Background

Hypospadias is an abnormality of anterior urethral and penile development in which the urethral
opening is ectopically located on the ventral aspect of the penis proximal to the tip of the glans
penis, which, in this condition, is splayed open. [1] The urethral opening may be located as far
down as in the scrotum or perineum. The penis is more likely to have associated ventral
shortening and curvature, called chordee, with more proximal urethral defects.

The earliest medical text describing hypospadias dates back to the second century CE and was
the work of Galen, the first to use the term. During the first millennium, the primary treatment
for hypospadias was amputation of the penis distal to the meatus. Since that time, many have
contributed to development of modern hypospadias repair. More than 300 different types of
repairs have been described in the medical literature. Although most reports have been in the past
60 years, most basic techniques were described more than a century ago.

Modern anesthetic techniques, fine instrumentation, sutures, dressing materials, and antibiotics
have improved clinical outcomes and have, in most cases, allowed surgical treatment with a
single-stage repair within the first year of life on an outpatient basis.

Pathophysiology
Hypospadias is a congenital defect that is thought to occur embryologically during urethral
development, between 8 and 20 weeks' gestation. The external genital structures are identical in
males and females until 8 weeks' gestation; the genitals develop a masculine phenotype in males
primarily under the influence of testosterone. As the phallus grows, the open urethral groove
extends from its base to the level of the corona.

The classic theory is that the urethral folds coalesce in the midline from base to tip, forming a
tubularized penile urethra and median scrotal raphe. This accounts for the posterior and middle
urethra. The anterior or glanular urethra is thought to develop in a proximal direction, with an
ectodermal core forming at the tip of the glans penis, which canalizes to join with the more
proximal urethra at the level of the corona. The higher incidence of subcoronal hypospadias
supports the vulnerable final step in this theory of development.

In 2000, Baskin proposed a modification of this theory in which the urethral folds fuse to form a
seam of epithelium, which is then transformed into mesenchyme and subsequently canalizes by
apoptosis or programmed cell resorption. [2] Similarly, this seam theoretically also develops at the
glanular level, and the endoderm differentiates to ectoderm with subsequent canalization by
apoptosis.

The prepuce normally forms as a ridge of skin from the corona that grows circumferentially,
fusing with the glans. Failure of fusion of the urethral folds in hypospadias impedes this process,
and a dorsal hooded prepuce results. On rare occasions, a glanular cleft with intact prepuce may
occur, which is termed the megameatus intact prepuce (MIP) variant.
Chordee (ventral curvature of the penis) is often associated with hypospadias, especially more
severe forms. This is thought to result from a growth disparity between the normal dorsal tissue
of the corporal bodies and the attenuated ventral urethra and associated tissues. Rarely, the
abortive spongiosal tissue and fascia distal to the urethral meatus forms a tethering fibrous band
that contributes to the chordee. (See the image below.)

Severe penile chordee. Note extreme

ventral curvature of penile shaft.
View Media Gallery

The location of the abnormal urethral meatus classifies the hypospadias. Although several
different classifications have been described, most physicians use the one proposed by Barcat
and modified by Duckett, which describes the location of the meatus after correction of any
associated chordee. [3, 4] Descriptive locations described include the following:

 Anterior (glanular and subcoronal)

 Middle (distal penile, midshaft, and proximal penile)
 Posterior (penoscrotal, scrotal, and perineal)

The location is anterior in 50% of cases, middle in 20%, and posterior in 30%; the subcoronal
position is the most common overall. (See the images below.)
 Proximal shaft hypospadias. Note deficient ventral
foreskin, blind urethral pit at glanular level, and lighter pigmented urethral plate extending to
true meatus at proximal shaft level.
View Media Gallery

Proximal shaft hypospadias. Note

typical dorsal hood of foreskin and ventral penile skin deficiency.
View Media Gallery
 Penoscrotal hypospadias. Note associated ventral chordee
and true urethral meatus located at scrotal level.

Etiology
Several etiologies for hypospadias have been suggested, including genetic, endocrine, and
environmental factors.

Genetic factors

A genetic predisposition has been suggested by the eightfold increase in incidence of

hypospadias among monozygotic twins as compared with singletons. This finding may relate to
the demand of two fetuses for human chorionic gonadotropin (HCG) produced by a single
placenta, with an inadequate supply during critical periods of urethral development.

A familial trend has been noted with hypospadias. The prevalence of hypospadias in male
children of fathers with hypospadias has been reported as 8%, and 14% of brothers of children
with hypospadias are also affected. The inheritance is likely polygenic.

Endocrine factors

A decrease in available androgen or an inability to use available androgen appropriately may

result in hypospadias. In a 1997 report by Aaronson et al, 66% of boys with mild hypospadias
and 40% with severe hypospadias were found to have a defect in testicular testosterone
biosynthesis. [5]
Mutations in the 5-alpha reductase enzyme, which converts testosterone (T) to the more potent
dihydrotestosterone (DHT), have been associated with hypospadias. A 1999 report by Silver et al
found that nearly 10% of boys with isolated hypospadias had at least one affected allele with a 5-
alpha reductase mutation. [6] Although androgen receptor deficits, quantitative or qualitative,
have been shown to result in hypospadias, this is thought to be relatively uncommon, and other
factors are more commonly implicated.

A higher incidence of hypospadias in winter conceptions has also been proposed. Theoretically,
this may be related to the effect of daylight on pituitary function, which, in turn, affects the
maternal and fetal hormonal milieu; however, other authors have not noticed this association.

A fivefold increased risk of hypospadias appears to exist in males born through in-vitro
fertilization (IVF) in comparison with a control group. This may reflect maternal exposure to
progesterone, which is commonly administered in IVF protocols. Progesterone is a substrate for
5-alpha reductase and acts as a competitive inhibitor of the T-to-DHT conversion.

Other factors that contribute to infertility, such as underlying endocrinopathies or fetal endocrine
abnormalities, may play a role.

Environmental factors

Endocrine disruption by environmental agents is gaining popularity as a possible etiology for

hypospadias and as an explanation for its increasing incidence.

Estrogens have been implicated in abnormal penile development in many animal models.
Environmental substances with significant estrogenic activity are ubiquitous in industrialized
society and are ingested as pesticides on fruits and vegetables, endogenous plant estrogens, in
milk from lactating pregnant dairy cows, from plastic linings in metal cans, and in
pharmaceuticals.

A study by Hadziselimovic reported increased estradiol concentration in placental basal

syncytiotrophoblasts of boys with undescended testes as compared with a control population. [7]
Undescended testes and hypospadias have been associated, but increased estradiol concentration
has not been implicated in hypospadias per se. This may support the association of hypospadias
with increasing parity, increasing maternal age, and low birth weight noted in some studies in
relation to lifelong exposure to environmental disruptors and a possible cumulative effect.

Combination theory

A growing body of evidence suggests that the development of hypospadias has a two-hit etiology
involving a genetic predisposition coupled with fetal exposure to an environmental disruptor.

Epidemiology
United States statistics

Hypospadias occurs in approximately 1 in every 250 male births in the United States. The
incidence doubled from 1970 to 1993. Although some have suggested that this doubling actually
reflects increased reporting of minor grades of hypospadias, increases in severe hypospadias
have also been noted. Increasing sensitivity of surveillance systems alone cannot explain this
twofold increase. However, some reports have linked the increased rate of hypospadias in boys
born prematurely and small for gestational age and boys with low birth weight.

International statistics

In several countries, the incidence of hypospadias may be rising. In general, the frequency seems
rather constant, at 0.26 per 1000 live births in Mexico and Scandinavia and 2.11 per 1000 live
births in Hungary. [10]

Race-related demographics

The incidence of hypospadias is higher in whites than in blacks, and the condition is more
common in those of Jewish and Italian descent. A genetic component may be present in certain
families; the familial rate of hypospadias is about 7%.

Prognosis
With modern anesthetics, instruments, sutures, dressing materials, and antibiotics, hypospadias
repair has become quite successful. Long-term studies on the outcomes of hypospadias using
current practices are limited. Although some earlier studies have been discouraging, these reflect
an era with poorer technical outcomes, increased number of operations, and a lack of
appreciation for the psychological morbidity associated with intervention at an older age.

Subsequent long-term studies have suggested that despite having decreased satisfaction with
their genital appearance, patients having undergone hypospadias repair are more satisfied with
their sex lives compared to healthy controls. [11] Newer scoring systems to lend an objective
measure to long-term outcomes are becoming more popular. [12, 13]

Although the techniques of hypospadias repair continue to evolve, the broader future of
hypospadias treatment is truly promising. Nontraditional tissue adherence techniques are being
developed, including tissue glues and laser-activated soldering techniques that have been shown
to improve wound healing and to reduce fistula formation.

Urethral substitutes, which may obviate the difficulties associated with severe hypospadias and
poor tissue availability, are currently under investigation. These substitutes are generally
acellular synthetic or natural matrices that can incorporate the patient's normal urethral cellular
components.
The embryology of hypospadias is being elucidated, and the understanding of its causes is
improving; with new information, an exciting new paradigm shift to hypospadias prevention or
antenatal intervention may occur.

Patient Education
Because most patients with hypospadias are surgically treated at a very young age, parental
teaching and reassurance is very important to ensure a satisfactory experience for the families of
these patients . Evidence suggests that online support groups can play an important role in how
parents and patients cope with hypospadias.

Surgical Care
The goals of surgical treatment of hypospadias are as follows:

 To create a straight penis by repairing any curvature (orthoplasty)

 To create a urethra with its meatus at the tip of the penis (urethroplasty)
 To re-form the glans into a more natural conical configuration (glansplasty)
 To achieve cosmetically acceptable penile skin coverage
 To create a normal-appearing scrotum

The resulting penis should be suitable for future sexual intercourse, should enable the patient to
void while standing, and should present an acceptable cosmetic appearance.

Timing of surgery

Before 1980, hypospadias repair was performed in children older than 3 years because of the
larger size of the phallus and a technically easier procedure; however, genital surgery at this age
(genital awareness occurs at about age 18 months) can be associated with significant
psychological morbidity, including abnormal behavior, guilt, and gender identity confusion.

