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Penile Lengthening After Previous Repair of Epispadias
0022-534 7/79/1214-0527$02. 00/0 Vol. 121, April
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1979 by The Williams & Wilkins Co.
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS W. HARDY HENDREN* From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
ABSTRACT
Unsatisfactory appearance and function of the penis are common after repair of complete epispadias. Reoperation can benefit the majority of these patients. The principles of repair in secondary cases as well as primary cases include wide mobilization of the corpora to gain length, resection of dorsal chordee to straighten the shaft, augmentation of urethral length with a graft, secure closure of the corpora over the urethra to avoid a fistula, wide mobilization of the mons to place hair-bearing skin in proper location and Z-plasty closure. There are many boys and young men with unsatisfactory appearance and function of the penis after an operation in early childhood for epispadias with or without bladder exstrophy. Although a boy may go through early childhood preoccupied with other matters, such as gaining urinary control or living with a urinary diversion bag depending on how the urinary tract has been managed, at puberty quite understandably he focuses great attention on the penis, especially if it is stubby and tethered upward to the lower abdominal wall. An operation can improve greatly the self-image of these patients as well as give them the ability to perform sexually, if additional penile length is gained and the penis can be made to suspend in a more normal manner.
ureteral reimplantation. This was done simultaneously in 3 patients and in the 1 patient with functionally closed bladder exstrophy the incontinence operation and penile reconstruction were done separately. In the remaining 13 patients only the penis was repaired. The usual anatomy in complete epispadias with incontinence consists of a cleft penis, the prepuce beneath the glans, a deficient bladder neck and separated pubic bones (fig. 1, A). Usually, the ureters are laterally placed, with a "hockey stick" configuration and vesicoureteral reflux. A vertical incision exposes the entire length of the urethra and interior of the bladder (fig. 1). The ureters are reimplanted by the cross-trigone advancement technique of Cohen (fig. 2, A and B). 7 The mucosa on the dorsum of the penis is mobilized by sharp dissection from the corpora (fig. 2, C). This maneuver alone frequently attains impressive length, especially when there is considerable scarring of this tissue from a previous closure. Most previous descriptions of epispadias repair simply rolled this tissue in as a urethral tube. 8 • n However, in some cases this can result in
THE ANATOMIC PROBLEM
Although there are many gradations in severity, in complete epispadias with or without bladder exstrophy the pelvic bones are separated, joined by the fibrous interpubic ligament. The penile corpora that attach to the pubic rami are pulled laterally, which causes shortening of penile length. In addition, there is dorsal chordee with upward tethering of the corpora to the interpubic ligament. Frequently, an operation during infancy has not corrected the attachment of the corpora to the pubic rami so that upward chordee remains. There is also postoperative scarring between the dorsum of the penis and the mons. Previous articles on this subject have emphasized that penile lengthening requires thorough mobilization of the corpora as a fundamental step in attaining adequate length. 1- 5 An experience with reoperation in 17 male subjects to improve appearance and function of the penis is described herein. Of these patients 14 had previous bladder exstrophy. Two were doing well with ureterosigmoidostomies, 10 had had a prior ileal loop and all but 1 were converted to a nonrefluxing colon conduit, which was still later joined to the rectosigmoid. 6 One patient had functionally closed bladder exstrophy, which was rudimentary and incontinent, and another had functionally closed exstrophy with continence. There were 3 patients with complete epispadias who had persisting incontinence.
Vertical incision
\ µ<;:.---~)( ·~o '.
r '.I//
A
bl.neck
• • •, ) • ,,,/ ·.J.\'I\I // / /
~-::.:::-:::.~-;.,, ii;~{~ \ : /
'"
/,Y ,
,,/
',<:_~Ureteral orifices too laterali Separated symphysis
short tunnels
Rectus
8
I nterpubic ligament
OPERATIVE TECHNIQUE
Sound in urethra
The operative steps used in the most extensive repair that may be needed, that is a case with epispadias and incontinence, are shown in figures 1 to 10. These patients require penile lengthening, reconstruction of the bladder outlet and Accepted for publication July 28, 1978. Read at annual meeting of American Urological Association, Washington, D. C., May 21-25, 1978. * Requests for reprints: Massachusetts General Hosopital, Boston, Massachusetts 02114. 527
Fm. 1
\
Small \ bladder I
528
HENDREN
tethering of the penis because, as emphasized by Horton and Devine, 10 and Williams, 11 this tissue is too short. This mobilization is carried back to the point at which the corpora diverge toward the pubic rami but not undermining the verumontanum, which risks cutting the ejaculatory ducts. A fine pointed cautery device is used for hemostasis. The groove between the corpora is incised deeply down to Buck's fascia on the underside of the penis to prepare a bed with ample room for a urethral graft (fig. 3, A). A tunnel is made through the distal glans, locating the meatus on the ventral aspect of the glans adjacent to the frenum. The skin is then mobilized completely from the corpora, leaving all of the subcutaneous adventitia with the skin to maintain skin flaps with adequate blood supply, laying bare the corpora (fig. 3, B). A vital aspect
A
of the repair consists in meticulous dissection of the corpora away from the pubic rami down to their insertion near the ischial tuberosities (fig. 4, A). Great care must be taken to avoid injury to the pudenda! vessels and nerves that emerge from Alcock's canal and run along the dorsum of the corpora. In some cases, with a lot of scar tissue along the upper aspect of the corpora, easy identification of these vessels and nerves may be difficult. In that circumstance it is best to carry the dissection along the underside of the corpora, approaching their insertion on the pubes laterally where there is less chance for injury to the vital structures. The spermatic cords lie just lateral to the field of dissection and they should be identified to prevent injury to them. After mobilizing the urethra, freeing the skin from the corpora and dissecting the corpora away from the pubic rami considerable length is attained in most cases (fig. 4, B). A tubed skin graft is inserted into the groove between the corpora and it is brought out Spermatic cord
I
Original position
mucosa
Corpus freed from pubic ramus
Int. pudenda!
art, vein, N
B Reim plants complete
.._
....
