45  Hypospadias
Christopher J. Long, MD, Mark R. Zaontz, MD, and Douglas A. Canning, MD
I
n 1995 John Duckett, MD, a hypospadias pioneer, wrote: “Hypo-
spadiology requires an in-depth study of the contributions of our
predecessors as well as a thorough knowledge of the methods and
maneuvers of current experts. To study at a medical center where
large numbers of hypospadias cases are managed is more beneficial
than the anguish of learning through one’s mistakes” (Duckett, 1995).
As outlined in this chapter, we have made significant advancements
since his passing, but we still have a lot of work to do to improve
outcomes. Expanding Dr. Duckett’s vision, meticulous documentation,
collaboration across multiple centers, and honest outcome reporting
will further advance this field.
EMBRYOLOGY
The classic triad of hypospadias, a dorsally hooded foreskin, a
proximal urethral meatus, and ventral penile curvature, arises from
an arrest in penile development. A more extensive review of penile
embryology is covered elsewhere in this volume, so we will focus
on the most relevant concepts.
The initial stages of external genital developmental are similar for
both sexes. Mesenchymal ingrowth at the cloacal membrane on the
ventral surface of the fetus divides it into two halves, forming the
foundation for anal and genital anatomy development (Glenister,
1954; Kurzrock et al., 1999). Migrating cells coalesce over the cloacal
membrane to form several folds, including the cloacal folds, which
merge to form the genital tubercle. The urogenital diaphragm forms
below the genital tubercle as the anal and genital systems separate
at 7 weeks of gestation. The urogenital folds and the more lateral
labioscrotal folds emerge lateral to the urogenital membrane (Fig. 45.1).
The exact mechanism of urethral development is debated. In
1954 Glenister proposed the “ectodermal ingrowth theory,” in which
the stratified squamous lining of the glanular urethra originates
independently via retrograde ectodermal ingrowth from cannulation
of the glans. The glanular urethra then fuses at the coronal margin
with the endodermal penile urethral plate, which is forming simultane-
ously as a result of antegrade fusion of the urethral folds (Glenister,
1954). This process occurs sequentially from the anus to the distal
glans during gestation weeks 8 to 16, the critical period for external
genitalia development.
Alternatively, recent evidence supports an entirely endodermal
origin for the urethra (Kurzrock et al., 1999). According to this
newer theory, the urethral plate formation is contiguous from
the penile urethra into the glans and undergoes differentiation
to form the stratified squamous layer (Kurzrock et al., 1999). The
authors have further refined their theory on urethral development
to describe the two-zipper hypothesis of urethral development. This
begins with the “unzipping” event, stemming from proliferation
of the flat urethral plate into a canalized urethral groove, which is
lined with flanking urethral folds. This process is seen in the male
and female urethra. The “closing zipper” is androgen dependent
and unique to the male urethra. As opposed to a simple fusion
of the urethral folds, the fusion process occurs in an interlacing
fashion at varying rates and levels, forming the contiguous urethra
(Li et al., 2015; Shen et al., 2016). Disruption of this process results
in the large spectrum of variants of hypospadias (Fig. 45.2).
Prepuce development coincides with and is dependent on
normal urethral development. At approximately 8 weeks of gestation,
preputial folds first appear on the distal penile shaft at the base of
the corona (Glenister, 1954). These folds first fuse dorsally, then
with further penile growth, join ventrally to envelop the developing
glans. This process is limited by the developing urethra. Ventral
fusion is not completed until the urethral folds fuse, typically
around 20 weeks of gestational age (Baskin and Ebbers, 2006).
Therefore incomplete urethral fold fusion results in an ectopic
meatus accompanied by incomplete foreskin, seen in 95% of boys
with hypospadias (Baskin and Ebbers, 2006). Contrary to this theory,
megameatus with an intact prepuce (MIP) is seen in 5% of cases.
The precise cause of this variant, characterized by a widened, distal
urethral meatus with a normal foreskin and a lack of severe curvature,
is unclear, but we know that complete development and midline
fusion of the preputial folds are not always accompanied by an
intact urethra (Duckett and Keating, 1989).
External genital development occurs in two phases, a hormone-
independent phase and a hormone-dependent stage, beginning at
the eighth week of gestation. Expression of the sex-determining
region of Y chromosome (SRY) gene product between 7 and 8
weeks of gestation results in masculinization of the undifferentiated
gonad, testosterone production, regression of female structures,
and elongation of the genital tubercle (Baskin and Ebbers, 2006).
Leydig cell–produced testosterone is locally converted by 5α-reductase
type 2 to dihydrotestosterone (DHT) by cells in the developing
external genitalia, further promoting growth (Kim et al., 2002). The
growth factor Sonic Hedgehog (Shh) plays a critical role in genital
tubercle formation and urethral plate formation (Miyagawa et al.,
2011). Androgen receptors have been localized to the penile skin,
inner prepuce, urethra, and stromal cells of the corpus spongiosum
during the early gestation period, suggesting an equally vital role
for androgens in urethral development (Kim et al., 2002). In
patients with a lack of androgen receptor stimulation, there is a
lack of midline fusion of the scrotal and/or urethral folds, as
occurs in female development, which lacks DHT and androgen
receptors.
KEY POINTS
• Dihydrotestosterone at the 8- to 12-week gestational phase
is a key mediator in the proper development of the penis.
• Male urethra closure is an androgen-dependent process,
contrasting its formation to that of the female urethra.
DIAGNOSIS OF HYPOSPADIAS
Hypospadias is one of the most common congenital anomalies in
boys, occurring in 1 in 150 to 300 live births (Elliott et al., 2011;
Springer et al., 2015). Diagnosis is made on physical exam and
includes the following three criteria: an ectopic, ventrally located
urethral meatus; ventral penile curvature; and an incomplete,
dorsally hooded foreskin. Hypospadias is a broad term, however,
and the severity of each component can vary greatly from boy to boy.
The latter two elements are not always present. Up to 5% of boys
with hypospadias have an intact prepuce (the previously mentioned
MIP variant) and are not diagnosed until the foreskin becomes
retractable or reduced at the time of circumcision (Fig. 45.3). Because
the intact prepuce can hide the presence of incomplete urethral
905
906 PART III  Pediatric Urology

A. Indifferent stage

Urogenital
Genital membrane
tubercle Urogenital
fold
Cloacal
fold

Cloacal Perineum
membrane Labioscrotal
swelling Anal fold and
membrane

6th week 7th week Late 7th week

B. Male

Endoderm

Urethral
groove

Urethral
plate
Epithelial
tag
Epithelial
Urethral
invagination
folds

Penile
urethra

Fig. 45.1.  (A) The relationship of the genital tubercle, urogenital fold, urogenital membrane, and labioscrotal
swellings in the undifferentiated state of development. (B) In males the urogenital folds fuse while the
genital tubercle elongates, forming the penis and glans. The lower image depicts the epithelial invagination
theory of distal urethra formation, as opposed to the recently proposed endodermal source for formation
of the entire urethra. The labioscrotal folds swell and fuse in the midline to form the scrotum.

