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Blackwell Science, LtdOxford, UKBJUBJU International1465-5101BJU InternationalJune 2004
93Supplement 3
Original Article
CURRENT CONCEPTS IN HYPOSPADIOLOGY
P.D.E. MOURIQUAND and P.-Y. MURE
Current concepts in hypospadiology
P.D.E. MOURIQUAND and P.-Y. MURE
INTRODUCTION
Hypospadias can be defined as a hypoplasia of
the tissues forming the ventral aspect (ventral
radius) of the penis beyond the division of the
corpus spongiosum. It is characterized by a
ventral triangular defect whose summit is the
division of the corpus spongiosum, whose
sides are represented by the two pillars of
atretic spongiosum and whose base is the
glans itself (Fig. 1).
In the middle of this triangle, from the tip to
the base of the penis there are: a widely open
glans, the urethral plate which extends from
the ectopic urethral meatus up to the apex of
the glans, the ectopic urethral meatus, and a
segment of variable length of atretic urethra
(not surrounded by any spongiosum) which
starts where the corpus spongiosum divides
[1].
Therefore it is possible to distinguish two
main types of hypospadias: (i) one with a
distal division of the corpus spongiosum with
little or no chordee when the penis is erect
(Fig. 2); and one with a proximal division of
the corpus spongiosum with a marked degree
of hypoplasia of the tissues forming the
ventral radius, with a significant degree of
chordee (Fig. 3). Besides these two main types
there is also hypospadias for which several
procedures have failed (hypospadias cripple;
Fig. 4).
The causes of hypospadias are essentially
unknown although several avenues have
been explored to explain this anomaly:
(i) Some endocrine disorders have been
described in relation to hypospadias, mainly
caused by an insufficient secretion of
androgens, or insufficient response by the
target tissues. However, in very few cases
can these disorders be detected [2–4]; (ii)
some genetic disorders [5] could explain
why hypospadias can be found in several
members of the same family; (iii) young and
old mothers are more prone to carry a baby
with hypospadias; babies of low birth-
weight [6] and twins also have a higher risk
of having hypospadias, possibly explained
by a placental insufficiency [7]; (iv) the
significant increase of hypospadias in the
population over the last 20 years [8] raises
the role of possible environmental factors
[9], e.g. hormonal disruptors and pesticides,
etc.; (v) abnormal or insufficient growth
factors [10] could also be responsible for
these penile anomalies and could explain
the significant complication rate after
surgery.
THE THREE MAIN STEPS OF
HYPOSPADIAS SURGERY
Following the anatomical description, three
surgical steps characterize hypospadias
surgery [11]:
(i) The correction of chordee, which is
essentially the result of the atresia of the
ventral radius. The penile curvature is often a
result of the tethering of the hypoplastic skin
beyond the division of the corpus spongiosum
onto the underlying structures, mainly the
hypoplastic urethra. Degloving the penis
represents the first step of this surgery and
straightens the penis in 80% of cases. In 15%
the persistent curvature is caused by an
abnormal tethering of the urethral plate and
the hypoplastic urethra onto the ventral
aspect of the corpora. Freeing the urethral
plate from the corpora is a valuable additional
manoeuvre which straightens the penis in
most cases (Fig. 5). Finally, in 5% of cases the
penis remains curved although all the tissues
forming the ventral radius have been freed.
The residual chordee is caused by asymmetric
corpora cavernosa and requires a dorsal
corporoplasty (dorsal shortening of the
albuginea of the corpora cavernosa; Fig. 6). In
most cases the urethral plate can be
preserved, although there are situations
where the urethral strip is very poor and the
two corpora twist around it; in these cases the
urethral plate is usually sacrificed.
(ii) Once the penis is straight the missing
urethra should be replaced. The technique
chosen depends on the size and quality of the
urethral plate. (a) If the urethral plate is wide
and healthy, it can be tubularized following
the Thiersch-Duplay technique (Fig. 7) [12,13];
(b) If it is too narrow to be tubularized, the
Snodgrass urethrotomy [14] is one option
(Fig. 8) or additional tissue can be laid
on the urethral plate using a rectangle of
pedicled preputial mucosa (onlay
urethroplasty; Fig. 9 [15]), or a flap of ventral
penile skin (Mathieu; Fig. 10 [16]); (c) If the
segment of urethra to be replaced is short
(< 2 cm) and if the distal urethra is not
hypoplastic, complete mobilization of the
whole penile urethra may be adequate to
bridge the defect (Fig. 11). This technique [17],
like the Thiersch-Duplay, has the advantage of
avoiding the use of non-urethral tissue; (d) If
the urethral plate is not preservable, a tube
needs to be made to replace the missing
urethra, using either a pedicled rectangle of
preputial mucosa (Asopa-Duckett) [18]
(Fig. 12) or buccal mucosa (Fig. 13) [19]; (e)
Finally, in cripple hypospadias, no definite
guidelines can be given, although the
principles of repeat surgery are very similar to
those of primary surgery. The procedures used
are the same and sometimes need to be
associated (composite urethroplasty) in
complex situations.
(iii) Once the urethroplasty is completed the
ventral radius of the penis needs to be
reconstructed. This includes: (a) Meatoplasty
creating a slit-shaped meatus; (b)
glanuloplasty to reconstruct the ventral
aspect of the glans; (c) creating a mucosal
collar around the glans [20]; (d) coverage of
the neourethra using the lateral pillars of
spongiosum (spongioplasty); (e) skin cover
with a redistribution of the skin shaft bringing
the excess dorsal skin to the ventrum; (f) and
some prefer to reconstruct the foreskin,
others favour circumcision.