_- -Corpora longer after, .;,
~
f.)
"
mobilizing urethro
2. ) freeing skin of shoft
B
3. ) dissecting them oway from pubic rami and interpubic ligament ( avoiding vessels at base )
Fm. 2
Fm. 4
A
Incise groove between corpora to make bed for urethra
New meatus at tip; tunnel through distal glans
Fm. 3
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS
through the tunnel in the glans (fig. 5, A). This graft is taken using a Reese dermatome set at about 20/1,000 of an inch to obtain a nearly full thickness graft that will not have a tendency to contract. In practice we harvest a graft that is about twice as long and twice as wide as needed to be sure that it is large enough. It is taken from a non-hair-bearing area, such as the upper inner ann or the upper outer quadrant of the buttocks. If the donor site shows bare fat, as it may in a patient with thin skin, no attempt is made to suture the graft site. Instead, a thin graft (10/1,000) is taken from the opposite side of the arm and laid on the donor site which will soon heal. When the graft is laid into its bed between the corpora the suture line should lie posteriorly against the intact Buck's fascia to minimize the chance for fistula formation. The patient's urethra is closed with inverting sutures (fig. 5, B). In .~nostomosis of graft to urethra
Thick free graft
Silostic stent ( many holes ) Interrupted sutures Running inverting suture to tubulorize graft Triangles of mucosa to remove Close urethra
B
Trim graft ond complete meotus
cases with bladder incontinence a Leadbetter bladder neck narrowing and plication procedure is done. 12 The 2 halves of the glans are approximated with horizontal mattress sutures; the skin at the edges of the glans must be mobilized and everted to expose enough glans tissue to approximate accurately and obtain a rounded glans of normal appearance (fig. 6, A and B). Most of the secondary cases we have seen had an unsightly cleft on the dorsum of the glans because the 2 halves had not been brought together properly. The 2 corpora are then closed over the graft. This closure is carried back to the point at which they diverge, burying the graft and its anastomosis of the end of the patient's urethra. Mucosa of the bladder neck segment is resected (fig. 6, C) and an overlapping closure of muscle is accomplished (fig. 7). The interpubic ligament is rejoined with several heavy sutures, which is facilitated by lateral compression of the patient's hips (fig. 8). A suprapubic tube is placed. Anatomic approximation of the pubic bones in the midline is not done, since it is not necessary and can lead to shortening of the corpora. In some cases the pubic bones will be pulled downward and inward if they are rotated toward the midline. The skin of the shaft is mobilized and the frenum is opened out and creating Byar's flaps to wrap around the distal shaft (fig. 9). The skin flaps are incised to form multiple triangular flaps to be interdigitated across the dorsum of the penis (fig. 10, _A). This a greater length of skin and avoids a straight line closure that can tether the penis upward. As this closure is carried upwards to the mons, the skin and subcutaneous fat of the mons are undermined far laterally to the anterior superior iliac spine to bring ample skin and subcutaneous fat into the mons area (fig. 10, B). This hair-bearing skin will give a more normal pubic escutcheon. Otherwise, these patients have a bald area just above the penis, with the hair diverging abnormally on each side. Before these skin flaps are brought together the subcutaneous fat beneath them can be separated and brought together in an overlapping fashion over the symphysis to fill in that space, which lessens the likelihood of fistula formation from the bladder or urethra. Another technique for filling in the mons and getting normal hair distribution is by rotating a sizable pedicle flap of lower abdominal wall from each lower quadrant. 13 Drainage is provided sump suction catheters. The penis is dressed with a circumferential, gentle compression bandage. The tip is suspended from the end of an inverted plastic cup that is placed over it. The tip of the penis, which protrudes from the dressing, is covered with a thick layer of antibiotic ointment to keep the air away from it. This prevents crusting of secretions around the meatus, which can block serous drainage and can result in infection along the graft. CASE REPORTS
Fm. 5
A
Case 1 . The patient was seen as a neonate with bladder
Bringing halves of glans together (horizontal mattress sutures)
Inverting closure of mucosa strip
j C
Close corpora over graft
B
Triangle of mucosa excised on each side
Fm. 6
530
HENDREN
B Overlapping muscle closure
Fm. 7
I nterpubic ligament rejoined with braided wire sutures
Completed bladder closure
Fm. 8
exstrophy in 1960. The bladder was rudimentary (fig. 11, A). When the child was 1 year old an ilea! loop urinary diversion :was done (in retrospect unfortunate and certainly not a method in current use). Penile repair and cystectomy were done when he was 18 months old. The mucosa on the dorsum of the corpora was rolled into a urethral tube but no tissue was added to it. When the boy was 15 years old the result was unsatisfactory (fig. 11. Bl. Although the glans was adequate there was dorsal tethering at the base of the penis caused by straight line closure plus inadequate mobilization of the corpora and failure to elongate the urethra. These maneuvers were not a part of the repair in those days. The ilea! loop was removed when the boy was 12 years old, because there was progressive upper tract deterioration seen in so many longterm ilea! loop diversions, 10 and a non-refluxing colon conduit was done instead. However, because the upper tracts were dilated, it was elected not to risk hooking the non-refluxing colon conduit into the fecal stream at a later date in this case. At age 15 years the patient underwent an extensive re-repair, using the principles shown in figures 1 to 10, which resulted in a much more normal appearance of the penis (fig. 11. C and Dl and a happier boy. Case 2 (figs. 12 and 13). A 20-year-old man was referred for secondary penile reconstruction and further management of
