- Email: [email protected]
Duplex Ultrasonography After Prostaglandin E1 Injection of the Clitoris in a Case of Hyperreactio Luteinalis
Val. 153. 1237-1238, April 1995 Printed in U.S.A.
DUPLEX ULTRASONOGRAPHY AFTER PROSTAGLANDIN E l INJECTION OF THE CLITORIS IN A CASE OF HYPERREACTIO LUTE INALIS EMRE AKKUS, SERGE CARRIER, CHARLES TURZAN, TO-NAO WANG
AND
TOM F. LUE*
From the Department of Urology, University of California School of Medicine, San Francisco and Department of Surgery, Kaiser Hospital, Oakland, California
ABSTRACT
We report an unusual case of persistent postpartum clitorimegaly due to ovarian hyperreactio luteinalis. Duplex ultrasonography of the clitoris after intracorporeal injection of prostaglandin E l revealed marked clitoral erection and increased arterial flow, as in the penis. KEYWORDS:clitoris, prostaglandins E,ultrasonography, pregnancy, testosterone Hyperreactio luteinalis is a moderate to severe polycystic benign ovarian disease seen rarely during pregnancy.'-6 It is generally the result of overproduction of ovarian stromal androgens secondary to the stimulation of elevated gonadotropins.'.2 Since the first description in 1938 by B ~ r g e ronly ,~ sporadic cases of hyperreactio luteinalis have been reported.' Marked maternal virilization may be accompanied by clitorimegaly, which usually resolves post partum and does not recur in subsequent p r e g n a n c i e ~ Unresolved . ~ ~ ~ ~ ~ ~clitori~ megaly due to hyperreactio luteinalis after pregnancy is an extremely infrequent We report an unusual case in which we injected the enlarged clitoris with prostaglandin E l and performed duplex ultrasonography to determine the hemodynamics of the clitoris. CASE REPORT
A 45-year-old woman (gravida 11, para 11) who had virilFIG. 1. Markedly enlarged 4 X 1 cm. clitoris due to hyperreactio ization during both pregnancies presented with marked clitoral enlargement and tenderness that were unresolved 15 luteinalis. years after the second pregnancy. Maternal virilization due to ovarian hyperreactio luteinalis was revealed by ovarian biopsies performed a t both deliveries, which were cesarean sections. During each pregnancy the patient complained of deepening of the voice, acne on the chest and back, and enlargement of the clitoris. Peripheral maternal total testosterone level increased to as high as 4,000 ng./dl. during the second pregnancy and was 10,000 ng./dl. at delivery. Umbilical cord blood testosterone levels remained within normal range. Neither female neonate showed any signs of virilization. Maternal signs of virilization completely resolved post partum except for persistent clitorimegaly. Clitoral enlargement and tenderness caused a severe interruption in sexual life. Physical examination revealed a markedly enlarged 4 x 1 cm. clitoris with no other accompanying virilization signs (fig. 1).Complete blood count, urinalysis and hormonal analysis, including testosterone level, were within normal range. Surgical correction of clitorimegaly with general anesthesia was elected. FIG. 2. Duplex ultrasonography of clitoris after intracorporeal inDuplex ultrasonography of the clitoris was performed before and after preoperative intracorporeal injection of 10 pg. jection of 10 pg. prostaglandin El with 41 cm. per second maximal systolic velocity. prostaglandin E l . In the flaccid state 2 corpora cavernosa were identified and erection occurred after injection, as in the penis. Arterial flow was increased with a maximal systolic velocity of 41 cm. per second (fig. 2). Intracavernous pressure was 45 mm. Hg and the erection was judged to be about 75% of a rigid penis. Clitoroplasty with sparing of the neurovascular bundle and glans was performed (fig. 3,A). No compliAccepted for publication August 19, 1994. cations were noted postoperatively. Clitorimegaly and tenSupported in part by Fonds de Recherche en Sante Quebec. * Re uests for reprints: Department of Urology, U-575,University derness were c jmpletely corrected (fig. 3,B ) and sexual life returned to normal 6 months postoperatively. of Caliqbrnia, San Francisco, California 94143-0738. 1237