COMMON PROCEDURES USED IN
PRIMARY CASES OF HYPOSPADIAS
(i) The urethroplasty procedure does not need
to chosen before straightening the penis, and
requires a very clear assessment of the quality
of the urethral plate. (a) The incision lines are
always the same, following each side of the
urethral plate from the tip of the glans down
to the division of the corpus spongiosum
(Fig. 14); (b) The two wings of the glans are
dissected deeply and laterally until the
corporeal surface is clearly identified; (c) On
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FIG. 1. Anatomy of the ventral radius of the penis.
Urethral plate
Hypoplastic urethra
(no surrounded by spongiosum)
Normal urethra
(surrounded by spongiosum)
Border between the
preputial skin and mucosa
Preputial hood
Division of the
corpus spongiosum
Ectopic urethral meatus
FIG. 2. Hypospadias with a distal division of the
corpus spongiosum.
FIG. 3. Hypospadias with a proximal division of the
corpus spongiosum.
FIG. 4. Hypospadias cripple.
FIG. 5. The urethral plate is lifted off the ventral
aspect of the corpora cavernosa.
FIG. 6. Dorsal plication of the corpora cavernosa.
each side, the incision line follows the border
between the mucosal aspect and the
cutaneous aspect of the preputial hood; (d) If
the foreskin is not preserved the incision line
continues on the dorsum following the same
landmark; (e) The penis is fully degloved and a
first erection test performed. As noted, the
penile curvature is corrected in most cases but
in ª20% it persists and for some the
dissection of the urethral plate is by lifting it
from the anterior aspect of the corpora. For
others the urethral plate is kept attached to
the ventral aspect of the corpora and a dorsal
plication of the corpora performed using the
Nesbitt principle.
(ii) If the urethral plate is wide and healthy,
the Thiersch-Duplay (Fig. 7) procedure is
used, where the urethral plate is tubularized
around an 8 F catheter for children aged
<3 years, using a 6/0 or 7/0 resorbable
running suture.
(iii) If the urethral plate is healthy but too
narrow to be tubularized around an 8 F
catheter, either the urethral plate is incised
longitudinally on its midline from the ectopic
meatus up the glans and subsequently
tubularized around an 8 F catheter (Fig. 8)
(the Snodgrass procedure, which leaves a
dorsal raw area in the urethra which
subsequently epithelialises); or non-urethral
tissues can be laid on the urethral plate to
create a conduit. These comprise: The Mathieu
procedure (Fig. 10), where the incision line
delimits a perimeatal-based skin flap that is
folded over and sutured to the edges of the
urethral plate using 6/0 or 7/0 resorbable
running sutures; the Onlay procedure (Fig. 9)
where a rectangle of preputial mucosa is
pedicled down to the base of the penis and
transferred to the ventrum of the penis to be
laid on the urethral plate; a rectangle of
buccal mucosa harvested from the inner
aspect of the lower lip or the inner aspect of
the cheek after identifying the Stenon duct.
The buccal graft is laid on the urethral plate
following the same principles as above
(Fig. 13). If the segment of urethra to
reconstruct is short (<2 cm) and the length of
hypoplastic urethra proximal to the ectopic
meatus is short, a full mobilization of the
P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E
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FIG. 7.
Thiersch-Duplay procedure.
(d)
(b)
(a)
(c)
FIG. 8.
Snodgrass procedure.
(d)
(b)
(a)
(c)
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FIG. 9.
Onlay procedure.
(a)
(b)
(c)
(d)
(e)
(f)
FIG. 10.
Mathieu procedure.
(d)
(b)
(a)
(c)
P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E
3 0 © 2 0 0 4 B J U I N T E R N A T I O N A L
penile urethra can be used following Koff’s
technique (Fig. 11).
(iv) The urethral plate is too poor to be kept a
full tube urethroplasty is needed using either
a pedicled rectangle of preputial mucosa
(Fig. 12) or a rectangle of buccal mucosa. The
main disadvantage of these techniques is that
a proximal circular urethral anastomosis is
used, which exposes the repair to the risk of
urethral stenosis.
(v) Once the penis is straightened and the
urethroplasty completed the ventral radius of
the penis is reconstructed following the steps
mentioned above.
OTHER PROCEDURES
There are many other procedures described to
repair the missing urethra, which clearly
shows that no one is totally satisfactory.
(i) The MAGPI [21] procedure was very
fashionable in the 1980s to repair distal
hypospadias; in fact it is not a meatal
advancement but a flattening of the glans,
which gives the illusion that the meatus
reaches the glans apex. Secondary retraction
of the meatus are quite common and
therefore this procedure has become less
popular.
(ii) Various flip-flap procedure have been
described using the Mathieu principle. The
Devine-Horton [22] procedure was quite
fashionable in the 1980s until it was noted
that the urethral plate should be preserved in
most cases.
(iii) The bladder graft urethroplasty (Fig. 15)
[23] seemed to be a logical option in the late
1980s as it is the same cell lining as the
urethra. Unfortunately complications were
common and with the use of buccal mucosa,
this technique has become obsolete.
