Male Epispadias

0022-5347/95/1546-2150$03.00/0

Vol. 154, 2150-2155, December 1995 Printed in U.S.A.

THE JOURNAL OF UROLOCY

Copyright 0 1995 by AMERICAN UROLOCICAL ASS~CIATION, INC.

Review Article MALE EPISPADIAS DAVID A. DIAMOND

AND

PHILIP G . RANSLEY

From the Department of Surgery, University of Massachusetts Medical Center, Worcester, Massachusetts

KEY WORDS: epispadias, penis, urethra

Nearly a century ago Cantwell described the first surgical repair of the genitourinary anomaly known as male epispadias.1 Since then our understanding of the embryology and anatomy of this rare congenital lesion has evolved. Our surgical approach has evolved as well, such that it is now expected that every male individual with epispadias should appear and function reasonably normally. INCIDENCE AND EMBRYOLOGY

Isolated male epispadias is a n exceedingly rare anomaly. It seems worth bearing in mind that studies of its incidence have been few, and have reflected specific eras and geographic distributions. Dees reported male epispadias in 1 of 118,000 live male births.2 As a result, fetuses in various stages of epispadiac development have not been available for embryological study. Thus, existing embryological theories are based on our understanding of normal development of the external genitalia as it relates to the spectrum of clinical variants of epispadias. However, in 90% of cases male epispadias is associated with bladder exstrophy, which occurs once in every 50,000 live births.3 Thus, from a practical standpoint the embryology of the exstrophy-epispadias complex is inseparable from that of epispadias alone. Embryological theories of epispadias have sought to explain the eventration of the urethra and bladder in the combined exstrophy-epispadias complex, and the dorsal location of the penile defect as opposed to the ventral defect in hypospadias. Virtually all modern theories regarding the development of the exstrophy-epispadias complex have 2 principles in common. The embryology of the disorder is not consistent with simple developmental arrest, as is the case with hypospadias. In addition, the embryology of the exstrophy-epispadias complex in some way relates to an inhibition of the normal migration of mesoderm toward the midline from the infraumbilical abdominal wall. The differences between theories relate primarily to the mechanism responsible for abnormal mesodermal migration.

Perhaps the most widely accepted embryological theory is that of Muecke, who developed a chick model of cloacal exstrophy.4 He postulated that under normal circumstances the cloacal membrane regressed in size to allow the mesoderm to reinforce the central aspect of the infraumbilical abdominal wall. If it persisted, a n abnormally large cloacal membrane could serve as a mechanical barrier to prevent normal mesodermal migration and proper development of the lower abdominal wall structure (fig. 1).Muecke demonstrated that surgical insertion of a n inert plastic graft into the cloacal primordium of the chick prevented normal mesodermal migration and resulted in cloacal exstrophy with an associated “splayed phallus.” Presumably the splayed phallus noted by Muecke resulted from aberrant development of the genital folds and may well correspond to the rudimentary hemi-phalli in cloacal exstrophy in man. According to the Muecke model exstrophy may or may not be associated with a bifid phallus depending on the ability of the original raised genital folds to meet in the midline a t the cephalad aspect of a large persistent cloacal membrane. An alternative theory proposed by Thomalla et a1 is that the exstrophy-epispadias complex arises as a result of premature dehiscence of the cloacal membrane with the anatomical level of dehiscence accounting for the variability of clinical defects.5 They showed that laser induced injury of the chick cloacal membrane could result in cloacal exstrophy if injury was induced early enough in gestation. Laser injury a t 68 hours of gestation could produce cloacal exstrophy but injury induced at 76 hours of gestation did not result in a n exstrophic defect. This theory is consistent with those of other embryologists who acknowledge that the cloacal membrane, which is an interface between the ectoderm and entoderm, would be prone to rupture without mesodermal ingrowth. While the theories of Muecke and Thomalla et a1 regarding eventration of the lower urinary tract seem plausible, neither fully explains the dorsal nature of the urethral defect, as

-3

.m (cut)