Currently, most physicians attempt to repair hypospadias when the child is aged 4-18 months,
with a trend toward earlier intervention. This has been associated with an improved emotional
and psychological result. A benefit in wound healing with earlier repair has also been perceived
and may have a basis in the reduced proinflammatory cytokine production noted at younger ages.
[20]

Late hypospadias repair, in the pubertal and postpubertal period, is associated with
complications, primarily urethrocutaneous fistula, in nearly half of patients. [21] Some reports cite
a higher rate of complications in 5-year-old patients than in 1-year-old patients, suggesting that
earlier repair is generally better. [22]
Types of repair

The specific techniques for hypospadias repair are beyond the scope of this article (see
Urogenital Reconstruction, Penile Hypospadias); however, the types of repairs can be generically
grouped, and the approach to the repair is relatively standard.

After a full assessment of the penile anatomy, the shaft skin of the penis is degloved to eliminate
any skin tethering, and an artificial erection is performed to rule out any curvature. Mild-to-
moderate chordee may be repaired by excising any ventral fibrous tethering tissue or by plicating
the dorsal tunics of the corporal bodies, compensating for any ventral-to-dorsal disproportion.

More severe chordee may require grafting of the ventral corporal bodies using synthetic, animal
(small intestinal subunit), cadaveric, or autologous tissues (tunica vaginalis or dermal grafts) to
avoid excessive shortening of penile length. On rare occasion, the urethral plate may be tethered
and transection of the plate may be required, precluding the use of native urethral tissues for
urethroplasty.

The urethra may be extended by using various techniques. These techniques are generally
categorized as primary tubularizations, local pedicled skin flaps, tissue grafting techniques, or
meatal advancement procedures.

The tubularized incised plate (TIP) repair has become the most commonly used repair for both
distal and midshaft hypospadias. This technique is a primary tubularization of the urethral plate,
with incision of the posterior wall of the plate, which allows it to hinge forward (see the image
below). This creates a greater diameter lumen than would otherwise be possible, obviating the
routine use of a flap or graft to bridge a short narrow segment of urethral plate.
 Tubularized incised plate (TIP) technique. Urethral plate has
been incised in dorsal midline; this expands width of plate and allows it to hinge forward for
tubularization.

View Media Gallery

The TIP repair has proved adaptable to various settings, and current surveys indicate that it is the
procedure of choice for most repairs by most urologists.

Various sutures have been used in the repair of hypospadias, but polyglycolic acid–based sutures
may offer the best balance of resilience when exposed to urine, without excessive time to
absorption resulting in a foreign body reaction. [23]

Studies support the general concept that increasing the layers of tissue between the urethra and
overlying skin coverage makes subsequent development of urethrocutaneous fistula less likely.
[24]
Temporary urethral stents are a common adjunct to hypospadias repair and are felt to decrease
the likelihood of fistula formation. Various drainage tubes have been utilized for this purpose. [25]
To stent or not to stent is an ongoing controversy, balancing the risk of irritative symptoms and
urinary tract infection with the risk of urinary retention. [26]

In a retrospective-prospective observational study of 189 patients that compared 1 week of

transurethral bladder catheterization after hypospadias repair with 3 weeks of catheterization,
Daher et al found the longer catheterization period to be associated with better outcomes and
fewer complications. [27]
In the setting of repeat repair after unsuccessful surgery for hypospadias when local tissues are
unavailable, buccal mucosa has been used for urethral grafting. This tissue is well suited for this
purpose because of its availability, characteristics that favor graft success, and resilience to a
moist environment. Urethral stents are generally used for bladder drainage while healing occurs
in all but the most distal hypospadias repairs.

Steps of repair

Glans flaps are generally mobilized to cover the distal urethral repair, bringing the divergent
ventral components to the midline and creating a more conical configuration. The excess dorsal
skin is mobilized to the deficient ventral aspect of the penis for final skin coverage.

The repair of penoscrotal transposition is often performed as a staged procedure because the
necessary incisions may compromise the vascular pedicle to skin flaps used in the primary
urethroplasty. The repair of penoscrotal transposition is usually deferred at least 6 months to
allow for adequate formation of collateral blood supply.

The repair of hypospadias is generally planned as a single-stage procedure, but excessive

chordee (especially if transection of the urethral plate is required), poor skin availability, and
small phallic size may be better approached in a staged manner. The chordee is repaired and the
skin is mobilized to the ventral penile shaft during the first stage, and the urethroplasty and
glansplasty are repaired after the first stage has completely healed.

Adjuvant hormonal therapy

Although no corrective medical therapy for hypospadias is known, hormonal therapy has been
used as an adjuvant to surgical therapy in infants with exceptionally small phallic size.
Preoperative treatment with testosterone injections or creams, as well as human chorionic
gonadotropin (HCG) injections, has been used to promote penile growth; some have reported
improvement in chordee with lessening in the severity of hypospadias. That prepubertal
androgen therapy may limit normal genital growth at puberty is a concern, but this has not been
confirmed clinically.

In a study of 182 children with midshaft or distal hypospadias (mean age, 30 months) who
underwent TIP repair for hypospadias, Asgari et al found preoperative parenteral testosterone
administration to be beneficial in decreasing complication rates (from 13.18% to 5.45%).

Complications
It is clear that repairs that are more proximal are associated with a greater incidence of
complications. [29] Older age at surgery and low surgical experience have also been associated
with poorer outcomes. A study from England by Wilkinson et al found that staged repairs were
associated with higher complication rates and that high-volume centers had lower complication
rates. [30]
With longer follow-up, it is apparent that late complications can occur, and thus, most advocate
continued evaluation through puberty. [31, 32, 33, 34]

Immediate postoperative concerns

Local edema and blood spotting can be expected early after repair and generally do not cause a
significant problem.

Postoperative bleeding rarely occurs and is usually controlled with a compressive dressing.
Infrequently, reexploration may be required to evacuate a hematoma and to identify and treat the
source of bleeding.

Infection is a rare complication of hypospadias repair in the modern era. Skin preparation and
perioperative antibiotics are generally used. Patients are often maintained on an antibiotic course
until any stents are removed, though this has not clearly been shown to be beneficial. [35]

Long-term issues

Urethrocutaneous fistulization is a major concern in hypospadias repair. The rate of fistula

formation is generally less than 10% for most single-stage repairs but rises with the severity of
hypospadias, approaching 40% with complex reoperative efforts. Fistulas rarely close
spontaneously and are repaired by using a multilayered closure with local skin flaps 6 months
after the initial repair. After repair, fistulas recur in approximately 10% of patients. (See the
image below.)
 Urethrocutaneous fistula has appeared after
hypospadias repair. Note one stream from true urethral meatus and second stream through more
proximal fistula.

View Media Gallery

Meatal stenosis, or narrowing of the urethral meatus, can occur. A urethral stent prevents any
problems initially, but a fine-spraying urinary stream that is associated with straining to void
likely requires operative meatal revision.

Urethral strictures may develop as a long-term complication of hypospadias repair. These are
generally repaired operatively and may require incision, excision with reanastomosis, or patching
with a graft or pedicled skin flap.

Urethral diverticula may also form and are evidenced by ballooning of the urethra while voiding.
A distal stricture may cause outflow obstruction and may result in a urethral diverticulum.
Diverticula can form in the absence of distal obstruction and are generally associated with graft-
or flap-type hypospadias repairs, which lack the subcutaneous and muscular support of native
urethral tissue. The redundant urethral tissue is generally excised, and the urethra is tapered to an
appropriate caliber.

Hair-bearing skin is avoided in hypospadias reconstruction but was used in the past. When
incorporated into the urethra, it may be problematic and can result in urinary tract infection or
stone formation at the time of puberty. This generally requires cystoscopic depilation using a
laser or cautery device or, if severe, excision of hair-bearing skin and repeat hypospadias repair.
Some surveys have suggested that milder forms of erectile dysfunction may be more common
with more proximal hypospadias repairs.

Consultations
Consultation with a pediatric endocrinologist is indicated in cases where a child may be
suspected of having a disorder of sex development.

Physical Examination
Although the diagnosis of hypospadias has been made with both antenatal fetal ultrasonography
and magnetic resonance imaging (MRI), the diagnosis is generally made upon examination of the
newborn infant. [15]

A dorsal hood of foreskin and glanular groove are evident, but upon closer inspection, the
prepuce is incomplete ventrally and the urethral meatus is noted in a proximally ectopic position.
Rarely, the foreskin may be complete, and the hypospadias is revealed at the time
of circumcision. If hypospadias is encountered during neonatal circumcision, after the dorsal slit
has been performed, the procedure should be halted, and the patient should be referred for
urologic evaluation.

Chordee may be readily apparent or may be discernible only during erection. Proximal
hypospadias is commonly associated with a bifid scrotum and penoscrotal transposition (see the
image below), in which the rugated scrotal skin begins lateral to the penis rather than in its
normal posterior origin.
 Penoscrotal transposition. Note rugated scrotal skin
lateral to penis, cephalad to its normal position.

Surgical Therapy
The aims of the surgical procedures are as follow:

 Widening of the meatus

 Correction of the curvature
 Reconstruction of the missing portion of the urethra
 Restoration of the normal aspect of the external genitalia

Surgery differs according to the severity of the malformation.

A meatotomy is required if the size of the external urethral meatus is inferior to that considered
normal according to the age of the patient.

The distal urethra missing in glanular hypospadias, usually without recurvatum, is well
reconstructed with local flaps based on the meatus (eg, Santanelli procedure, Flip Flap, MAGPI
[meatal advancement and glanuloplasty]), including preputium plasty at the same sitting (see
images below).
 Distal hypospadias. Incision lines are shown.
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The spatulated flap is turned over and sutured to the glans.

View Media Gallery
 The urethra is reconstructed and sutured between the
glanular flaps.
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Preputium plasty. Reconstruction of the inner layer.