(iv) Two-stage procedures have been
popularized again by Bracka [24], who
resurrected the Cloutier [25] procedure. In a
first step the penis is straightened after
complete removal of the urethral plate. The
ventral radius of the penis is grafted by
preputial mucosa or buccal mucosa and then
tubularized a few weeks later. Although these
techniques are not commonly used they
certainly have solid indications in severe cases
or repeat surgery.
SURGICAL TIPS
The age at surgery for primary hypospadias
repair is usually 6–24 months. Hormonal
stimulation of the penis using bhCG or
testosterone or dihydrotestosterone is
sometimes indicated in patients with a small
penis, or for repeat surgery; it is unclear how
safe these treatments are in the long-term.
General anaesthesia is the rule, often
FIG. 11.
Koff procedure.
(a)
(b)
(c)
(d)
(e)
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associated with caudal or penile anaesthesia.
Magnification is commonly used in this
surgery but coagulation is often not needed
when the tourniquet is used, followed by a
slightly compressive dressing. Other surgeons
prefer bipolar coagulation or adrenaline
injection before incision. Antibiotics
protocols are extremely variable among
centres, and again the efficacy needs to be
confirmed. The use of urine drainage via a
suprapubic catheter, a transurethral bladder
catheter or dripping urethral stent varies
among surgeons; some use no drainage. The
dressing is essential after surgery and also
varies. The ‘daisy dressing’ (Fig. 16) is our
favourite as it is very comfortable for the
patient and contains postoperative bleeding.
Others prefer Opsite, silastic foam or
Tagaderm dressings. Pain control is essential
after surgery using morphine instillations,
anti-inflammatory medications,
anticholinergics and diazepam to reduce
bladder spasms.
RESULTS
Assessing hypospadias surgery requires
extensive experience and honesty, as there are
no reliable objective methods to evaluate
functional and cosmetic results. Very few
publications report long-term outcomes of
these various procedures. There are also large
differences between what the patient (or
family) thinks and the surgeon’s evaluation.
Urinary flow studies are often abnormal even
after a successful hypospadias repair, as quite
often the child changes his bladder behaviour
after repair. However, the aim should be a
straight penis with no redundant skin, with a
slit-shaped meatus, with regular scars and an
aesthetically reconstructed ventral aspect of
the glans. The child should be able to pass
water while standing, with no spraying, no
straining, no pain, in a single stream from the
apex of the glans. The penis should be straight
when erected.
MAIN COMPLICATIONS
A poor cosmetic result is the most common
complication, often related to irregular and
asymmetric scars with skin ‘blobs’ and an
excess of ventral skin (Fig. 17). The mucosal
collar is an important addition to improve
penile cosmesis.
Urethral fistula is the second most common
problem met in this surgery (Fig. 18),
often lateral at the coronal level. The
temptation to simply close the fistula is
dangerous, as recurrent fistulae are quite
common at this level. Often it is more
advisable to repeat the whole urethroplasty.
Urethral fistula can also be associated
with a urethral stricture, which should be
treated concomitantly. The causes of
fistulae remains unknown although it is
likely that local infection, local ischaemia,
an inadequate procedure or the poor
FIG. 12.
Asopa-Duckett procedure.
(a)
(b)
(c)
(d)
(e)
(f)
P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E
3 2 © 2 0 0 4 B J U I N T E R N A T I O N A L
FIG. 15. (a) Bladder graft urethroplasty: anterior
aspect of the bladder after detrusorotomy. (b)
Bladder graft urethroplasty (tube).
(b)
(a)
FIG. 16. Daisy dressing and double nappies.
FIG. 17. A poor cosmetic result.
FIG. 18. Urethral fistula.FIG. 13. (a) Buccal graft urethroplasty: the inner
aspect of the lower lip. (b) Onlay buccal graft
urethroplasty (hypospadias cripple).
(a)
(b)
FIG. 14. The incision lines follow each edge of the
urethral plate from the apex of the glans down to
the division of the corpus spongiosum. The lines
following the border between the preputial skin and
the preputial mucosa cross where the corpus
spongiosum divides.
FIG. 19. (a) Urethrocele with a distal stenosis of the
urethra. (b) X-ray of the urethra of the same patient.
(a)
(b)
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healing abilities of the patient might be
involved.
Urethral strictures are less common as onlay
urethroplasties are more popular and avoid
circular anastomosis, which certainly
increases this risk. Urethral dilatations are
very poorly accepted in children. Internal
urethrotomies have a poor record and often
a segmental urethroplasty is needed using a
patch onlay with buccal mucosa. In other
cases a full repeat urethroplasty is
preferable. Meatal stenosis remains a
common complication and requires either
dilatation or better, a meatotomy or
meatoplasty.
A urethrocele (Fig. 19) is caused by the
progressive distension of the reconstructed
urethra, possibly resulting from a distal
urethral stenosis or the difference of
compliance of the tissues used for
urethroplasty compared to the native
urethra. Excision of the redundant tissue
is the usual treatment, with a good
backing of the neourethra with the
surrounding tissues to avoid further
urethral distension.
Balanitis xerotica obliterans is an annoying
complication as it tends to recur; it causes
recurrent meatal stenosis and sometimes
pain on voiding when it develops inside
the reconstructed urethra. Its cause is
unknown.