I

E p i s p o d i o c q - T penis primord ium

A

B

V

. Unsupported membrane ruptures over- extensively

2151

MALE EPISPADIAS

opposed to the normal ventral relationship of the urogenital sinus to the phallus. Interestingly, Patten and Barry suggested that an understanding of the development of the epispadiac phallus enabled one to understand the associated dorsal eventration of urethra and bladder in the exstrophyepispadias complex.6 They noted that the genital tubercles normally arise cephalad to the urogenital orifice and carry a dense mass of mesenchymal tissue, which becomes the infraumbilical abdominal wall. They suggested that in epispadias the paired primordia of the genital tubercles arise caudal to their normal position, resulting in misdirection of the mesoderm caudal to the cloacal membrane. In this event the infraumbilical abdominal wall would not be reinforced by mesoderm, which would leave edoderm and entoderm in contact with each other without a supporting mesodermal layer, resulting in an unstable embryological situation and predisposing the cloacal membrane to rupture. According to this theory the urogenital portion of the cloaca would be located cephalad and the anal portion would be located caudal to the genital tubercle. Thus, with rupture of the urogenital segment of the cloacal membrane the outlet of the urogenital sinus comes to lie on the cranial aspect of the penis, resulting in the epispadiac defect (fig. 2). Variation in the degree of caudal displacement of the paired primordia of the genital tubercles could account for the Wering severity of the exstrophy-epispadiascomplex. Thus,Patten and Barry provide a unified theory to explain the dorsal relationship of the urogenital sinus relative to the phallus as well as the vulnerability of the lower urinary tract to eventration based upon the abnormally caudal origin of the genital tubercles. However, unlike the previous 2 theories an animal model consistent with the hypothesis of Patten and Barry has not been developed. ANATOMY

A number of important anatomical considerations in epispadias have a bearing on its functional significance and surgical repair. Perhaps the foremost of these is the location of the urethral orifice. The most common form of the anomaly is complete or penopubic epispadias in which the defect begins at the bladder neck and involves the length of the phallus

and urethral plate. In addition, this describes the anatomy of male patients with the combined exstrophy-epispadias complex who have undergone initial bladder closure and, thus, conversion to complete epispadias. In general, these patients are totally incontinent of urine and are likely to require bladder neck reconstruction. The more distal the urethral orifice, the less likely that the bladder neck is involved and the child will be incontinent. The 2 milder forms of the disorder, which are usually associated with urinary continence, are penile (the everted urethral plate begins on the penile shaft and extends to the tip of glans) and balanitic epispadias (only the glans is involved).3 In 1973 Culp reviewed the records of 28 male individuala with epispadias who had not undergone repair. Patients were Only 1 older than age 3 years and some were adole~cents.~ patient with complete epispadias and a patulous bladder neck was totally incontinent. A total of 20 patients was described as “classicsubsymphyseal”of whom 11 were totally incontinent, while 9 had some degree of urinary continence. Of the 6 patients with penile epispadias 3 were incontinent and 3 had an element of continence.“he patient with balanitic epispadias was continent. In no other series but that of Culp has continence been correlated with urethral orifice location. The high percent of continence in the subsymphysealgroup may be a function of the older age of his patients, some of whom were pubertal. All patients with complete or penile epispadias have a variable degree of dorsal chordee, which at its worst may result in an erection firmly directed against the a b d o d wall, making sexual function impossible. Historically such chordee was believed to be related to tethering of the corpora by the urethral plate. However, Woodhouse and Kellett demonstrated that there is an intrinsic deformity of the corporeal bodies in epispadias that is independent of the urethra.8 Of interest and importance in penile dissection is the altered course of the neurovascular bundles in the epispadiac phallus. Hurwitz et al noted that, rather than being dorsal and adjacent to the midline as is normal, the neurovascular bundles take a progressively ventrolateral course along the corpora, consistent with a medial-to-lateral rotational deformity.9 At the level of the symphysis the n e u r o v d a r bun-

Metanephric duct

Mesoneohric duct

RG. 2. Hypothetical stage in genesis of exstrophy of bladder and epispadias demonstrating relationship of genital tubercle to urogenital sinus. Reprinted with permission?