 Preputium plasty. Reconstruction of the outer surface.

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Normal mobility of the preputium after its reconstruction.

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In penile and penoscrotal types of malformation, resection of the chordee and reconstruction of
the missing part of the urethra are performed with a single-stage procedure (eg, Duckett,
Standoli, Scuderi, modified Koyanagi).

In some clinical situations (eg, perineal hypospadias, genital ambiguity, significant hypospadias
with previous circumcision), more extensive operations are necessary, and the former multistage
operations may be of occasional use.

A study by Arnaud et al indicated that in proximal penile hypospadias repair, the presence of a
bifid scrotum predicts the need to transect the urethral plate. Of 18 children with a bifid scrotum,
plate transection was considered necessary in 15, compared with two out of 11 children without a
bifid scrotum. [7]

Single-stage procedure

See the list below:

 Place a traction suture through the glans and extend a coronal incision around the meatus (see
images below).

Distal hypospadias: spatulated glans, ventrally cleft

preputium. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted
with permission of the editor.)

View Media Gallery

 Distal hypospadias. A traction suture is placed
through the glans. Incision lines according to Scuderi repair: a coronal incision extended up to
and around the meatus. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73.
Reprinted with permission of the editor.)

View Media Gallery

 Lift the penile skin, including the prepuce, and raise it in the plane between the Buck and dartos
fascia.
 Deglove the meatus and penile urethra and separate them from the corpora cavernosa to the
point that normal spongy tissue is detected. Excise the hypoplastic stenotic portion of the
urethra.
 Perform an artificial erection with intracavernous injection of saline solution to assess the
presence and degree of curvature (see images below). Perform chordectomy and straightening
of the penile shaft when needed.
 Artificial erection. An intracavernous injection of
saline is performed while controlling the back flow at the basis of the penis with an elastic band.
(Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with
permission of the editor.)

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 Artificial erection achieved with normal saline
injected in the corpora cavernosa while controlling the backward flow. (Published in Scand J
Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

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 At this point, evaluate the actual urethral defect and begin the reconstruction. Harvest a peno-
preputial skin flap, which may include both sides of the apron to increase its length,
longitudinally along the penile vascular axis according to Scuderi and Koyanagi (Scuderi
technique, see images below). The preputial flap can also be raised transversally from the
ventral surface (according to Duckett) [5] or from the dorsal aspect of the apron (according to
Standoli). [8]
 Two 5/0 traction sutures are placed on either side of
the preputial apron. Outlining of the vertical preputial flap after resection of the chordee and
evaluation of the urethral defect. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24
(1): 67-73. Reprinted with permission of the editor.)

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 Vertical preputium flap after Scuderi repair. Note the
"V" shape pattern on the inferior part of the cutaneous flap. (Published in Scand J Plast Reconstr
Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

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 Mobilize the flap with a large subcutaneous pedicle from the dartos fascia to ensure an
appropriate vascularization (see images below).
 The vertical preputial flap is elevated on the dorsal
cutaneous side of the penis, vertically disposed along the vascular axis (Scuderi technique), to
comply with the defect to be reconstructed. The flap is mobilized with a large subcutaneous
pedicle.

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 Elevation of the vertical preputial flap with its
subcutaneous pedicle.

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 In the Scuderi procedure, a buttonhole incision is performed bluntly into the pedicle (see first 2
images below), and the flap is transposed ventrally by passing the penile body through the
pedicle (see second 2 images below).
 Buttonhole incision along the midline of the
subcutaneous pedicle of the vertical preputial flap, along the axis of the blood vessel, taking care
not to jeopardize the vascularization. (Published in Scand J Plast Reconstr Surg Hand Surg 1990;
24 (1): 67-73. Reprinted with permission of the editor.)

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 Buttonhole incision along the median line of the
pedicle. (Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with
permission of the editor.)

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 The flap is transposed ventrally through the
buttonhole incision without tractioning or twisting the pedicle, which may impair the blood
supply. Key suture between the flap and the urethra. (Published in Scand J Plast Reconstr Surg
Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

View Media Gallery

 Key suture between the flap and the urethra.
(Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with
permission of the editor.)

View Media Gallery

 If raised according to Koyanagi, the skin flap is divided into two portions at the 12 o'clock
position to form a Y-shape, whereas in the modified Koyanagi repair, a button-hole is made
trough the pedicle. [6]
 If raised according to Duckett or Standoli, ventrally transpose the flap by its rotation around the
corpora cavernosa. [5, 8] To reduce the incidence of stenosis of the proximal urethral
anastomosis, the preputial flap must be V-shaped proximally and joined to a distal incision of 5
mm performed on the ventral wall of the urethra along its medial line.
 Continue suturing between the proximal side of the flap and the urethra (see first 2 images
below) and extend it by rolling the flap into a tube around a 12F or 14F silicone catheter (see
third image below).
 The lower edge of the flap is sewn all around the
urethral orifice.

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 The flap is transferred ventrally, and the lower edge
of the flap is sewn all around the urethral orifice.

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 The flap is tubed around a urinary catheter. In this
manner, the anastomosis is covered by the pedicle and the longitudinal suture lies deep
between the corporeal bodies, reducing the possibility of fistula occurrence. (Published in Scand
J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

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 Remove a vertical strip of tissue from the ventral surface of the glans (see first 2 images below)
and raise two triangular flaps to cover the terminal part of the neo-urethra (see third image
below).
 The flap is rolled into a tube. (Published
in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the
editor.)

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 The glans is split to permit the placement of the exit
of the neo-meatus at the tip of the glans. A vertical strip of tissue from the ventral surface of
glans is removed, and two thick triangular flaps are raised to cover the distal part of the neo-
urethra. A well-closed urethra during intercourse is achieved.

View Media Gallery

 Splitting of the glans. (Published in Scand J Plast
Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

View Media Gallery

 Carry out the distal anastomosis. At the end of the procedure, discharge redundant poorly
vascularized foreskin (see first 2 images below) and pull up the penile skin and suture it to the
corona, creating an appearance similar to a circumcised penis (see second 2 images below).
 Trimming of the preputium in excess.

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The excess preputium is removed to provide

the penis a circumcised appearance.
View Media Gallery

Final appearance at the end of the operation.

View Media Gallery

 Final appearance. (Published in Scand J Plast Reconstr
Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the editor.)

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 Stent the urethra and apply a mild compressive dressing.

Preoperative Details
See the list below:

 An accurate physical examination of the external genitalia of the patient is required to

assess the severity of the malformation. Check the position of the meatus, dimension of
the penis, and presence of the testicles.
 If a hypospadias condition is associated with impalpable testes, obtain appropriate tests
(eg, complete endocrine screen, chromosome analysis, ultrasonography) to exclude an
intersex condition.

Intraoperative Details
See the list below:

 After penile degloving, an artificial erection with intracavernous injection of saline

solution is performed to assess the presence and degree of curvature.
  The external urethral meatus is then resected together with the most distal hypoplastic
portion of the urethra.
 Cordectomy and straightening of the penile shaft is performed if required.
 At this stage, the actual urethral defect shows and the urethral reconstruction is planned.
 A penile-preputial flap is harvested according to the preferred technique, taking into
consideration the age-related urethral size. The flap is inset to the proximal urethral
stump and tubed around a silicone catheter.
 The glans is split and two flaps that reach the tip of the glans are elevated to cover the
distal part of the neo-urethra.
 The redundant foreskin is discharged and the penile skin is pulled up and sutured to the
corona to achieve a final aspect similar to a circumcised penis.

Next: Postoperative Details

Postoperative Details
See the list below:

 Restraints for arm and legs may be necessary.

 Remove the urethral stent after 48 hours.
 The dressings remain in place for 4 days if no problems occur.
 Remove the diverting urinary catheter after 8-10 days.
 Discharge the patient after removal of the urinary diversion and when spontaneous
voiding occurs without difficulties.

Follow-up
See the list below:

 Patients are observed with fluximetry tests including registration of the micturition
volume, maximum flow, medium flow, and micturition time.
 A pressure-flow study with urethrogram and endoscopy (see image below) before
removal of the urinary diversion may be indicated to evaluate the detrusorial pressure and
the morphologic and urodynamic aspects of the newly reconstructed urethra.
 Poor flow rate in a junctional
stenosis (above). Normal flow rate (down) 1 year after dilatation. (Published in Scand J
Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with permission of the
editor.)

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 Perform a flow rate study at 3 weeks postoperatively (see image below). Examine
patients showing a normal flow rate again at 3 and 12 months postoperatively. In patients
exhibiting mild stenosis, urethra dilatation is indicated; repeat the flow rate study after 3
weeks.
 Endoscopic view of the anastomosis.
(Published in Scand J Plast Reconstr Surg Hand Surg 1990; 24 (1): 67-73. Reprinted with
permission of the editor.)

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 At the 3-month follow-up visit, question patients about the persistence of the curvature or
other problems.
 It is reported that the majority (range, 50-81%) of hypospadias repair complications
(fistula, glans dehiscence, meatal stenosis, urethral stricture, and diverticulum) are
diagnosed by the first year after surgery. Nevertheless, late complications are detected in
patients with symptoms. [9]
 Long-term follow-up care is necessary at least through puberty to exclude late failure
caused by hypertrophic urethral scarring or undetected chronic inflammation.

Complications
See the list below:

 Early complications include bleeding, infection, wound separation, flap necrosis, and
edema.
 Temporary stenosis from edema or hypertrophic scarring at the anastomotic site may
occur in 7% of repairs. Normalization is achieved after dilatation with urethral probes of
progressive caliber and stabilization of the healing process.
  Early urinary leakage from delayed healing of the urethral suture has been reported with
an incidence of 3-9%. Spontaneous resolution occurs by keeping the suprapubic diversion
for a longer time.
 Urethrocutaneous fistulas with urinary leakage from the new urethra range from 0.6-23%
in the one-stage operation and from 2-37.3% in the two-stage operation.
 Urethral stricture complicates approximately 8.5% of hypospadias repairs.
 Persistent chordee caused by incomplete excision requires secondary surgical excision of
all fibrous tissue.