Urethral prolapse (bladder mucosa), urethral
stones (hairy skin), sticking meatus (bladder
mucosa), meatal retraction (MAGPI) are also
reported but are probably less common with
modern techniques.
Results with some of the most common
techniques include: (a) The Mathieu
procedure gives excellent results, with 1%
meatal stenosis and ª5% fistula rate [26]. One
common criticism of the Mathieu procedure is
its half-moon shaped meatus. (b) The Koff
urethral mobilization has a very low fistula
rate but meatal stenosis is common, especially
if the distal urethra left in place is hypoplastic.
In our experience the meatal stenosis rate is
ª20% [27]. (c) The Duplay-Snodgrass
procedure has a much higher complication
rate in proximal hypospadias (about a third of
patients required complementary surgery)
[28]. (d) The Onlay procedure has a
complication rate of 15–50%; Elbackry [29]
reported a 7% breakdown, 23% fistulae, 9%
strictures and 3% diverticulae. (e) Buccal graft
urethroplasties often require subsequent
surgical revisions. One group reported a 57%
complication rate with five (of 30) with
meatal stenosis, seven strictures, two fistulae
and one breakdown over a 5-year follow-up
[30]. (f) The Asopa/Duckett tube has a poor
long-term record, as does bladder mucosa
graft urethroplasty; 66% of Ransley’s patients
required 1–9 additional procedures to treat
complications [31]. The most common
complications were meatal stenosis and/or
urethral prolapse. (g) Two-stage procedures
produce a good cosmetic result but
significant urethral complications, e.g.
stenosis in 7% [24]. (h) Composite repair
using the prepuce has a reoperation rate of
almost half [32].
CONCLUSIONS
Hypospadias surgery remains a difficult
challenge, as several factors contributing to
success remain unknown. One of the most
intriguing is the variation in the ‘healing
ability’ among patients. With the development
of tissue engineering it is hoped that urethral
substitution using the patient’s urethral tissue
might be a future avenue to resolve the
current difficulties. A long-term follow-up of
these patients appears to be crucial for
assessing and validating the various
techniques currently available. The problem
is how to follow these patients. Clinical
examination of the penis is highly subjective;
assessing the urinary stream is difficult, as
urine flow studies are very often abnormal
after urethral reconstruction. Finally, the
experience and honesty of the paediatric
urologist remain the two most important
factors in hypospadiology. Collaboration with
paediatric endocrinologists is also important
to increase the chances of surgical success.
Treatment before and after surgery may be
helpful for improving the patient’s ‘healing
ability’.
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18 Asopa HS, Elhence EP, Atria SP, Bansal
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32 Jayanti VR, McLorie GA, Khoury AE,
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Correspondence: Professor P.D.E.
Mouriquand, Department of Paediatric
Urology, Debrousse Hospital, 29, rue Soeur
Bouvier, 69322 Lyon Cedex 05, France.
e-mail: pierre.mouriquand@chu-lyon.fr

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CURRENT CONCEPTS

  • 1. 2 6 © 2 0 0 4 B J U I N T E R N A T I O N A L | 9 3 , S U P P L E M E N T 3 , 2 6 – 3 4 Blackwell Science, LtdOxford, UKBJUBJU International1465-5101BJU InternationalJune 2004 93Supplement 3 Original Article CURRENT CONCEPTS IN HYPOSPADIOLOGY P.D.E. MOURIQUAND and P.-Y. MURE Current concepts in hypospadiology P.D.E. MOURIQUAND and P.-Y. MURE INTRODUCTION Hypospadias can be defined as a hypoplasia of the tissues forming the ventral aspect (ventral radius) of the penis beyond the division of the corpus spongiosum. It is characterized by a ventral triangular defect whose summit is the division of the corpus spongiosum, whose sides are represented by the two pillars of atretic spongiosum and whose base is the glans itself (Fig. 1). In the middle of this triangle, from the tip to the base of the penis there are: a widely open glans, the urethral plate which extends from the ectopic urethral meatus up to the apex of the glans, the ectopic urethral meatus, and a segment of variable length of atretic urethra (not surrounded by any spongiosum) which starts where the corpus spongiosum divides [1]. Therefore it is possible to distinguish two main types of hypospadias: (i) one with a distal division of the corpus spongiosum with little or no chordee when the penis is erect (Fig. 2); and one with a proximal division of the corpus spongiosum with a marked degree of hypoplasia of the tissues forming the ventral radius, with a significant degree of chordee (Fig. 3). Besides these two main types there is also hypospadias for which several procedures have failed (hypospadias cripple; Fig. 4). The causes of hypospadias are essentially unknown although several avenues have been explored to explain this anomaly: (i) Some endocrine disorders have been described in relation to hypospadias, mainly caused by an insufficient secretion of androgens, or insufficient response by the target tissues. However, in very few cases can these disorders be detected [2–4]; (ii) some genetic disorders [5] could explain why hypospadias can be found in several members of the same family; (iii) young and old mothers are more prone to carry a baby with hypospadias; babies of low birth- weight [6] and twins also have a higher risk of having hypospadias, possibly explained