2152

MALE EPISPADIAS

dles are slightly lateral to the midline at the 11and 1o'clock approach, which avoided devascularizing the urethra.12 In positions. As the nerves are followed to the coronal margin, the Young technique the urethra was detached from the right they are noted as far ventrally as the 8 and 4 o'clock posi- corpus cavernosum but was left entirely attached to the left tions. Thus, given the lateral location of the nerves distally, corporeal body and simply rotated ventrally beneath the there is little likelihood of injury during dissection of the corpora. Young reported excellent results in 2 cases and urethra from the corporeal bodies. However, because of the noted that the procedure had "the great merit of practically more anterior position of the nerves proximally, they would restoring the urethra to its normal location."12 A different be vulnerable during freeing of the corpora from the symphy- modification of the Cantwell technique was proposed in 1903 by Bullitt, who encountered di%culty in reconstituting the sis pubis. Typically the epispadiac glans has a flattened configura- native urethral plate alone and, thus, incorporated ventral tion with excess irregular glans epithelium adjacent to the preputial skin in distal urethral reconstruction. l 3 Between 1918 and 1948 little was published about the mucosal urethral plate, which terminates on the dorsum. Excess glans epithelium is a source of difficulty in glans surgical approach to male epispadias. Although Young stated repair since, unless it is trimmed suf€iciently, a normal con- in 1918 that "the great amount of literature which is found ical configuration will not result. In addition, because the concerning the operative treatment of both hypospadias and urethral plate terminates on the dorsum, a simple approxi- epispadias is eloquent proof of the unsatisfactory results mation of glans wings would not produce a urethral meatus which have been usually obtained,"'* one presumes that considerable difficulty was encountered with the aforementioned centrally located within the glans. techniques. In the 1940s and 1950s entirely new approaches to correcting male epispadias were proposed, which concentrated on the 2 areas of greatest functional significance: EVOLUTION OF SURGICAL REPAIR OF EPISPADIAS In an era when technological advances have totally trans- urethral reconstruction and the correction of dorsal chordee. In 1948 Mays bridged the gap between the early and modformed our surgical approach to many urological diseases the history of the surgical repair of male epispadias is a n anom- ern approaches to epispadias repair by combining a Cantwell aly. The original urethroplasty described a century ago in- type tubularization and ventral rotation of the urethral plate volved concepts that were abandoned in a n attempt to find with creation of a distal urethra using ventral preputial skin, more successful alternative approaches. However, given the borrowing the idea of Bullitt.I4 Unlike the Cantwell and difficulty in consistently achieving surgical success we have Young techniques in which the urethra terminated in the recently returned to the original conceptual approach in a glans, the Mays technique converted epispadias to hypospamuch improved form, which has enabled us to approximate dias and resulted in a urethra terminating at the distal penile shaft on the ventrum. consistent surgical success. In 1958 Swenson was among the first to abandon the The earliest surgery for male epispadias repaired the peThiersch-Duplay approach of tubularizing the urethral nile defect without attempting to restore function of the epispadiac urethra. In 1845 Dieffenbach freshened lateral plate.I5 Instead he applied the Denis-Browne principle, tissue edges and placed approximating sutures,IO and in proved successful in hypospadias repair, to male epispadias. 1869 Thiersch simply rotated local skin flaps to cover the He simply buried the longitudinal strip of urethral mucosa urethral defect." It was not until 1895 that Cantwell per- and approximated the corpora cavernosa dorsal to it. As surgical approaches to hypospadias in the 1970s and formed the first true urethroplasty for complete epispadias.' In a n attempt to reconstitute the epispadiac anatomy to 1980s became more sophisticated and successful, they were normal Cantwell completely mobilized the dorsal urethral applied to the repair of male epispadias. In 1972 Devine et plate from the corpora cavernosa and transplanted the tubu- a1 applied their highly successful technique of free graft larized urethra below the corpora cavernosa, which were urethroplasty to epispadias.1" In the process they made an rotated dorsally and approximated in the midline (fig. 3). important observation about the relationship of the urethra While he originally described a successful outcome in 2 cases, to dorsal chordee, noting a dense band of fibrous connective difficulties were encountered with the repair that were at- tissue on the dorsal surface of the corpora cavernosa that tributed to poor vascular supply of the fully mobilized ure- extended to the urethral groove. Dividing the urethra and excising this fibrous tissue often corrected chordee but not thral tube. In 1918 Young described his modification of the Cantwell consistently, which suggested that the tunica albuginea of the corpora might be short and require lengthening by incising tunica and placing a dermal graft into the defect. This technique was the forerunner of the more revolutionary Woodhouse approach to chordee correction but it also supported the concept that division of the urethral plate was necessary to correct dorsal chordee.'; In 1984 Lepor et a1 reported their experience with a modified Young urethroplasty in which the urethra remained dorsal to the corpora and was tubularized in continuity in Thiersch-Duplay fashion from prostate to glans.18 They were among the first t o be candid enough to report their fistula rate (21%')and incidence of persistent upward penile deviation on erection (47%0.These figures were similar to those reported by Kramer et a1 (30%.fistula rate, 33% upward curvature on standing) although the angles of erections were not described in their series.19 These similarities are of interest in that Kramer et a1 routinely divided the urethral plate and Lepor et al did not. In 1984 Thomalla2('and in 1987 Monfort" et a1 applied the highly successful Duckett and Asopa transverse island pediFIG. 3. Cantwell epispadias repair. Dorsal urethal plate I B Jmobi. cle p a f t techniques for hypospadias repair to ninle epispalized from corpora and tubularized.Corpora cavernosa rotated dorsal dias. According to Thomalla et a1 the abilitv to drop the to urethra and approximated. Reprinted with permission.' urethral plate proximally ,ifforded excellent opportunity for