the urinary tract. During infancy primary functional closure of the bladder had been done but he was incontinent. Later a ureterosigmoidostomy was done. After multiple bouts of pyelonephritis an ileal loop diversion was done but there was progressive dilatation of the upper tracts through the years. Therefore, the ileal loop was removed and a non-refluxing colon conduit was substituted. The upper tract improved and 18 months later the colon conduit was anastomosed to the rectosigmoid. The patient then elected to have the penis revised, since attempted intercourse had proved to be impossible. The operation consisted of the principles outlined in figures 1 to 10, in addition to grafting the shaft of the penis with a relatively thick (18/1,000 of an inch) Thiersch's graft taken from the buttocks because there was inadequate local skin to cover the shaft. Revision of the penis improved the self-image and made sexual activity possible. Marriage is contemplated. It has been now more than 3 years since the staged ureterosigmoid diversion and penile repair, and the excretory urogram (IVP) is satisfactory and stable. Case 3 (fig. 14). A 16-year-old boy was referred for further genital operation and possible removal of an ileal conduit. During infancy primary bladder closure had been done but this was followed by hydronephrosis and upper tract deterioration. When he was 4 years old an ileal conduit was done. The ilea! loop was removed and a non-refluxing colon conduit was created. Nine months later genital revision was done. A fistula on the dorsum of the penis was circumscribed, mobilizing the urethra that lay proximal to it. The corpora were mobilized widely. A free skin graft was inserted between the patient's short urethral segment and the tip of the penis. The skin of the shaft and mans was rearranged and closed with the Z-plasty technique, which was satisfactory. The colon conduit was taken down from the abdominal wall and anastomosed to the rectosigmoid. At followup 1 year later the patient is well. Case 4 (figs. 15 and 16). A 6-year-old boy was referred for correction of incontinence and revision of the genitalia. He was born with complete epispadias and incontinence and 2 gracilis sling procedures had been unsuccessful. A cystogram showed a small capacity bladder and no bladder neck (fig. 16, A). An IVP was normal. In a single lengthy procedure complete repair was done, incorporating all of the steps shown in figures 1 to 10, that is penile lengthening, creation of a distal urethra with skin graft, ureteral reimplantation, tubularization and narrowing of the bladder neck. A cystogram 1 month later showed satisfactory bladder capacity, a more
Resutu red interpubic ligament
C Skin mobilized completely from shaft, and ventral prepuce opened
Incision to make lateral flaps
FIG. 9
B
Subcutcmeous sump suction
t
Completed vertical closure
Sump suction in prevesicol space
FIG. 10
532
HENDREN
The principles outlined for secondary genital repair are equally applicable to new cases, as will be illustrated in a final case. Case 5 (fig. 17). A 3-year-old boy with complete epispadias was referred for penile repair. It was not certain whether there was total urinary incontinence, although it seemed likely. Initially, the penis was repaired but nothing was done to the bladder neck. At the first stage dorsal chordee was removed and preputial skin was transferred to the dorsum to provide additional length for creating a satisfactory penile urethra. At the second stage the corpora were mobilized widely, accomplishing skin closure by a multiple Z-plasty technique as shown in figures 1 to 10. (Today this would be done in a single operation, using a free graft to lengthen the urethra.) When the boy was 8 years old the bladder neck was narrowed to improve continence but there was still some stress incontinence. Therefore, this was repeated 1 year later,
FIG. 11. Case 1. A, appearance of genitalia as neonate. Rudimentary bladder is not suited for functional closure. B, when patient was 15 years old glans was satisfactory but note marked tethering upward to abdominal wall by dense scar at base of penis. C, after penile lengthening, which included insertion of free graft for distal urethra, note multiple Z-plasty closure of skin over shaft and mons. D, penis hangs in normal manner and there is better hair distribution 3 years postoperatively.
FIG. 13. Case 2, 3 years later. A, patient passing No. 16 Hegar dilator into graft urethra to demonstrate its patency. B, satisfactory appearance of urethral meatus at tip of penis. Pale color of skin graft covering undersurface of shaft is apparent. C, penis hangs normally with adequate size and length for intercourse.
FIG. 12. Case 2. A, note stubby appearance of penis, which lies upward against abdominal wall, precluding intercourse. B, manually pulling penis downward reveals inadequate length and diffuse scar of dorsum of penis and mons. Note cleft glans. Penis with this appearance causes severe psychological problems. C, intraoperative photograph shows elongated corpora and tubed skin graft to elongate urethra (for ejaculation). Corpora are then closed over urethral graft to bury it. D, since there was insufficient skin to cover shaft relatively thick (18/1,000 inch) Thiersch's graft was applied. Note Zplasty closure of mons with scar replaced by hair-bearing skin with subcutaneous fat beneath it and satisfactory length of shaft.
~ormal appearance of the prostatic urethra and bladder neck, and satisfactory caliber of the grafted distal urethra (fig. 16, B). Surprisingly, voluntary control of the urine was accomplished 2 months after this extensive revision.
FIG. 14. Case 3. A, preoperatively, penis tethered to abdominal wall is being held down with sound in previously tubularized urethra. Note fistula. B and C, postoperative views 3 months after secondary penile repair.