A B C

Fig. 45.2.  (A) A boy with a proximal hypospadias variant with hypoplastic ventral shaft skin, a penoscrotal
meatus, and severe ventral penile curvature. The glans appears small and with a poorly developed groove.
(B) A boy who has a subcoronal meatus with a flat glans groove and a lack of ventral foreskin. (C) A boy
with severe ventral penile curvature.
 Chapter 45  Hypospadias 907

A B

Fig. 45.3.  (A) This boy has what appears to be an intact prepuce and normal penis. (B) Once the foreskin
is retracted, the hypospadias is obvious. There is separation of the glans wings and a distal penile shaft
meatus, representing the megameatus with an intact prepuce (MIP) variant.

development in a newborn infant, it is critical to retract the foreskin

before circumcision to avoid missing this anomaly and potentially
injuring the incomplete urethra or removing foreskin that could be
incorporated in an ensuing urethral reconstruction (Hatch et al.,
1989). Some have compared the outcomes of patients with a MIP
variant for a prior circumcision to no prior circumcision, concluding
that prior circumcision did not negatively affect hypospadias repair
(Chalmers et al., 2014; Snodgrass and Khavari, 2006). In our experi-
ence a circumcision can be performed if the penis is carefully assessed
to rule out a more severe variant of hypospadias with either a ventral
skin deficiency or ventral penile curvature. In these instances a
circumcision should be avoided because it may be necessary to correct
these anomalies.
Another variant includes boys who have penile curvature without
hypospadias. These boys have all the elements of the hypospadias
complex but have an orthotopic meatus. In these cases, if circumcision
is desired, it should be deferred until definitive surgical management
of the curvature can be performed in the operating room because
the foreskin may be needed for reconstruction.
The urethral meatus location has classically been used to define
severity of hypospadias (Snodgrass et al., 2011). Using this criteria,
the majority of boys (70%–85%) have a mild, distal meatus variant
(Borer et al., 2001; Duckett, 1989; Pfistermuller et al., 2015; Rushton
and Belman, 1998; Fig. 45.4). Proximal hypospadias occurs in 10% Fig. 45.4.  Various potential locations for the ectopic urethral meatus in
to 25% of patients and presents several unique management chal- hypospadias. The classical description was based on the location of the
lenges to the surgeon (Bergman et al., 2015; Manzoni et al., 2004). urethral meatus and divided into distal, midshaft, and proximal hypospadias.
A classification based solely on the location of the urethral meatus
oversimplifies the hypospadias phenotype and may even be mislead-
ing. Boys who have what appears to be a distal urethral meatus may
have severe ventral curvature, hypoplastic tissue overlying a more in some boys. A classification system that includes the location
proximal urethral meatus, and a lack of ventral penile shaft skin, of the urethral meatus as well as the degree of penile curvature
increasing the complexity of the repair and the risk of complication after degloving leads to a more definitive and relevant diagnosis.
(Fig. 45.5). Thus defining hypospadias complexity simply based The GMS score (glans meatus and penile shaft [curvature])
upon the location of the urethral meatus underestimates the severity incorporates physical exam findings in the operating room, assessing
908 PART III  Pediatric Urology
A
B more proximal hypospadias (Hjertkvist et al., 1989; John Radcliffe
Fig. 45.5.  (A and B) Hypospadias classification based solely on the location
of the urethral meatus can be deceiving. These two patients have what
appears to be a distal hypospadias with a subcoronal meatus, but insertion
of a bougie à boule reveals hypoplastic ventral shaft skin and absent corpus
spongiosum to the level of the penoscrotal junction. Both of these boys have
what we consider to be a severe or proximal hypospadias variant in spite of
the initial location of the urethral meatus.
the quality of the glans and urethral plate, the location of the urethral
meatus, and the degree of penile curvature, to objectively assign
scores to stratify severity (Fig. 45.6; Arlen et al., 2015; Merriman
et al., 2013). Indeed, higher GMS scores correlate with an increased
risk of developing a complication (Arlen et al., 2015; Merriman et al.,
2013). The GMS score assessment was developed for use in the
operating room because office measurements have proven to be less
accurate at assessing severity, particularly the degree of ventral penile
curvature. It remains to be seen whether an office-based exam can
provide the same degree of granularity that is obtained in the operating
room, but for now the latter remains the gold standard measurement
of phenotype severity.
The initial evaluation of boys with hypospadias requires a careful
history and physical examination. In addition to the triad of hypo-
spadias, boys may have associated anomalies, including penile torsion,
penoscrotal webbing, and penoscrotal transposition, that must be
considered when planning the repair. Physical examination may
note dysplastic ventral tissue in boys with hypospadias. Ventral
curvature from lack of ventral shaft skin may be immediately obvious
on exam. Anatomic studies have shown worsened curvature and
shorter penile length in boys with proximal hypospadias. Ultrasonic
elastography studies have found less elastic tissue and androgen
resistance in boys with proximal hypospadias, suggesting more
dysplastic tissue, which not only causes penile curvature but can
also complicate surgical reconstruction (Bush et al., 2013a; Camoglio
et al., 2016; Snodgrass et al., 2014a).
Glans (G) Score:
1. Glans good size; healthy urethral plate, deeply grooved
2. Glans adequate size; adequate urethral plate, grooved
3. Glans small in size; urethral plate narrow, some fibrosis or flat
4. Glans very small; urethral plate indistinct, very narrow or flat
Meatus (M) Score:
1. Glanular
2. Coronal sulcus
3. Mid or distal shaft
4. Proximal shaft, penoscrotal
Shaft (S) Score:
1. No chordee
2. Mild (<30˚) chordee
3. Moderate (30−60˚) chordee
4. Severe (>60˚) chordee
Fig. 45.6.  The glans meatus and penile shaft (GMS) score is assessed at
the time of the procedure. Each element assigns a higher number score for
increasing severity. A small, flatted glans with a poorly defined urethral plate
achieves a higher glans score, whereas a proximal location a higher meatus
score, and more severe ventral curvature for the shaft score. The components
are additive to assign a summary score. (From Merriman LS: The GMS
hypospadias score. J Pediatr Urol 9:707–712, 2013.)
The most common anomalies associated with hypospadias are
inguinal hernia and/or hydrocele and cryptorchidism. Inguinal hernia
and/or hydrocele is more common, ranging from 9% to 16%, but
no increased incidence is noted with severity of hypospadias (Wu
et al., 2002). Cryptorchidism is found in approximately 7% of
patients with hypospadias. This increases to nearly 10% with
Hospital Cryptorchidism Study Group, 1992; Shima et al., 1979;
Sorber et al., 1997; Weidner et al., 1999; Wu et al., 2002). The AUA
cryptorchidism guideline recommends that all boys with unilateral
or bilateral undescended testes and severe proximal hypospadias
must undergo additional testing to rule out a disorder of sexual
differentiation (DSD), which occurs much more frequently in
these settings (Kaefer et al., 1999; Kolon et al., 2014; Moreno-Garcia
and Miranda, 2002; Rajfer and Walsh, 1976; Rohatgi et al., 1987).
Guidelines introduced in 2006 included severe hypospadias as a
form of 46,XY DSD (Hughes et al., 2006). The concern for DSD is
particularly high in a boy with hypospadias and a nonpalpable testis.
Kaefer et al. (1999) examined 79 patients with a history of hypospa-
dias and cryptorchidism. Those with a nonpalpable testis(es), either
unilateral or bilateral, had a threefold increased risk for having
an underlying DSD diagnosis identified (approximately 50% vs.
15%). There was a significantly increased risk of DSD with proximal
hypospadias compared with distal hypospadias (64% vs. 7%)
(Kaefer et al., 1999). A normal 46,XY karyotype may be misleading,
particularly in boys with the most severe variants. The surgeon must
be alert for contributing diagnoses such as 5α-reductase deficiency,
androgen receptor insensitivity, decreased testosterone production
associated with testicular dysgenesis, and ovotesticular DSD. Workup
may include ultrasound (to rule out müllerian remnants), karyotype,
and hormonal analysis (testosterone, FSH, LH, AMH). These data
remind surgeons to look carefully for signs of DSD when planning
surgery in a boy with proximal hypospadias.
ETIOLOGY OF HYPOSPADIAS
Hypospadias is multifactorial in origin, including genetic and
environmental concerns. Risk factors include premature birth, infants
small for gestational age (<10th percentile for weight, length, and/
or head circumference), and intrauterine growth restriction. All have
been associated with an increased risk for having a newborn with
hypospadias (Gatti et al., 2001; Ghirri et al., 2009; Hussain et al.,
2002). In particular there is a trend toward premature birth (<37

