Keywords
epispadias - redo surgery - penile disassembly
Introduction
Epispadias presents the most severe congenital anomaly of the penis that requires
advanced surgical skills for successful repair and satisfactory outcome. The main
hallmark of the epispadias is partially or completely opened urethra on the dorsal
side of the penis, usually combined with marked dorsal curvature. Glans is always
opened dorsally and complete penile body is significantly shortened.[1] The goal of epispadias repair is reconstruction of functionally and cosmetically
acceptable penis while attaining the maximum corporal length as possible. Despite
constant improvement in surgical approach, high incidence of failure rate is widely
present due to rarity of this anomaly and severity of penile deformities. Usually,
patients undergo several surgical procedures in childhood with questionable results.
Outcome can be severely worsened after penile growth in puberty due to increasing
of residual dorsal curvature. Inadequate penile length becomes more obvious in adolescents
and their expectations for functional and aesthetically acceptable penis impose successful
redo epispadias surgery. Our aim was to present a radical approach for correction
of all penile deformities in patients after epispadias repair in childhood.
Patients/Materials and Methods
Patients/Materials and Methods
Between January 2006 and January 2011, 13 patients, aged 13 to 22 years, underwent
redo surgery due to failed epispadias repair in childhood. The number of previous
repairs in childhood ranged from one to five (median 2.2). All patients had severe
dorsal curvature and short penile urethra and were continent before redo surgery ([Fig. 1]). Mean preoperative penile length was 9.2 ± 1.1 cm (ranged 6.9 to 10.3 cm). Patients
were operated in two-stage procedure. First stage included penile disassembly and
grafting procedure for complete straightening and lengthening of the penis, and second
stage included reconstruction of the penile urethra using buccal mucosa graft and
scrotal hairless skin flap.
Fig. 1 Appearance of the treated epispadias in childhood. Severe curvature is visible in
erect penis.
Operative Technique
Standard circumferential incision was made under the glans. Complete penile degloving
was performed carefully to avoid injury of the skin. All scars around corporal bodies,
from the previous surgeries, were released. After degloving, severely deformed corporal
bodies were identified, and the urethra was very short and positioned dorsally. Lifting
of the neurovascular bundle began on the ventrolateral side of the penis. After lifting
the neurovascular bundle, subtotal glans mobilization was performed for additional
release of corporal bodies. Subcoronal plexus of the glans was preserved by meticulous
dissection. Separation of the corporal bodies was done along with the dissection of
the urethra. In this way, penile disassembly was completed. Artificial erection or
erection induced by prostaglandin E1 injection revealed all deformities of the corporal
bodies. Short urethra, which was limiting factor for straightening of the penis, was
transected at subcoronal level. Complete straightening of the corporal bodies was
achieved by incision made on the dorsal surface of the tunica albuginea, directly
opposite to the site of maximal curvature. The defects of the tunica albuginea were
closed by bovine or equine pericardium ([Fig. 2]). Erection was re-established, and good result of penile curvature correction was
confirmed. Corporal bodies were sutured together and neurovascular bundle was fixed
on its anatomical position. Urethral remnant was transposed ventrally and urethral
orifice was fixed at the base of the penis ([Fig. 3]). Gap between glanular part of the urethra and urethral stump was left for the second
stage repair. Penile entities were reassembled in normal anatomical relationship.
Penile skin was reconstructed using remaining skin flaps. At the end of the first
stage, complete straightening and lengthening of the penis was achieved while urethral
orifice was positioned at the base of the penis on its ventral side. After 6 months,
patients underwent second stage procedure that included repair of resultant hypospadias
([Fig. 4]). Longitudinal scrotal island skin flap with abundant vascular pedicle was carefully
harvested. If needed, patient underwent laser epilation of the scrotal skin before
surgery. Buccal mucosal graft was taken from the inner patient's cheek and fixed to
the corporal bodies to create dorsal part of the newly reconstructed urethra. Previously
harvested longitudinal island scrotal skin flap was joined with mucosal graft over
16-Fr catheter using 5/0 poliglecaprone 25 running suture and anastomosed to glanular
part of urethra ([Fig. 5]). In this way, missing urethra was formed. A very wide and abundant pedicle flap
covered all suture lines to prevent fistula formation. Remaining penile and scrotal
skin was used to cover penile shaft. Finally, penis of satisfactory size and shape
was created together with complete reconstruction of the urethra ([Fig. 6]). Urethral stent was left for 2 weeks, while urinary drainage was enabled by suprapubic
tube.
