- Email: [email protected]
Phallic Reconstruction in Cloacae Exstrophy
0022" 5347 /89/1422-0563$02,00/0 Vol. 142, Printed in
THE JOURNAL OF
Copyright© 1989 by AMERICAH UROLOGICA:..,
ASSOCll.'TI0:L~,
INC.
PHALLIC RECONSTRUCTION
CLOACAL EXSTROPHY
D. A. HUSMANN, G. A. McLORIE
AND
B. M. CHURCHILL
From the University of Texas Southwestern A1edical Center, Dallas, Texas, and The Hospital /or Sick Children, Toronto, Ontario, Canada
ABSTRACT
During the last 25 years 8 genotypic male subjects with doacal exstrophy have undergone phallic reconstruction. AU 8 patients had phallic inadequacy after completion of the reconstruction with penile lengths at or below 2 standard deviations below the mean. Of the patients 4 have reached puberty and 2 have documented physiological impotence by nocturnal penile tumescence testing. Three patients have required intensive psychiatric counseling, ranging from 18 months to 3 years because of feelings of sexual inadequacy. Of 2 patients who have attempted sexual relations 1 is unable to penetrate the vagina because of phallic size and 1 is presently engaged in successful vaginal intercourse. As a consequence of phallic inadequacy and its associated psychological trauma in these individuals we strongly recommend sexual conversion as part of the initial management plan. (J. Urol., part 2, 142: 563-564, 1989) The genotypic male patient with cloacal bladder exstrophy in our experience has had associated absent or diminuitive penis (30 per cent), or bifid phallic structures (70 per cent). 1 Although we usually perform sexual conversion on these individuals, as recommended Tank and Lindenauer in 1970, 2 we occasionally have maintained the male sex assignment in children with cloacal bladder exstrnphy associated with bifid lie structures when parents admnently demanded phallic reconstruction rather than sexual conversion. This review is presented to give the parents and physicians managing these individuals the proper perspective of the eventual functional outcome. MATERIALS AND METHODS
We evaluated 8 genotypic male patients 3 to 22 old with cloacal bladder exstrophy who were managed phallic reconstruction during the last 25 years. Of the patients 4 were prepubertal and 4 were postpubertal. In 3 cases reconstruction had been performed before the recommendation for sexual conversion-by Tank and Lindenauer, and 5 had undergone ohallic reconstruction following parenteral refusal to allow ~exual conversion. A retrospective review of all available medical, psychiatric and n-n,cM,rn,,a records along with current history and physical examinations were done. Stretched dorsal length from the pubic "'""'v,m, to the tip was noted in all 1.iaut,uc:,; and ret'.OI':le ~,,n=,>:,sar.!U
563
RESULTS
Urethroplasty with a 2-staged reconstruction technique resulted in significant complications requiring further operative repairs in 3 patients (38 per cent): 1 required closure of a urethrocutaneous fistula and 2 required multiple endoscopic procedures along with repeated attempts at urethral reconstruction for intractable stricutres. Unfortunately, 1 of these patients remains a technical failure and he has elected to pursue management of the outlet obstruction by permanent suprapubic tube placement. After completion of the phallic reconstruction stretched penile lengths were at or below 2 standard deviations below the mean on standard penile growth nomograms in all cases (see figure). Evaluations for pubertal changes revealed that 4 patients had Tanner stage VI pubic hair development. Serum testosterone determinations in these 4 individuals were within normal adult male limits with values ranging from 540 to 750 ng.f cc. Sexual histories revealed feelings of sexual inadequacy in all 4 patients, 3 of whom have undergone intensive psychiatric counseling, ranging from 18 months to 3 years. The remaining patient underwent a preliminary psychiatric investigation but he refused to participate in further counseling. In 2 of our postpubertal patients the sexual histories revealed co:mi:11e1;e absence of erectile capability, which was confirmed by nocturnal penile tumescence testing. Both patients had been born with coexisting cloacal exstrophy and lumbosacral myePhysical examinations revealed absence of S2-S4 dermatome se11sa,t1c,n and both refused sacral and
18
...................................... +2S.D.