KEY POINTS
• Hypospadias is diagnosed by physical exam and includes
the following three criteria: an ectopic, ventrally located
urethral meatus; ventral penile curvature; and an
incomplete, dorsally hooded foreskin.
• The classic definition of hypospadias severity focused on
the location of the urethral meatus. A classification system
that includes the location of the urethral meatus as well as
the degree of penile curvature after degloving leads to a
more definitive and relevant diagnosis.
• The presence of hypospadias and unilateral or bilateral
cryptorchidism should raise concern for a DSD and
prompt an appropriate workup.
weeks) and low birth weight in those with a combination of proximal
hypospadias and cryptorchidism (Sekaran et al., 2013). One theory
to explain this finding is placental insufficiency, which frequently
contributes to small-for-gestational-age boys. Decreased placental
weight at birth is associated with a twofold increase in hypospadias
development and a 1.5 times risk of cryptorchidism (Arendt et al.,
2016). Use of assisted reproduction techniques are also associated
with higher rates of hypospadias (Wennerholm et al., 2000). Although
these infants also have higher rates of prematurity, low birth weight,
and twinning, multivariate analysis suggests that assisted reproduction
remains an independent factor (Funke et al., 2010).
Epidemiologic analysis has identified strong familial associations
of hypospadias, suggesting a genetic component (Fredell et al., 2002;
Schnack et al., 2008). Studies of twin boys reveal a concordance
rate of 9% to 27% in a twin brother, whereas an affected boy has
a 9% to 15% chance of having a first-degree relative with hypo-
spadias (brother, father) (Asklund et al., 2007; Bauer et al., 1979).
A nationwide Danish registry of more than 1 million boys identified
5380 boys with hypospadias, implicating maternal and paternal
transmission and found an elevated risk for even third-degree relatives
(Schnack et al., 2008).
In spite of these findings, attempts at elucidating a “one-hit” gene
mutation for sporadic hypospadias have been disappointing, because
discrete chromosomal abnormalities are found in only up to 30%
of boys with hypospadias (Bouty et al., 2015). Detailed review of
the genetic basis of hypospadias is beyond the scope of this chapter
but warrants review (Bouty et al., 2015; Kalfa et al., 2011). Various
genes have been implicated and segregating them into broader
categories is feasible. Homeobox genes (HOX) and fibroblast growth
factor (FGF) have been identified as important members of the cascade
for penile development. Wilms tumor 1, SOX9, DMRT1, and GATA4
affect gonad development and have been associated with hypospadias.
Finally, mutations within the androgen cascade, androgen produc-
tion and reception, have been implicated. These include all ele-
ments of androgen production, including the luteinizing hormone
(LH) receptor gene, 5α-reductase gene, the androgen receptor,
and normal testicular development (Baskin et al., 2001; Fredell
et al., 2002; Silver and Russell, 1999).
Nearly 200 syndromes with known genetic causes have an associ-
ated hypospadias, although these represent a fraction of those boys
with idiopathic forms (Kalfa et al., 2011). The most commonly
encountered associations include WAGR (Wilms tumor [WT]), Aniridia
genitourinary abnormalities, and developmental delay [R]), Denys-
Drash syndrome (genitourinary malformations, renal failure, and
high risk for WT), and Smith-Lemli-Opitz syndrome (malformations
of the heart, lungs, kidneys, gastrointestinal tract, and genitalia)
(Bouty et al., 2015).
Since the 1970s, several large population studies across the world
have reported rising rates of hypospadias, some as high as 3% per
year (Chul Kim et al., 2011; Lund et al., 2009; Paulozzi, 1999; Toppari
et al., 2010). Others have disputed this trend, reporting stable
incidence and explaining perceived increased rates reported by others
as increased reporting of more mild variants (Bergman et al., 2015;
Chapter 45  Hypospadias 909
Lane et al., 2017). On the other hand, a study in Sweden over a
40-year period reported a rising trend in incidence for distal and
proximal variants (Nordenvall et al., 2014). Taken together, these
data suggest the rate is either stable or increasing. This fact,
combined with concerns for a concurrent rise in the rate of
cryptorchidism and a decrease in sperm quality, has created concern
for a common cause, likely an environmental source, to explain
these genital-specific findings.
The testicular dysgenesis syndrome (TDS) hypothesizes that
this series of increasingly frequent male reproductive developmental
disorders, including poor semen quality, increased risk of testicular
cancer, cryptorchidism, and hypospadias, are secondary to a fetal
exposure that directly affects the androgen cascade and genital
development (Skakkebaek et al., 2001). This constellation is thought
to be multifactorial in origin and includes environmental and genetic
insults resulting in testicular dysgenesis, originated from abnormal
testicular development in utero (Skakkebaek et al., 2001). Animal
studies have identified a direct correlation between pesticide
exposure, disruption of the androgen cascade, and abnormal
genital development (Gray and Kelce, 1996; Hayes et al., 2002;
Toppari et al., 1996). Potential causative agents under investigation
include pesticides (exposure in the workplace and in foods with
elevated levels of pesticides), hormones (birth control medication,
diethylstilbestrol [DES]), phthalates (found in plastics and hairspray),
and phytoestrogens consumed in a vegetarian diet (Baskin et al.,
2001; Kalfa et al., 2015). These environmental disrupting chemicals
(EDC), in theory, competitively inhibit androgen receptor binding
and disrupt external genital development, although efforts at establish-
ing a link in humans have been unsuccessful (Baskin et al., 2001;
Gaspari et al., 2012; Toppari et al., 1996). In a study performed in
southern France, after removing patients with hypospadias and
age-matched controls, the authors were able to identify a significant
risk for developing hypospadias after parental exposure to EDC,
specifically detergents, pesticides, and cosmetics, during pregnancy.
The authors found an increased risk for environmental exposure
alone, but this risk was higher when combined with occupational
exposures (Kalfa et al., 2015). Because these chemicals are ubiquitous,
it is challenging to avoid them to study them in isolation. Nevertheless,
understanding their role in hypospadias development is vital.
The anogenital distance (AGD) is measured from the center of
the anus to the most caudad midline border of the penoscrotal
junction. This distance, which is thought to be a measure of
disrupted genital development, is shorter in boys with suspected
exposure to EDC (Swan et al., 2005). Corroborating reports in
animal models, shorter AGD in males has been associated with
infertility, cryptorchidism, and hypospadias. In addition, shorter
AGD is directly proportional to hypospadias severity (Cox et al.,
2017; Eisenberg et al., 2011; Hsieh et al., 2012; Jain and Singal, 2013).
KEY POINTS
• There is an increased risk of hypospadias in births
resulting from assisted reproduction and monozygotic
twins.
• The cause of hypospadias is multifactorial, but placental
insufficiency and androgen disruption, in part via
environmental exposures, are potential culprits in what is
thought to be an increasing rate of hypospadias
development. Anogenital distance is a reflection of
disruption of this cascade and may correlate with
phenotype severity.
SURGICAL ASSESSMENT
The goals of surgical reconstruction in boys with hypospadias
include correction of penile curvature to ensure a long, straight
erection, advancement of the urethra to ensure normal passage
of urine and semen through the glans, and the creation of a
cosmetically pleasing penis. The surgeon must assess the potential
910 PART III  Pediatric Urology
long-term significance of the defect and have an objective discussion
with the boy’s parents as to whether a surgical repair should be
performed. In some cases, in which the penis is straight while erect
and the urethral meatus is distal enough to allow for urination
while standing, there may be limited benefit to a repair. Repair
should be accomplished with the minimum number of procedures
to ensure an adequate long-term result, extending into adulthood.