Outcome and Prognosis

Functional results obtained with one-stage correction of hypospadias in terms of overall success
rate, incidence of fistulas or stenosis, and mean hospitalization time have proved to be superior
than those obtained with multistage procedures, and the prognosis is good.

The use of a well-nourished and innervated flap with a long and pliable pedicle is the reason for
the high success rate reported in the literature when using one-stage reconstruction.

Glanuloplasty and residual preputial trimming are always associated with urethral repair and
allow the reconstruction of a cosmetically acceptable glans with a neomeatus at the tip, closed
during intercourse, and with a final appearance close to a normal circumcised penis.

The different techniques of preputial flap (Duckett, Standoli, Scuderi, Koyanagi) allow good
functional results in primary hypospadias or in physically disabled patients where prepuce is no
longer available and the flap can be harvested from the dorsal preputial skin.

PENDAHULUAN

Hipospadia merupakan kelainan abnormal dari perkembangan uretra anterior dimana muara dari

uretra terletak ektopik pada bagian ventral dari penis proksimal hingga glands penis. Muara dari

uretra dapat pula terletak pada skrotum atau perineum. Semakin ke proksimal defek uretra maka

penis akan semakin mengalami pemendekan dan membentuk kurvatur yang disebut “chordee”

(Djakovick, 2008).

Pada abad pertama, ahli bedah dari Yunani Heliodorus dan Antilius, pertama-tama yang

melakukan penanggulangan untuk hipospadia. Dilakukan amputasi dari bagian penis distal dari

meatus. Selanjutnya cara ini diikuti oleh Galen dan Paulus dari Agentia pada tahun 200 dan
tahun 400 (Djakovick, 2008). Hipospadia terjadi 1:300 kelahiran bayi laki-laki hidup di Amerika

Serikat. Kelainan ini terbatas pada uretra anterior. Pemberian estrogen dan progestin selama

kehamilan diduga meningkatkan insidensinya. Jika ada anak yang hipospadia maka

kemungkinan ditemukan 20% anggota keluarga yang lainnya juga menderita hipospadia.

Meskipun ada riwayat familial namun tidak ditemukan ciri genetik yang spesifik (Djakovick,

2008).

Berdasarkan hasil survei, diketahui bahwa hipospadia hanya terjadi pada laki-laki yang dibawa

sejak lahir. Insidensinya 3:1000 atau 3 dari 1000 kelahiran (Sadler, 2006; Sjamsuhidajat,

2006;Djakovic, e t all. , 2008). Berdasarkan data yang dicatat oleh Metropolitan Atlanta

Congenital Defects Program (MACDP) dan Birth Defects Monitoring Program (BDMP)

insidensi hipospadia mengalami dua kali peningkatan antara 1970-1990. Prevalensi yang

dilaporkan antara 0,3% menjadi 0,8% sejak tahun 1970an. Tahun 1993 BDMP melakukan

survey mengenai insidensi hipospadia, dari hasil survei tersebut diketahui bahwa kasus

hipospadia mengalami peningkatan menjadi 20,2 per 10.000 kelahiran hidup pada 1.970-

39,7 per 10.000 kelahiran. Insidensi kasus hipospadia terbanyak adalah Eropa. BDMP

menyatakan bahwa insdensi hipospadia meningkat menjadi 20,2 per 10.000 kelahiran

hidup pada 1.970-39,7 per 10 000 kelahiran hidup pada tahun 1993. Kajian populasi yang

dilakukan di empat kota Denmark tahun 1989-2003 tercatat 65.383 angka kelahiran bayi laki-

laki dengan jumlah kelainan alat kelamin (hipospadia) sebanyak 319 bayi (Djakovick, 2008).

ANATOMI ORGANA GENETALIA EKSTERNA MASKULINA

Organa genetalia maskulina eksterna terdiri dari skrotum dan penis.

1. Scrotum
 Scrotum merupakan kantong yang menonjol keluar dari bagian bawah dinding anterior

abdomen. Scrotum berisi testis, epididymis, dan ujung bawah funiculus spermaticus (Snell,

2006).

a. Dinding scrotum memiliki lapisan

1) Cutis

2) Fascia superficialis, musculus dartos (otot polos) menggantikan panniculus adiposus.

Musculus dartos dipersarafi oleh serabut saraf simpatis dan berfungsi untuk

pengerutan kulit di atasnya. Pada saat dingin. Tunika dartos akan mengadakan

kontraksi sehingga testis akan mendekati tubuh yang temperaturnya lebih tinggi

sehingga temperatur dalam testis akan sama dengan temperatur tubuh. Pada saat

panas. Tunika dartos mengalami relaxasi sehingga testis akan menjauhi tubuh,

scrotum menjadi turun (Snell, 2006).

3) Fascia spermatica externa, cremasterica dan spermatica interna

Fascia spermatica externa berasal dari aponeurosis musculus obliquus externus

abdominis. Sedangkan musculus obliquus internus abdominis akan membentuk fascia

cremasterica. Fascia spermatica interna berasal dari fascia transversalis (Snell, 2006).

4) Tunica vaginalis

Terletak dalam fascia spermatica dan meliputi permukaan anterior, media, dan

lateralis masing-masing testis. Merupakan bagian bawah processus vaginalis dan

biasanya sesaat sebelum lahir menutup dan memisahkan diri dari bagian atas

processus vaginalis dan cavitas peritonealis  kantung tertutup, diinvaginasi dari

belakang oleh testis (Snell, 2006).

b. Aliran limfe
 Cairan limfe dari tunica vaginalis akan dialirkan ke nodi lymphoidei inguinales superficialis

(Snell, 2006).

c. Vaskularisasi scrotum (Snell, 2006)

1) R. scrotalis anterior

2) A.spermatica externa

3) R. scrotalis posterior

d. Inervasi (Snell, 2006)

1) Rr. Scrotales anterior

2) N pudendus externa

3) Rr. Scrotalis posterior

4) N. cutaneus femoris posterior

2. Penis

a. Definisi

Merupakan organ genetalia laki-laki yang berfungsi sebagai alat kopulasi. Dibedakan atas pars

fixa dan pars libera. Pars fixa terdiri dari radix penis (crus penis dan bulbus penis). Pars libera

atau batang penis terdiri dari 2 corpora cavernosum penis, 1 corpus cavernosum urethra dan 1

glands penis (Snell, 2006).

b. Bagian penis

1) Radix penis.

Dibentuk dari tiga massa jaringan erektil : bulbus penis dan crus penis dextra et

sinistra. Bulbus penis terletak di garis tengah dan melekat pada permukaan bawah
 diaphragma urogenital. Bulbus penis ditembus oleh urethra dan permukaan luarnya

dibungkus oleh musculus bulbospongiosus (Snell, 2006).

Masing-masing crus penis melekat pada pinggir arcus pubis dan diliputi oleh

musculus ischiocavernosus pada permukaan luarnya. Bulbus melanjutkan diri ke

depan sebagai corpus penis dan membentuk corpus spongiosum penis. Di anterior

kedua crus saling mendekat dan di bagian dorsal corpus penis terletak berdampingan

membentuk corpus cavernosum penis (Snell, 2006).

2) Corpus penis

Terdiri dari tiga jaringan erektil yang diliputi sarung fascia berbentuk tubular (fascia

buck). Jaringan erektil dibentuk dari dua corpora cavernosa penis yang terletak di

dorsal dan satu corpus spongiosum penis yang terletak pada permukaan ventralnya.

Pada bagian distal corpus spongiosum penis melebar  glans penis yang meliputi

ujung distal corpora cavernosa penis (Snell, 2006).

Pada ujung glans penis terdapat celah yang merupakan muara urethra disebut meatus

urethra externus. Preputium penis merupakan lipatan kulit seperti kerudung yang

menutupi glans penis. Preputium dihubungkan dengan glans penis oleh lipatan yang

terdapat tepat di bawah muara urethra dan dinamakan frenulum preputii (Snell, 2006).

c. Vaskularisasi

1) Arteri (Snell, 2006)

a) Corpora cavernosa : a. profunda penis cabang a. pudenda interna

b) Corpus spongiosum penis : a. bulbi penis cabang a. pudenda interna dan a. dorsalis

penis cabang a. pudenda interna.

2) Vena (Snell, 2006)

 Vena bermuara ke vena pudenda interna

3) Limfe

Cairan limfe dialirkan ke nodi superomedialis dan nodi inguinalis superfisicales.

Struktur profunda penis mengalirkan cairan life ke nodi iliaci interni (Snell, 2006).

4) Persarafan

Persarafan berasal dari nervus pudendus dan plexus pelvicus (Snell, 2006).

ETIOLOGI

Penyebabnya sebenarnya sangat multifaktor dan sampai sekarang belum diketahui penyebab

pasti dari hipospadia. Namun, ada beberapa faktor yang oleh para ahli dianggap paling

berpengaruh antara lain :

1. Gangguan dan ketidakseimbangan hormon

Hormon yang dimaksud di sini adalah hormon androgen yang mengatur organogenesis

kelamin (pria) atau bisa juga karena reseptor hormone androgennya sendiri di dalam tubuh yang

kurang atau tidak ada. Sehingga walaupun hormone androgen sendiri telah terbentuk cukup akan

tetapi apabila reseptornya tidak ada tetap saja tidak akan memberikan suatu efek yang

semestinya. Atau enzim yang berperan dalam sintesis hormon androgen tidak mencukupi pun

akan berdampak sama.

2. Genetika.

Terjadi karena gagalnya sintesis androgen. Hal ini biasanya terjadi karena mutasi pada

gen yang mengode sintesis androgen tersebut sehingga ekspresi dari gen tersebut tidak terjadi.
3. Lingkungan.

Biasanya faktor lingkungan yang menjadi penyebab adalah polutan dan zat yang bersifat

teratogenik yang dapat mengakibatkan mutasi.

