Surgery to the external genitalia



Surgery to the external genitalia
Surgery to the external genitalia
Antonio Macedo Jr and Miguel Srougi
Surgery to the external genitalia is one of the most challenging
chapters of reconstructive urology, due to the need to correct
complex problems such as hypospadias or epispadias
(isolated or associated to exstrophies). Although this specialty
has shown a continuous technical advancement there is no
consensus as to the most efficient surgical procedure or to the
best strategy to treat these pathologies. The papers reviewed
here reflect these statements and reinforce the idea that
surgeons' good sense, allied with their experience in the area,
are the factors that define the choice of the ideal surgical
option for each case. Curr Opin Urol 11:585±590. # 2001 Lippincott
Williams & Wilkins.
Department of Urology, Federal University of SaÄo Paulo, SaÄo Paulo, Brazil
Correspondence to Antonio Macedo Jr MD, Federal University of SaÄo Paulo, Rua
Maestro Cardim, 560/cj 215, 01323-000 SaÄo Paulo, Brazil
E-mail: [email protected]
Current Opinion in Urology 2001, 11:585±590
intracytoplasmic sperm injection
# 2001 Lippincott Williams & Wilkins
External genitalia surgery is used for reconstructive
treatment of a group of problems, encompassing
hypospadias, epispadias with or without exstrophy,
and intersex anomalies, and also for a group of day-today problems such as circumcision and testicular
pathologies. While in the second group there are few
technical divergences concerning the surgery itself, in
the ®rst group there is a great disparity in the
management, depending on the occasion and the most
appropriate technique for the correction. For example,
there are at least 300 different techniques to treat
hypospadias and exstrophy; epispadias can be treated in
several stages or, as suggested recently, in a single
stage. The objective of this paper is to focus on recent
publications in this area in order to obtain a better
understanding of the more accepted tendencies in
external genitalia pathologies, but without the claim of
de®ning the best management.
Neonatal circumcision is a routine procedure in the USA,
as opposed to the situation in Europe. In a series of 129
boys forwarded consecutively for circumcision during a
period of 3 years [1], the authors found 30 (23%) with
inconspicuous penis, a designation that includes situations such as buried penis, trapped penis, concealed
penis and micropenis. According to the authors, just as in
hypospadias and epispadias, the inconspicuous penis
must be considered a contraindication for neonatal
The treatment of penile adhesions after circumcision is
unde®ned due to the possibility of spontaneous resolution. The authors evaluated 254 circumcised boys,
classifying the degree of adherence and the age of
presentation into four groups: less than 12 months,
between 13 and 60 months, between 61 and 108 months
and more than 109 months. The authors found
adherence rates of 71, 28, 8 and 2%, respectively. They
concluded that there is no need for manual lysis of these
adherences [2].
Anesthetic blockades with or without vasoconstrictors
are used commonly in genital surgery. A case of
accidental injection of epinephrine leading to intense
vasoconstriction of the penile skin was treated successfully with a series of subcutaneous injections of
phentolamine, in doses of 0.1±0.2 mg/kg [3]. The
authors recommend this treatment in cases of signi®cant
ischemia after an accidental injection of epinephrine.
586 Paediatric urology
Circumcision is the classical treatment of phimosis.
When foreskin preservation is desired, an incision only
in the ventral face of the prepuce and not in the dorsal
face allows the release of the obstruction and the
maintenance of genital esthetics indistinguishable from
the non-circumcised penis [4].
Neonatal circumcision, lysis of labial adhesions and
meatotomy are procedures performed with local anesthesia. The parent perception of discomfort was
evaluated in the parents of 99 children submitted for
these procedures, and was classi®ed as light, moderate
or severe [5]. The authors concluded that parent
perception of discomfort was greater when the parents
followed the procedures of meatotomy and lysis of
labial adhesions than in circumcision. However, 96% of
the parents were satis®ed with not having chosen
general anesthesia [5].
Undescended testes
The non-palpable testis is ideally diagnosed by laparoscopy due to the high accuracy of this method, reported
at between 95 and 100%. The trocar access is the critical
point of laparoscopy and complications are calculated to
occur in 7.8% with the use of the Veress needle and in
3.8% with an open incision. In obese patients this
procedure can be even more dif®cult. de Filippo et al. [6]
described two cases of non-palpable testis in obese
children, identi®ed in the inguinal region with magnetic
resonance imaging, dismissing the use of laparoscopy.