Achievement of this goal includes preparation of the family and
patient for the proper procedure, accurate anatomic assessment,
and an honest discussion about surgical outcomes and potential
complications.
Surgical timing is important. The timing of the repair should
balance the potential adverse psychological effects of surgery, the
anesthetic risk to the child, the degree of penile development that
will facilitate a successful repair, and wound healing differences as
boys age (Bermudez et al., 2011). Genital awareness begins to occur
around 18 months of age and progresses with advancing age (Schultz
et al., 1983). Boys undergoing earlier repair (usually before 12 months
of age) experienced less anxiety and have improved psychosexual
outcomes compared with boys undergoing repair at older ages
(Belman and Kass, 1982; Perlmutter et al., 2006). Boys operated on
at a younger ages may also experience fewer complications, a finding
that reinforces the need for early correction (Perlmutter et al., 2006).
Comparatively speaking, hypospadias surgery in adults may be
associated with a higher complication rate (Hensle et al., 2001).
Based on these studies, in 1996 the American Academy of Pediatrics
Section on Urology recommended that elective hypospadias repairs
occur between the ages of 6 and 12 months, within which a few
exceptions remain in our practice today (American Academy of
Pediatrics, 1996).
Parents frequently ask about the safety of anesthesia when
contemplating surgery in their infant son. Data in animals suggest
varying degrees of neurotoxicity in response to agents used for general
anesthesia (Jevtovic-Todorovic et al., 2003). Because studies that
include brain biopsies in animals are impossible to replicate in
human subjects, we must rely on longitudinal observational studies
in children. Children exposed to anesthesia (general vs. regional
block) for hernia repair had no detectable neurodevelopmental delays
at 2 years of age (Davidson et al., 2016). Although research continues,
based on current evidence it appears that there are few consequences
after a single anesthesia exposure with short-term follow-up (Sun
et al., 2016). As additional clinical studies are completed, we will
have more information with which to counsel families (Pinyavat
et al., 2016). As a result, the preoperative surgical consultation
with the boy’s parents must include a careful assessment of the
benefits of a surgical repair versus an age-based discussion of the
risk of general anesthesia.
The initial evaluation of boys with hypospadias requires a careful
history and physical examination. A focused history should identify
prior surgeries (such as circumcision, bladder, or scrotal or hypo-
spadias surgery), urinary tract infections (UTIs), voiding dysfunction,
the presence of penile curvature with erection, and any associated
medical diseases that may complicate care, such as asthma or a
bleeding diatheses. Physical exam should include a measurement
of penile length and careful groin examination to assess for unde-
scended testis on either side. Assessment of anogenital distance may
also prompt a workup for androgen insensitivity, which could have
implications during puberty.
Routine radiographic assessment with voiding cystourethrogram
(VCUG) or renal bladder ultrasound (RBUS) in patients with hypo-
spadias is not recommended. In a systematic analysis, the most
common associated anomalies included vesicoureteral reflux (VUR),
hydronephrosis, and renal position anomalies. These studies were
done before the era of routine fetal imaging and the findings were
of minimal clinical significance (Chariatte et al., 2013). Boys with
a history of other global systemic anomalies should be considered
for evaluation with a screening RBUS for the presence of associated
urinary tract abnormalities (Khuri et al., 1981). A prostatic utricle
may be present, more commonly in boys with proximal hypospadias,
and may pose a risk for voiding dysfunction or UTI because of
urine entrapment and stasis after the repair (Devine et al., 1980).
Surgeons may consider cystoscopy and removal of the utricle at
the time of the repair, but the fact that the majority of boys with
utricles are asymptomatic and the high risk for injury to the vas
deferens make this a rare indication for intervention (Hester and
Kogan, 2017).
The preoperative physical examination should assess severity,
which will guide surgical planning. The assessment includes the
degree of penile curvature, the location of the urethral meatus, the
quality of the urethral plate, and the quality of penile skin for
reconstruction (Merriman et al., 2013). Although it is difficult to
precisely determine the severity of the phenotype before surgery,
particularly the location of the meatus and degree of penile
curvature, the surgeon should try to assess the need for a two-stage
repair, which is best discussed with the family before the day of
surgery (Snodgrass et al., 2011). If hypospadias is encountered at
the time of newborn circumcision, careful evaluation of the severity
should be performed and circumcision aborted if there is curvature
or skin deficiency. As discussed earlier, boys with unilateral or bilateral
undescended testes, perineal hypospadias, or ambiguous genitalia
must undergo additional workup to rule out a disorder of sexual
differentiation, which occurs much more frequently in this setting
(Kaefer et al., 1999; Moreno-Garcia and Miranda, 2002; Rajfer and
Walsh, 1976; Rohatgi et al., 1987).
Preoperative Androgen Stimulation
The use of preoperative androgen stimulation in hypospadias surgery
is controversial. Bush et al. (2015) reported that a small glans size
(defined as a width of less than 14 mm) at the time of surgical
repair has been associated with increased risk for complications.
Although this group and others have since questioned the importance
of glans width and attributed higher complications to technical errors,
many surgeons remain concerned that urethroplasty in boys with
a small glans results in more complications (Faasse et al., 2016).
Preoperative androgen stimulation, in the form of DHT, human
chorionic gonadotrophin (hCG), or testosterone, can be used
in prepubertal boys to increase the size of the glans and the
penis (Asgari et al., 2015; Gearhart and Jeffs, 1987; Luo et al., 2003;
Nerli et al., 2009). Increasing glans size is thought to decrease the
amount of tension on the glansplasty and increase the amount
of tissue available for urethroplasty, potentially decreasing the
complication rate.
Concerns for androgen stimulation in these boys include aggressive
behavior, increased erections, skin discoloration, and secondary male
characteristics, all of which are transient and resolve spontaneously
within 6 months after the last dose (Asgari et al., 2015; Paiva et al.,
2016). Some surgeons prefer to avoid preoperative testosterone
because of a perceived increased risk for bleeding as a result of
increased angiogenesis. Others believe that poor wound healing may
follow androgen exposure (Ashcroft and Mills, 2002; Gilliver et al.,
2009). Therefore androgen use is controversial (Gorduza et al., 2011;
Menon et al., 2017). To complicate the discussion further, variable
degrees of androgen insensitivity may be present in boys with severe
proximal hypospadias, which may lead to abbreviated response to
androgen stimulation (Snodgrass et al., 2014a).
Several meta-analyses and systemic analyses have been per-
formed in an attempt to answer the question as to whether
androgen stimulation reduces or increases the complication rate
(Kaya et al., 2008; Netto et al., 2013; Wong and Braga, 2015).
Unfortunately, the reported data are variable, specifically the mode
of administration, severity of hypospadias, and lack of standardized
outcome assessment, precluding meaningful conclusion. Two recent
randomized trials have reported fewer complications after preoperative
testosterone administration (Asgari et al., 2015; Kaya et al., 2008).
A survey of pediatric urologists suggests that preoperative androgen
therapy was common before hypospadias repair in boys with proximal
hypospadias or in those with a small glans (Malik and Liu, 2014).
We use testosterone in some boys, particularly if the glans is small.
If we use it, we administer preoperative testosterone cypionate
intramuscularly (IM) 5 and 2 weeks before the planned surgery date
to boys with a glans width of less than 15 mm. We feel as though