by a placental insufficiency [7]; (iv) the significant increase of hypospadias in the population over the last 20 years [8] raises the role of possible environmental factors [9], e.g. hormonal disruptors and pesticides, etc.; (v) abnormal or insufficient growth factors [10] could also be responsible for these penile anomalies and could explain the significant complication rate after surgery. THE THREE MAIN STEPS OF HYPOSPADIAS SURGERY Following the anatomical description, three surgical steps characterize hypospadias surgery [11]: (i) The correction of chordee, which is essentially the result of the atresia of the ventral radius. The penile curvature is often a result of the tethering of the hypoplastic skin beyond the division of the corpus spongiosum onto the underlying structures, mainly the hypoplastic urethra. Degloving the penis represents the first step of this surgery and straightens the penis in 80% of cases. In 15% the persistent curvature is caused by an abnormal tethering of the urethral plate and the hypoplastic urethra onto the ventral aspect of the corpora. Freeing the urethral plate from the corpora is a valuable additional manoeuvre which straightens the penis in most cases (Fig. 5). Finally, in 5% of cases the penis remains curved although all the tissues forming the ventral radius have been freed. The residual chordee is caused by asymmetric corpora cavernosa and requires a dorsal corporoplasty (dorsal shortening of the albuginea of the corpora cavernosa; Fig. 6). In most cases the urethral plate can be preserved, although there are situations where the urethral strip is very poor and the two corpora twist around it; in these cases the urethral plate is usually sacrificed. (ii) Once the penis is straight the missing urethra should be replaced. The technique chosen depends on the size and quality of the urethral plate. (a) If the urethral plate is wide and healthy, it can be tubularized following the Thiersch-Duplay technique (Fig. 7) [12,13]; (b) If it is too narrow to be tubularized, the Snodgrass urethrotomy [14] is one option (Fig. 8) or additional tissue can be laid on the urethral plate using a rectangle of pedicled preputial mucosa (onlay urethroplasty; Fig. 9 [15]), or a flap of ventral penile skin (Mathieu; Fig. 10 [16]); (c) If the segment of urethra to be replaced is short (< 2 cm) and if the distal urethra is not hypoplastic, complete mobilization of the whole penile urethra may be adequate to bridge the defect (Fig. 11). This technique [17], like the Thiersch-Duplay, has the advantage of avoiding the use of non-urethral tissue; (d) If the urethral plate is not preservable, a tube needs to be made to replace the missing urethra, using either a pedicled rectangle of preputial mucosa (Asopa-Duckett) [18] (Fig. 12) or buccal mucosa (Fig. 13) [19]; (e) Finally, in cripple hypospadias, no definite guidelines can be given, although the principles of repeat surgery are very similar to those of primary surgery. The procedures used are the same and sometimes need to be associated (composite urethroplasty) in complex situations. (iii) Once the urethroplasty is completed the ventral radius of the penis needs to be reconstructed. This includes: (a) Meatoplasty creating a slit-shaped meatus; (b) glanuloplasty to reconstruct the ventral aspect of the glans; (c) creating a mucosal collar around the glans [20]; (d) coverage of the neourethra using the lateral pillars of spongiosum (spongioplasty); (e) skin cover with a redistribution of the skin shaft bringing the excess dorsal skin to the ventrum; (f) and some prefer to reconstruct the foreskin, others favour circumcision. COMMON PROCEDURES USED IN PRIMARY CASES OF HYPOSPADIAS (i) The urethroplasty procedure does not need to chosen before straightening the penis, and requires a very clear assessment of the quality of the urethral plate. (a) The incision lines are always the same, following each side of the urethral plate from the tip of the glans down to the division of the corpus spongiosum (Fig. 14); (b) The two wings of the glans are dissected deeply and laterally until the corporeal surface is clearly identified; (c) On
  • 2. C U R R E N T C O N C E P T S I N H Y P O S P A D I O L O G Y © 2 0 0 4 B J U I N T E R N A T I O N A L 2 7 FIG. 1. Anatomy of the ventral radius of the penis. Urethral plate Hypoplastic urethra (no surrounded by spongiosum) Normal urethra (surrounded by spongiosum) Border between the preputial skin and mucosa Preputial hood Division of the corpus spongiosum Ectopic urethral meatus FIG. 2. Hypospadias with a distal division of the corpus spongiosum. FIG. 3. Hypospadias with a proximal division of the corpus spongiosum. FIG. 4. Hypospadias cripple. FIG. 5. The urethral plate is lifted off the ventral aspect of the corpora cavernosa. FIG. 6. Dorsal plication of the corpora cavernosa. each side, the incision line follows the border between the mucosal aspect and the cutaneous aspect of the preputial hood; (d) If the foreskin is not preserved the incision line continues on the dorsum following the same landmark; (e) The penis is fully degloved and a first erection test performed. As noted, the penile curvature is corrected in most cases but in ª20% it persists and for some the dissection of the urethral plate is by lifting it from the anterior aspect of the corpora. For others the urethral plate is kept attached to the ventral aspect of the corpora and a dorsal plication of the corpora performed using the Nesbitt