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS
533
flap of the prepuce. :i In certain cases it is best to be prepared to use any one of these well established principles of plastic surgery according to what skin is available. In most cases the penis hangs down in a normal position. Erection is at an angle that permits intercourse, whereas some male subjects with
FIG. 15. Case 4. A, preoperatively, there were scars on leg and mans secondary to gracilis sling operations. Penis is essentially unrepaired. Note prominent preputial skin hanging from undersurface which will be used to resurface shaft. penis, urethra and bladder are opened to begin repair. been mobilized. Free skin graft for distal urethra is to be anastomosed to patient's urethral which has been proximally. Ureters have been reimplanted by Cohen technique. D, conclusion of operation.
making the bladder neck more narrow and tubularizing it to a higher level. The ureters also were reimplanted at a higher point in the trigone. Slowly, the patient gained complete urinary continence during the next 4 years. Penile appearance was satisfactory. The patient is 17 years old and has normal sexual function. DISCUSSION
The operative approach described herein for these difficult problems consists of long established surgical principles. As emphasized previously others there must be wide mobilization of the corpora from the pubic rami and the interpubic ligament. 1- 5 , 11 I have not dared to detach completely the corpora from the pubis, leaving them attached only at the neurovascular bundles as described by and Eraklis,-, for fear oflosing the blood supply to the penis, which would be an utter catastrophy. Although perhaps the shaft can be made somewhat longer by that technique I have found that reasonable length can be attained without completely detaching the corpora and without running the risk of losing all by trying to get an extra bit oflength. When lengthening is performed it is possible to leave the urethral opening at the base of the penis. However, I prefer to construct simultaneously a distal urethra using a free graft because these patients have the potential to ejaculate and this is better through the tip of the penis than on the mons. A free graft technique has been used by some surgeons for hypospadias since before the turn of the century. 10 A free graft must be relatively thick, since a thin one will contract. I have taught these patients or a parent to pass a Hegar dilator gently through the graft each day for several months postoperatively to be certain that there is no problem with stenosis. Perhaps this is unnecessary since no thick graft has become stenotic. Skin from the prepuce can be used to make the graft but I prefer to save that for shaft closure. I also have used bladder mucosa when functional use of the bladder was not contemplated. The force of ejaculation in these patients depends on whether there is a bladder remnant proximal to the verumontanum, which can cause retrograde ejaculation. Multiple Z-plasty closure of the dorsum of the penis usually is better than straight line closure, since it gives greater length and is less apt to contract. A Z-plasty scar is less noticeable in time, in contrast to a straight line closure that can form a bridle-like scar. Johnston and Kogan described other techniques to avoid straight line closure, includh1g \l-Y va,,u""""' of the skin and use of a reverse Nesbit
FIG. 16. Case 4. A, preoperative cystogram shows wide bladder neck and prostatic urethra as well as small capacity of bladder. B, voiding cystourethrogram by percutaneous suprapubic needle 6 weeks postoperatively demonstrates normal appearance of bladder neck, which has been tubularized and patent grafted distal urethra.
FIG. 17. Case 5-new case of compiete epispadias with incontinence repaired using principles outlined. A, preoperative appearance when patient was 3 years old. B, patient at age 17 years has satisfactory sexual function and continence.
534
HENDREN
tethering of the penis to the abdominal wall find that impossible. If there is too little skin to cover the shaft a free graft can be used, as shown in case 2. The initial cosmetic result of a free graft is not pleasing in some cases, since hypertrophy of the scar can occur at the seams. This softens in time and as the graft becomes more supple its appearance becomes acceptable. The color match of grafted skin is not perfect but these patients have not seemed to be worried much about that. Use of these long established principles can give a better initial repair in boys with epispadias, as well as greatly improve the appearance and function in patients when the initial result proves unsatisfactory.
6. 7. 8. 9. 10. 11.
REFERENCES
1. Johnston, J. H.: Lengthening of the congenital or acquired short penis. Brit. J. Urol., 46: 685, 1974. 2. Johnston, J. H.: The genital aspects of exstrophy. J. Urol., 113: 701, 1975. 3. Johnston, J. H. and Kogan, S. J.: Monograph: the exstrophic anomalies and their surgical reconstruction. In: Current Problems in Surgery. Chicago: Year Book Medical Publishers, Inc., 1974. 4. Tanagho, E. A.: Male epispadias: surgical repairs ofurethropen. ile deformity. Brit. J. Urol., 48: 127, 1976. 5. Kelley, J. H. and Eraklis, A. J.: A procedure for lengthening
12. 13. 14. 15.
the phallus in boys with exstrophy of the bladder. J. Ped. Surg., 6: 645, 1971. Hendren, W. H.: Exstrophy of the bladder-an alternative method of management. J. Urol., 115: 195, 1976. Cohen, S. J.: Ureterozystoneostomie: Eine neue antireflux technik. Aktuel. Urol., 6: 1, 1975. Gross, R. E. and Cresson, S. L.: Treatment of epispadias: a report of18 cases. J. Urol., 68: 477, 1952. Culp, 0. S.: Treatment of epispadias with and without urinary incontinence: experience with 46 patients. J. Urol., 109: 120, 1973. Horton, C. E. and Devine, C. J., Jr.: Hypospadias and epispadias. Clinical symposia. CIBA, vol. 24, No. 3, 1972. Williams, D. I.: Epispadias and exstrophy. In: Surgical Pediatric Urology. Edited by H. B. Eckstein, R. Hohenfellner and D. I. Williams. Stuttgart: Georg Thieme Publishers, pp. 298-312, 1977. Leadbetter, G. W. and Fraley, E. E.: Surgical correction for total urinary incontinence: 5 years after. J. Urol., 97: 869, 1967. Allen, T. D., Spence, H. M. and Salyer, K. E.: Reconstruction of the external genitalia in exstrophy of the bladder: preliminary communication. J. Urol., 111: 830, 1974. Middleton, A. W., Jr. and Hendren, W. H.: Ileal conduits in children at the Massachusetts General Hospital from 1955 to 1970. J. Urol., 115: 591, 1976. Horton, C. E.: Plastic and Reconstructive Surgery of the Genital Area. Boston: Little, Brown & Co., 1973.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1979 by The Williams & Wilkins Co.