1238
PROSTAGLANDIN E l INJECTION O F CLITORIS IN HYPERREACTIO LUTEINALIS
FIG.3. A, neurovascular bundles and glans of clitoris preoperatively. B, postoperative appearance of clitoris
DISCUSSION
Because hyperreactio luteinalis usually regresses sponta~3~ neously within a few weeks post p a r t ~ r n ' - ~ 3conservative treatment is generally p r e f e ~ ~ e d . ' However, .~.~ in our case a surgical approach for unresolved clitoral enlargement was necessary. To our knowledge no previous report describes postpartum persistent clitorimegaly that necessitated surgical correction of the clitoris. Duplex ultrasonography after injection of an intracavernous pharmacological vasoactive agent is widely used in the diagnosis of impotence." To our knowledge injection of prostaglandin El and duplex ultrasonography of the human clitoris have not been reported previously. Clitoral erection with arterial flow of 41 cm. per second after prostaglandin El injection demonstrated hemodynamic and physiological responses similar to those of the penis. Diederichs et al described a similar clitoral response in female dogs after stimulation of the cavernous nerves." We suggest that in select cases intracorporeal prostaglandin E 1 injection and duplex ultrasonography can be used effectively to gain an understanding of the hemodynamics of clitoral erection. ADDENDUM
Since our report was submitted for publication Drs. Robert Mevorach and Barry Kogan of University of California, San Francisco, have treated another patient in whom injection of a vasoactive agent (papaverine) was used as an aid to clitoroplaty. A 5-year-old girl with the adrenogenital syndrome and a 1.5 X 0.6 X 0.6 cm.clitoris was hospitalized for reduction clitoroplasty.With the patient under general anesthesia 0.2 ml. papaverine was injected into the side of the clitoris and a needle was inserted to measure intracorporeal pressure. After 10 minutes engorgement of the clitoris to twice the size was noted but intracorporeal pressure remained 0 mm. Hg.
REFERENCES
1. Wajda, K. J., Lucas, J. G. and Marsh, W. L., Jr.: Hyperreactio luteinalis. Benign disorder masquerading a s a n ovarian neoplasm. Arch. Path. Lab. Med., 113:921,1989. 2. VanSlooten, A. J.,Rechner, S. F. and Dodds, W. G.: Recurrent maternal virilization during pregnancy caused by benign androgen-producing ovarian lesions. Amer. J . Obst. Gynec., 167: 1342,1992. 3. Burger, K.:Bilateral ovarian lutein cysts associated with hydrops of fetus and placenta. Int. Congr. Verlask. Gynec., 2 440, 1938. 4. Fayez, J . A., Bunch, T. R. and Miller, G. L.: Virilization in pregnancy associated with polycystic ovary disease. Obst. Gynec., 44:511, 1974. 5. Magendantz, H. G.,Jones, D. E. and Schomberg, D. W.: Virilization during pregnancy associated with polycystic ovary disease. Obst. Gynec., 40 156,1972. 6. Clement, P. B.: Tumor-like lesions of the ovary associated with pregnancy. Int. J. Gynec. Path., 12: 108,1993. 7. Scully, R. E.: Multiple luteinized follicle cysts a n d o r corpora lutea. In: Tumors of the Ovary and Maldeveloped Gonads. Washington, D. C.: Armed Forces Institute of Pathology, pp. 377-379, 1979. 8. Okadome, M., Kaku,T., Tsukamoto, N., Saito, T., Matsukuma, K., Uehira, K., Hirakawa, T. and Nakono, H.: Hyperreactio luteinalis in normal singleton pregnancy. Int. J . Gynaec. Obst., 29 365,1989. 9. Berger, N.G., Repke, J. T. and Woodruff, D. J.: Markedly elevated serum testosterone in pregnancy without fetal virilization. Obst. Gynec., 63: 260, 1984. 10. Lue, T. F.,Hricak, H., Marich, K. W. and Tanagho, E. A,: Evaluation of arteriogenic impotence with intracorporeal injection of papaverine and the duplex ultrasound scanner. Sem. Urol., 3:43, 1985. 11. Diederichs, W., Lue, T. F. and Tanagho, E. A,: Clitoral response to cavernous nerve stimulation in dogs. Int. J . Impot. Res., 3: 7,1991.
