http://emedicine.medscape.

com/article/1015227-overview#showall
http://www.brazjurol.com.br/novembro/baskin_621_629.pdf
http://www.lipteh.com/Study-Notes/Urogenital/Snodgrass%20method.pdf

Hypospadias Repair: An Overview of the Actual Techniques

Ramnath Subramaniam, F.R.C.S., F.E.A.P.U.,1 Anne Francoise Spinoit, M.D.,2 and Piet Hoebeke, M.D., Ph.D.2

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3312185/

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. 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 over 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.[1]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.

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. [2, 3] 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.

Proximal shaft hypospadias. Note typical dorsal

hood of foreskin and ventral penile skin deficiency.

Penoscrotal hypospadias. Note associated ventral chordee and true

urethral meatus located at scrotal level.

Epidemiology
Frequency
United States
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
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.[4]

Mortality/Morbidity
The treatment for hypospadias is surgical repair. Hypospadias is generally repaired for functional and cosmetic
reasons. The more proximally ectopic the position of the urethral meatus, the more likely the urinary stream is
to be deflected downward, which may necessitate urination in a seated position. Any element of chordee can
exacerbate this abnormality. Fertility may be affected. The abnormal deflection of ejaculate may preclude

effective insemination, and significant chordee can preclude vaginal insertion of the penis or can be associated
with inherently painful erections.
Although the most minor forms of hypospadias are insignificant in physiologic terms, they too may merit repair
on the basis of the potential psychological stress associated with having a genital anomaly.

Race
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%.

History
Obtain a thorough history and physical examination, including any history of a familial pattern of hypospadias,
any past medical history or comorbidity, and a physical assessment focusing on the meatal location, glans
configuration, skin coverage, and chordee.
A history of infertility and treatment should also be documented; in vitro fertilization (IVF) has been associated
with a higher incidence of hypospadias.

Physical
Although the diagnosis of hypospadias has been made with both prenatal fetal ultrasonography and magnetic
resonance imaging (MRI), the diagnosis is generally made upon examination of the newborn infant. [5]
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.

Causes
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 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. [6]
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 nearly
10% of boys with isolated hypospadias had at least one affected allele with a 5-alpha reductase mutation.

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.
[7]

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 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.[8] 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.[9, 10]

Diagnostic Considerations
Undescended testes and inguinal hernias are the anomalies most commonly associated with hypospadias; look
for undescended testes and inguinal hernias during the review of symptoms and physical examination. In 1981,
a review by Khuri of more than 1000 patients with hypospadias reported that the incidence of undescended
testes and inguinal hernias was 9% for each.[11] With more severe forms of hypospadias, the incidence of
undescended testes exceeded 30%, and the incidence of inguinal hernias approached 20%.

The combination of hypospadias and undescended testis can be an indicator of an underlying disorder of
sexual development (DSD). In a 1999 study by Kaefer et al, DSD states were identified in approximately 30%
of patients with unilateral or bilateral undescended testes and hypospadias, and more proximal meatal location
carried a higher association of DSD states than more distal meatal location. [12] If any gonad was nonpalpable,
the incidence rose to 50%; however, if both gonads were palpable, the incidence was only 15%.
A prostatic utricle is occasionally noted when catheterization of the urethra is attempted in patients with
hypospadias.

Differential Diagnoses

Circumcision

Disorders of Sexual Development

Genital Anomalies

maging Studies
Upper urinary tract anomalies are rarely associated with hypospadias and do not justify routine imaging in
these patients unless other organ system anomalies are present. Other associated findings are more common
(eg, enlarged prostatic utricle, low-grade vesicoureteral reflux) but are of little consequence clinically unless
other symptoms merit evaluation.[13]

Medical Care
Minor cases of hypospadias, in which the meatus is located up toward the tip of the glans, may not require
surgical repair and may simply be managed with observation.

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. [14]
Late hypospadias repair, in the pubertal and postpubertal period, is associated with complications, primarily
urethrocutaneous fistula, in nearly half of patients.[15]Some reports cite a higher rate of complication in 5-yearold patients than in 1-year-old patients, suggesting that earlier repair is generally better.[16]

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.
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 acidbased sutures may offer the
best balance of resilience when exposed to urine, without excessive time to absorption resulting in a foreign
body reaction.[17](See the image below.)

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.

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.[18] 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. [19] 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. [20]
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. [21]
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 for 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%). [22]

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

Complications
It is clear that repairs that are more proximal are associated with a greater incidence of complications. Older
age at surgery and low surgical experience have also been associated with poorer outcomes. With longer
follow-up, it is apparent that late complications can occur and most advocate continued evaluation through
puberty.[23, 24, 25, 26]

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. [27]

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.

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 inurinary 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.

Newer surveys have suggested milder forms of erectile dysfunction may be more common with more proximal
hypospadias repairs.[28]

Prognosis
Current 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.[29]Newer scoring systems to lend an objective measure to long-term outcomes are becoming
more popular.[30, 31]

Future prognosis
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 patients with hypospadias.
Evidence suggests that online support groups can play an important role in how parents and patients cope with
this condition.[32]