2163

MALE EPISPADIAS

chordee correction in penile lengthening. They reported a 25% fistula rate and Monfort et al reported an overall fistula rate of 55% with interposition of the vascularized pedicle

tube graft. Monfort et a1 alluded to long-term difEculties with fistulas and stenosis in this technique at both extremities of the tube where vascularity was suboptimal. A long-term problem that has become apparent with this form of reconstruction that patients requiring intermittent catheterization may encounter difficulty as a result of anastomotic narrowing and urethral tortuosity. CHORDEE CORRECTION

than a Nesbitt plication ventrally, which would further shorten the phallus. "his extensive corporeal dissection required full mobilization of the urethral plate from the corpora and identification of the course of the neurovascular bundles to avoid iqjury to them during the fascial release.

1tunica albuginea dorsally rather

CURRENT RECONSTRUCTIVE APPROACH TO MALE EPISPADIAS

In the pediatric age group the synthesis of the Woodhouse and Kellett concepts with those of Cantwell resulted in the evolution of a new approach to male epispadias.m "his a p proach, known as the modified Cantwell procedure, entails 3 major components: 1)corporeal rotation dorsally with cavernocavernostomy, 2) urethral mobilization and reconstruction that maintains an intact urethral plate, and 3) improved glanuloplasty and dorsal skin coverage.