1
DUPLEX ULTRASONOGRAPHY AFTER PROSTAGLANDIN E l INJECTION OF THE CLITORIS IN A CASE OF HYPERREACTIO LUTE INALIS EMRE AKKUS, SERGE CARRIER, CHARLES TURZAN, TO-NAO WANG
AND
TOM F. LUE*
From the Department of Urology, University of California School of Medicine, San Francisco and Department of Surgery, Kaiser Hospital, Oakland, California
ABSTRACT
We report an unusual case of persistent postpartum clitorimegaly due to ovarian hyperreactio luteinalis. Duplex ultrasonography of the clitoris after intracorporeal injection of prostaglandin E l revealed marked clitoral erection and increased arterial flow, as in the penis. KEYWORDS:clitoris, prostaglandins E,ultrasonography, pregnancy, testosterone Hyperreactio luteinalis is a moderate to severe polycystic benign ovarian disease seen rarely during pregnancy.'-6 It is generally the result of overproduction of ovarian stromal androgens secondary to the stimulation of elevated gonadotropins.'.2 Since the first description in 1938 by B ~ r g e ronly ,~ sporadic cases of hyperreactio luteinalis have been reported.' Marked maternal virilization may be accompanied by clitorimegaly, which usually resolves post partum and does not recur in subsequent p r e g n a n c i e ~ Unresolved . ~ ~ ~ ~ ~ ~clitori~ megaly due to hyperreactio luteinalis after pregnancy is an extremely infrequent We report an unusual case in which we injected the enlarged clitoris with prostaglandin E l and performed duplex ultrasonography to determine the hemodynamics of the clitoris. CASE REPORT
A 45-year-old woman (gravida 11, para 11) who had virilFIG. 1. Markedly enlarged 4 X 1 cm. clitoris due to hyperreactio ization during both pregnancies presented with marked clitoral enlargement and tenderness that were unresolved 15 luteinalis. years after the second pregnancy. Maternal virilization due to ovarian hyperreactio luteinalis was revealed by ovarian biopsies performed a t both deliveries, which were cesarean sections. During each pregnancy the patient complained of deepening of the voice, acne on the chest and back, and enlargement of the clitoris. Peripheral maternal total testosterone level increased to as high as 4,000 ng./dl. during the second pregnancy and was 10,000 ng./dl. at delivery. Umbilical cord blood testosterone levels remained within normal range. Neither female neonate showed any signs of virilization. Maternal signs of virilization completely resolved post partum except for persistent clitorimegaly. Clitoral enlargement and tenderness caused a severe interruption in sexual life. Physical examination revealed a markedly enlarged 4 x 1 cm. clitoris with no other accompanying virilization signs (fig. 1).Complete blood count, urinalysis and hormonal analysis, including testosterone level, were within normal range. Surgical correction of clitorimegaly with general anesthesia was elected. FIG. 2. Duplex ultrasonography of clitoris after intracorporeal inDuplex ultrasonography of the clitoris was performed before and after preoperative intracorporeal injection of 10 pg. jection of 10 pg. prostaglandin El with 41 cm. per second maximal systolic velocity. prostaglandin E l . In the flaccid state 2 corpora cavernosa were identified and erection occurred after injection, as in the penis. Arterial flow was increased with a maximal systolic velocity of 41 cm. per second (fig. 2). Intracavernous pressure was 45 mm. Hg and the erection was judged to be about 75% of a rigid penis. Clitoroplasty with sparing of the neurovascular bundle and glans was performed (fig. 3,A). No compliAccepted for publication August 19, 1994. cations were noted postoperatively. Clitorimegaly and tenSupported in part by Fonds de Recherche en Sante Quebec. * Re uests for reprints: Department of Urology, U-575,University derness were c jmpletely corrected (fig. 3,B ) and sexual life returned to normal 6 months postoperatively. of Caliqbrnia, San Francisco, California 94143-0738. 1237
