Conti13 histologically confirmed the existence of these “polsters” and postulated that they act as arterial and venous valves under the influence of the lumbar sympathetics (Fig. 1). In the flaccid state the constricting arterial polsters distal to the arteriovenous anastomosis decrease the penile arterial caliber, and blood is shunted away from the corpora. Drainage is completed by relaxation of the venous polsters. Erection occurs following constriction of the polsters within the arteriovenous shunts and veins along with relaxation of the arterial cushions, resulting in engorgement of the corpora cavernosa. Recently, however, others have suggested that erection occurs with increased arterial flow rate alone and is independent of venous obstruction. I4 Idiopathic priapism refers to a pathologic erection which is unaccompanied by disease that may cause or maintain the condition. The first patient described falls within this category, and there is no apparent etiologic factor contributing to its occurrence. A urethral stricture was possibly related to the previous catheterizations and was probably unrelated to the episodic prolonged erections. on the other hand, is Secondary priapism, caused by factors directly or indirectly impairing the normal mechanisms of detumescence, such as in Case 2 following perineal trauma. Hematoma followed by corporal compression and perhaps
493
Deep dorsal
Corpora
cavernosa
Profundq
’
A. FIGURE 1.
‘-‘
a. ‘\\
Muscular
Efferent
Mechanism sf normal
v. erection and detumescence.
thrombosis subsequently led to a persistent erection that required an alternative to the normal venous outflow for relief. Whether normal erections will occur in this patient remains unknown. Priapism in children is rare and is most frequently caused by hematologic disorders. However, the condition has been reported to be related to congenital syphilis, l5 retroperitoneal fibrosarcoma,16 trauma,i’ and unknown causes.i8 Leukemic priapism is secondary to the blood abnormality and is probably related to the sludging of leukemic cells in the corpora cavernosa. Clinicians generally agree that therapy should be directed toward reduction of splenomegaly and decreasing the number of leukemic cells.1g*20 Likewise, children with priapism secondary to sickle cell disease are usually managed by measures directed at reduction of blood sludging within the vascular spaces21 However, shunting procedures have been used when conservative therapy was unsuccessful.” If conservative forms of management have been unsuccessful, surgical intervention should be instituted within seventy-two hours after the onset of priapism. Hinman23 reported that prolonged venous stasis results in irreversible fibrosis of the corpora cavernosa and increases the likelihood of impotence. Corporosaphenous
494
v.
BloodJow
in Jaccid penis (A) and in erect penis
bypass provides alternative venous drainage of the corpora cavernosa, thus relieving the painful engorgement of the penis. Its use is indicated in idiopathic and selected cases of secondary priapism. Northwestern
University Medical School 303 East Chicago Avenue Chicago, Illinois 60611 (DR. RESNICK)
References 1. CALLAWAY, T. : Unusual case of priapism, London Med. Repos. 21: 286 (1824). 2. MCKAY, R. W., and COLSTON, J. A. L.: Priapism: a new method of treatment, J. Urol. 19: 121 (1928). 3. BELL, W. R., and PILRAY, W. R.: Management of priapism by therapeutic defibrination, N. Engl. J. Med. 280: 649 (1969). 4. SMITH, K. H.: Priapism: use of Dicumarol in persistent priapism, J. Urol. 64: 400 (1950). 5. BURT, F. B., SCHIRMER, H. K., and SCOTT,W. W.: A new concept in the management ofpriapism, ibid. 83: 60 (1960). 6. ULM, A. H.: Treatment of primary priapism with Arfonad, ibid. 81: 291 (1959). 7. PRICE,R. A., and PENNA, 0. J. : Effectiveness of drugs in presenting postoperative penile erections, Surgery 24: 980 (1948). 8. ROLNICK, D., COTTRELL, T. L., and LLOYD, F.
UROLOGY I APRIL 1975 / VOLUME V. NUMBER4
9.
10.
11. 12.
13. 14.
15. 16.
A.: Priapism. Report of two cases and discussion of treatment, Ill. Med. J. 112: 129 (1957). GRAYHACK, J. T., MCCULLOUGH, W., O’CONOR, V. J., Venous by-pass to control JR., and TRIPPEL, 0.: priapism, Invest. Ural. 1: 509 (1964). QUACKELS, R.: Cure of a patient suffering from priapism by caverno-spongiosa anastomosis, Acta Urol. Belg. 32: 5 (1964). GALE, A. S.: Ketamine prevention of penile turgescence, J.A.M.A. 219: 1629 (1972). STIEVE, H. : Harn und Geschlachtsappart, in MiillenAnadorif, W. V., Ed. : Handbuch der mikroskopischen tomic des Menschen, Berlin, Springer-Verlag, 1930. CONTI, G.: L’erection du penis suman et ses bases morphologicovasculaires, Acta Anat. 14: 217 (1952). MALVER, T., BARON, T., and CLARK, S. S.: Assessment of potency with the Doppler flowmeter, Urology 2: 396 (1973). NUCKOLS, C. H.: Congenital syphilitic priapism, Louisville News, Cincinnati Clinic, 1876. LATTIMER, J. K.: The male genital tract, in Mustard,
UROLOGY
/
APRIL 1975 /
VOLUME V. NUMBER4
17. 18.