While the issue of chordee correction in male epispadias is not truly separable from that of urethral reconstruction, it was somewhat delayed in receiving the surgical attention originally directed toward urethroplasty. In 1958 HinmAn was among the first to focus on straightening and lengthening the epispadiac phallus through full mobilization and reCORPOREAL ROTATION AND CAVERNOCAVERNOSTOMY cession of the urethral plate relative to the corpora.22 SusIn the course of freeing the corporeal bodies completely pensory ligaments of the penis were divided and ventral preputial skin was rotated dorsally to cover the defect pro- from the urethra and adjacent soft tissue to perform lengthening phalloplasty a number of observations were made with duced with urethral recession. A similar accomplished was proposed by Michalowski and time. Rotating the corporeal bodies could, indeed, correct Modelski, who accomplished penile lengthening by perform- chordee as noted by Koff and Eakins.m Yet surprisingly it ing mobilization and 2-plasty of the urethral plate.23 To was noted that dorsal rotation of the corpora in the direction achieve penile length, which was believed to be compromised opposite to that described by Koff and Eakina seemed to by the course of the corpora in traversing the distance be- produce superior correction. Thia observation was exactly tween divergent ischiopubic rami, Kelly and Eraklis first what Cantwell had originally described but he never disproposed a more aggressive approach to corporeal mobiliza- cussed the issue of chordee correction in his original article, tion from the symphysis pubis.% The corpora were com- only restoration of the normal anatomical relationships of the pletely dissected from their attachment to the ischiopubic corpora to urethra. As described by Woodhouse and Kellett, rami. In time this aggressive mobilization was noted to have in phalloplasty a graft of lyophilized dura mater was origithe associated risk of devasculmking the corporeal bodies. nally used to fill the corporeal defeet p d u c e d by the dorsal As a result, more modest approaches to freeing the corpora relaxing incision.8 After Jakob Kreuzfeld disease was refrom the symphysis pubis were proposed by Johnston,26and Jordan and Gilbert.26 An altemative approach to overcoming the divergent symI physis pubis in epispadias to enhance penile length was proposed by Schjllingerand Wiley, who demonstratedthat division of the sacrotuberousand sacrospinousligaments in conjunction with posterior pelvic osteotomiescould reduce the intrasymphyseal distance.27 They observed that phallic length was significantly improved when the intrasymphyseal distance was reduced to less than 3 cm. However, this creative aggressive approach has not gained widespread acceptance. Koff and Eakins took an entirely different approach to chordee correction in epispadias, recognizing that rotation of the corporeal bodies could correct dorsal curvature.28 Their technique entailed dissection of the corpora from the urethra and inferior pubic ramus, and medial to lateral rotation of the corporeal bodies in opposite directions with ventral fixation. The urethra remained dorsally located. In 1984 the study by Woodhouse and Kellett provided the conceptual breakthrough to explain the limitations of previous approaches and serve as the basis for the current approach to chordee correction in epispadias repair.8 They studied epispadias patients with cavernosography and computerized tomography, and concluded that the epispadiac corpora were inherently short. The shortness was further exaggerated by the corporeal length required to reach the midline from the divergent inferior pubic rami. In addition, the inferior pubic rami did not lie in the normal anatomical plane but were rotated downward, parallel to the floor. In addition, it was apparent that when the corpora emerged from the perineum they coursed sharply upward. Thus, given FIG.4. Modified Cantwell epiapadias regajr. A, bilateral transthese inherent abnormalities, it was understandable why corporeal incisions after complete mo hzahon of c o ~ p and o ~ previous techniques directed simply at improved mobiliza- verse neurovascularbundles, F d urethral m n s t r u c t i o n B be tion of the corpora to correct chordee were not successful. cavernoeavernost.omy yth corpora overi -rni~=f To correct the intrinsic dorsal curvature of the corporeal Reprinted with on from Marshay.F.: Textbook of OperW.B. s a d CO., in preee. bodies Woodhouse and Kellett preferred a fascial release of ative urology.

A

B

2154

MALE EPISPADIAS

ported to have been transmitted through a dural graft this material was abandoned and an alternative substance was sought. Cavernocavernostomy in which the 2 separated corporeal bodies were approximated dorsally in the midline provided a solution (fig. 4). This maneuver combined the 2 steps of filling the corporeal defect and approximating the corporeal bodies into 1 body. It also resulted in solid durable dorsal corporeal approximation, which individual approximating sutures had not consistently achieved. Complete freeing of the urethra was necessary for corporeal mobilization and chordee correction yet it became apparent with time that the fully mobilized urethral plate was quite elastic in nature. While it may have contributed to chordee in its dorsal location, when rotated ventral to the corpora it appeared to have sufficient length to prevent penile tethering. The course of the neurovascular bundles in male epispadias, as defined by Hurwitz et al,9 was a n important consideration in corporeal reconstruction. To avoid damage to the neurovascular bundle in the process of making the transverse corporeal releasing incision and approximating the corpora dorsally the neurovascular bundles were mobilized off of the corpora. This maneuver afforded uncompromised exposure and access for aggressive corporeal reconstruction. Long-term followup of chordee correction using dorsal corporeal rotation with cavernocavernostomy has consistently produced a downward angled phallus.29 URETHRAL RECONSTRUCTION