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS W. HARDY HENDREN* From the Division of Pediatric Surgery, Massachusetts General Hospital and the Department of Surgery, Harvard Medical School, Boston, Massachusetts
ABSTRACT
Unsatisfactory appearance and function of the penis are common after repair of complete epispadias. Reoperation can benefit the majority of these patients. The principles of repair in secondary cases as well as primary cases include wide mobilization of the corpora to gain length, resection of dorsal chordee to straighten the shaft, augmentation of urethral length with a graft, secure closure of the corpora over the urethra to avoid a fistula, wide mobilization of the mons to place hair-bearing skin in proper location and Z-plasty closure. There are many boys and young men with unsatisfactory appearance and function of the penis after an operation in early childhood for epispadias with or without bladder exstrophy. Although a boy may go through early childhood preoccupied with other matters, such as gaining urinary control or living with a urinary diversion bag depending on how the urinary tract has been managed, at puberty quite understandably he focuses great attention on the penis, especially if it is stubby and tethered upward to the lower abdominal wall. An operation can improve greatly the self-image of these patients as well as give them the ability to perform sexually, if additional penile length is gained and the penis can be made to suspend in a more normal manner.
ureteral reimplantation. This was done simultaneously in 3 patients and in the 1 patient with functionally closed bladder exstrophy the incontinence operation and penile reconstruction were done separately. In the remaining 13 patients only the penis was repaired. The usual anatomy in complete epispadias with incontinence consists of a cleft penis, the prepuce beneath the glans, a deficient bladder neck and separated pubic bones (fig. 1, A). Usually, the ureters are laterally placed, with a "hockey stick" configuration and vesicoureteral reflux. A vertical incision exposes the entire length of the urethra and interior of the bladder (fig. 1). The ureters are reimplanted by the cross-trigone advancement technique of Cohen (fig. 2, A and B). 7 The mucosa on the dorsum of the penis is mobilized by sharp dissection from the corpora (fig. 2, C). This maneuver alone frequently attains impressive length, especially when there is considerable scarring of this tissue from a previous closure. Most previous descriptions of epispadias repair simply rolled this tissue in as a urethral tube. 8 • n However, in some cases this can result in
THE ANATOMIC PROBLEM
Although there are many gradations in severity, in complete epispadias with or without bladder exstrophy the pelvic bones are separated, joined by the fibrous interpubic ligament. The penile corpora that attach to the pubic rami are pulled laterally, which causes shortening of penile length. In addition, there is dorsal chordee with upward tethering of the corpora to the interpubic ligament. Frequently, an operation during infancy has not corrected the attachment of the corpora to the pubic rami so that upward chordee remains. There is also postoperative scarring between the dorsum of the penis and the mons. Previous articles on this subject have emphasized that penile lengthening requires thorough mobilization of the corpora as a fundamental step in attaining adequate length. 1- 5 An experience with reoperation in 17 male subjects to improve appearance and function of the penis is described herein. Of these patients 14 had previous bladder exstrophy. Two were doing well with ureterosigmoidostomies, 10 had had a prior ileal loop and all but 1 were converted to a nonrefluxing colon conduit, which was still later joined to the rectosigmoid. 6 One patient had functionally closed bladder exstrophy, which was rudimentary and incontinent, and another had functionally closed exstrophy with continence. There were 3 patients with complete epispadias who had persisting incontinence.
Vertical incision
\ µ<;:.---~)( ·~o '.
r '.I//
A
bl.neck
• • •, ) • ,,,/ ·.J.\'I\I // / /
~-::.:::-:::.~-;.,, ii;~{~ \ : /
'"
/,Y ,
,,/
',<:_~Ureteral orifices too laterali Separated symphysis
short tunnels
Rectus
8
I nterpubic ligament
OPERATIVE TECHNIQUE
Sound in urethra
The operative steps used in the most extensive repair that may be needed, that is a case with epispadias and incontinence, are shown in figures 1 to 10. These patients require penile lengthening, reconstruction of the bladder outlet and Accepted for publication July 28, 1978. Read at annual meeting of American Urological Association, Washington, D. C., May 21-25, 1978. * Requests for reprints: Massachusetts General Hosopital, Boston, Massachusetts 02114. 527
Fm. 1
\
Small \ bladder I
528
HENDREN
tethering of the penis because, as emphasized by Horton and Devine, 10 and Williams, 11 this tissue is too short. This mobilization is carried back to the point at which the corpora diverge toward the pubic rami but not undermining the verumontanum, which risks cutting the ejaculatory ducts. A fine pointed cautery device is used for hemostasis. The groove between the corpora is incised deeply down to Buck's fascia on the underside of the penis to prepare a bed with ample room for a urethral graft (fig. 3, A). A tunnel is made through the distal glans, locating the meatus on the ventral aspect of the glans adjacent to the frenum. The skin is then mobilized completely from the corpora, leaving all of the subcutaneous adventitia with the skin to maintain skin flaps with adequate blood supply, laying bare the corpora (fig. 3, B). A vital aspect
A
of the repair consists in meticulous dissection of the corpora away from the pubic rami down to their insertion near the ischial tuberosities (fig. 4, A). Great care must be taken to avoid injury to the pudenda! vessels and nerves that emerge from Alcock's canal and run along the dorsum of the corpora. In some cases, with a lot of scar tissue along the upper aspect of the corpora, easy identification of these vessels and nerves may be difficult. In that circumstance it is best to carry the dissection along the underside of the corpora, approaching their insertion on the pubes laterally where there is less chance for injury to the vital structures. The spermatic cords lie just lateral to the field of dissection and they should be identified to prevent injury to them. After mobilizing the urethra, freeing the skin from the corpora and dissecting the corpora away from the pubic rami considerable length is attained in most cases (fig. 4, B). A tubed skin graft is inserted into the groove between the corpora and it is brought out Spermatic cord
I
Original position
mucosa
Corpus freed from pubic ramus
Int. pudenda!
art, vein, N
B Reim plants complete
.._
....