19.
20.
21.
22.
23.
W. T., et al., Eds. : Pediatric Surgery, 2nd ed., Chicago, Year Book Publishers, 1969, p. 1267. FRASEN, W. T.: A case of priapism, Br. Med. J. 2: 419 (1955). HOWE, G. E., PRENTISS, R. J., COLE, J, W., and MASTERS, R. H.: Priapism: a surgical emergency, J. Urol. 101: 576 (1969). SCHREIBMAN, S. M., GEE, T. S., and GRABSTALD, H. : Management of priapism in patients with chronic granulocytic leukemia, ibid. 111: 786 (1974). GRAIVIER, L., et al. : Priapism in a 7 year old infant with chronic granulocytic leukemia, ibid. 105: 137 (1971). ROTHFELD, S. u., and MAZOL, D.: Priapism in children: a complication of sickle cell disease, ibid. 105: 307 (1971). SNYDER, G. B., and WILSON, C. B.: Surgical management of priapism and its sequelae in sickle cell disease, South. Med. J. 59: 1393 (1966). HINMAN, F., JR.: Priapism: reasons for failure of therapy, J. Urol. 83: 420 (1960).
495
IN BOYS with Cavernosaphenous
MARTIN I. RESNICK, JAMES
M. HOLLAND,
LOWELL
Shunt
M.D. M.D.
R. KING, M.D.
JOHN T. GRAYHACK,
M.D.
From the Department of Urology, Northwestern University Medical School, and Division of Urology, Children’s Memorial Hospital, Chicago, and Evanston Hospital, Evanston, Illinois
ABSTRACT - Two children with priapism are presented; one case was secondary to trauma and the other was idiopathic in nature. Both boys were effectively treated by bilateral corporosaphenous shunts. The mechanism of normal erection and the pathophysiology of priapism are discussed.
,Priapism is an abnormally prolonged, frequently painful erection of the penis unaccompanied by sexual desire. Typically the corpora cavernosa are distended and firm; however, the glans penis and corpus spongiosum remain flaccid. If left untreated, impotence results because of the eventual fibrosis of the cavernosus septa. Treatment prior to the introduction of the shunting procedures in the 1960s had been varied and generally not uniformly effective. In the early nineteenth century Callaway’ described the use of leeches, enemas, emetics, and cold solutions. However, when all failed, he recommended incision and drainage of the corporus cavernosum which led to detumescence but was associated with impotence. McKay and Colston in 1928’ reported a favorable response with aspiration and irrigation of the corpora cavernosa. Other recommended forms of treatment have been the use of intravenous fibrinolytic therapy,3 anticoagulant therapy,4 hypotensive agents5 ligation of the pudendal arteries6 and use of estrogenic’ and adrenocortical’ hormones. Following the introduction of the corporosaphenous shunt9 and the spongiocavernosum shunt in 1964 a more uniformly acceptable form of treatment had been made available.lO When performed in
492
the appropriate patient, these procedures frequently provide a satisfactory result. Shunting procedures for priapism in children are rarely indicated because the majority of patients have sustained erections secondary to either leukemia or sickle cell disease that are best treated by correction of the hematologic abnormality. We recently encountered 2 children with priapism, one secondary to trauma and the other idiopathic in nature, who were effectively treated by bilateral corporosaphenous bypass. Their histories are reported herein. Case Reports Case 1 A thirteen-year-old white boy was transferred to Children’s Memorial Hospital with a history of priapism for forty-eight hours. He had two previous episodes.at one and one-half years and three weeks prior to admission that began when he had the desire to urinate but did not because it was not convenient at the time. After the onset of priapism, however, he was unable to urinate. Both episodes responded satisfactorily to conservative therapy, such as urethral catheterization, sedation, and application of ice packs. The
UROLOGY
/ APRIL 1975 / VOLUME
V, NUMBER4
present episode began in the same manner as previous ones had but was not associated with urinary retention. Physical examination was unremarkable except for a fully erect penis without turgidity of the corpus spongiosum and glans penis. The urinary bladder was not palpable. Complete blood count, urinalysis, blood urea nitrogen, serum creatinine, and hemoglobin electrophoresis were all within normal limits. Ketamine anesthesia was administered because of the reported benefit of this agent in the treatment of penile turgescence. l1 Priapism persisted, and spinal anesthesia was used which was also ineffective in reducing the erection. The corpora were then irrigated with a dilute heparin solution until all the dark, nonclotted blood was removed and typical arterial blood appeared. The erection was not reduced, and it was elected to perform bilateral corporosaphenous shunts. Following surgery the erection gradually subsided, and at the time of discharge six days postoperatively the penis had become flaccid. Postoperatively the patient complained of weakness of the urinary stream, and a retrograde urethrogram performed one month following surgery revealed a stricture of the bulbous urethra. The patient underwent a successful