As noted, the elasticity of the urethral plate can only be appreciated with its complete mobilization off of the corporeal bodies. With ventral rotation of the urethra relative to the corpora cavernosa the length of the urethral plate was consistently sufficient to extend from prostate to glans without tension. Ventral rotation provided a more direct route from bladder to glans. Along with the maintenance of urethral continuity it has afforded ease of intermittent catheterization. Urethral reconstruction has been performed with interrupted absorbable sutures to promote maximal flexibility of the urethra. Dorsal coverage of the urethral suture line by the corporeal bodies has resulted in a markedly decreased fistula rate (8%)relative to that of dorsally located urethroplasty.30 Of interest is the fact that no urethra has been devascularized in the course of this dissection, as was the original concern of Young with the Cantwell technique.

FIG. 5. Epispadias skin coverage using transverse preputial island flap distally and Z-plasty of midline incision proximally. Reprinted with permission from Marshall, F. F.: Textbook of Operative Urology. Philadelphia: W. B. Saunders Co., in press.

ing only the proximal dorsal aspect of the penile shaft to be covered. Kramer and Jackson described the use of bilateral rhomboid flaps to aid in proximal dorsal coverage.":*A simple effective alternative approach to proximal shaft coverage has been Z-plasty in which triangular flaps of skin from the lateral aspect of the midline abdominal incision are based distally and reconfigured to close the distal abdominal incision as well as cover the proximal penile shaft. Z-plasty helps to fill in the suprapubic hollow that sometimes results and also disrupts the midline incision, which can contract and cause dorsal tethering of the phallus (fig. 5 ) . BLADDER NECK RECONSTRUCTION IN MALE EPISPADIAS

GLANULOPLASTY AND SKIN COVERAGE

An aspect of epispadias repair that has historically received little attention is cosmesis. Indeed, in 1 report cosmesis was assessed in terms of phallic straightness and length. Certainly the achievement of a healthy urethral tube within a straight phallus has been a daunting surgical task but more consistent success in these areas has allowed u s to focus on further cosmetic aspects of repair.jl The 2 primary components are glans reconstruction and skin coverage.3z The urethral plate on the epispadiac glans terminates on the dorsum. Thus, to achieve a terminal, centrally located urethral meatus the urethra must be extended distally toward the ventrum of the glans. The epispadiac glans wings have a bulk of irregular tissue lateral to the urethral plate on either side. To reconstruct the glans in a conical configuration these protrusions of tissue must be excised aggressively. A 2-layer glans closure may then be performed with a superficial subcuticular closure, producing superior cosmetic results. Dorsal skin coverage in epispadias repair can be difficult and a reliable source of healthy skin is of great value. A transverse island pedicle flap from the ventral prepuce rotated to the dorsum serves this purpose well. The remaining ventral prepuce provides ventral and lateral coverage, leav-

The vast majority of male individuals with complete (penopubic) epispadias and a percent of those with penile epispadias will be incontinent and require surgery for urinary control. Classically surgery has entailed a version of the Young-Dees-Leadbetter approach to bladder neck reconstruction although alternative approaches, such as the artificial urinary sphincter, have been used.2.34-36 However, it is of interest that urethroplasty has had a significant role in the process of achieving urinary continence. Kramer and Kelalis noted that of 43 male patients with penopubic epispadias and complete or partial urinary incontinence 5 achieved total continence after urethroplasty alone (2 at pubertyl37 Of 15 patients with penile epispadias and partial or total urinary incontinence 7 achieved continence with urethroplasty alone ( 5 a t puberty). Ritchey et a1 noted that urinary continence, which develops at puberty in male epispadias patients, does not reliably occur in the male exstrophy population.:'S However, in our experience with minor degrees of urinary continence associated with good bladder storage and emptying incontinence may resolve with puberty in the exstrophy patient. Studies of the older epispadias patient raise the philosophical issue of whether the modein approach of achieving early urinary continence with bladder neck reconstruction is preferable to watchful waiting until puherty in thca hope t.hat continence