1238
PROSTAGLANDIN E l INJECTION O F CLITORIS IN HYPERREACTIO LUTEINALIS
FIG.3. A, neurovascular bundles and glans of clitoris preoperatively. B, postoperative appearance of clitoris
DISCUSSION
Because hyperreactio luteinalis usually regresses sponta~3~ neously within a few weeks post p a r t ~ r n ' - ~ 3conservative treatment is generally p r e f e ~ ~ e d . ' However, .~.~ in our case a surgical approach for unresolved clitoral enlargement was necessary. To our knowledge no previous report describes postpartum persistent clitorimegaly that necessitated surgical correction of the clitoris. Duplex ultrasonography after injection of an intracavernous pharmacological vasoactive agent is widely used in the diagnosis of impotence." To our knowledge injection of prostaglandin El and duplex ultrasonography of the human clitoris have not been reported previously. Clitoral erection with arterial flow of 41 cm. per second after prostaglandin El injection demonstrated hemodynamic and physiological responses similar to those of the penis. Diederichs et al described a similar clitoral response in female dogs after stimulation of the cavernous nerves." We suggest that in select cases intracorporeal prostaglandin E 1 injection and duplex ultrasonography can be used effectively to gain an understanding of the hemodynamics of clitoral erection. ADDENDUM
Since our report was submitted for publication Drs. Robert Mevorach and Barry Kogan of University of California, San Francisco, have treated another patient in whom injection of a vasoactive agent (papaverine) was used as an aid to clitoroplaty. A 5-year-old girl with the adrenogenital syndrome and a 1.5 X 0.6 X 0.6 cm.clitoris was hospitalized for reduction clitoroplasty.With the patient under general anesthesia 0.2 ml. papaverine was injected into the side of the clitoris and a needle was inserted to measure intracorporeal pressure. After 10 minutes engorgement of the clitoris to twice the size was noted but intracorporeal pressure remained 0 mm. Hg.
REFERENCES
1. Wajda, K. J., Lucas, J. G. and Marsh, W. L., Jr.: Hyperreactio luteinalis. Benign disorder masquerading a s a n ovarian neoplasm. Arch. Path. Lab. Med., 113:921,1989. 2. VanSlooten, A. J.,Rechner, S. F. and Dodds, W. G.: Recurrent maternal virilization during pregnancy caused by benign androgen-producing ovarian lesions. Amer. J . Obst. Gynec., 167: 1342,1992. 3. Burger, K.:Bilateral ovarian lutein cysts associated with hydrops of fetus and placenta. Int. Congr. Verlask. Gynec., 2 440, 1938. 4. Fayez, J . A., Bunch, T. R. and Miller, G. L.: Virilization in pregnancy associated with polycystic ovary disease. Obst. Gynec., 44:511, 1974. 5. Magendantz, H. G.,Jones, D. E. and Schomberg, D. W.: Virilization during pregnancy associated with polycystic ovary disease. Obst. Gynec., 40 156,1972. 6. Clement, P. B.: Tumor-like lesions of the ovary associated with pregnancy. Int. J. Gynec. Path., 12: 108,1993. 7. Scully, R. E.: Multiple luteinized follicle cysts a n d o r corpora lutea. In: Tumors of the Ovary and Maldeveloped Gonads. Washington, D. C.: Armed Forces Institute of Pathology, pp. 377-379, 1979. 8. Okadome, M., Kaku,T., Tsukamoto, N., Saito, T., Matsukuma, K., Uehira, K., Hirakawa, T. and Nakono, H.: Hyperreactio luteinalis in normal singleton pregnancy. Int. J . Gynaec. Obst., 29 365,1989. 9. Berger, N.G., Repke, J. T. and Woodruff, D. J.: Markedly elevated serum testosterone in pregnancy without fetal virilization. Obst. Gynec., 63: 260, 1984. 10. Lue, T. F.,Hricak, H., Marich, K. W. and Tanagho, E. A,: Evaluation of arteriogenic impotence with intracorporeal injection of papaverine and the duplex ultrasound scanner. Sem. Urol., 3:43, 1985. 11. Diederichs, W., Lue, T. F. and Tanagho, E. A,: Clitoral response to cavernous nerve stimulation in dogs. Int. J . Impot. Res., 3: 7,1991.
1