the IM route of administration achieves a more consistent increase
in glans width and is tolerated well by children.
Intraoperative Assessment and Management
Use of a regional anesthetic is an important feature of a balanced
anesthetic in boys undergoing repair for hypospadias. Regional
blockade, with either a caudal or circumferential penile block, reduces
narcotic requirement during the procedure and improves pain control
in the immediate postoperative period (Morrison et al., 2014; Soliman
et al., 1978). A survey of practicing urologists found that regional
blocks are commonly used in hypospadias surgery, with more urolo-
gists preferring a caudal block (Kim et al., 2016). For longer proce-
dures, a second caudal dose can be administered before concluding
the case to prolong the analgesic effect in the postoperative period
(Chhibber et al., 1997; Samuel et al., 2002). Recently, use of a
pudendal nerve block has emerged as an alternative method of
regional blockade. The benefits of the pudendal block include a
wider coverage area than a penile block, including the ventral penis,
perineum, and scrotum. It also reduces the risk of the potential side
effects of a caudal block, such as lower extremity weakness, which
makes the use of a caudal block less attractive in older children
(Kendigelen et al., 2016). In addition, ketorolac (0.5 mg/kg IV), given
as a single dose 30 minutes before the conclusion of the procedure,
can be an effective adjunctive analgesic, depending upon the surgeon’s
comfort level with the concern for postoperative bleeding (Morrison
et al., 2014; Watcha et al., 1992).
Recent groups have released conflicting reports regarding the
secondary effects of caudal anesthesia and surgical hypospadias
repair. Kundra et al. (2012) noted a higher rate of fistula formation
in boys who underwent caudal compared with penile block (19.2%
vs. 0, P < .001). They documented a 27% increase in penile volume
10 minutes after the caudal was placed, theorizing that the penile
engorgement leads to increased swelling and compromised wound
healing. These authors also found penile block to provide better
pain control after distal hypospadias repair. Other single-institution
studies have confirmed similar concerns for an increased compli-
cation development when a caudal block is administered (Kim
et al., 2016; Saavedra-Belaunde et al., 2017; Taicher et al., 2017). On
the other hand, others have found no association between caudal
blockade and complications after hypospadias repair (Braga et al.,
2017; Zaidi et al., 2015). Both papers that refute the association
between complications and caudal anesthesia believe that boys
undergoing caudal had more severe forms of hypospadias and
therefore higher complication rates. All of these reports are
limited by marginal statistical power, randomization, and patient
numbers, leaving the question of an increased complication risk
with caudal use unanswered. We still routinely use caudal blocks
for hypospadias repair.
Surgical planning continues after the induction of anesthesia.
After antiseptic preparation and administration of intravenous
antibiotics, the genitalia are carefully examined to determine surgical
approach. We do not perform routine cystoscopy except in cases of
severe proximal hypospadias or reoperative procedures. Prostatic
utricles, if present, can make catheter placement difficult. Utricles
occur more frequently in proximal hypospadias. If a large utricle is
suspected, because of severity of the hypospadias or from prior
imaging, a cystoscopic examination may be useful to prepare for a
more complicated catheter placement (Ciftci et al., 1999).
Preoperative assessment of the hypospadias should proceed as
follows. With the boy asleep the preputial glanular adhesions are
released and the location of the urethral meatus, quality of ventral
shaft tissue, and degree of penile curvature are assessed. Sometimes
a proximal hypospadias masquerades as a distal hypospadias with
severe penile curvature and a hypoplastic ventral shaft skin (see Fig.
45.5). The surgeon must recognize this before incision to ensure
that the appropriate repair is performed. A circumscribing incision
is then made and the penis is degloved, partially or completely,
depending upon the degree of penile curvature. Care should be
taken to develop a mucosal collar, rotating inner shiny preputial
tissue from dorsolateral skin to the ventrum where it is lacking. This
Chapter 45  Hypospadias 911
will aid in ventral shaft skin coverage and yield an improved cosmetic
result (Firlit, 1987).
KEY POINTS
• The goals of hypospadias reconstruction include
achievement of a straight phallus with proper urinary and
sexual function, as well as a cosmetically pleasing
appearance.
• The conversation about the risk of hypospadias repair
should include the risks of early anesthesia exposure
versus the benefits of surgery at a younger age.
• Routine preoperative imaging is not recommended in boys
with nonsyndromic hypospadias.
• Preoperative androgen stimulation can transiently increase
the penile length and size for surgery. Its impact on
complication development is controversial.
• Physical examination in a patient with hypospadias may
reveal a ventral deficient prepuce, downward glans tilt,
deviation of the median penile raphe, ventral curvature,
scrotal encroachment onto the penile shaft, scrotal cleft,
and penile scrotal transposition. Each of these must be
considered for the surgical reconstruction; each has its
own degree of impact.
• Regional blockade is an important adjunct to the surgical
procedure. Caudal anesthesia can decrease the amount of
anesthetic delivered and does not appear to increase the
complication rate.
Assessment and Management of Ventral Penile Curvature
Penile curvature, or chordee, occurs in the presence or absence of
hypospadias. The degree of curvature is a major determinant in
the selection of a one-stage versus a two-stage repair. The decision
to treat curvature centers around the potential functional and
cosmetic concerns that males can have as they mature into adult-
hood. Assessment of males with untreated congenital curvature
or those with Peyronie disease suggests that as little as 20 to 30
degrees of ventral curvature may result in significant morbidity
for patients, including difficulties with intercourse and the patient’s
dissatisfaction of the appearance of the penis (Menon et al., 2016;
Walsh et al., 2013).
Curvature may result from shortened ventral skin, a short
urethra, or from intrinsic curvature of the erectile bodies. The
cause of curvature is extremely difficult to assess outside of the
operating room. Parents should be asked if they note a history of
curvature with erections or may even document this in their son
with photos, but the definitive assessment is done via artificial
erection in the operating room after the penis has been degloved.
In 1973 Devine and Horton developed a staging system based on
the degree of dysplasia of Buck fascia, dartos, and corpus spongiosum
(Devine and Horton, 1973). Donnahoo et al. (1998) expanded this
further, dividing curvature into a classification system of 4 groups:
skin tethering; dysgenetic Buck and dartos fascia; corporal dispropor-
tion; and congenitally short urethra. Each component can be present
to varying degrees, increasing the complexity of the defect and repair.
Although this system was used to describe chordee in boys without
hypospadias, its concepts are relevant to the curvature noted in
hypospadias.
An attempt to assess the degree of curvature should be made
in the operating room before skin incision. Passage of a catheter
or bougie à boule into the meatus will provide an assessment of
the overlying tissue, indicating the quality of the urethra and the
ventral skin (see Fig. 45.5). A circumscribing incision is made and
the penis is degloved as far as the penoscrotal junction, to excise
dysplastic dartos tissue. Artificial erection should then be performed,
typically with a tourniquet placed at the penoscrotal junction
and injection of sterile normal saline (Gittes and McLaughlin,
1974). Alternatively, in small boys the surgeon can compress
the corpora at the base of the penis to approximate an erection
912 PART III  Pediatric Urology