Johanson two-stage urethroplasty. Eight months after the bypass procedure he had not experienced any recurrent episodes of priapism; however, spontaneous erections have occurred. Case 2 A six-year-old white boy was admitted to Evanston Hospital with a prolonged penile erection. Two days previously he fell from a stepladder and sustained a straddle injury on the side of a chair following which a persistent nonpainful erection was noted. He did not have difficulty voiding. On physical examination a superficial ecchymosis was noted over the penoscrotal junction. The penis was nontender and approximately 80 per cent of being fully erect. The corpora were not apparently ruptured or thrombosed. The glans penis and corpus spongiosum were not turgid. Complete blood count, urinalysis, blood urea nitrogen, serum creatinine and hemoglobin electrophoresis were within normal limits. The patient was placed on bedrest, ice packs and scrotum, were applied to the penis, and slight improvement was noted. perineum, Prostatic massage had no effect. He was sent home with the belief that this traumatic partial priapism would gradually subside with conservative therapy. However, when he was seen one
UROLOGY
/ APRIL 1975 / VOLUME
V, NUMBER4
week after injury the penis appeared slightly more erect, and at seventeen days following injury the penis was again at its initial state of an 80 per cent erection. Surgical intervention was elected at this time. Irrigation of the corpora cavernosa with a dilute heparin solution was successful in reducing the priapism slightly, but when the irrigating needles were removed the erection returned despite firm compression. Therefore, a bilateral corporosaphenous shunt was performed. In three to four hours following surgery the penis was 30 to 40 per cent erect, and at the time of discharge one week following surgery the penis was flaccid. At six months follow-up, the patient had not had any recurrence, however, he has not been noted to experience any spontaneous erections. Comment A discussion of the pathophysiology of priapism requires a review of the mechanism of normal erection and detumescence. Stieve in 193012 first described the muscular pillars or cushions lying longitudinally within the small arteries and veins of the penis. In 1952 Conti13 histologically confirmed the existence of these “polsters” and postulated that they act as arterial and venous valves under the influence of the lumbar sympathetics (Fig. 1). In the flaccid state the constricting arterial polsters distal to the arteriovenous anastomosis decrease the penile arterial caliber, and blood is shunted away from the corpora. Drainage is completed by relaxation of the venous polsters. Erection occurs following constriction of the polsters within the arteriovenous shunts and veins along with relaxation of the arterial cushions, resulting in engorgement of the corpora cavernosa. Recently, however, others have suggested that erection occurs with increased arterial flow rate alone and is independent of venous obstruction. I4 Idiopathic priapism refers to a pathologic erection which is unaccompanied by disease that may cause or maintain the condition. The first patient described falls within this category, and there is no apparent etiologic factor contributing to its occurrence. A urethral stricture was possibly related to the previous catheterizations and was probably unrelated to the episodic prolonged erections. on the other hand, is Secondary priapism, caused by factors directly or indirectly impairing the normal mechanisms of detumescence, such as in Case 2 following perineal trauma. Hematoma followed by corporal compression and perhaps
493
Deep dorsal
Corpora
cavernosa
Profundq
’
A. FIGURE 1.
‘-‘
a. ‘\\
Muscular
Efferent
Mechanism sf normal
v. erection and detumescence.
thrombosis subsequently led to a persistent erection that required an alternative to the normal venous outflow for relief. Whether normal erections will occur in this patient remains unknown. Priapism in children is rare and is most frequently caused by hematologic disorders. However, the condition has been reported to be related to congenital syphilis, l5 retroperitoneal fibrosarcoma,16 trauma,i’ and unknown causes.i8 Leukemic priapism is secondary to the blood abnormality and is probably related to the sludging of leukemic cells in the corpora cavernosa. Clinicians generally agree that therapy should be directed toward reduction of splenomegaly and decreasing the number of leukemic cells.1g*20 Likewise, children with priapism secondary to sickle cell disease are usually managed by measures directed at reduction of blood sludging within the vascular spaces21 However, shunting procedures have been used when conservative therapy was unsuccessful.” If conservative forms of management have been unsuccessful, surgical intervention should be instituted within seventy-two hours after the onset of priapism. Hinman23 reported that prolonged venous stasis results in irreversible fibrosis of the corpora cavernosa and increases the likelihood of impotence. Corporosaphenous
494
v.