principle. (ii) If the urethral plate is wide and healthy, the Thiersch-Duplay (Fig. 7) procedure is used, where the urethral plate is tubularized around an 8 F catheter for children aged <3 years, using a 6/0 or 7/0 resorbable running suture. (iii) If the urethral plate is healthy but too narrow to be tubularized around an 8 F catheter, either the urethral plate is incised longitudinally on its midline from the ectopic meatus up the glans and subsequently tubularized around an 8 F catheter (Fig. 8) (the Snodgrass procedure, which leaves a dorsal raw area in the urethra which subsequently epithelialises); or non-urethral tissues can be laid on the urethral plate to create a conduit. These comprise: The Mathieu procedure (Fig. 10), where the incision line delimits a perimeatal-based skin flap that is folded over and sutured to the edges of the urethral plate using 6/0 or 7/0 resorbable running sutures; the Onlay procedure (Fig. 9) where a rectangle of preputial mucosa is pedicled down to the base of the penis and transferred to the ventrum of the penis to be laid on the urethral plate; a rectangle of buccal mucosa harvested from the inner aspect of the lower lip or the inner aspect of the cheek after identifying the Stenon duct. The buccal graft is laid on the urethral plate following the same principles as above (Fig. 13). If the segment of urethra to reconstruct is short (<2 cm) and the length of hypoplastic urethra proximal to the ectopic meatus is short, a full mobilization of the
  • 3. P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E 2 8 © 2 0 0 4 B J U I N T E R N A T I O N A L FIG. 7. Thiersch-Duplay procedure. (d) (b) (a) (c) FIG. 8. Snodgrass procedure. (d) (b) (a) (c)
  • 4. C U R R E N T C O N C E P T S I N H Y P O S P A D I O L O G Y © 2 0 0 4 B J U I N T E R N A T I O N A L 2 9 FIG. 9. Onlay procedure. (a) (b) (c) (d) (e) (f) FIG. 10. Mathieu procedure. (d) (b) (a) (c)
  • 5. P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E 3 0 © 2 0 0 4 B J U I N T E R N A T I O N A L penile urethra can be used following Koff’s technique (Fig. 11). (iv) The urethral plate is too poor to be kept a full tube urethroplasty is needed using either a pedicled rectangle of preputial mucosa (Fig. 12) or a rectangle of buccal mucosa. The main disadvantage of these techniques is that a proximal circular urethral anastomosis is used, which exposes the repair to the risk of urethral stenosis. (v) Once the penis is straightened and the urethroplasty completed the ventral radius of the penis is reconstructed following the steps mentioned above. OTHER PROCEDURES There are many other procedures described to repair the missing urethra, which clearly shows that no one is totally satisfactory. (i) The MAGPI [21] procedure was very fashionable in the 1980s to repair distal hypospadias; in fact it is not a meatal advancement but a flattening of the glans, which gives the illusion that the meatus reaches the glans apex. Secondary retraction of the meatus are quite common and therefore this procedure has become less popular. (ii) Various flip-flap procedure have been described using the Mathieu principle. The Devine-Horton [22] procedure was quite fashionable in the 1980s until it was noted that the urethral plate should be preserved in most cases. (iii) The bladder graft urethroplasty (Fig. 15) [23] seemed to be a logical option in the late 1980s as it is the same cell lining as the urethra. Unfortunately complications were common and with the use of buccal mucosa, this technique has become obsolete. (iv) Two-stage procedures have been popularized again by Bracka [24], who resurrected the Cloutier [25] procedure. In a first step the penis is straightened after complete removal of the urethral plate. The ventral radius of the penis is grafted by preputial mucosa or buccal mucosa and then tubularized a few weeks later. Although these techniques are not commonly used they certainly have solid indications in severe cases or repeat surgery. SURGICAL TIPS The age at surgery for primary hypospadias repair is usually 6–24 months. Hormonal stimulation of the penis using bhCG or testosterone or dihydrotestosterone is sometimes indicated in patients with a small penis, or for repeat surgery; it is unclear how safe these treatments are in the long-term. General anaesthesia is the rule, often FIG. 11. Koff procedure. (a) (b) (c) (d) (e)
  • 6. C U R R E N T C O N C E P T S I N H Y P O S P A D I O L O G Y © 2 0 0 4 B J U I N T E R N A T I O N A L 3 1 associated with caudal or penile anaesthesia. Magnification is commonly used in this surgery but coagulation is often not needed when the tourniquet is used, followed by a slightly compressive dressing. Other surgeons prefer bipolar coagulation or adrenaline injection before incision. Antibiotics protocols are extremely variable among centres, and again the efficacy needs to be confirmed. The use of urine drainage via a suprapubic catheter, a transurethral bladder catheter or dripping urethral stent varies among surgeons; some use no drainage. The dressing is essential after surgery and also varies. The ‘daisy dressing’ (Fig. 16) is our favourite as it is very comfortable for the patient and contains postoperative bleeding. Others prefer Opsite, silastic foam or Tagaderm dressings. Pain control is essential after surgery using morphine instillations, anti-inflammatory medications, anticholinergics and diazepam to reduce bladder spasms. RESULTS Assessing hypospadias surgery requires extensive