Penile degloving,
artificial erection

Resolution of
Persistent curvature
curvature

Mild ventral Severe ventral

Proceed with curvature, curvature,
urethroplasty <30 degrees ≥30 degrees

Corporal lengthening Corporal lengthening

Dorsal shortening
procedure, procedure,
procedure (plication)
2-stage repair 2-stage repair

Proceed with
urethroplasty

Fig. 45.7.  Algorithm for management of penile curvature. Once the penis is degloved, artificial erection
is performed. If the curvature is resolved, the urethroplasty can be completed. If there is persistent penile
curvature, we use a measurement of 30 degrees as the defining measurement for performing a dorsal
plication or a corporal lengthening procedure. If the surgeon is concerned about the quality of the ventral
shaft skin in spite of curvature of less than 30 degrees, a corporal lengthening procedure can be considered.

without injection. Curvature can occur at the base of the penis and
can be obscured by the presence of a tourniquet. Pharmacologic
erection with prostaglandin can be induced as an alternative to
saline injection (Kogan, 2000).
Penile curvature has traditionally been treated by the surgeon
in a subjective manner, typically categorized as mild (<30 degrees),
moderate (30–45 degrees), or severe (≥45 degrees). We, along with
several other institutions, use an orthopedic device called a goniometer
that functions as a protractor to objectively measure the degree of
curvature (Fig. 45.7). Our experience with this device is that the
degree of curvature was being underestimated when the “eyeball”
test alone was used. Electronic alternatives, which include apps for
use on phones and tablet devices, are starting to emerge as well.
Regardless of the approach we feel that the assessment should be
standardized.
The degree of penile curvature drives the decision for a single
versus two-stage repair (Fig. 45.8). Although no consensus exists
for treatment of specific degrees of curvature, most seem to agree
that a dorsal plication is sufficient for curvature less than 30 degrees
(Springer et al., 2011). If curvature is more severe than 30 degrees,
the next step would be to divide the urethra. Some severe cases of
ventral curvature have a meatus proximal to the point of maximal
bend so that urethral division is not necessary. Persistent curvature
greater than 30 degrees at this point would warrant a corporal
lengthening procedure, which in our hands requires transection of
the corpora spongiosum distal to the urethra or transection of the
urethra (Steven et al., 2013).
Dorsal shortening techniques, first described by Nesbit, involve
excising an ellipsoid segment of tunica albuginea and closing this
defect in a transverse orientation, shortening the dorsum of the
penis (Fig. 45.9) (Nesbit, 1965). Anatomic studies by Baskin
identified an area on the dorsal midline that was free of any
neurovascular tissue, allowing a modified plication by placement
of a single, midline plication suture through the tunica albuginea
to correct curvature (Baskin et al., 1998). Contrary to Baskin’s original
description, we prefer to incise the tunica albuginea in the midline
dorsally until the corporal tissue is visualized. The defect is then
closed to approximate the raw edges transversely in a Heineke-Mikulicz
fashion. This is performed using either a nonabsorbable or a long-
lasting absorbable suture. Advantages of the plication approach are