The conclusion of their study was that, in the obese
patients, magnetic resonance imaging should be performed as routine before laparoscopy [6].
In a Dutch study [7], the authors reported on a case
where a small abdominal testis was found by laparoscopy, but it was noticed that the vas deferens, with
small caliber vessels, was entering the internal ring. At
inguinal exploration, the vessels and the vas deferens
were found to end in a tiny round nubbin ®rmly attached
to the upper scrotum by the gubernaculum. The authors
concluded that, if only an inguinal exploration was
performed, the intra-abdominal testis would have been
left in the peritoneal cavity, with the known risks of
malignancy, thus reinforcing the systematic indication of
laparoscopy in non-palpable testes.
The classical open exploration of the inguinal testis
mandates a second incision to ®x the testis in the
scrotum. Forty-eight consecutive patients (60 orchiopexies) with testis below the external ring were treated
by the Bianchi single high scrotal incision technique in
both primary and secondary surgeries. The conversion
into inguinal incision was needed in only four cases and
all the others were treated adequately by a high scrotal
incision. One of the authors observed that the initial
identi®cation of the testis, in cases undergoing an
operation, can be even easier due to access without
previous inguinal incision ®brosis [8].
Undescended testis and ectopic testis are situations
caused by a different physiopathologic mechanism,
although the testis has an anomalous position in both
cases; in the ectopic testis the abnormality of terminal
testicular descent happens after the passage of the
external inguinal ring. The ectopic testis can be found
in the Denis Browne super®cial inguinal pouch, the
perineum or the penis, or lateral to the scrotum, the
pubic region, the thigh or the contralateral scrotum. If
this ®nal incidence of ectopic testis occurs due to a
mechanical obstruction and not due to endocrinopathy,
it would be expected that the total germ cell count and
testicular volume would be greater than in the
undescended testis. Furthermore, a lower rate of patent
processus vaginalis and malformed epididymides would
be present in patients with an ectopic testis rather than
with an undescended testis. Hutcheson et al. [9]
compared the pathological ®ndings in 17 cases of
ectopic testis, which were not in the Denis Browne
super®cial inguinal pouch, with those of age-matched
patients with unilateral undescended testis. The
authors found no difference in total germ cell count,
testicular volume, processus vaginalis patency or
epididymal abnormalities. The conclusion of this study
was that boys with an ectopic testis also showed an
increased incidence of subfertility and testicular malignancy.
The initial hormonal treatment of cryptorchidism with
human chorionic gonadotropin or gonadotropin releasing
hormone has a success rate of around 20% and allows the
distinction of truly undescended from retractile testes.
The knowledge that the subfertility, translated by a
decrease in the number of spermatogonia and gonocytes
found in distopic testes, might be reduced by an early
surgical intervention has anticipated the age recommendation for orchiopexy. The unphysiological exposure to
follicle-stimulating hormone, luteinizing hormone and
testosterone, due to human chorionic gonadotropin or
gonadotropin releasing hormone therapy, may harm 1- to
3-year-old boys with cryptorchidism. Cortes et al. [10]
measured the number of spermatogonia per tubule at
orchiopexy in 72 consecutive boys with cryptorchidism
who underwent simultaneous testicular biopsy. Among
these patients, 27 had received previous hormone
treatment. In these patients, the authors found a lower
number of spermatogonia per tubule than in those
treated only by surgery and suggested that in 1- to 3year-old boys with chryptorchidism the gonadotropic
releasing hormone or human chorionic gonadotropin,
given for testicular descent, may suppress the number of
germ cells.