16
E 14
2
.,:;:
0, !::
"'
.-~==-Mean
12
10
_J
.!?
·c., Q_
00
8
·············:····
••••
-2 S.D.
/_.'iii/
6 4
.................,...···········-g·······"·····························/
2 2
4
6
8
10
12
14 16
18 20 22 24
Age (years)
Standard penile length nomogram
564
HUSMANN, MCLORIE AND CHURCHILL
penile vascular testing that would have confirmed the etiology of the impotence. The remaining 2 postpubertal patients were capable of penile erections, which was confirmed by history and nocturnal penile tumescence documentation. Both have attempted sexual relationships: 1 is unable to penetrate the vagina due to phallic inadequacy, and 1 is married and engaging in successful vaginal intercourse. Unfortunately, 1 of these 2 patients has a chronic indwelling suprapubic tube for intractable urethral strictures. Semen analysis revealed aspermia with retrograde ejaculation. In the other patient 2 semen evaluations revealed sperm counts ranging from 20 to 40 million with normal motility and morphologic parameters. However, no documented history of paternity exists. One further note of concern regarding eventual fertility in this patient population is the frequent occurrence of cryptorchidism. Of 16 testes 13 (81 per cent) required orchiopexy because of failure of testicular descent.
cloacal exstrophy individual who was believed to have committed suicide as a result of phallic inadequacy. 8 In conclusion we wish to emphasize that, despite limited success enabling 25 per cent of our postpubertal patients to be capable of vaginal intercourse and fertility, the complications of phallic inadequacy and its associated psychological morbidity are substantial. We strongly recommend sexual conversion as part of the initial management plan of male subjects with cloacal exstrophy. REFERENCES 1. Husmann, D. A., McLorie, G. A. and Churchill, B. M.: Management
2. 3.
DISCUSSION
The purpose of this paper is to enable the physician to inform the parents adequately of the possible complications regarding failure to comply with the recommendations for sexual conversion in their male child with cloacal exstrophy. In reviewing our data we find that almost all patients had inadequate phallic size after completion of reconstruction. Furthermore, 50 per cent of the postpubertal patients were unable to obtain an erection, probably as a consequence of secondary neurological injury from the coexisting myelodysplasia. In addition, the psychiatric component of phallic inadequacy at maturity cannot be over stressed. All 4 of our postpubertal patients have had significant psychiatric morbidity. To stress this point further, Howell and associates reported on a genotypically male
4.
5. 6. 7. 8.
of the hindgut in cloacal exstrophy: terminal ileostomy versus colostomy. J. Ped. Surg., 23: 1107, 1988. Tank, E. S. and Lindenauer, S. M.: Principles of management of exstrophy of the cloaca. Amer. J. Surg., 119: 95, 1970. Lee, P. A., Mazur, T., Danish, R., Amrhein, J., Blizzard, R. M., Money, J. and Migeon, C. J.: Micropenis. I. Criteria, etiologies and classifications. Johns Hopkins Med. J., 146: 156, 1980. Underwood, L. E. and Van Wyck, J. J.: Hormones in normal and aberrant growth. In: Textbook of Endocrinology, 6th ed. Edited by R. H. Williams. Philadelphia: W. B. Saunders Co., chapt. 28, p. 1169, 1981. Johnston, J. H. and Kogan, S. J.: The exstrophic anomalies and their surgical reconstruction. Curr. Prob. Surg., pp. 1-39, Aug. 1974. Koff, S. A. and Eakins, M.: The treatment of penile chordee using corporeal rotation. J. Urol., 131: 931, 1984. Brzezinski, A., Homsy, Y. L. and Laberge, I.: Orthoplasty in epispadias. J. Urol., 136: 259, 1986. Howell, C., Caldamone, A., Snyder, H., Ziegler, M. and Duckett, J.: Optimal management of cloacal exstrophy. J. Ped. Surg., 18: 365, 1983.