Fig. 2 Penile disassembly was done. Corpora cavernosa were lengthened and straightened by
penile tunical incision and grafting.
Fig. 3 Corpora cavernosa are joined, neurovascular bundles are placed dorsally, and glans
is fixed in proper position. Short urethra is repositioned ventrally at the penoscrotal
angle.
Fig. 4 Appearance 6 months after surgery. Hypospadiac urethra is visible at the base of
the penis.
Fig. 5 Urethral lengthening is done by combined buccal mucosa graft and longitudinal scrotal
skin flap.
Fig. 6 Appearance at the end of surgery. Penis is straightened and lengthened with urethral
meatus positioned at the tip of the glans.
Results
Mean follow-up was 33 months (ranged from 12 to 60 months). There was no injury of
the neurovascular bundles and no sign of corporal or glans necrosis. Patients were
followed by treating surgeon at 1, 3, 6, and 12 months and yearly thereafter. Follow-up
included measurement of the penile length as well as voiding function. Last appointment
included a questionnaire about surgery outcome. Ten patients have completely straightened
and lengthened penis. Three patients manifested mild curvature that did not require
further treatment. Nine patients were sexually active and they reported satisfactory
sexual intercourse. One patient developed fistula that was closed 4 months later,
while other patients reported regular voiding with no difficulties ([Table 1]).
Table 1
Surgical outcome 12 months after surgery
|
No. of patient
|
Age (years)
|
Preoperative penis length in erection (cm)
|
Postoperative penis length in erection (cm)
|
Residual penile curvature present
|
Sexually active
|
|
1
|
16
|
10.1
|
11.7
|
–
|
–
|
|
2
|
17
|
10.3
|
12.4
|
–
|
Yes
|
|
3
|
13
|
6.9
|
9.6
|
Yes, mild
|
–
|
|
4
|
18
|
9.3
|
10.2
|
–
|
Yes
|
|
5
|
15
|
9.6
|
11.5
|
–
|
–
|
|
6
|
19
|
10.1
|
12.7
|
–
|
Yes
|
|
7
|
18
|
9.8
|
11.9
|
Yes, mild
|
Yes
|
|
8
|
14
|
7.6
|
9.8
|
–
|
–
|
|
9
|
21
|
10.2
|
12.8
|
–
|
Yes
|
|
10
|
17
|
8.9
|
10.1
|
–
|
Yes
|
|
11
|
22
|
9.4
|
11.2
|
–
|
Yes
|
|
12
|
16
|
7.8
|
9.3
|
Yes, mild
|
Yes
|
|
13
|
18
|
9.5
|
11.7
|
–
|
Yes
|
Discussion
Different surgical approaches have been applied in attempt to successfully repair
epispadias, as one of the most severe penile anomaly. Regardless of the technique
used, the goal of epispadias repair is reconstruction of functionally and aesthetically
acceptable penis while attaining the maximum corporal length. Level of the defect
and degree of the curvature impose the complexity of the surgical procedure. Although
a large number of different surgical approaches were published, two major principles
were widely accepted. The modified Cantwell–Ransley technique includes partial mobilization
and tabularization of the urethra followed with rotation of the corporal bodies.[2]
[3] Additional straightening was achieved by transversal incision of the tunica albuginea
and longitudinal suturing of joined corporal bodies (cavernosocavernostomy). Surgical
centers published their favorable results with modified Cantwell–Ransley procedure.[4]
[5] Another widely accepted procedure was published by Mitchell and Bägli and includes
penile disassembly with complete dissection of the urethral plate and separation of
corporal bodies with its hemiglans.[6] Complete dividing of the corporal bodies enables adequate medial rotation of the
corpora with proper ventralization of the tubularized urethral plate and correction
of the dorsal curvature. Many centers reported satisfactory results with complete
disassembly procedure.[7]
[8]
[9] Lengthening of the penis in disassembly procedure is achieved by excessive mobilization
of freed corporal bodies. The urethra is brought to the glans if adequate length is
present; otherwise, the resultant hypospadias can be repaired in a second stage. According
to the published data, resultant hypospadias is fairly common and it was reported
in 36 to 77% cases that underwent Mitchell–Bagli repair.[9]
[10]
[11]
[12] Several modifications of standard disassembly procedure have been reported.[13]
[14] In contrast to Mitchell–Bagli disassembly procedure, cornerstones of Perovic disassembly
technique include complete detachment of corporal bodies from the glans and lifting
of the neurovascular bundles.[13] Marked dorsal curvature of the corporal bodies is corrected by incision on dorsal,
concave ridge of the corpora, and grafting the defects. Perovic disassembly technique
probably allows maximum of the possible lengthening because corpora are completely
detached from urethra, glans, and neurovascular bundles. After that maneuver corporal
bodies can be easily incised and grafted to obtain maximum length and complete straightening.
