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16 Male Epispadias
mean of 2.3cm without any short-term side effects. The increased length and girth
with increased vascularity have the potential to improve outcomes and decrease
vascular injury to the urethral plate and skin [15].
Makedonsky described the use of intramuscular HCG administered to patients
with epispadias, bladder exstrophy combined with cryptorchidism. HCG was
administered intramuscularly at 250–500 IU twice a week for 3 weeks prior to
reconstruction. Compared to controls, mean penile length was seen to increase by a
mean of 1.8cm and penile circumference by a mean of 1.2cm. Epidermal growth
factor (EGF) and human epidermal growth factor receptor (Her2/neu) were found to
be decreased in exstrophy/epispadias compared to controls, and both EGF and
Her2/neu increased by 10% after administration of HCG [16].
261
Pelvic Osteotomy
Pelvic osteotomy is recommended for proximal epispadias involving the bladder
neck as this allows for decreased tension on the tissues of the bladder neck and
proximal urethra decreasing complications such as dehiscence and stulae [17].
Techniques of osteotomy are described in another section of this book.
Modified Cantwell-Ransley Repair
This repair entails three components: (1) corporal rotation dorsally with cavernocavernostomy, (2) urethral mobilization and reconstruction, and (3) improved glanuloplasty and dorsal skin coverage [18] (Fig.16.2).
Corporeal Rotation andCavernocavernostomy
The corporeal bodies are freed completely from the urethra except the distal part of
the corpora and hemiglans leaving the mucosa attached to the glans distally therefore assuring improved blood supply and preventing shortening of the urethra.
The corporeal bodies are rotated dorsally (medially) with cavernocavernostomy
to secure the corpora and to transpose the urethra ventrally.
In order to avoid damage to the neurovascular bundle, they are mobilized off the
corpora. Current modications avoid the routine mobilization of the neurovascular
bundles, since in most of the cases, the incision and the anastomosis of the corporeal
bodies can be safely done without their mobilization [19].
Urethral Reconstruction
The urethral plate is dissected to the penile base and distally to the tip of the penile
glans with creation of glandular wings. The urethral reconstruction is performed
with interrupted absorbable sutures. Dorsal coverage of the urethral suture line by
262
P. A. Merguerian
a
b
Fig. 16.2 Modied Cantwell-Ransley repair. (a) Bilateral transverse corporal incisions are made
after complete mobilization of the corpora and neurovascular bundles, and urethral reconstruction.
(b) Beginning of cavernocavernostomy with corpora overlying reconstructed urethra
the corporal bodies results in a lower stula rate. The glandular urethra and the
meatus are advanced by using a modication of the MAGPI procedure for hypospadias, named IPGAM, which involves a vertical incision in the distal urethral plate
and transverse advancement with absorbable sutures.
Glanuloplasty andSkin Coverage
The two main components of the repair that allow normal cosmetic results are the
glansplasty and skin coverage.
The epispadias glans wings usually have a bulk of irregular tissue just lateral to
the urethral plate. To reconstruct the glans in a conical conguration, this tissue
should be aggressively excised and a 2 layer closure of the glans performed with
subcuticular absorbable sutures.
16 Male Epispadias
Dorsal skin coverage can be difcult, and several methods have been used including a transverse prepucial island ap that is rotated dorsally [11], bilateral rhomboid
aps to aid in proximal dorsal coverage, and Z-plasty brought from the lateral aspect
of the midline abdominal incision.
The most common approach is by using reverse Byars aps and a longitudinal
dorsal suture line [20].
263
Bladder Neck Reconstruction
The majority of patients with penopubic epispadias and a percent of penile epispadias patients will be incontinent and require reconstruction. The two most common
bladder neck reconstructions performed today are the Young-Dees-Leadbetter repair
and the Mitchell modication.
The bladder neck reconstruction is described elsewhere in this book.
The Mitchell Repair
Bagli and Mitchell first reported results using this technique in 1996 [13]. It
was felt that the planes of dissection were such that this technique allows for
better access to the bladder neck and to bladder neck reconstruction. Moreover,
completely disassembling the urethral plate and the corporal bodies allowed
for a more normal appearance of penis and allowed the urethra to be placed
ventrally. Because the corporal bodies are completely disassembled, there is no
crossed blood supply and the blood supply of each corpus is based on the central artery. The urethral and spongiosal blood supplies come from the proximal
urethra. The dorsal nerve complex is not dissected from the corpora cavernosal bodies.
Initial Dissection
The dissection is usually started on the ventral aspect of the penis. The initial plane
of dissection occurs just above Buck’s fascia. As the dissection progresses medially,
the plane will shift from above Buck’s fascia to above the tunica albuginea on the
ventromedial aspect of each corporal body. The urethral plate with the corpus spongiosum is dissected from the dorsomedial aspect of the corpora cavernosa on the
tunica albuginea preserving all of the spongiosal tissue (Fig.16.3).
264
Fig. 16.3 Plane of dissection between the urethral plate and the corpora cavernosa
P. A. Merguerian
Careful lateral dissection of the penile shaft skin and dartos fascia from the corporal bodies is performed above Buck’s fascia ensuring to keep the neurovascular
bundle intact. They are located laterally on the corpora within Buck’s fascia.