that it is a simpler procedure and does not limit subsequent ventral
penile shaft skin coverage options. Disadvantages include possible
injury to the neurovascular bundle (if the plication is performed off
the midline axis) and the potential for recurrence of curvature. Elevat-
ing the neurovascular bundle before placing plication sutures may
reduce the risk of neural injury (Dean et al., 2000). Plication may
be less effective in boys with more severe curvature, particularly
when the tunica albuginea is not incised. Of particular concern is
that recurrent curvature at puberty is more common after plication
compared with ventral lengthening (Ozkuvanci et al., 2017). In our
experience, plication works well for mild curvature (<15 degrees),
and many use this technique for up to 30 degrees of curvature (see
Fig. 45.7).
Several options exist for ventral corporal lengthening procedures
(Fig. 45.10). The basis for each procedure involves single or multiple
ventral incisions opposite the point of maximal curvature, releasing
the ventral tension on the tunica albuginea to straighten the
phallus. Multiple superficial incisions into, but not through, the
tunica albuginea of corpus cavernosa to release the tension on the
ventral surface of the penis are called “fairy cuts.” Several (1 to 3)
full-thickness incisions into the tunica albuginea without grafting
are referred to as transverse corporotomies and have been popularized
in the STAG technique, as described in the proximal hypospadias
section (see Fig. 45.10A) (Pippi Salle et al., 2016; Snodgrass and
Bush, 2017a). A single full-thickness corporotomy extending from
9 o’clock to 3 o’clock or until there is mild reverse hinging of the
penis, with subsequent grafting, is our preferred method for ventral
lengthening (Videos 45.1 and 45.2). Subsequent graft options include
A B
C
Chapter 45  Hypospadias 913
either autologous tissue (dermal graft [Devine and Horton, 1975],
tunica vaginal flap [Braga et al., 2007b] or graft) or nonautologous
tissue (AlloDerm, small intestinal submucosa (Braga et al., 2008;
Castellan et al., 2011; Fig. 45.10B). The dermal graft procedure is
depicted in Fig. 45.10C–F.
The choice of corporal lengthening approach is left to the
discretion of the surgeon: no series has formally compared the
three ventral lengthening procedures. Benefits of the corporal
lengthening procedure(s) include an increase in length of the penis
and improved results for severe curvature. Concerns include a longer
and more complicated procedure compared with plication, a limita-
tion in ventral skin coverage options, and a potential higher risk of
erectile dysfunction. In our experience, patients having undergone
pediatric penile lengthening procedures have not had issues in
adulthood with erectile function.
Curvature may worsen as these boys progress through puberty
and undergo more significant penile growth. Therefore it is
important to properly identify and correct curvature at the time
of the initial repair (Barbagli et al., 2006; Braga et al., 2008). Direct
comparison of the approaches to curvature described earlier is difficult
for two reasons: first, there is a lack of standardized definitions of
the degree of curvature (lack of objective instrumentation) and second,
there are few well-designed and executed comparative studies. In a
study of 51 patients with perineal hypospadias with severe ventral
curvature with a minimum follow-up of 5 years, 5 of 23 (21%) of
patients who underwent dorsal plication developed recurrent cur-
vature, compared with 0 of 11 patients who underwent a two-stage
repair with corporal grafting (Gershbaum et al., 2002). In a series
Fig. 45.8.  (A) A penis that is degloved to the peno-
scrotal junction and undergoing artificial erection.
Sterile saline is injected via a butterfly needle with
a tourniquet in place at the base of the penile shaft.
(B,C) The goniometer with a protractor-like element
present to precisely measure the degree of penile
curvature. Continued
914 PART III  Pediatric Urology
F displayed without a measuring device. (D–F courtesy
of 100 boys operated upon in Toronto, Braga found an increased
rate of recurrent penile curvature after dorsal shortening with
plication when compared with boys who had ventral lengthening
with corporal grafting (28% vs. 9%, P = .03) (Braga et al., 2008).
These results corroborate our clinical experience in that corporal
lengthening procedures better correct more severe curvature. This is
of particular importance as boys with persistent penile curvature
after primary repair, in pre- and postpubertal age groups are chal-
lenging to correct, sometimes requiring several staged procedures
to achieve an acceptable outcome. This makes it imperative that
penile curvature is properly diagnosed and corrected. Long-term
assessment is going to be essential for these boys to determine the
ideal approach to ensure lasting success and proper function.