_- -Corpora longer after, .;,
~
f.)
"
mobilizing urethro
2. ) freeing skin of shoft
B
3. ) dissecting them oway from pubic rami and interpubic ligament ( avoiding vessels at base )
Fm. 2
Fm. 4
A
Incise groove between corpora to make bed for urethra
New meatus at tip; tunnel through distal glans
Fm. 3
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS
through the tunnel in the glans (fig. 5, A). This graft is taken using a Reese dermatome set at about 20/1,000 of an inch to obtain a nearly full thickness graft that will not have a tendency to contract. In practice we harvest a graft that is about twice as long and twice as wide as needed to be sure that it is large enough. It is taken from a non-hair-bearing area, such as the upper inner ann or the upper outer quadrant of the buttocks. If the donor site shows bare fat, as it may in a patient with thin skin, no attempt is made to suture the graft site. Instead, a thin graft (10/1,000) is taken from the opposite side of the arm and laid on the donor site which will soon heal. When the graft is laid into its bed between the corpora the suture line should lie posteriorly against the intact Buck's fascia to minimize the chance for fistula formation. The patient's urethra is closed with inverting sutures (fig. 5, B). In .~nostomosis of graft to urethra
Thick free graft
Silostic stent ( many holes ) Interrupted sutures Running inverting suture to tubulorize graft Triangles of mucosa to remove Close urethra
B
Trim graft ond complete meotus
cases with bladder incontinence a Leadbetter bladder neck narrowing and plication procedure is done. 12 The 2 halves of the glans are approximated with horizontal mattress sutures; the skin at the edges of the glans must be mobilized and everted to expose enough glans tissue to approximate accurately and obtain a rounded glans of normal appearance (fig. 6, A and B). Most of the secondary cases we have seen had an unsightly cleft on the dorsum of the glans because the 2 halves had not been brought together properly. The 2 corpora are then closed over the graft. This closure is carried back to the point at which they diverge, burying the graft and its anastomosis of the end of the patient's urethra. Mucosa of the bladder neck segment is resected (fig. 6, C) and an overlapping closure of muscle is accomplished (fig. 7). The interpubic ligament is rejoined with several heavy sutures, which is facilitated by lateral compression of the patient's hips (fig. 8). A suprapubic tube is placed. Anatomic approximation of the pubic bones in the midline is not done, since it is not necessary and can lead to shortening of the corpora. In some cases the pubic bones will be pulled downward and inward if they are rotated toward the midline. The skin of the shaft is mobilized and the frenum is opened out and creating Byar's flaps to wrap around the distal shaft (fig. 9). The skin flaps are incised to form multiple triangular flaps to be interdigitated across the dorsum of the penis (fig. 10, _A). This a greater length of skin and avoids a straight line closure that can tether the penis upward. As this closure is carried upwards to the mons, the skin and subcutaneous fat of the mons are undermined far laterally to the anterior superior iliac spine to bring ample skin and subcutaneous fat into the mons area (fig. 10, B). This hair-bearing skin will give a more normal pubic escutcheon. Otherwise, these patients have a bald area just above the penis, with the hair diverging abnormally on each side. Before these skin flaps are brought together the subcutaneous fat beneath them can be separated and brought together in an overlapping fashion over the symphysis to fill in that space, which lessens the likelihood of fistula formation from the bladder or urethra. Another technique for filling in the mons and getting normal hair distribution is by rotating a sizable pedicle flap of lower abdominal wall from each lower quadrant. 13 Drainage is provided sump suction catheters. The penis is dressed with a circumferential, gentle compression bandage. The tip is suspended from the end of an inverted plastic cup that is placed over it. The tip of the penis, which protrudes from the dressing, is covered with a thick layer of antibiotic ointment to keep the air away from it. This prevents crusting of secretions around the meatus, which can block serous drainage and can result in infection along the graft. CASE REPORTS
Fm. 5
A
Case 1 . The patient was seen as a neonate with bladder
Bringing halves of glans together (horizontal mattress sutures)
Inverting closure of mucosa strip
j C
Close corpora over graft
B
Triangle of mucosa excised on each side
Fm. 6
530
HENDREN
B Overlapping muscle closure
Fm. 7
I nterpubic ligament rejoined with braided wire sutures
Completed bladder closure
Fm. 8
exstrophy in 1960. The bladder was rudimentary (fig. 11, A). When the child was 1 year old an ilea! loop urinary diversion :was done (in retrospect unfortunate and certainly not a method in current use). Penile repair and cystectomy were done when he was 18 months old. The mucosa on the dorsum of the corpora was rolled into a urethral tube but no tissue was added to it. When the boy was 15 years old the result was unsatisfactory (fig. 11. Bl. Although the glans was adequate there was dorsal tethering at the base of the penis caused by straight line closure plus inadequate mobilization of the corpora and failure to elongate the urethra. These maneuvers were not a part of the repair in those days. The ilea! loop was removed when the boy was 12 years old, because there was progressive upper tract deterioration seen in so many longterm ilea! loop diversions, 10 and a non-refluxing colon conduit was done instead. However, because the upper tracts were dilated, it was elected not to risk hooking the non-refluxing colon conduit into the fecal stream at a later date in this case. At age 15 years the patient underwent an extensive re-repair, using the principles shown in figures 1 to 10, which resulted in a much more normal appearance of the penis (fig. 11. C and Dl and a happier boy. Case 2 (figs. 12 and 13). A 20-year-old man was referred for secondary penile reconstruction and further management of