Penile Disassembly
The penis is disassembled into three components: the right and left corporal bodies with
their respective hemiglans and the urethral plate with its underlying corpus spongiosum.
This provides easier access to the proximal urethra and the bladder neck.
The plane of dissection is at the level of the tunica albuginea on the corpora.
Once the plane is between the urethral plate/corpus spongiosum and the corporal
bodies, the dissection is carried distally to separate the three components from each
other. It is important to keep the corpus spongiosum attached to the urethral plate as
the blood supply of the urethra is based on the spongiosal tissues. Para-exstrophy
aps cannot be utilized in this technique as this maneuver will devascularize the
distal urethra (Fig.16.4).
16 Male Epispadias
Fig. 16.4 Urethral
tubularization in continuity
with bladder neck closure
265
Proximal Dissection
Dissection of the urethral and spongiosum from the corporal bodies is crucial for
posterior placement of the bladder neck and proximal urethra. A deep incision of the
intersymphyseal ligaments posterior and lateral to the urethral plate is necessary to
allow the bladder neck and posterior urethra to be positioned deep in the pelvis.
Bladder Neck Reconstruction
For penopubic epispadias with involvement of the bladder neck, the bladder neck
reconstruction is performed next (described in another chapter).
266
P. A. Merguerian
Primary Closure
Once the bladder neck is reconstructed and the corporal bodies and urethral plate
with corpus spongiosum are dissected, the closure is started. A suprapubic tube is
recommended for the proximal repairs.
The urethra is then tubularized in two layers using monolament suture in an
interrupted fashion so as not to shorten the urethra. The tubularized urethra is then
placed ventrally. The corporal bodies are rotated medially. Buck’s fascia of each
corporal body is then reapproximated in the midline. This medial rotation is important to assist in correcting the dorsal curvature.
If osteotomies were performed, the pubic symphysis is reapproximated using
two 0.0 polydioxanone sutures with the knots tied anteriorly to prevent erosion into
the bladder neck and proximal urethra (Fig.16.5).
Dorsally, glans wedges are excised to create a conical appearance of the glans
penis. The hemiglans are then reapproximated using subcuticular interrupted monolament sutures.
The urethra is then brought up to each hemiglans ventrally to create an orthotopic meatus. Occasionally, the urethra may not reach as it is left in a hypospadias
location for future reconstruction. The glans is recongured ventrally with interrupted monolament sutures.
Fig. 16.5 Symphysis
pubis is reapproximated
after the urethra and
bladder neck have moved
posteriorly underneath the
corporal bodies
16 Male Epispadias
267
Skin Closure
Dorsal skin coverage in epispadias repair can be difcult. Reverse Byars aps are
used to transpose the ventral skin dorsally for coverage.
A transverse island pedicle ap from the ventral prepuce rotated to the dorsum
can also be utilized. The remaining ventral prepuce is then used for ventral and lateral coverage. Kramer and Jackson described the use of bilateral rhomboid aps to
aid in proximal dorsal coverage [21].
An alternative approach to proximal shaft coverage has been Z-plasty in which
triangular aps of skin from the lateral aspect of the midline abdominal incision are
based distally and recongured to close the distal abdominal incision as well as
cover the proximal penile shaft.
Modification totheMitchell-Bagli Epispadias Repair
A modication of the Mitchell-Bagli repair is described in the Video Journal by
Pippi Salle etal. [22] (https://doi.org/10.1016/j.urolvj.2022.100127).
It is felt that the most critical aspect of achieving continence is allowing for
adequate bladder outlet resistance to allow bladder cycling and increased bladder
capacity. Special attention is paid to tailoring the bladder neck so that it has a nearnormal funneled conguration.
Another step which is not always achieved with the Mitchell and the CantwellRansley repair is dorsal penile curvature correction and maximizing penile length.
This leads to dissatisfaction of cosmetic appearance in adolescence and adulthood.
Instead of internally rotating the corporal bodies, external rotation has been shown
to achieve a more normal lie of the penile shaft. This requires extensive dissection
of the corporal bodies and separation from the urethra [23].
In order to reduce the risk of creating a dimple at the penopubic area, mobilization of fat aps from the mons pubis on either side to lay on top of the pubic closure
at the penopubic angle allows for a atter penopubic junction.
Finally, the previously described methods of skin closure have the potential of
increasing the risk of dorsal curvature recurrence secondary to retraction of the
dorsal midline scar. Moreover, redundancy and ventral skin ischemia have been
reported after the use of transverse preputial island aps. The rhomboid aps have
the disadvantage of having hair-bearing skin on the penile shaft.
In an attempt to prevent these complications, Pippi Salle described the use of a
ventrally based skin ap that is then rotated to cover the penile shaft with the suture
line being on the lateral aspect of the penis, thus preventing the complications
related to the abovementioned repairs [24] (Fig.16.6).