THE JOURNAL OF
Copyright© 1989 by AMERICAH UROLOGICA:..,
ASSOCll.'TI0:L~,
INC.
PHALLIC RECONSTRUCTION
CLOACAL EXSTROPHY
D. A. HUSMANN, G. A. McLORIE
AND
B. M. CHURCHILL
From the University of Texas Southwestern A1edical Center, Dallas, Texas, and The Hospital /or Sick Children, Toronto, Ontario, Canada
ABSTRACT
During the last 25 years 8 genotypic male subjects with doacal exstrophy have undergone phallic reconstruction. AU 8 patients had phallic inadequacy after completion of the reconstruction with penile lengths at or below 2 standard deviations below the mean. Of the patients 4 have reached puberty and 2 have documented physiological impotence by nocturnal penile tumescence testing. Three patients have required intensive psychiatric counseling, ranging from 18 months to 3 years because of feelings of sexual inadequacy. Of 2 patients who have attempted sexual relations 1 is unable to penetrate the vagina because of phallic size and 1 is presently engaged in successful vaginal intercourse. As a consequence of phallic inadequacy and its associated psychological trauma in these individuals we strongly recommend sexual conversion as part of the initial management plan. (J. Urol., part 2, 142: 563-564, 1989) The genotypic male patient with cloacal bladder exstrophy in our experience has had associated absent or diminuitive penis (30 per cent), or bifid phallic structures (70 per cent). 1 Although we usually perform sexual conversion on these individuals, as recommended Tank and Lindenauer in 1970, 2 we occasionally have maintained the male sex assignment in children with cloacal bladder exstrnphy associated with bifid lie structures when parents admnently demanded phallic reconstruction rather than sexual conversion. This review is presented to give the parents and physicians managing these individuals the proper perspective of the eventual functional outcome. MATERIALS AND METHODS
We evaluated 8 genotypic male patients 3 to 22 old with cloacal bladder exstrophy who were managed phallic reconstruction during the last 25 years. Of the patients 4 were prepubertal and 4 were postpubertal. In 3 cases reconstruction had been performed before the recommendation for sexual conversion-by Tank and Lindenauer, and 5 had undergone ohallic reconstruction following parenteral refusal to allow ~exual conversion. A retrospective review of all available medical, psychiatric and n-n,cM,rn,,a records along with current history and physical examinations were done. Stretched dorsal length from the pubic "'""'v,m, to the tip was noted in all 1.iaut,uc:,; and ret'.OI':le ~,,n=,>:,sar.!U
563
RESULTS
Urethroplasty with a 2-staged reconstruction technique resulted in significant complications requiring further operative repairs in 3 patients (38 per cent): 1 required closure of a urethrocutaneous fistula and 2 required multiple endoscopic procedures along with repeated attempts at urethral reconstruction for intractable stricutres. Unfortunately, 1 of these patients remains a technical failure and he has elected to pursue management of the outlet obstruction by permanent suprapubic tube placement. After completion of the phallic reconstruction stretched penile lengths were at or below 2 standard deviations below the mean on standard penile growth nomograms in all cases (see figure). Evaluations for pubertal changes revealed that 4 patients had Tanner stage VI pubic hair development. Serum testosterone determinations in these 4 individuals were within normal adult male limits with values ranging from 540 to 750 ng.f cc. Sexual histories revealed feelings of sexual inadequacy in all 4 patients, 3 of whom have undergone intensive psychiatric counseling, ranging from 18 months to 3 years. The remaining patient underwent a preliminary psychiatric investigation but he refused to participate in further counseling. In 2 of our postpubertal patients the sexual histories revealed co:mi:11e1;e absence of erectile capability, which was confirmed by nocturnal penile tumescence testing. Both patients had been born with coexisting cloacal exstrophy and lumbosacral myePhysical examinations revealed absence of S2-S4 dermatome se11sa,t1c,n and both refused sacral and
18
...................................... +2S.D.