Pembesaran dari tuberkel genitalis dan perkembangan yang mengikutinya dari phallus

dan urethra tergantung dari tingkat testosteron selama embriogenesis. Jika testis gagal dalam

menghasilkan testosteron dalam jumlah yang mencukui atau sel dari struktur genitalia tidak

memiliki reseptor androgen yang mencukupi atau androgen-converting enzyme 5 alpha-

reductase, akan menghambat proses virilisasi dan akan menimbulkan hipospadia (Santanelli,

2010)

Faktor genetik dan non genetik berpengaruh dalam terjadinya hipospadia, dengan terjadinya

hipospadia familial terjadi pada 28% kasus Mekanisme genetik yang sebenarnya sangat rumit

dan bervariasi. Adanya kemungkinan dari penurunan gen autosomal dominan sedang

diperdebatkan, hipotesis ain adalah penurunan gen autosomal resesif dengan manifestasi

inkomplit. Aberasi kromosomal ditemukan secara sporadic (Santanelli, 2010).

Faktor non-genetik utama yang dihubungkan dengan hipospadia adalah pemberia hormon sexual;

peningkatan insiden hipospadia ditemukan pada bayi ang lahir yang ibunya terpapar terapi

estrogen selama kehamilan. Prematuritas juga memiliki kejadian yang lebih besar dengan

hipospadia dibandingkan dengan populasi umum (Fabio dan Grippaudo, 2010).

PATOFISIOLOGI

Lokasi abnormal dari hipospadia terletak pada daerah ventral dari penis, atau di skrotum dan

perineum.
Penis akan terbentuk sekitar minggu kelima kehamilan dalam pengaruh testosteron. Lekukan

urethra akan bergabung dengan urethral groove, dan ketika minggu ke-14 proses ini akan selesai

(lihat gambar dibawah). Pertumbuhan ke dalam dari ujung glans akan berlanjut kedalam untuk

bertemu dengan urethral tube pada fossa navicularis. Preputium kemudian terbentuk pada akhir

dari proses perkembangan (Sadler, 1996).

Gambar 1. Keterangan Gambar: KIRI. Genitalia external pada stadium belum

terdifferensiasi.TENGAH. Genitalia externa laki-laki pada minggu ke-9. Dari Atas ke

Bawah. Potongan melintang pada area genital selama perkembangan dari saluran

urethra.

Hipospadia terjadi etika penggabungan dari leukan urethra terhenti pada ujung proximal

dari glans penis dan dapat terjadi di mana saja sepanjang urethral groove. Bentuk hipospadia

yang paling parah disertai dengan pemendekan urethral groove, yang akan menimbulkan

terikatnya penis, yang dinamakan chordee (Fabio dan Grippaudo, 2010).

 Deformitas yang terjadi memiliki tingkat keparahan yang berbeda tergantung dengan

perkembangan embriologis yag terganggu. Meatus dapat berjenis glanular (60%), penile (35%),

atau scrotoperineal (5%) dan secara klinis inadekuat pada 75% pasien dan sering stenotik.

DIAGNOSIS

Diagnosis hipospadia biasanya jelas pada pemeriksaan inspeksi. Kadang-kadang

hipospadia dapat didiagnosis pada pemeriksaan ultrasound prenatal. Jika tidak teridentifikasi

sebelum kelahiran, maka biasanya dapat teridentifikasi pada pemeriksaan setelah bayi lahir.

Pada orang dewasa yang menderita hipospadia dapat mengeluhkan kesulitan untuk

mengarahkan pancaran urine. Chordee dapat menyebabkan batang penis melengkung ke ventral

yang dapat mengganggu hubungan seksual. Hipospadia tipe perineal dan penoscrotal

menyebabkan penderita harus miksi dalam posisi duduk, dan hipospadia jenis ini dapat

menyebabkan infertilitas.

Beberapa pemeriksaan penunjang yang dapat dilakukan yaitu urethtroscopy dan cystoscopy

untuk memastikan organ-organ seks internal terbentuk secara normal. Excretory urography

dilakukan untuk mendeteksi ada tidaknya abnormalitas kongenital pada ginjal dan ureter.

DIAGNOSIS BANDING
1. Ambiguous Genitalia

2. Anomali Genitalia

Gejalanya adalah :

- Lubang penis tidak terdapat di ujung penis, tetapi berada di bawah atau di dasar penis

- Penis melengkung ke bawah

- Penis tampak seperti berkerudung karena adanya kelainan pada kulit depan penis

- Jika berkemih, anak harus duduk.

MANAJEMEN

Manajemen Hipospadia

Tujuan dilakukan manajemen dari hipospadia adalah antara lain untuk memperbaiki tampilan
kosmetik dan fungsional. Dalam hal fungsional, untuk memperlancar aktivitas berkemih dan
aktivitas seksual. Secara umum, langkah operasi yang dilakukan untuk manajemen pasien
hipospadia, antara lain:

- Memperlebar meatus

- Memperbaiki kurvatura

- Rekonstruksi bagian yang hilang dari uretra

- Restorasi aspek normal genitalia eksterna

Ada beberapa cara yang digunakan untuk manajemen hipospadia, antara lain:
- Jika tidak ditemukan uretra distal pada hipospadia tipe glanular (atau hipospadia tipe glanular
distal), maka manjemen yang bisa dilakukan adalah dengan menggunakan flap lokal dengan
basis meatus (meatotomi) , misalnya teknik Santanelli procedure, Flip Flap, MAGPI (Meatal
Advancement and Glanuloplasty).

- Cara yang akan ditunjukkan berikut ini adalah meatotomi. Prosedur meatotomi diperlukan jika
ukuran meatus uretra eksternal lebih rendah daripada normalnya sesuai dengan usia pasien.

- Jika hipospadia bentuk penil dan penoskrotal, maka manajemen yang bisa dilakukan adalah dengan
reseksi chordee dan rekonstruksi bagian yang hilang dari uretra, misalnya teknik Duckett,
Standoli, Scuderi, modified Koyanagi. Bisa dilakukan dengan jalan satu tahap atau dua tahap.
Untuk hasil yang lebih baik, biasanya dilakukan operasi dua tahap.
 Tahap pertama adalah setelah insisi dari hipospadia telah dilakukan dan flap telah diangkat,
maka seluruh jaringan yang dapat mengakibatkan bengkok diangkat dari sekitar meatus dan
dibawah glans. Setelah itu dilakukan tes ereksi artificial. Bila korde tetap ada,maka diperlukan
reseksi lanjutan.

Tahap kedua adalah rekonstruksi uretra atau urethroplasty. Pada tahap kedua bisa
digunakan suatu teknik MAGPI seperti pada hipospadia tipe glanular distal. Tahap ini dilakukan
jika penis sudah terlihat lurus menggunakan tes ereksi artifisial. Pertama dilakukan insisi
sirkumsisi secara paralel tiap sisi uretra sampai glans, kenudian dibuatlah uretra di bagian
tengah. Jika uretra sudah terbentuk akan ditutup menggunakan bagian lateral flap kulit preputium
ke ventral bertemu di median.

Komplikasi Hipospadia

Jangka pendek
- Edema lokal dan bintik-bintik perdarahan dapat terjadi segera setelah operasi dan biasanya tidak
menimbulkan masalah yang berarti
- Perdarahan postoperasi jarang terjadi dan biasanya dapat dikontrol dengna balut tekan. Tidak jarang
hal ini membutuhkan eksplorasi ulang untuk mengeluarkan hematoma dan untuk
mengidentifikasi dan mengatasi sumber perdarahan.
- Infeksi merupakan komplikasi yang cukup jarang dari hipospadia. Dengan persiapan kulit dan
pemberian antibiotika perioperatif hal ini dapat dicegah.

Jangka panjang
- Fistula : Fistula uretrokutan merupakan masalah utama yang sering muncul pada operasi
hpospadia.Fistula jarang menutup spontan dan dapat diperbaiki dengna penutupan berlapis dari
flap kulit lokal.
- Stenosis meatus : Stenosis atau menyempitnya meatus uretra dapat terjadi. Adanya aliran air seni
yang mengecil dapat menimbulkan kewaspadaan atas adanya stenosis meatus.
- Striktur : Keadaan ini dapat berkembang sebagai komplikasi jangka panjang dari operasi
hipospadia.Keadaan ini dapat diatasi dengan pembedahan, dan dapat membutuhkan insisi, eksisi
atau reanastomosis.
- Divertikula : Divertikula uretra dapat juga terbentuk ditandai dengan adanya pengembangan uretra
saat berkemih. Striktur pada distal dapat mengakibatkan obstruksi aliran dan berakhir pada
divertikula uretra. Divertikula dapat terbentuk walaupun tidak terdapat obstruksi pada bagian
distal. Hal ini dapat terjadi berhubungan dengan adanya graft atau flap pada operasi hipospadia,
yang disangga dari otot maupun subkutan dari jaringan uretra asal.
- Terdapatnya rambut pada uretra : Kulit yang mengandung folikel rambut dihindari digunakan
dalam rekonstruksi hipospadia. Bila kulit ini berhubungan dngan uretra, hal ini dapat
menimbulkan masalah berupa infeksi saluran kemih dan pembentukan batu saat pubertas.
Biasanya untuk mengatasinya digunakan laser atau kauter, bahkan bila cukup banyak dilakukan
eksisi pada kulit yang mengandung folikel rambut lalu kemudian diulang perbaikan hipospadia.

Abstract
Nonsystemic review of the literature was done for timing of surgery, preoperative evaluation and
plan, anesthesia, suture materials, magnification, tissue handling, stent and diversion problems,
intra and postoperative care, dressing, and follow-up protocol. The best time for hypospadias
repair is between 6 and 18 months. Preoperative evaluation in proximal hypospadias includes
hormonal and radiological examination for intersex disorders, as well as for upper tract
anomalies along with routine evaluation. General anesthesia is a rule but local blocks help in
reducing the postoperative pain. Magnification, gentle tissue handling, use of microsurgical
instruments, and appropriate-sized stent for adequate period help in improving the results.
Hormonal stimulation is useful to improve growth and vascularity of urethral plate and decrease
the severity of chordee in poorly developed urethral plate with severe curvature. Urethral plate
preservation urethroplasty with spongioplasty is the procedure of choice in both proximal and
distal hypospadias. Algorithms are proposed for management of hypospadias both with curvature
and without curvature. Two-stage urethroplasty has its own indications. A good surgical outcome
may be achieved following basic surgical principles of microsurgery, fine suture materials,
choosing one or two-stage repair as appropriate, proper age of surgery, and with good
postoperative care. Future of hypospadiology is bright with up coming newer modalities like
laser shouldering, robotics, and tissue engineering.