Surgery to the external genitalia Macedo and Srougi 587
The etiology of hypospadias is still the object of
discussion. The implied factors include testosterone
biosynthesis defects, 5 alpha-reductase type 2 mutations,
androgen receptor mutations, in-vitro fertilization (progesterone administration or endocrine abnormalities
associated with infertility), and environmental agents
that can disrupt the male sex hormone axis. Two papers
have been published reviewing the etiology of hypospadias [11,12 . .]. Baskin [12 . .] reviewed the current
scienti®c reports on the etiology of hypospadias and
presented the embryology and possible mechanisms of
urethral and penile formation. The author proposed a
new theory of glandular human urethral development via
endodermal cellular differentiation to replace the classic
explanation of ectodermal intrusion. A Finnish epidemiological study was conducted to de®ne the prevalence
of hypospadias in the country. The authors of this study
found 28.1 surgically treated patients per 10 000 male
live births [13]. With the development of conjugal
infertility treatment techniques, the last decade has
shown an increase in the number of births after
intracytoplasmic sperm injection (ICSI). An interesting
Swedish study was conducted to determine the incidence of congenital malformations in children born
after ICSI [14 .]. The medical records of 1139 infants
were reviewed: 736 singletons, 200 sets of twins and one
set of triplets. The total number of infants with an
identi®ed anomaly was 87 (7.6%), 40 of which were
minor. The incidence of malformations in children born
after ICSI was also compared with all births in Sweden
using data from the Swedish Medical Birth Registry and
the Registry of Congenital Malformations. The only
speci®c malformation that was found to occur in excess
in children born after ICSI was hypospadias (relative risk
3.0, 95% con®dence interval 1.09±6.50), which may be
related to paternal subfertility [14 .].
The use of the urethral plate has become popular in the
last 10 years, as has the perception that techniques such
as onlay preputial ¯ap, the glans approximation procedure and, more recently, the tubularized incised plate
repair are associated with a lower rate of complications.
The presence of a marked ventral curvature is one of the
factors that can de®ne the surgical correction in one or
two stages, as in the less severe cases a Nesbit dorsal
plication recti®es the penis and allows use of the urethral
plate in urethroplasty. Despite the growing clinical use
of the urethral plate, its histological characteristics have
not been well described.
Two important studies [15,16] have been published
emphasizing the urethral plate anatomy. The San
Francisco group [15] evaluated the urethral plate of a
newborn with hypospadias and of two fetuses with distal
hypospadias, with 30 normal fetal penises as controls.
The specimens were embedded in paraf®n and serially
sectioned (6 mm) after formalin ®xation. Hematoxylin
and eosin staining as well as immunohistochemical
evaluation of the nerves, smooth muscles, blood vessels
and epithelium were performed. According to the
authors' ®ndings, the urethral plate is well vascularized,
has a rich nerve supply and an extensive muscular and
connective tissue backing, supporting its use for
hypospadias reconstruction. In a similar study [16],
subepithelial biopsies of the urethral plate were carried
out in 17 boys, ®ve of them with ventral penile
curvature. All the biopsies showed well vascularized
connective tissue comprised of smooth muscle and
collagen. There was no evidence of ®brous bands or
dysplastic tissue. These histological ®ndings support
reasonable efforts to preserve the urethral plate, even in
cases of mild to moderate penile curvature.
The anatomy of the neurovascular bundle is another
important feature in the performance of a dorsal plication
to correct penile curvature. Historical experience suggests that mobilization of the neurovascular bundle is
anatomically possible. The perforating branches into the
corporal bodies have been studied in 35 normal human
fetal penile specimens, gestational age 8±35 weeks, and
three hypospadic specimens, 33±41 weeks of gestation.
Baskin et al. [17] concluded that perforating branches
from the dorsal lateral neurovascular bundles do not
exist, based on serial step sectioning and microscopic
examination of male genital specimens. It is possible
surgically to elevate the neurovascular bundle, but the
dissection needs to remain directly on top of the tunica
albuginea to prevent nerve injury. Small perforating
branches into the urethral spongiosum may be injured
with unknown signi®cance. The authors continued,
however, to advocate plication in the nerve-free zone
at the 12 o'clock position for correction of penile
curvature [17].
The Koyanagi technique for hypospadias repair is based
on parameatal ¯aps that extend distally around the distal
shaft to incorporate the inner layer of the prepuce. A
reoperation rate as high as 50% has limited its use to
date. The Columbia group [18] reported their modi®cation of the technique, attempting to preserve the blood
supply to the ¯aps and to improve results. The authors
treated 20 boys with proximal hypospadias and found
four urethrocutaneous ®stulas (20%) with a mean followup of 34 months.