16
E 14
2
.,:;:
0, !::
"'
.-~==-Mean
12
10
_J
.!?
·c., Q_
00
8
·············:····
••••
-2 S.D.
/_.'iii/
6 4
.................,...···········-g·······"·····························/
2 2
4
6
8
10
12
14 16
18 20 22 24
Age (years)
Standard penile length nomogram
564
HUSMANN, MCLORIE AND CHURCHILL
penile vascular testing that would have confirmed the etiology of the impotence. The remaining 2 postpubertal patients were capable of penile erections, which was confirmed by history and nocturnal penile tumescence documentation. Both have attempted sexual relationships: 1 is unable to penetrate the vagina due to phallic inadequacy, and 1 is married and engaging in successful vaginal intercourse. Unfortunately, 1 of these 2 patients has a chronic indwelling suprapubic tube for intractable urethral strictures. Semen analysis revealed aspermia with retrograde ejaculation. In the other patient 2 semen evaluations revealed sperm counts ranging from 20 to 40 million with normal motility and morphologic parameters. However, no documented history of paternity exists. One further note of concern regarding eventual fertility in this patient population is the frequent occurrence of cryptorchidism. Of 16 testes 13 (81 per cent) required orchiopexy because of failure of testicular descent.
cloacal exstrophy individual who was believed to have committed suicide as a result of phallic inadequacy. 8 In conclusion we wish to emphasize that, despite limited success enabling 25 per cent of our postpubertal patients to be capable of vaginal intercourse and fertility, the complications of phallic inadequacy and its associated psychological morbidity are substantial. We strongly recommend sexual conversion as part of the initial management plan of male subjects with cloacal exstrophy. REFERENCES 1. Husmann, D. A., McLorie, G. A. and Churchill, B. M.: Management
2. 3.
DISCUSSION
The purpose of this paper is to enable the physician to inform the parents adequately of the possible complications regarding failure to comply with the recommendations for sexual conversion in their male child with cloacal exstrophy. In reviewing our data we find that almost all patients had inadequate phallic size after completion of reconstruction. Furthermore, 50 per cent of the postpubertal patients were unable to obtain an erection, probably as a consequence of secondary neurological injury from the coexisting myelodysplasia. In addition, the psychiatric component of phallic inadequacy at maturity cannot be over stressed. All 4 of our postpubertal patients have had significant psychiatric morbidity. To stress this point further, Howell and associates reported on a genotypically male
4.
5. 6. 7. 8.
of the hindgut in cloacal exstrophy: terminal ileostomy versus colostomy. J. Ped. Surg., 23: 1107, 1988. Tank, E. S. and Lindenauer, S. M.: Principles of management of exstrophy of the cloaca. Amer. J. Surg., 119: 95, 1970. Lee, P. A., Mazur, T., Danish, R., Amrhein, J., Blizzard, R. M., Money, J. and Migeon, C. J.: Micropenis. I. Criteria, etiologies and classifications. Johns Hopkins Med. J., 146: 156, 1980. Underwood, L. E. and Van Wyck, J. J.: Hormones in normal and aberrant growth. In: Textbook of Endocrinology, 6th ed. Edited by R. H. Williams. Philadelphia: W. B. Saunders Co., chapt. 28, p. 1169, 1981. Johnston, J. H. and Kogan, S. J.: The exstrophic anomalies and their surgical reconstruction. Curr. Prob. Surg., pp. 1-39, Aug. 1974. Koff, S. A. and Eakins, M.: The treatment of penile chordee using corporeal rotation. J. Urol., 131: 931, 1984. Brzezinski, A., Homsy, Y. L. and Laberge, I.: Orthoplasty in epispadias. J. Urol., 136: 259, 1986. Howell, C., Caldamone, A., Snyder, H., Ziegler, M. and Duckett, J.: Optimal management of cloacal exstrophy. J. Ped. Surg., 18: 365, 1983.