Keywords: Algorithm, diversion, general considerations, hormonal stimulation, hypospadias,

postoperative care, preoperative evaluation, repair, stent, suture material, urethroplasty

Hypospadiology is still recognized as an expanding and evolving speciality. The results of

hypospadias repair have improved in the last three decades. Davis long back said that “I believe
the time has arrived to state that the surgical repair of hypospadias is no longer dubious,
unreliable, or extremely difficult. If tried and proven methods are scrupulously followed, a good
result should be obtained in every case. Anything less than this suggests that the surgeon is not
temperamentally fitted for this kind of surgery.”[1] One of John Ducket's many enduring
legacies has been the emergence of the ‘hypospadiologist’, i.e., a surgeon committed to
excellence in hypospadias surgery with a case load sufficient to develop and maintain a high
level of specialist expertise. As a result the era of the ‘occasional’ hypospadias surgeon is fast
disappearing. While science undoubtedly merits a higher profile within ‘hypospadiology,’ for the
child with this condition what matters most remains the commitment and skill of the surgeon.
The traditional saying that “see one, assist one and do one” does not hold true for the
hypospadias surgeon; it should be “see many, assist many, do many, and then teach many.” Who
should operate the hypospadias? General surgeons, general urologists, pediatric surgeons,
pediatirc urologists, or hypospadialogists? As hypospadias surgery is technically demanding, any
one of them can, provided the surgeon has a temperament for hypospadias surgery, has mastered
six common techniques in hypospadias and has at least 40-50 cases to operate per year.
Experience in hypospadias surgery has a definite co-relation with a successful outcome. There is
a significant difference in outcome of hypospadias surgery done by pediatric urologists vs. other
surgical specialists.[2,3]

John Duckett had said “There are many successful methods, no single procedure works for all
hypospadias cases, choose a suitable technique for individual case.”

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TIMING OF SURGERY
Most males with hypospadias are often diagnosed just after birth or identified during
examination before a newborn circumcision. Rarely, the ventral foreskin will be normal in
appearance and the hypospadias will be noted later in life when the foreskin is retracted or after a
circumcision is performed. Treatment starts with birth of the child, the first and foremost step
being to inform and console the parents about the congenital anomaly, timing and outcome of
surgical procedures, and establishing a bond of confidence between parents and the surgeon.
This helps in removing the worry, guilt, and fear of the unknown to parents and in better
planning of surgical treatment of the child. Meatal dilatation should be done at the time of first
examination if hypospadiac opening is associated with meatal stenosis. The timing of surgery is
chosen after considering milestones of development, size of penis, child response to surgery,
anesthesia risk, and toilet training. The infant develops good tolerance to surgery and anesthesia
by the age of 6 months. The penile length at 1 year is on an average 0.8 cm less than at preschool
age. The child is well aware about his genitalia and toilet training by the age of 18 months. So
the most suitable age for operation of hypospadias is between 6 and 18 months. Another
opportunity is at 3-4 years if the previous optimal age is missed.[4] The American Academy of
Pediatrics review suggest that the ideal age for genital surgery is between 6 and 12 months.[5]
Others prefer to operate even earlier on an adequate-sized phallus at 4 months of age as healing
is quicker with minimal scars and the infant overcomes the stress of surgery easily.[6] Age of
presentation (mean age 5 years) to the hospital in the developing countries is higher than in the
western part of world because of ignorance, illiteracy, and unaffordability, so patients may be
operated whenever the child is brought to the hospital after the age of 4 years.[7]

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PREOPERATIVE EVALUATION
The preoperative assessment includes not only the medical checkup of the child including history
of problems, but also counseling of the parents. Parents should be told about the goals of the
surgery, plan of surgical repair, likely modifications during surgery, common complications and
their treatments, period of hospitalization, postoperative protocol including catheter care,
dressings and medications. The perineum is inspected for diaper rash or infection and if present
then surgery is to be postponed till such infection is cleared off.

Preoperative examination includes measurement of the size of the penis, shape of the glans,
location and size of the meatus, urethral plate for it's development, width and length, severity of
hypospadias, length of hypoplastic urethra, chordee and it's severity, size of dorsal hood, shape
of the scrotum, and associated anomalies like undescended testis, inguinal hernia or penile
torsion. Sometimes multiple pinpoint dimples may be present on the surface of the urethral plate
in addition to a hypospadiac meatus and in such cases location of the meatus should be
confirmed by a probe.[6] Occasionally probing may confirm the partial duplication of urethra
that should be laid open to convert it to one urethra. According to the location of meatus the
hypospadias is divided in to anterior (glanular and subcoronal 50%), middle (Distal penile, mid-
shaft, and proximal penile 30%) and posterior (Penoscrotal, scrotal and perineal 20%).[8]

Other congenital anomalies associated with severe hypospadias are pelvi ureteric junction
obstruction, vesicoureteric reflux, renal agenesis, persistent Mullerian structures and intersex
disorders, undescended testis and inguinal hernia with or without hydrocele.[9] Associated
anomalies with hypospadias increase with severity of the disease. Patients with severe
hypospadias require complete evaluation including ultrasonography for upper tract anomalies
and internal sex organs, karyotyping, micturating cystourethrogram and pandoscopy.[4,10]
Urethrogram or endoscopy is needed for proper assessment of prostatic utricle which may create
problems in catheterization during surgery. Patients with hypospadias of any degree with
impalpable one or both gonads should be evaluated for intersex disorders. Such patients should
have karyotyping and ultrasonography of the urinary tract and internal genital organs.[10]
Accurate assessment of type of hypospadias, severity of curvature, and the urethral quality is
often possible under anesthesia and, therefore decision of surgical plan may have to be changed
on the table.[4]

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HORMONAL STIMULATION
There is no general agreement on the use of hormonal stimulation in hypospadias surgery. Use of
βHCG 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 longterm.[11] HCG
is best suited in cases of patients with undescended testis. But if one suspects a
hypogonadotrophic etiological factor of hypospadias then HCG should be used cautiously as
experimental micropenis model supports delaying hormonal therapy until puberty.[12]
Local testosterone cream 5% twice a day for 5 weeks is preferred by most of the pediatric
urologists and others favour systemic testosterone, as per Koff's regimen (two injections a week
for 5 weeks). Hormonal stimulation increases length of penis significantly, increases vascularity
and thickness of corpus spongiosum and decreases the severity of hypospadias.[13]

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MAGNIFICATION
Key of success in hypospadias surgery is proper dissection and meticulous approximation of
tissues. So magnification becomes an important tool in hypospadias surgery in small
children.[11] Various magnification tools are high-powered simple glasses, loupes and operating
microscope. The choice depends upon the availability and acclimatization of the surgeon to use
the magnification.

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ANESTHESIA
General anesthesia is the rule, often associated with caudal or penile anesthesia. The evidence
examined shows an increased duration of analgesia with caudal bupivacaine, clonidine, ketamine
and midazolam. However, routine use of these adjuvants in the setting of elective outpatient
surgery shows an improved patient outcome. It is unclear if the potential for neurotoxicity is
outweighed by clinical benefits. Further testing, including large clinical trials, is required before
recommending routine use of nonopioid additives for caudal blockade in children.[14] Routine
local penile block at the beginning and ending of surgery significantly improves relief from
postoperative pain.[15]

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SUTURE MATERIAL
The composition of suture material and the technique of suture placement may contribute
significantly in the outcome of hypospadiac surgery. Significantly low fistula rate (4.95% vs.
16.6%) were noted by Ulman and co-workers in subcuticular repair compared to full thickness
through and through technique,[16] while others are of the opinion that sutures used either
subcuticular or through and through does not affect the results provided polyglactin suture is
used.[17] Late absorbable sutures may be the cause for small fistulae. Usually polyglactin
absorbable sutures are useful for the inner most layer closure with epithelial inversion, while
polyglyconate sutures are used for other layers.[2] The author is of the opinion that when the
technique involves passing the sutures through the epithelium of urethral plate or skin, then early
absorbable suture like Vicryl rapid should be used and in subcuticular suturing, any of the
absorbable or late absorbable suture material can be used.
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TISSUE HANDLING
General principles in hypospadias surgery include minimal tissue trauma, minimal and pin point
use of cautery, tension free repair in all layers, use of well-vascularized tissue closure in as many
layers as possible, and single-stage repair with epithelial inversion.[4]

Tissue trauma can be minimized by proper handling of the tissues by using stay sutures, skin
hooks, microsurgical instruments and dissecting the tissues in proper plane, maintaining the
proper vascularity of the flap for neo-urethra, and skin is very important in prevention of
ischemic complications. During penile degloving plane of dissection is kept at the level of Buck's
fascia [Figure 1] and while dissecting the inner prepucial flap it is between two layers of Dartos
fascia [Figure 2]. For mobilizing the urethral plate and urethra, a plane of dissection is created,
beginning at the level of Buck's fascia in normal urethra and then proceeding distally in the same
plane. Incision for glanular wings should be in continuity with corpus spongiosum [Figure 3].

Figure 1

Showing deep plane of dissection at Buck's fascia

Figure 2

Showing superficial plane of dissection at two layers of dartos fascia

Figure 3

Showing mobilization of corpus spongiosum and urethral plate in to glans

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URINARY DIVERSION
Use of stents and diversion is still a debatable issue. In a multicentric retroscopic review of
Mathieu's repair, no difference was noted in fistula rate in stented vs. nonstented repair and none
of the patients, even in caudal anesthesia group, had urinary retention postoperatively.[17]
Others had successful stent free repair with Snodgrass modification.[18] According to some
authors, there was significant difference in outcome of stented vs. unstented patients[4] while
others claim no difference in outcome.[19] In author's opinion using silastic catheter of adequate
size according to the age of child, just inside the bladder for about a week is safer and improves
the results. The stent can be left in the diapers and patient can be sent home the same day in day
care centers.