MALE EPISPADIAS

WiU develop spontaneously. Indeed, if continence developed reliably with puberty, such an approach would seem worthwhile. Should continence not develop spontaneously with time, apart from the embarrassment of incontinence, the prospect of a pubertal epispadias patient requiring additional reconstruction seems particularly unappealing. For the majority of epispadias patients who remain wet after urethroplasty Peters et al noted enhanced bladder capacity following the increased bladder outlet resistance resulting from the modified Young urethroplasty.39 In this respect urethroplasty has contributed to the ease of bladder neck reconstruction and, presumably, its likelihood of BUCeess. However, one wonders if performing the classic YoungDees-Leadbetter reconstruction with parallel incisions through the trigone may risk denervation of the epispadiac bladder. Although such a procedure may be necessary in exstrophy, it may be counterproductive for the patient with isolated male epispadias. Hollowell et al demonstrated that epispadiac bladders with good contractility before YoungDees-Leadbetter reconstruction had poor contractility after bladder neck reconstruction.qo Therefore, it seems reasonable to consider alternative approaches to the achievement of continence in the patient with isolated male epispadias. Indeed, 1 of us (P.G. R.) has abandoned the classic YoungDees-Leadbetter bladder neck reconstruction in the complete epispadias patient and instead elects polydimethylsiloxane iqjection in the prostatic urethra to achieve urinary continence.