the urinary tract. During infancy primary functional closure of the bladder had been done but he was incontinent. Later a ureterosigmoidostomy was done. After multiple bouts of pyelonephritis an ileal loop diversion was done but there was progressive dilatation of the upper tracts through the years. Therefore, the ileal loop was removed and a non-refluxing colon conduit was substituted. The upper tract improved and 18 months later the colon conduit was anastomosed to the rectosigmoid. The patient then elected to have the penis revised, since attempted intercourse had proved to be impossible. The operation consisted of the principles outlined in figures 1 to 10, in addition to grafting the shaft of the penis with a relatively thick (18/1,000 of an inch) Thiersch's graft taken from the buttocks because there was inadequate local skin to cover the shaft. Revision of the penis improved the self-image and made sexual activity possible. Marriage is contemplated. It has been now more than 3 years since the staged ureterosigmoid diversion and penile repair, and the excretory urogram (IVP) is satisfactory and stable. Case 3 (fig. 14). A 16-year-old boy was referred for further genital operation and possible removal of an ileal conduit. During infancy primary bladder closure had been done but this was followed by hydronephrosis and upper tract deterioration. When he was 4 years old an ileal conduit was done. The ilea! loop was removed and a non-refluxing colon conduit was created. Nine months later genital revision was done. A fistula on the dorsum of the penis was circumscribed, mobilizing the urethra that lay proximal to it. The corpora were mobilized widely. A free skin graft was inserted between the patient's short urethral segment and the tip of the penis. The skin of the shaft and mans was rearranged and closed with the Z-plasty technique, which was satisfactory. The colon conduit was taken down from the abdominal wall and anastomosed to the rectosigmoid. At followup 1 year later the patient is well. Case 4 (figs. 15 and 16). A 6-year-old boy was referred for correction of incontinence and revision of the genitalia. He was born with complete epispadias and incontinence and 2 gracilis sling procedures had been unsuccessful. A cystogram showed a small capacity bladder and no bladder neck (fig. 16, A). An IVP was normal. In a single lengthy procedure complete repair was done, incorporating all of the steps shown in figures 1 to 10, that is penile lengthening, creation of a distal urethra with skin graft, ureteral reimplantation, tubularization and narrowing of the bladder neck. A cystogram 1 month later showed satisfactory bladder capacity, a more
Resutu red interpubic ligament
C Skin mobilized completely from shaft, and ventral prepuce opened
Incision to make lateral flaps
FIG. 9
B
Subcutcmeous sump suction
t
Completed vertical closure
Sump suction in prevesicol space
FIG. 10
532
HENDREN
The principles outlined for secondary genital repair are equally applicable to new cases, as will be illustrated in a final case. Case 5 (fig. 17). A 3-year-old boy with complete epispadias was referred for penile repair. It was not certain whether there was total urinary incontinence, although it seemed likely. Initially, the penis was repaired but nothing was done to the bladder neck. At the first stage dorsal chordee was removed and preputial skin was transferred to the dorsum to provide additional length for creating a satisfactory penile urethra. At the second stage the corpora were mobilized widely, accomplishing skin closure by a multiple Z-plasty technique as shown in figures 1 to 10. (Today this would be done in a single operation, using a free graft to lengthen the urethra.) When the boy was 8 years old the bladder neck was narrowed to improve continence but there was still some stress incontinence. Therefore, this was repeated 1 year later,
FIG. 11. Case 1. A, appearance of genitalia as neonate. Rudimentary bladder is not suited for functional closure. B, when patient was 15 years old glans was satisfactory but note marked tethering upward to abdominal wall by dense scar at base of penis. C, after penile lengthening, which included insertion of free graft for distal urethra, note multiple Z-plasty closure of skin over shaft and mons. D, penis hangs in normal manner and there is better hair distribution 3 years postoperatively.
FIG. 13. Case 2, 3 years later. A, patient passing No. 16 Hegar dilator into graft urethra to demonstrate its patency. B, satisfactory appearance of urethral meatus at tip of penis. Pale color of skin graft covering undersurface of shaft is apparent. C, penis hangs normally with adequate size and length for intercourse.
FIG. 12. Case 2. A, note stubby appearance of penis, which lies upward against abdominal wall, precluding intercourse. B, manually pulling penis downward reveals inadequate length and diffuse scar of dorsum of penis and mons. Note cleft glans. Penis with this appearance causes severe psychological problems. C, intraoperative photograph shows elongated corpora and tubed skin graft to elongate urethra (for ejaculation). Corpora are then closed over urethral graft to bury it. D, since there was insufficient skin to cover shaft relatively thick (18/1,000 inch) Thiersch's graft was applied. Note Zplasty closure of mons with scar replaced by hair-bearing skin with subcutaneous fat beneath it and satisfactory length of shaft.
~ormal appearance of the prostatic urethra and bladder neck, and satisfactory caliber of the grafted distal urethra (fig. 16, B). Surprisingly, voluntary control of the urine was accomplished 2 months after this extensive revision.
FIG. 14. Case 3. A, preoperatively, penis tethered to abdominal wall is being held down with sound in previously tubularized urethra. Note fistula. B and C, postoperative views 3 months after secondary penile repair.