The preputial island ¯ap onlay technique continues to
be one of the most popular techniques for the treatment
of hypospadias due to the excellent cosmetic results and
acceptable complication rates. The double onlay preputial ¯ap is an alternative to the conventional techniques because it guarantees neourethra coverage with
588 Paediatric urology
well vascularized preputial skin. In a study of 47 children
with hypospadias, 30 penoscrotal and ®ve perineal [19],
the authors found 17% ®stula, 9% diverticula, 4% meatal
recession and 4% persistent penile curvature requiring a
repeated dorsal plication.
The tubularized incised-plate repair urethroplasty
(Snodgrass procedure) was the subject of several papers
that examined different features of the technique. A
prospective Austrian study [20] comparing the Snodgrass
technique with the Mathieu technique in 60 children
showed superiority of the ®rst technique, both in terms
of less complications and reduced surgery time. The
Australian experience with this technique in 60 cases
showed urethral ®stulas in six patients (10%) and meatal
stenosis in three (5%). A good or satisfactory ®nal
cosmetic and functional result was achieved in 58
patients (98%) [21]. Tubularized incised plate urethroplasty has also been used as a procedure to correct
complications after hypospadias repair. A Chinese report
on six secondary Snodgrass procedures showed good
functional results in all patients. A small ®stula in one
patient and a mild meatal retraction in another were
observed [22]. As surgeons became more experienced
with the Snodgrass technique, meatal stenosis became
the most frequent complication of this method. There is
doubt about the need to dilate the urethra postoperatively to decrease this complication. An Egyptian study
[23] reviewing 27 patients with hypospadias, including
®ve glanular, 16 coronal, two recurrent cases of
hypospadias after a failed Mathieu repair, and four
patients who required a second-stage repair, tried to
answer this question. Of the ®rst seven patients, four had
a small ®stula associated with meatal stenosis. By regular
dilatation of the glanular urethra, all ®stulas resolved
spontaneously. Dilatation was instituted in all the
remaining patients and no ®stula or meatal stenosis
occurred. The author of this study suggests that a routine
urethral dilatation is important to prevent adhesions
between both sides of the incised plate, which can result
in meatal stenosis and ®stula. The group's experience
with this technique con®rms the observation seen by
several other authors.
There is a concept that ¯aps are superior to grafts in
proximal hypospadias surgery. The San Diego group
reviewed their experience using preputial skin ¯aps or
free grafts [24 .]. The records of 142 patients were
reviewed and repairs were subdivided into tubularized
and onlay reconstructions within the groups. Two-thirds
of the repairs were performed with free grafts. A
proximal stricture developed in eight patients who
underwent free tubularized graft and in none of the
free onlay repair patients. Otherwise there was no
signi®cant difference in the complication rate of the
various types of repairs. Of the 43 patients who had
stricture, ®stula or meatal stenosis, 29 (67%) presented
with the problem more than 1 year after surgery. The
authors concluded that there is a signi®cantly higher
proximal stricture rate when a tube and not an onlay free
graft is used and that a longer follow-up (more than 1
year after surgery) may be necessary to assess completely
the outcome of proximal hypospadias surgery.
Hypospadias cripples can be de®ned as patients with
remaining functional complications after previous hypospadias repair. A retrospective review of a group of 94
patients showed that 82 had major meatal dystopia
(87%), 43 (46%) had residual curvature of the penile
body, 19 (20%) showed meatal stenosis and only ®ve had
one or more ®stulas [25]. In the absence of genital skin
for the urethroplasty, the oral mucosa is the best option
for urethral replacement. This tissue is easily obtained
and has excellent immunohistochemical properties,
similar to those of the urethral epithelium [26]. As
shown in the free graft tendency, it should be used as an
onlay reconstruction and not tubularized and covered by
a Scarpa ¯ap in the dorsal region. The meatus should not
be brought to the glans tip, but must be placed in the
coronal or proximal region in order to achieve functional
success in patients who have already been operated on
several times. The dressing must be kept occluded for at
least 5±7 days, minimizing local manipulation. A
suprapubic cystostomy tube should be left indwelling
routinely as the best urinary diversion and a ®ne silicone
catheter used as a drain for just the urethral secretions
[27,28]. The success rate of severe hypospadias treatment with the use of oral mucosa is between 70 and
80%. The favorable results obtained with oral mucosa for
treatment of hypospadias cripples has encouraged some
investigators also to use the tissue as a primary repair for
one-stage or two-stage treatment. The results have been
excellent and are supported by the major histological
similarities between oral mucosa and normal urethral
tissue, compared with penile skin of the dorsal region.