2165

Behandlung der Epispadie. Arch. Heilk., 10: 20,1869. 22. Young, H. H.: A new operation for epispadias. J. Urol., 2 237, 1918. 13. Bullitt, J. B.: Epispadias: report of a case operated on by a modification of Cantwell's method. J A M & 41: 297,1903. 14. Mays, H. B.: Epispadias: a plan of treatment. J. Urol., 101:251, 1972. 15. Swenson. 0.:Pediatric Surgery. New York: Appleton-Century KroRs, 1968. 16. Devine, C. J., Jr., Horton, C. E. and SCaB,J. E., Jr.: Epispadias. Urol. Clin.N. Amer., 7:465,1980. 17. Woodhouse, C. R. J.: The management of erectile deformity in adults with exstrophy and epispadias. J. Urol., 1 s 932,1986. 18. Lepor, H.,Shapim, E. and Jeffs, R. D.: Urethral reconstruction in boys with classical bladder exstrophy. J. Urol., 131: 512, 1984. 19. Kramer, S.A.,Membian, H.4. J. and Kelalia, P.P.: Long-term followup of cosmetic appearance and genital function in male epispadias: review of 70 patients. J. Urol., 1 s 543,1986. 20. Thomalla, J. V. and Mitchell, M.E.: Ventral preputial island flap technique for the repair of epispadias with or without exstrophy. J. Urol., 132 985,1984. 21. Monfort, G., Morrison-Lacombe,G., Guys, J. M. and Coquet, M.: Transverse island flap and double flap procedure in the treat ment of congenital epispadias in 32 patients. J. Urol., 138: 1069,1987. 22. Hinman. F., Jr.: A method of lengthening and =pairing the penis in exatrophy of the bladder. J. Urol-, ls: 237,1958. 23. Michalowski, E. and Modelski, W.: The surgical treatment of epispadias. Surg.,Gynec. k Obst., 117:465,1963. 24. Kelley, J. H. and Eraklie. A J.: A procedure for lengthening the phallus in boys with exstrophy of the bladder. J. Ped. Surg.,8: BLADDER AUGMENTATION IN MALE EPISPADIAS 645,1971. J. H.:The genital aspects of exatrophy. J. Urol., 113: Some epispadias and exatrophy patients remain wet after 25. Johnston, 701,1975. urethroplasty and bladder neck reconstruction. Often inade- 26. Jordan, G. H. and Gilbert, D. A: Operative p d u r e 8 for episquate bladder capacity remains a major cause, raising the pa& and exstrophy of the bladder. Sem. Urol., 6:243,1987. issue of bladder augmentation. In general the inclination to 27. SchiUinger, J. F. and Wiley, M. J.: Bladder exstrophy: penile augment epispadiac bladders has been far less than to auglengthening procedure. Urology, U:434,1984. ment exstrophic bladders that are inherently more abnormal. 28. Koff, S.A. and Ealrins, M.: The treatment of penile chordeeusing corporeal rotation. J. Urol., 131: 931,1984. In the series by Ritchey et a1 8 of 17 exstrophy bladders were augmented compared to only 3 of 45 epispadiac b1adde1-s.~~ 29. Kqjbafzadeh, A. M., DufFy, P. G. and Ranaley, P. G.: The evolution of penile reconstruction in epispadias repair: a report of Thus,the hope is that the better quality epispadias bladder 180 cases. J. Urol., part 2,1&4.858,1995. will achieve adequate capacity to store and empty effectively 30. Gearhart, J. P.,Leonard,M.P., Burgers, J. K.and Jeffi, R. D.: with time. The Cantwell-Ransley technique for repair of epispadias. J. Urol.. 148:851, 1992. REFERENCES 31. Vorstman, B., Horton, C. E. and Winslow, B. H.: Repair of secondary genital deformities of epispadiadexstrophy. Clin. 1. Cantwell, F. V.: operative treatment of epispadias by transplanPlaat. Surg., 16: 381.1988. tation of the urethra. Ann. Surg.,M: 689, 1895. 2. Dees, J . E.: Congenital epispadias with incontinence. J. Urol., 32. Diamond, D.A. and Ransley, P. G.: Improved glanuloplasty in epispadias repair: tachnical aspects. J. Urol., l a 1243,1994. 62 513,1949. 3. Duckett, J. W., Jr.: Epispadias. Urol. Clin. N. h e r - , 6 107, 33. Knuner, S. A. and Jackson, I. T.: Bilateral rhomboid flap for reconstruction of the external genitalia in epispadias-esstaw 1978. phy. Plast. Reconstr. Surg., T7: 621,1986. 4. Muecke, E. C.: The role of the cloacal membrane in exstrophy: the first successful experimental study. J. Urol., Sa:659,1964. 34. Young, H. H.: An operation for the cure of incontinence associated with epispadias. J. Urol., ?: 1,1922. 5. Thomalla. J. V.. Rudobh. R. A,, Rink,R. C. and Mitchell, M. E.: Induction of cloacal 'ex;ltrophy in the chick embryo using the 35. Leadbetter, G.W., Jr.: Surgical correction of total urinary incontinence. J. Urol.. 91: 261.1964. CO2 laser. J. Urol., 1W.991,1985. 6. Patten, B. M. and Barry, A.: The genesis of exstrophy of the 36. Light, J. K. and Sebtt, F.B.: "matment of the epispadias-exstrophy complex with the AS792 artificial urinary sphiocter. bladder and epispadias. Amer. J. Anat., 90: 35, 1952. J. Urol., 129:738, 1983. 7. Culp, 0. S.: Treatment of epispadias with and without urinary incontinence: experience with 46 patients. J . Urol., 109: 120, 37. Kramer, S. A. and Kelalis, P. P.: Assessment of urinary continence in epispadias: review of 94 patients. J. Urol., 1%: 290, 1973. 1982. 8. Woodhouse, C.R.J. and Kellett, M.J.: Anatomy of the penis and its deformities in exstrophy and epispadias. J. Urol., 132 38. Ritchey, M. L., Kramer, S. A. and Kelalis, P. P.: Vesical neck reconstructionin patients with epispadias-exstrophy.J. Urol., 1122,1984. 1 m 1278,1985. 9. Hurwitz, R. S..Woodhouse, C. R. J. and Ransley, P. G.: The anatomical course of the neurovascular bundles in epispadias. 39. Peters, C. A., Gearhart, J. P. and Jeffs, R. D.: Epispadias and incontinence: the challenge of the small bladder. J. Urol., 140. J. Urol., 1s8:68.1986. 1199,1988. 10. Dieffenbach, J. F.: Plastische Operationen an den Harwegan. Urethmplastice. Die Operative Chirurgie Leipzig: F. A. 40. Hollowell. J. G., Hill, P. D., Duffy, P. G. and Ransley, P. G.: Bladder function and dysfunctionin exstrophy and epispadias. Bockhaus., 1: 526, 1846. Lancet, 338:926, 1991. 11., Thiersch, C.: Ueber Die Entstehungsweise und operative