PENILE LENGTHENING AFTER PREVIOUS REPAIR OF EPISPADIAS
533
flap of the prepuce. :i In certain cases it is best to be prepared to use any one of these well established principles of plastic surgery according to what skin is available. In most cases the penis hangs down in a normal position. Erection is at an angle that permits intercourse, whereas some male subjects with
FIG. 15. Case 4. A, preoperatively, there were scars on leg and mans secondary to gracilis sling operations. Penis is essentially unrepaired. Note prominent preputial skin hanging from undersurface which will be used to resurface shaft. penis, urethra and bladder are opened to begin repair. been mobilized. Free skin graft for distal urethra is to be anastomosed to patient's urethral which has been proximally. Ureters have been reimplanted by Cohen technique. D, conclusion of operation.
making the bladder neck more narrow and tubularizing it to a higher level. The ureters also were reimplanted at a higher point in the trigone. Slowly, the patient gained complete urinary continence during the next 4 years. Penile appearance was satisfactory. The patient is 17 years old and has normal sexual function. DISCUSSION
The operative approach described herein for these difficult problems consists of long established surgical principles. As emphasized previously others there must be wide mobilization of the corpora from the pubic rami and the interpubic ligament. 1- 5 , 11 I have not dared to detach completely the corpora from the pubis, leaving them attached only at the neurovascular bundles as described by and Eraklis,-, for fear oflosing the blood supply to the penis, which would be an utter catastrophy. Although perhaps the shaft can be made somewhat longer by that technique I have found that reasonable length can be attained without completely detaching the corpora and without running the risk of losing all by trying to get an extra bit oflength. When lengthening is performed it is possible to leave the urethral opening at the base of the penis. However, I prefer to construct simultaneously a distal urethra using a free graft because these patients have the potential to ejaculate and this is better through the tip of the penis than on the mons. A free graft technique has been used by some surgeons for hypospadias since before the turn of the century. 10 A free graft must be relatively thick, since a thin one will contract. I have taught these patients or a parent to pass a Hegar dilator gently through the graft each day for several months postoperatively to be certain that there is no problem with stenosis. Perhaps this is unnecessary since no thick graft has become stenotic. Skin from the prepuce can be used to make the graft but I prefer to save that for shaft closure. I also have used bladder mucosa when functional use of the bladder was not contemplated. The force of ejaculation in these patients depends on whether there is a bladder remnant proximal to the verumontanum, which can cause retrograde ejaculation. Multiple Z-plasty closure of the dorsum of the penis usually is better than straight line closure, since it gives greater length and is less apt to contract. A Z-plasty scar is less noticeable in time, in contrast to a straight line closure that can form a bridle-like scar. Johnston and Kogan described other techniques to avoid straight line closure, includh1g \l-Y va,,u""""' of the skin and use of a reverse Nesbit
FIG. 16. Case 4. A, preoperative cystogram shows wide bladder neck and prostatic urethra as well as small capacity of bladder. B, voiding cystourethrogram by percutaneous suprapubic needle 6 weeks postoperatively demonstrates normal appearance of bladder neck, which has been tubularized and patent grafted distal urethra.
FIG. 17. Case 5-new case of compiete epispadias with incontinence repaired using principles outlined. A, preoperative appearance when patient was 3 years old. B, patient at age 17 years has satisfactory sexual function and continence.
534
HENDREN
tethering of the penis to the abdominal wall find that impossible. If there is too little skin to cover the shaft a free graft can be used, as shown in case 2. The initial cosmetic result of a free graft is not pleasing in some cases, since hypertrophy of the scar can occur at the seams. This softens in time and as the graft becomes more supple its appearance becomes acceptable. The color match of grafted skin is not perfect but these patients have not seemed to be worried much about that. Use of these long established principles can give a better initial repair in boys with epispadias, as well as greatly improve the appearance and function in patients when the initial result proves unsatisfactory.
6. 7. 8. 9. 10. 11.
REFERENCES
1. Johnston, J. H.: Lengthening of the congenital or acquired short penis. Brit. J. Urol., 46: 685, 1974. 2. Johnston, J. H.: The genital aspects of exstrophy. J. Urol., 113: 701, 1975. 3. Johnston, J. H. and Kogan, S. J.: Monograph: the exstrophic anomalies and their surgical reconstruction. In: Current Problems in Surgery. Chicago: Year Book Medical Publishers, Inc., 1974. 4. Tanagho, E. A.: Male epispadias: surgical repairs ofurethropen. ile deformity. Brit. J. Urol., 48: 127, 1976. 5. Kelley, J. H. and Eraklis, A. J.: A procedure for lengthening
12. 13. 14. 15.
the phallus in boys with exstrophy of the bladder. J. Ped. Surg., 6: 645, 1971. Hendren, W. H.: Exstrophy of the bladder-an alternative method of management. J. Urol., 115: 195, 1976. Cohen, S. J.: Ureterozystoneostomie: Eine neue antireflux technik. Aktuel. Urol., 6: 1, 1975. Gross, R. E. and Cresson, S. L.: Treatment of epispadias: a report of18 cases. J. Urol., 68: 477, 1952. Culp, 0. S.: Treatment of epispadias with and without urinary incontinence: experience with 46 patients. J. Urol., 109: 120, 1973. Horton, C. E. and Devine, C. J., Jr.: Hypospadias and epispadias. Clinical symposia. CIBA, vol. 24, No. 3, 1972. Williams, D. I.: Epispadias and exstrophy. In: Surgical Pediatric Urology. Edited by H. B. Eckstein, R. Hohenfellner and D. I. Williams. Stuttgart: Georg Thieme Publishers, pp. 298-312, 1977. Leadbetter, G. W. and Fraley, E. E.: Surgical correction for total urinary incontinence: 5 years after. J. Urol., 97: 869, 1967. Allen, T. D., Spence, H. M. and Salyer, K. E.: Reconstruction of the external genitalia in exstrophy of the bladder: preliminary communication. J. Urol., 111: 830, 1974. Middleton, A. W., Jr. and Hendren, W. H.: Ileal conduits in children at the Massachusetts General Hospital from 1955 to 1970. J. Urol., 115: 591, 1976. Horton, C. E.: Plastic and Reconstructive Surgery of the Genital Area. Boston: Little, Brown & Co., 1973.