However, there is still a lot of debate about the
technique, and some authors believe that free grafts
should be limited to circumstances where there is total
absence of genital skin suitable for urethral construction
[29]. We believe that oral mucosa is a good tissue option;
it is also very easy to harvest. The lower lip is the donor
site of choice, but tissue should be removed very
super®cially. We do not believe that this technique is
too aggressive for obtaining tissue, especially when there
is concern about the quality of the tissues in the genital
The great number of hypospadias repairs is also
combined with different types of hypospadias dressings.
As repairs become more complicated, dressings often
become more complicated as well. A randomized study
performed in 100 consecutive patients evaluated the
Surgery to the external genitalia Macedo and Srougi 589
success and complications of hypospadias with and
without dressings [30]. All but one repair preserved the
integrity of the urethral plate. The authors found similar
results in both groups and concluded that the success
rate for hypospadias repairs that preserve the urethral
plate is independent of dressing usage and that dressing
may not be indicated for all hypospadias repairs. The
authors, however, reinforce that these ®ndings do not
apply to hypospadias repairs that produce a potential
space between the urethral plate and the corpora, and to
graft urethroplasty.
Interest has increased in combining procedures during
reconstruction of bladder exstrophy in an effort to reduce
the number of procedures and to improve results.
Genitalia reconstruction, which in the past was delayed
until after bladder closure, is now often performed
together with the initial surgery. Gearhart and Mathews
[31] reported on 24 boys with classic bladder exstrophy
who underwent combined bladder closure and epispadias repair. The mean patient age was 20 months.
Eighteen boys had undergone a prior failed closure.
Osteotomies were performed in all patients. Urethrocutaneous ®stulas developed in seven patients but no
instances of bladder prolapse or dehiscence were noted.
The authors reinforce that boys presenting after failed
initial closure or who are older than 5 months of age may
be candidates for a combination of epispadias repair with
bladder closure [31]. The technique of complete
reconstruction in exstrophy and its results were reviewed
in a paper published by the Seattle group [32 . .]. From
1989 to 1997, the authors performed a complete primary
repair approach in 18 patients with bladder exstrophy
and six patients with cloacal exstrophy. Eighteen
patients underwent this procedure in the ®rst day of
life. No patients experienced dehiscence of the primary
The staged approach for female bladder exstrophy±
epispadias results in a vagina that remains in an abnormal
position on the anterior abdominal wall. The Oklahoma
group reported on seven female patients who underwent
total urogenital complex mobilization, with complete
disassembly of the pelvic diaphragm or ¯oor anterior to
the rectum, to reposition the urethra and vagina to their
proper anatomical positions in the perineum [33]. No
complications were noticed, all patients having adequate
vaginal caliber without evidence of stenosis. The authors
suggest that this procedure should be considered in the
initial approach, because the majority of female patients
with exstrophy will require surgical reconstruction of the
vagina and external genitalia later in life.
The Johns Hopkins 10-year experience with the
modi®ed Cantwell±Ransley procedure for exstrophy
and epispadias has now been reported [34]. The authors
reported on 93 males with a mean follow-up of 68
months. The incidence of ®stulas was 23% in the
immediate postoperative period and 19% at 3 months.
Urethral stricture at the proximal anastomotic area
developed in seven patients.
Continence after exstrophy and bladder neck reconstruction can be achieved without intermittent catheterization. The Indiana group reviewed 53 patients and found
an alarming frequency of clinical and urodynamic
problems related to voiding [35 . .]. The authors of this
report questioned the normalcy of the voiding pattern
and risks to achieve continence among patients with
exstrophy. We agree with these observations and, since
June 1998, we have been treating failed primary
exstrophy repairs with a continent catheterizable ileumbased reservoir, anastomosed to the bladder plate [36 . .].
The advances in surgery on the external genitalia are
related to a better understanding of embryology and
physiopathology of the urogenital tract congenital
anomalies. The techniques employed in the treatment
continue to evolve and change the treatment strategies.
Specialists in this area must pay attention to these
developments in order to be able to apply these new
concepts in clinical practice.
References and recommended reading
Papers of particular interest, published within the annual period of review, have
been highlighted as:
of special interest
of outstanding interest
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