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DRESSING
Hypospadias surgeons have different views about postoperative dressings; some concluded that
no dressing is required in plate preservation procedures,[19] while others have used various
innovative methods. The techniques described and found suitable include polyurethrane bio
occlusive foil, Cavi care, SANAV, glove-finger, Fibrin seal (Tisseal), Melolin, Peha-Haft, and
adhesive membrane dressings. Silicon foam dressing was found effective in restricting edema,
hematoma formation and stabilization with easy removal.[20] Pressure during the dressing
following hypospadias repair is a controversial issue. Excessive pressure may compromise the
blood supply of flap and skin which may lead to tissue necrosis while no pressure may lead to
hematoma, edema and infection increasing the incidences of complications. The author believes
that dressing is essential to control postoperative edema, prevent hematoma formation that
predisposes to infection and it works as a barrier from surroundings specially in third world
countries where the ward cleanliness and hygiene may not be ideal.

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SURGICAL TREATMENT
The goals in management of hypospadias repair are creating a straight penis, reconstructing slit-
like meatus at the tip of penis, creating a urethra of adequate length and uniform caliber,
symmetry in appearance of glans and penile shaft, projectile stream and normalization of
erections, and thereby imposing confidence in the child. These goals can be achieved by
meatoplasty and glanuloplasty, orthoplasty, urethroplasty, scrotoplasty and skin cover.

Meatoplasty and glanuloplasty

This helps in creating the conical glans, fish mouth wide meatus at the tip, giving projectile
stream and prevents meatal stenosis and fistula. V flap or W shape flap meatoplasty are
commonly done with flap urethroplasty. Circumcoronal incision is planned 5-7 mm away from
corona to raise para-glanular flaps, suturing of which will reduce the incidence of subcoronal
fistula [Figure 4]. An important point in meatoplasty and glanuloplasty is raising adequate length
of glanular wing to prevent pressure on the neo-urethra [Figure 5] that will reduce ischemic
complications.
Figure 4

Showing glanular and para-glanular flaps for glanuloplasty

Figure 5

Showing glanuloplasty with adequate space between neourethra and glanular flaps

Orthoplasty

Mclaughlin and Gitte's test (1974) is the most important mile stone in deciding the single-stage
repair. Straight penis is the first requirement for a successful repair and complete chordee
correction, should be tested on table before proceeding for the urethroplasty. Various method of
chordee correction are penile de-gloving, plication procedures, split and roll technique, extended
urethral mobilization, penile disassembly and tunica grafting procedures. Rational approach in
correction of chordee is described step-by-step by Bhat 2007[7] and Bhat et al. 2007.[21]
Pharmacological erection by intracorporeal injection of Prostaglandin E1 has been found useful
both intraoperative as well as in follow-up visits to check for correction of curvature.[22]

Urethroplasty

Various factors in deciding the type of urethroplasty are size of penis, chordee, location of the
meatus, size and configuration of the glans, development and width of urethral plate,
development of corpus spongiosum, length of hypoplastic urethra, ventral penile skin proximal
to the meatus and skin available on the dorsal hood and penile shaft.

Urethral plate preservation procedures are preferred as there is no substitute for urethra. Final
decision about the type of urethroplasty is to be taken only after correction of chordee. In distal
and middle hypospadias with minimal chordee or without chordee, the first choice of procedure
is TIP urethroplasty with inlay graft if required and second is onlay flap urethroplasty. An
algorithm [Figure 6] is proposed for choice of procedures in hypospadias without curvature. The
controversy still continues in management of proximal hypospadias. On one end of the spectrum
are one-stage procedures with utilization of urethral plate[7,23,24] and on the other are two-stage
procedures.[2,25,26] Lam et al. reported spraying of stream in 40%, milking of urethra after
voiding in 40%, milking of the ejaculate 42.9%, and painful ejaculation in 7.7% in spite of good
cosmetic results in two-stage procedures.[27] So hypospadias surgery should aim to preserve and
utilize the urethral plate and supplement with spongioplasty to improve the results. To avoid the
confusion, a rational approach is proposed [Algorithm 2, Figure 7] taking into consideration all
the factors influencing the repair with stress on preserving the urethral plate and one-stage
urethroplasty.[7,21,28]

Figure 6
Algorithm 1 for hypospadias without curvature

Figure 7

Algorithm 2 for hypospadias with curvature

Healthy tissue cover

Healthy-vascularized tissue cover over neourethra or corporal graft definitely helps in overall
surgical success. Poorer the tissue more is the need to provide healthy vascularized tissue to
optimize the chances of success. Various healthy and well-vascularized tissues used are
dorsal/ventral dartos flap, [Figure 8] tunica vaginalis, [Figure 9] denuded inner prepucial skin,
and spread out corpus spongiosum [Figure 10]. Dorsal dartos vascular pedicle is mobilized up to
root of penis to avoid torsion and tunica vaginalis requires adequate mobilization on its vascular
pedicle to prevent inherent sequelae of torque. Skin is to be denuded completely to prevent the
complication of buried skin inclusion dermoid. Spongioplasty is the most suitable healthy tissue
cover for neourethra and reconstructs a near normal urethra.

Figure 8

(a) and (b) Showing dartos flap as healthy tissue cover

Figure 9

Showing tunica flap as healthy tissue cover

Figure 10

Showing spongioplasty as healthy tissue cover

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PREPUTIOPLASTY
In distal hypospadias, surgery is done more for cosmetic appearance. Many parents and patients
demand prepucial reconstruction. Circumcision is less acceptable to both the general population
and medical profession; the prepuce can be preserved and refashioned to give good cosmetic
results.[29] Preputioplasty is feasible in patients where prepuce is not utilized in urethroplasty
and satisfies the patients and parents to have an uncircumcised penis. This adds about 20 extra
minutes to the operating time. Klijn et al. had higher complications of urethroplasty with
preputioplasty and they discouraged preputioplasty when circumcision is done.[30] While others
recommend preputioplasty since they had no difference in results with preputioplasty.[29,31,32]
Author is of the opinion that preputioplasty is to be added to urethroplasty in distal hypospadias
where prepuce is not utilized and parents demand preservation of prepuce.

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SKIN COVER
Dorsal hood is brought to ventrum by Byar's technique or by Nesbit's technique. Disadvantage of
Nesbit is the suture line on lateral side, but it helps in reducing the fistula rate. Author
recommends the midline suture simulating median raphae with trimming of skin margins to
avoid ischemic complications. In two-stage procedures or in redo cases, there may be tension on
suture line requiring dorsal releasing incision or some times nongenital skin graft is needed.

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POSTOPERATIVE CARE
The important points which require attention in postoperative period are dressing, catheter care,
analgesics and antibiotics. Postoperatively the child may experience incisional pain and pain
related to bladder spasms. We treat incisional pain with acetaminophen or acetaminophen with
codeine. Bladder spasms are best treated acutely with Oxybutynin (0.2 mg/kg/dose every 6 h).
Prophylactic antibiotics are advised till patient is on catheter drainage. The parents are instructed
to apply an antibiotic ointment to the tip of the glans penis and urethra meatus every time the
diapers are changed or after passing urine.

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FOLLOW-UP PTOTOCOL
Usual period of follow-up is for two years after surgery as it is expected that by this time most of
the complications will appear and follow-up beyond the period may not be cost effective. Any
patient with complications beyond this period will automatically present to the surgeon. Early
discharge is being justified on the grounds that it is best to let the patient forget his genital
abnormality and surgery. Repeated visits to the hospital will remind the child of his abnormality
and may have psychological implications. Only a section of surgeons like adult urologists have
an access to these patients up to teenage and may contribute a lot in long-term follow-up, actual
outcome of surgery and real incidence of chronic complications. An ideal follow-up will be at 1,
3, 6 months and then yearly up to 2 years and review follow-up at puberty and mid-teens by
which time genital maturity is at or near completion and patient can express his social and sexual
problems following genital surgery. A previous asymptomatic fistula too may start leaking,
chordee may appear due to failure of growth of scarred urethra, shape and size of the penis may
be of concern to the patient. These late complications may need to be treated.

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FUTURE
Though many new concepts and innovations have been added, but the importance of current
techniques are not going to be obsolete suddenly. Future of hypospadiology is directed toward
the new innovative techniques and use of developments in biotechnology like LASER
shouldering, robotics, and tissue engineering. Laser shouldering has been tried, but it still has to
prove its results better than conventional suturing. Urethral regeneration has been identified as
one of many potential applications of tissue engineering. Tubular acellular collagen matrices
seeded with urothelial cells have been used experimentally with success to repair a created
urethral defects in a rabbit model. Similarly others have grown and used the corpus
cavernosum.[33,34] But the complexity of urethral structure (urethral mucosa surrounded by
spongiosum) makes it difficult to do urethral replacement by tissue engineering. So it is unlikely
that there will be routine use of the technology in hypospadias surgery, though it could find a
limited role in complex salvage cases. Robotics can play a major role by removing the effects of
tremors for meticulous suturing.

Abstract
Hypospadias is a highly prevalent congenital anomaly. The impact of the defect and operative
interventions on sexual and reproductive function has been addressed by few publications. It is
essential to know the possible outcomes of intervention for appropriate counseling, operative
planning, and follow-up. English articles indexed in Pubmed dealing with the long-term sexual
and reproductive outcome following hypospadias repair from 1965 to 2007 were reviewed. To
our knowledge, there was no prospective trial comparing the impact of various techniques on
sexual outcome. There is considerable discordance in literature regarding the effects on sexual
function. A few publications report patient and partner dissatisfaction with the appearance of
genitalia. Sexual dissatisfaction is often attributed to penile size. Ejaculatory disturbances range
between 6 and 37% of operated individuals. There is no convincing evidence for impaired
fertility. The long-term follow-up is essential to identify problems and to address them
appropriately. Literature documenting the outcome of specific operative procedures and analysis
based on severity of hypospadias will be informative. The long-term follow-up of the newer
techniques which are more commonly used are awaited.