BloodJow
in Jaccid penis (A) and in erect penis
bypass provides alternative venous drainage of the corpora cavernosa, thus relieving the painful engorgement of the penis. Its use is indicated in idiopathic and selected cases of secondary priapism. Northwestern
University Medical School 303 East Chicago Avenue Chicago, Illinois 60611 (DR. RESNICK)
References 1. CALLAWAY, T. : Unusual case of priapism, London Med. Repos. 21: 286 (1824). 2. MCKAY, R. W., and COLSTON, J. A. L.: Priapism: a new method of treatment, J. Urol. 19: 121 (1928). 3. BELL, W. R., and PILRAY, W. R.: Management of priapism by therapeutic defibrination, N. Engl. J. Med. 280: 649 (1969). 4. SMITH, K. H.: Priapism: use of Dicumarol in persistent priapism, J. Urol. 64: 400 (1950). 5. BURT, F. B., SCHIRMER, H. K., and SCOTT,W. W.: A new concept in the management ofpriapism, ibid. 83: 60 (1960). 6. ULM, A. H.: Treatment of primary priapism with Arfonad, ibid. 81: 291 (1959). 7. PRICE,R. A., and PENNA, 0. J. : Effectiveness of drugs in presenting postoperative penile erections, Surgery 24: 980 (1948). 8. ROLNICK, D., COTTRELL, T. L., and LLOYD, F.
UROLOGY I APRIL 1975 / VOLUME V. NUMBER4
9.
10.
11. 12.
13. 14.
15. 16.
A.: Priapism. Report of two cases and discussion of treatment, Ill. Med. J. 112: 129 (1957). GRAYHACK, J. T., MCCULLOUGH, W., O’CONOR, V. J., Venous by-pass to control JR., and TRIPPEL, 0.: priapism, Invest. Ural. 1: 509 (1964). QUACKELS, R.: Cure of a patient suffering from priapism by caverno-spongiosa anastomosis, Acta Urol. Belg. 32: 5 (1964). GALE, A. S.: Ketamine prevention of penile turgescence, J.A.M.A. 219: 1629 (1972). STIEVE, H. : Harn und Geschlachtsappart, in MiillenAnadorif, W. V., Ed. : Handbuch der mikroskopischen tomic des Menschen, Berlin, Springer-Verlag, 1930. CONTI, G.: L’erection du penis suman et ses bases morphologicovasculaires, Acta Anat. 14: 217 (1952). MALVER, T., BARON, T., and CLARK, S. S.: Assessment of potency with the Doppler flowmeter, Urology 2: 396 (1973). NUCKOLS, C. H.: Congenital syphilitic priapism, Louisville News, Cincinnati Clinic, 1876. LATTIMER, J. K.: The male genital tract, in Mustard,
UROLOGY
/
APRIL 1975 /
VOLUME V. NUMBER4
17. 18.
19.
20.
21.
22.
23.
W. T., et al., Eds. : Pediatric Surgery, 2nd ed., Chicago, Year Book Publishers, 1969, p. 1267. FRASEN, W. T.: A case of priapism, Br. Med. J. 2: 419 (1955). HOWE, G. E., PRENTISS, R. J., COLE, J, W., and MASTERS, R. H.: Priapism: a surgical emergency, J. Urol. 101: 576 (1969). SCHREIBMAN, S. M., GEE, T. S., and GRABSTALD, H. : Management of priapism in patients with chronic granulocytic leukemia, ibid. 111: 786 (1974). GRAIVIER, L., et al. : Priapism in a 7 year old infant with chronic granulocytic leukemia, ibid. 105: 137 (1971). ROTHFELD, S. u., and MAZOL, D.: Priapism in children: a complication of sickle cell disease, ibid. 105: 307 (1971). SNYDER, G. B., and WILSON, C. B.: Surgical management of priapism and its sequelae in sickle cell disease, South. Med. J. 59: 1393 (1966). HINMAN, F., JR.: Priapism: reasons for failure of therapy, J. Urol. 83: 420 (1960).
495