268
P. A. Merguerian
AC
BD
A
C
D
B
AC
BD
Fig. 16.6 Ventral penoscrotal junction is identied and the incision line demarcated. Dissection
of the ventral penile skin ap is directed toward the penoscrotal junction. Mobilization is continued
until a ap with sufcient mobility is fashioned to enable dorsal rotation and lateral placement of
the longitudinal shaft skin suture line. Rotation of the ventral penile skin ap allows for lateral
placement of the suture line
16 Male Epispadias
269
Outcomes
Reported rates of success for epispadias repairs vary. The degree of epispadias, the
age of the patient, surgeon experience, and the presence of previously operated tissue all impact the successful outcome of epispadias repair.
Moreover, penile cosmetic result is a subjective measurement. Patient-reported
outcomes are lacking, and therefore, cosmetic success of these repairs is difcult to
validate and compare.
Fistula Formation
Urethrocutaneous stula is the most common urethroplasty-related complication.
The areas predisposed to stula formation are the penopubic junction and penoscrotal angle. The rate of urethrocutaneous stula formation varies from 2.4% to 28%
[20, 25, 26].
Gearhart etal. reported a 21% stula rate in 75 boys who underwent the CantwellRansley epispadias [19].
Thomas et al. [27] in a series of 30 male patients followed for a median of
18.5years reported a urethrocutaneous stula rate of 20%.
Braga etal. [28] reported a rate of 24% in 21 patients with penopubic epispadias.
A recent study by Bencic etal. reported a rate of 22.6% in 31 patients with isolated epispadias with a mean age of 17years. In this study, they also report urethrocutaneous stula occurring in 2 patients 10 years after closure undermining the
importance of long-term follow-up [29].
Urethral Stricture
The incidence of urethral stricture varies between 5% and 10% [27, 29, 30].
Djordjevic described repair of failed epispadias in 23 adult men with complete
penile disassembly after failed epispadias repair. The urethra was reconstructed
with genital skin or buccal mucosa. In this cohort, the rate of urethral stricture was
43.8% [31].
Residual Curvature
As there is a lack of patient-reported outcomes, the true incidence of residual curvature and especially the incidence of clinically signicant residual dorsal curvature
are lacking. Moreover, patient-reported outcomes are hard to obtain as shown in the
study by Reddy etal. evaluating the long-term sexual outcome of men with epispadias [32]. Out of 74 patients who met inclusion criteria, 15 (20%) agreed to ll out
a questionnaire on their sexual function.
270
P. A. Merguerian
Reddy etal. reported on 15 patients of which nine (60%) had persistent curvature
of the penis. Moreover, 8 of the 15 patients (53%) underwent chordee repair and
penile lengthening during adolescence [32].
Urinary Continence
The true incidence of incontinence after epispadias repair is lacking as the denition
of incontinence is not standardized. Also, the types of continence procedure vary
from study to study making it difcult to assess outcomes. Moreover, there are no
long-term patient-reported outcomes and denition of what is acceptable for patients
and their families.
Cendron etal. dened continence as dry intervals of 2h or more or on clean
intermittent catheterization. Of 4 patients with Glandular epispadias, 3 (75%) were
continent. Of 8 patients with penile epispadias, 5 (63%) were continent. Of 14
patients with penopubic epispadias, 9 (64%) were continent. In this series, the bladder neck was repaired using the Young-Dees-Leadbetter repair or sling bladder neck
suspension [33].
Thomas etal. dened continence as not needing to wear any protection (e.g.,
incontinence pad for urine leakage) with patient reporting to be dry between voids
or catheterizations. Of the 3 distal epispadias, 1 required a continence procedure
with a total continence rate of 100%. Of the 3 midshaft epispadias patients, 2
required a continence procedure with the total continence rate of 67% (2/3).
Continence surgery included injection of bulking agents (Macroplastique), bladder
neck reconstruction (Young-Dees-Leadbetter), bladder neck sling, Kelly procedure,
and a combination of above [27].
Braga etal. denition of continence was as follows: continent (dry intervals of at
least 4h), partially continent (2- to 4-h dry intervals), and incontinent (dry intervals
of less than 2h). They also stratied the continence rate based on the procedure
performed (Cantwell-Ransley repair versus Mitchell-Bagli repair). Continence was
100% in 3 glandular and 9 penile epispadias patients. Of 17 penopubic epispadias,
11 had undergone the Cantwell-Ransley repair and 6 had undergone the MitchellBagli repair. Of the 11 Cantwell-Ransley repairs, 8 (73%) were continent and 3
(27%) were partially continent. Of the 6 Mitchell-Bagli repairs, 5 (83%) were continent and 1(17%) was partially incontinent [28].
Bencic etal. dened urinary continence as dry for more than 2h. They reported
their results on 31 patients with isolated epispadias. Of the 19 patients with penopubic epispadias, 9 had a single-stage repair, 6 had 2 repairs, and 4 had multiple
repairs. Of these, 12 (63%) are continent and 1 (5.3%) is continent on clean intermittent catheterization. Of the 8 patients with penile epispadias, 7 had a single-stage
repair and 1 had 2-stage repair. Of these, 7 (87.5%) are continent and 1 (12.5%) is
incontinent. All 4 glandular epispadias patients had one repair, and they are all continent [29].