experience and honesty, as there are no reliable objective methods to evaluate functional and cosmetic results. Very few publications report long-term outcomes of these various procedures. There are also large differences between what the patient (or family) thinks and the surgeon’s evaluation. Urinary flow studies are often abnormal even after a successful hypospadias repair, as quite often the child changes his bladder behaviour after repair. However, the aim should be a straight penis with no redundant skin, with a slit-shaped meatus, with regular scars and an aesthetically reconstructed ventral aspect of the glans. The child should be able to pass water while standing, with no spraying, no straining, no pain, in a single stream from the apex of the glans. The penis should be straight when erected. MAIN COMPLICATIONS A poor cosmetic result is the most common complication, often related to irregular and asymmetric scars with skin ‘blobs’ and an excess of ventral skin (Fig. 17). The mucosal collar is an important addition to improve penile cosmesis. Urethral fistula is the second most common problem met in this surgery (Fig. 18), often lateral at the coronal level. The temptation to simply close the fistula is dangerous, as recurrent fistulae are quite common at this level. Often it is more advisable to repeat the whole urethroplasty. Urethral fistula can also be associated with a urethral stricture, which should be treated concomitantly. The causes of fistulae remains unknown although it is likely that local infection, local ischaemia, an inadequate procedure or the poor FIG. 12. Asopa-Duckett procedure. (a) (b) (c) (d) (e) (f)
  • 7. P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E 3 2 © 2 0 0 4 B J U I N T E R N A T I O N A L FIG. 15. (a) Bladder graft urethroplasty: anterior aspect of the bladder after detrusorotomy. (b) Bladder graft urethroplasty (tube). (b) (a) FIG. 16. Daisy dressing and double nappies. FIG. 17. A poor cosmetic result. FIG. 18. Urethral fistula.FIG. 13. (a) Buccal graft urethroplasty: the inner aspect of the lower lip. (b) Onlay buccal graft urethroplasty (hypospadias cripple). (a) (b) FIG. 14. The incision lines follow each edge of the urethral plate from the apex of the glans down to the division of the corpus spongiosum. The lines following the border between the preputial skin and the preputial mucosa cross where the corpus spongiosum divides. FIG. 19. (a) Urethrocele with a distal stenosis of the urethra. (b) X-ray of the urethra of the same patient. (a) (b)
  • 8. C U R R E N T C O N C E P T S I N H Y P O S P A D I O L O G Y © 2 0 0 4 B J U I N T E R N A T I O N A L 3 3 healing abilities of the patient might be involved. Urethral strictures are less common as onlay urethroplasties are more popular and avoid circular anastomosis, which certainly increases this risk. Urethral dilatations are very poorly accepted in children. Internal urethrotomies have a poor record and often a segmental urethroplasty is needed using a patch onlay with buccal mucosa. In other cases a full repeat urethroplasty is preferable. Meatal stenosis remains a common complication and requires either dilatation or better, a meatotomy or meatoplasty. A urethrocele (Fig. 19) is caused by the progressive distension of the reconstructed urethra, possibly resulting from a distal urethral stenosis or the difference of compliance of the tissues used for urethroplasty compared to the native urethra. Excision of the redundant tissue is the usual treatment, with a good backing of the neourethra with the surrounding tissues to avoid further urethral distension. Balanitis xerotica obliterans is an annoying complication as it tends to recur; it causes recurrent meatal stenosis and sometimes pain on voiding when it develops inside the reconstructed urethra. Its cause is unknown. Urethral prolapse (bladder mucosa), urethral stones (hairy skin), sticking meatus (bladder mucosa), meatal retraction (MAGPI) are also reported but are probably less common with modern techniques. Results with some of the most common techniques include: (a) The Mathieu procedure gives excellent results, with 1% meatal stenosis and ª5% fistula rate [26]. One common criticism of the Mathieu procedure is its half-moon shaped meatus. (b) The Koff urethral mobilization has a very low fistula rate but meatal stenosis is common, especially if the distal urethra left in place is hypoplastic. In our experience the meatal stenosis rate is ª20% [27]. (c) The Duplay-Snodgrass procedure has a much higher complication rate in proximal hypospadias (about a third of patients required complementary surgery) [28]. (d) The Onlay procedure has a complication rate of 15–50%; Elbackry [29] reported a 7% breakdown, 23% fistulae, 9% strictures and 3% diverticulae. (e) Buccal graft urethroplasties often require subsequent surgical revisions. One group reported a 57% complication rate with five (of 30) with meatal stenosis, seven strictures, two fistulae and one breakdown over a 5-year follow-up [30]. (f) The Asopa/Duckett tube has a poor long-term record, as does bladder mucosa graft urethroplasty; 66% of Ransley’s patients required 1–9 additional procedures to treat complications [31]. The most common complications were meatal stenosis and/or urethral prolapse. (g) Two-stage procedures produce a good cosmetic result but significant urethral complications, e.g. stenosis in 7% [24]. (h) Composite repair using the prepuce has a reoperation rate of almost half [32]. CONCLUSIONS Hypospadias surgery remains a difficult challenge, as several factors contributing to success remain unknown. One of the most intriguing is the variation in the ‘healing ability’ among patients. With the development of tissue engineering it is hoped that urethral substitution using the patient’s urethral tissue might be a future avenue to resolve the current difficulties. A long-term follow-up of these patients appears to be crucial for assessing and validating the various techniques currently available. The problem is how to follow these patients. Clinical examination of the penis is highly subjective; assessing the urinary stream is difficult, as urine flow studies are very often abnormal after urethral reconstruction. Finally, the experience and honesty of the paediatric urologist remain the two most important factors in hypospadiology. Collaboration with paediatric endocrinologists is also important to increase the chances of surgical success. Treatment before and after surgery may be helpful for improving the patient’s ‘healing ability’. REFERENCES 1 Mouriquand P, Mure PY. Hypospadias. In Gearhart J, Rink R, Mouriquand P eds. Pediatric Urology. Philadelphia: WB Saunders, 2001, 713–28 2 Ahmed SF, Cheng A, Hughes IA. Assessment of the gonadotrophin- gonadal axis in androgen insensitivity syndrome. Arch Dis Child 1999; 80: 324–9 3 Boehmer ALM, Nijman RJ, Lammers BA et al. Etiological studies of severe or familial hypospadias. J Urol 2001; 165: 1246–54 4 Feyaerts A, Forest MG, Morel Y et al. Endocrine screening in 32 consecutive patients with hypospadias. J Urol 2002; 168: 720–5 5 Stoll C, Alembik Y, Roth MP, Dott B. Genetic and environmental factors in hypospadias. J Med Genet 1990; 27: 559– 63 6 Gatti JM, Kirsch AJ, Troyer WA, Perez- Brayfield MR, Smith EA, Scherz HC. Increased incidence of hypospadias in small-for-gestational age infants in a neonatal intensive care unit. BJUInt 2001; 87: 548–50 7 Fredell L, Kockum I, Hansson E et al. Heredity of hypospadias and the significance of low birth weight. J Urol 2002; 167: 1423–7 8 Paulozzi LJ. International trends in rates of hypospadias and cryptorchidism. Envir Health Perspect 1999; 107: 297–302 9 Sultan C, Balaguer P, Terouanne B et al. Environmental xenooestrogens, antiandrogens and disorders of male sexual differentiation. Mol Cell Endocrinol 2001; 178: 99–105 10 El-Galley RE, Smith E, Cohen C, Petros JA, Woodard J, Galloway J. Epidermal growth factor (EGF) and EGF receptor in hypospadias. Br J Urol 1997; 79: 116–9 11 Mouriquand PDE, Persad R, Sharma S. Hypospadias repair: Current principles and procedures. Br J Urol 1995; 76: 9–22 12 Thiersch C. Uber die entstehungswise und operative behandlung der epispadie. Arch Heitkunde 1869; 10: 20–5 13 Duplay S. De l’hypospade périnéoscrotal et de son traitement chirurgical. Arch General Med 1874; 1: 613–57 14 Snodgrass W. Tubularized incised plate urethroplasty for distal hypospadias. J Urol 1994; 151: 464–9 15 Elder JS, Duckett JW, Snyder HM. Onlay island flap in the repair of mid and distal hypospadias with chordee. J Urol 1987; 138: 376–9 16 Mathieu P. Traitement en un temps de l’hypospade balanique et juxta-balanique. J Chir (Paris) 1932; 39: 481–4 17 Koff SA. Mobilization of the urethra in the surgical treatment of hypospadias. J Urol 1981; 125: 394–7 18 Asopa HS, Elhence EP, Atria SP, Bansal NK. One stage correction of penile
  • 9. P. D . E . M O U R I Q U A N D a n d P. - Y. M U R E 3 4 © 2 0 0 4 B J U I N T E R N A T I O N A L hypospadias using a foreskin tube. A preliminary report. Int Surg 1997; 55: 435–40 19 Dessanti A, Rigamonti W, Merulla V, Falchetti D, Caccia G. Autologous buccal mucosa graft for hypospadias repair: an initial report. J Urol 1992; 147: 1081–4 20 Firlit CF. The mucosal collar in hypospadias surgery. J Urol 1987; 137: 80–2 21 Duckett JW. MAGPI (meatoplasty and glanuloplasty): a procedure for subcoronal hypospadias. Urol Clin North Am 1981; 8: 513–9 22 Devine CJ, Horton CE. One stage hypospadias repair. J Urol 1961; 85: 166 23 Mollard P, Mouriquand P, Bringeon G, Bugmann P. Repair of hypospadias using a bladder mucosa graft in 76 cases. J Urol 1989; 142: 1548–50 24 Bracka A. Hypospadias repair: The two- stage alternative. Br J Urol 1995; 76: 31– 41 25 Cloutier A. A method for hypospadias repair. Plast Reconstr Surg 1962; 30: 368– 73 26 Minevich E, Pecha BR, Wacksman J, Sheldon CA. Mathieu hypospadias repair: Experience in 202 patients. J Urol 1999; 162: 2141–3 27 Paparel P, Mure PY, Garignon C, Mouriquand P. Translation uréthrale de Koff: a propos de 26 hypospades présentant une division distale du corps spongieux. Progrès en Urologie 2001; 11: 1327–30 28 Snodgrass WT, Lorenzo A. Tubularized incised-plate urethroplasty for proximal hypospadias. BJU Int 2002; 89: 90–3 29 Elbakry A. Complications of the preputial island flap-tube urethroplasty. BJU Int 1999; 84: 89–94 30 Duckett JW, Coplen D, Ewalt D, Baskin LS. Buccal mucosal urethral replacement. J Urol 1995; 153: 1660–3 31 Kinkead TM, Borzi PA, Duffy PG, Ransley PG. Long-term followup of bladder mucosa graft for male urethral reconstruction. J Urol 1994; 151: 1056–8 32 Jayanti VR, McLorie GA, Khoury AE, Churchill BM. Can previously relocated penile skin be successfully used for salvage hypospadias repair? J Urol 1994; 152: 740–3 Correspondence: Professor P.D.E. Mouriquand, Department of Paediatric Urology, Debrousse Hospital, 29, rue Soeur Bouvier, 69322 Lyon Cedex 05, France. e-mail: pierre.mouriquand@chu-lyon.fr