Complications after epispadias repair are not rare and include: fistula formation,
urethral stricture, meatal stenosis, glans or wound dehiscence, residual curvature,
skin necrosis, and severe ischemic injury with loss of the glans or corporeal body.[15] Available reports usually present complications in short time follow-up and some
of this reports are extended to 4 or 7 years postoperatively.[5]
[9] However, there is no available data about delayed outcomes in patients who reached
full sexual maturity. Some of the complications as residual dorsal curvature can become
worsen after penile growth during puberty. Several authors noted that several complications
after hypospadias repair may occur many years after initially successful outcome.[16]
[17] It should be emphasized that follow-up of the patients who underwent reconstructive
penile surgery should be extended until patients fulfill their sexual functionality.
Patients in our group underwent one to five procedures (median 2.2) during childhood
for epispadias repair. All of them had severe scar formations that made disassembly
procedure very difficult and challenging. Special care was taken to avoid iatrogenic
vascular injury with possible disastrous consequences. Complete straightening and
lengthening of the penis was imperative in our patients' expectations. Preoperatively,
all patients in our group had severe dorsal curvature that would make sexual intercourse
impossible or extremely awkward. Penile length is of major concern especially in pubertal
males. Complete penile disassembly, with lifting of neurovascular bundles and transecting
of short urethra, enabled full proximal mobilization of corporeal bodies. Finally,
additional straightening and lengthening was easily achieved by incision of tunica
albuginea on the dorsal side and grafting of the defects. Epispadiac penis is significantly
shorter because of marked congenital deficiency of anterior corporal tissue. Therefore,
maximum straightening and lengthening should be attempted to give the patients satisfactory
result. Plication of ventral convex side of the corpora should be avoided as it inevitably
leads to additional shortening of the penis. Potentially, the best results can be
obtained by maximal proximal mobilization of the corpora and grafting of the shorter
side of the corporal bodies. Postoperatively, usage of vacuum device was advised to
our patients to attain maximal penile length and to prevent retraction of the graft
and scar tissue.
Regardless of the technique used, resultant hypospadias repair can be a difficult
surgical task with high complication rate. Simultaneous use of hairless local skin
flap and buccal mucosa free graft enables one-stage urethral reconstruction in these
patients. Dorsal half of newly created urethra was formed by buccal mucosa graft and
ventral half by longitudinal skin flap, therefore minimizing possibility for urethral
strictures. Wide pedicle of the skin flap was fixed over the lateral suture lines
to prevent fistula formation.
Conclusion
Redo epispadias repair after failed surgery in childhood is very challenging and demanding
procedure. Lack of available healthy tissue and presence of scar formation impose
careful surgical approach. Complete penile disassembly enables full correction of
all deformities, primarily marked dorsal curvature, and short penile shaft. Radical
approach in redo epispadias repair is necessary to achieve functionally and aesthetically
satisfactory result, but it should be performed only by experienced and skilful team
in highly specialized surgical centers.