Keywords: Hypospadias, reproduction, sexual function

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INTRODUCTION
In hypospadias, the inherent difficulties to reconstruct the urethra, straighten the penis, and to
restore the appearance of the penis are evident from the number of techniques and modifications
described in the literature. However, the impact of the deformity extends beyond the realms of a
structural defect, by virtue of the diverse functions of the penis. To counsel parents and patients
appropriately, it is essential to know the effect on sexual function and reproduction. Literature on
the long-term outcome, impact on sexual function and reproduction continues to be sparse. We
reviewed the published literature on the sexual and reproductory outcome of hypospadias.

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GENITAL PERCEPTION
Publications on the psychological, social, and sexual development of patients operated on for
hypospadias are still rare and the results are somewhat discordant. The possible explanations for
these discrepancies are mainly methodological, with too small series, low rates of response to
questionnaires, study populations of different ages and above all the absence of a control group,
which prevents any comparison of the results with those of a reference population.[1] Another
possible reason for difficulty in long-term follow-up is that the patient, after growing up to be an
adult, often does not follow up with the initial surgeon. Moriya et al.[2] observed that the rate of
dissatisfaction with penile appearance was slightly higher in the hypospadias group than in age
matched controls which was not statistically significant (40.9% vs. 34.2%; P = 0.809). The
single reason for dissatisfaction in hypospadias group was smaller penile size. Mureau et al.[3]
interviewed 116 hypospadias patients and 88 controls who underwent hernia repair, between 9
and 18 years of age. They noted that approximately 25% of the patients reported dissatisfaction
with penile appearance compared to only about 5% of the controls. Scars, penile size, and
glanular shape were the most spontaneously reported reasons for dissatisfaction. Weber et al.[4]
used a genitalia perception score (GPS) that ranged from a minimum of 1 to a maximum of 10 to
evaluate self-perception of genitalia. Sixty-four patients between the age of 6 and 17 were asked
to rate the appearance of their penis with regard to the following criteria: Meatus, glans, penile
skin, penile straightness, and general appearance. Their GPS was almost as high as that of a
control group but similar rating done by urologists on the same population was significantly less
favorable. Liu et al.[5] observed that a higher percentage of those with proximal hypospadias
were dissatisfied with the penile appearance than those who were operated for distal hypospadias
(40% vs. 19.2%).

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PSYCHOSOCIAL AND SEXUAL DEVELOPMENT

Berg et al.[6] in the early 1980s noted that adult men operated on for hypospadias more
frequently recalled depression and anxiety in addition to poorer adjustment with peers during
childhood. The information pertaining to the childhood development of this group was based
upon adult recall of childhood events. Hence these data might be distorted by adult experiences.
Mureau et al.[3] compared the psychological adjustment following hypospadias surgery with a
control group who underwent hernia repair. They noted that psychosocial adjustment did not
significantly differ from the control group. Lesma et al.[7] assessed the psychosexual outcome in
30 patients using Center for Epidemiologic Studies Depression Scale (CES-D) and Self-rating
Anxiety Scale (SAS) and an original structured questionnaire. Absence of depression and anxiety
traits was noted in both populations. Sandberg et al.[8] compared 175 boys, 6- to 10-year old
who underwent hypospadias repair with a community sample. Though boys with hypospadias
were slightly lower in social involvement, they did not perform more poorly in schools.

Sexual sensation

Sexual sensation has not been well documented in most articles. Bubanj et al.[9] noted that self-
reported strength of libido was slightly better for controls compared to patients with hypospadias
but without a statistically significant difference. Moriya et al.[2] noted that only about 10% of
both patients and controls reported that their libido was low.

Sexual life

The social and sexual life of adults operated for hypospadias during childhood has been studied
by a few authors. Aho et al.[10] compared those who underwent hypospadias repair and
circumcision. There was no significant difference in sexual and social life. Almost the same
proportions reported that they were not inhibited in seeking sexual contacts. All participants
reported exclusive heterosexual orientation and they were mostly satisfied with their body image.
Conversely, Liu et al.[5] observed that 49 out of 102 patients complained that their penis had
been ridiculed by partners. There was no control group in this study. They also observed that
those with proximal hypospadias (60% vs. 36.5%, P < 0.05) and those with complications had
been more often ridiculed than those in the distal group and those without complications (78.9%
vs. 29.7%, P < 0.05). In a comparative study, Mureau et al.[11] compared 73 who underwent
hypospadias repair in childhood with 50 controls. Though hypospadias patients reported a more
negative genital appraisal than the controls, they did not have a different sexual adjustment.
Higher patient age at final operation had a negative impact on sociosexual development.

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ERECTION AND SEXUAL SATISFACTION

The erectile problems in hypospadias may be attributed to surgically correctable and
noncorrectable causes. More commonly encountered correctable causes include persistent
chordee, torsion, inadequate cosmetic outcome, etc. Commonest surgically uncorrectable cause
is the size of the penis. Achieving a straight penis is one of the objectives of hypospadias
correction. With a constant move toward achieving a normal-looking penis, the results of
contemporary repairs are likely to be different. Sommerlad[12] reviewed 60 adults who
underwent hypospadias repair, half of which were Ombredanne repair and the unsightly
redundant skin was a frequent source of complaint. Kenawi evaluated the sexual function in 82
subjects who underwent surgery for hypospadias and he noted that incomplete or incorrect
surgery resulted in sexual dissatisfaction.[13] Bubanj et al. observed that though the frequency of
intercourse during 4 weeks was significantly lesser for those who were operated for hypospadias,
there were no significant difference between patients with hypospadias and controls regarding
inhibition in seeking sexual contacts or patterns of sexual relationships. Those with distal
hypospadias were more satisfied with their sexual life.[9] Of the 76 sexually active adults, Liu et
al. noted that the commonest sexual complaints included short penis, increased curvature, painful
erection, and no erection. The erectile problems were more in those who had proximal
hypospadias.[5] They also felt that the main reason for dissatisfaction was penile size. Mureau et
al.[3] studied noted that the lesser size of the penis was noted to be a major cause for
dissatisfaction. Similar observation regarding penile size was made by Moriya et al.[2] Zaontz in
his editorial comment of this article has underscored the importance of penile size. Sexual
function and satisfaction in 10 adult patients who underwent oral buccal mucosa graft
urethroplasty was studied using International Index of Erectile Functioning (IIEF-15) by Nelson
et al. They noted that the long-term sexual function and satisfaction were excellent, in spite of
them having undergone multiple procedures.[14] The long-term efficacy of dorsal plication was
evaluated by Chertin et al.[15] Six of the 28 in whom the erection test was repeated later
required further plication. Yucel et al. noted that 10 out of 25 who underwent re-operation for
hypospadias had recurrence of chordee.[16] Bubanj et al. followed up patients following
hypospadias repair after puberty. They found that higher number of study patients had ventral
curvature during erection (40% vs. 18%) compared to controls.[9] This underscores the
importance of long-term follow-up to ensure that there are no significant deformities when they
become sexually active. Whether the generous utilization of procedures on the dorsal aspect of
the corpora in order to preserve the urethral plate has any effects on penile length, deformity, and
sexual function will be known when the long-term results of these procedures are available. A
few techniques, like the one described by van der Meulen have a tendency to produce penile
torsion.[17] With a decreasing trend toward using long tabularized prepucial island flaps for
proximal hypospadias, penile torsion, and associated difficulty in intercourse is likely to be less.
Studying whether any particular technique has an increased propensity to difficulties in erection
will be informative. Tubularized incised plate (TIP), meatal advancement and glanuloplasty
(MAGPI), and glanular reconstruction and prepucioplasty (GRAP) have a lesser incidence of
penile torsion and the mechanical difficulties are likely to be less.

Ejaculation after hypospadias repair

Inability to achieve satisfactory ejaculation is documented in almost all publications. Reported

incidence ranges from 6 to 37%. Problems reported include weak or dribbling ejaculation,
having to milk out ejaculate after orgasm, quantity of semen passing after intercourse,
anejaculation with or without orgasm, etc.[1] Liu et al.[5] observed that the rates of ejaculation
problems in the distal and proximal groups were 19.5% (8/41) and 48.6% (17/30), respectively;
in the one- and two-stage groups were 16.7% (7/42) and 52.9% (18/34), respectively. Olofsson et
al.[18] assessed the perspective of young men 20 years after surgery and they noted that 8 out of
22 had attenuated ejaculation. All these men underwent Byars’ two-stage technique. Bubanj et
al.[9] documented spraying or dribbling of ejaculate in a third of patients. Miller et al.[19]
studied the sexual and urinary function of 19 men who had undergone reconstruction for perineal
or perineoscrotal hypospadias. Though 15 of them had normal sexual function, only seven had
satisfactory ejaculation. In the proximal hypospadias, the neourethral tube is devoid of smooth
muscle and corpus spongiosum. Hence there may be little room for improvement in this group
with the operative techniques being used now. With the utilization of TIP, urethral plate
mobilization and spongioplasty for proximal hypospadias[20] whether there will be an
improvement in ejaculation remains to be seen.

Fertility

Literature is scant on the fertility of men who had hypospadias. Aho et al. found that men who
had hypospadias during childhood were less likely to live with a partner and that they had fewer
children (0.8 vs. 1.1).[10] The difference was not statistically significant. Bracka evaluated the
semen analysis of 169 men who were operated for hypospadias in childhood.[21] Of the 32 who
had fathered a child and only one had sperm count of < 20 million/ml. But 40 of the 137 (29%)
whose fertility was not proven had sperm counts < 20 million/ml. Two recent publications on
sexual function following hypospadias have not assessed fertility.