Some have referred to boys with severe ventral curvature with
a normal urethra as having “congenital penile curvature” (Das-
kalopoulos et al., 1993; Devine and Horton, 1973; Kramer et al.,
1982). Biopsies of tunica albuginea tissue in these boys show that
the extracellular matrix and collagen fibers are misaligned and
disrupted (Darewicz et al., 2001). Reports have delineated a progressive
assessment and treatment algorithm that begins with penile degloving
and plication, whereas severe cases may require transection of the
urethra at the point of maximal bend, followed by one of the various
Fig. 45.8, cont’d. (D–F) The degree of penile curvature
Rafael Gonzalbez.)
grafting techniques (Bhat et al., 2008; Tang et al., 2007). This approach
requires a staged repair to reconnect the urethra after the chordee
has been corrected. We have developed a successful technique that
allows the normal urethra to be mobilized in situ, allowing a
corporotomy and graft in one operation, thus avoiding a secondary
procedure (Zaontz and Dean, 2016; Fig. 45.11). We believe this will
provide a more secure correction of the curvature while obviating
a second repair, an experience shared by others (McQuaid et al.,
2016). Recurrent curvature can occur in 2% to 15% of these boys,
particularly with plication technique, a reality that should be discussed
with parents before repair (Donnahoo et al., 1998; McQuaid et al.,
2016; Tang et al., 2007).
Finally, a number of children may have dorsal and/or lateral penile
curvature, which can be corrected most of the time with simple plication
techniques. However, in rare instances of severe dorsal and or lateral
curvature, a patch graft may be necessary as well as the potential need
for urethral and/or neurovascular bundle mobilization. Others have
noted a higher complication rate with plication in boys with lateral,
dorsal, or spiral anomalies that result in a curved penis (McQuaid
et al., 2016). These lateral areas of the corpora are theorized to have
a thinner albuginea when compared with the dorsum of the penis,
limiting the relative effectiveness of plication techniques.
 Chapter 45  Hypospadias 915

Nesbit
(excision)

Plication

Heineke-Mikulitz
technique (incision)
B Midline dorsal plication for penile curvature
A in a nerve-free zone

Fig. 45.9.  (A) Description of three different plication techniques to correct penile curvature. Nesbit plication
technique: an excisional biopsy of dorsal tunica albuginea is made opposite the point of maximal curvature.
In a similar fashion, others have described performing a plication without incising the tunica albuginea. The
Heineke-Mikulitz technique involves a vertical incision in the tunica albuginea and horizontal closure of the
defect to shorten the dorsum of the penis. For each of these techniques the incisions and/or plications
are made in parallel on either side of the neurovascular bundle, only one side is displayed for simplicity.
(B) The midline (Baskin) plication technique, similar to the Heineke-Mikulitz approach. A single midline
plication is performed in the avascular plane of the penis. (A, From Nyirady P: Management of congenital
penile curvature. J Urol 179:1495–1498, 2008; B, From Baskin LS: Anatomical studies of hypospadias.
J Urol 160:1108–1115, 1998.)

B Vaginalis flap Vaginalis graft Dermal graft

A C

Fig. 45.10.  (A) The multiple ventral tunica albuginea corporotomies without grafting as described in the
staged tubularized autograft (STAG) repair. Arrows are pointing to the transverse corporotomies, which
are made full thickness through the albuginea; in this particular example three incisions have been made.
(B) Three approaches to ventral penile lengthening. A tunica vaginalis flap, tunica vaginalis graft, and
dermal graft are depicted. Each approach is performed after division of the urethral plate and incision of
the tunica albuginea. (C–F) show four live surgical images of a dermal graft. First a single, full-thickness
transverse incision is made in the tunica albuginea, opposite the point of maximal curvature (C).
Continued
916 PART III  Pediatric Urology

Fig. 45.10, cont’d. A dermal graft is then measured 20% larger than the defect in the tunica to allow
for graft contracture without shortening the penis (D). This is then sutured in place in a watertight fashion
(E). Repeat artificial erection is performed to confirm that penile curvature is fully corrected (F). (A, From
Snodgrass W: Staged tubularized autograft repair for primary proximal hypospadias. J Urol 198:680–686,
2017. B, From Braga LH: Outcome analysis of severe chordee correction using tunica vaginalis as a flap
in boys with proximal hypospadias. J Urol 178(suppl4):1693–1697, 2007.)

no such benefit (Bush et al., 2013b; Ziada et al., 2011). A systematic

KEY POINTS
• Curvature less than 30 degrees can be straightened by
dorsal plication without clinically apparent shortening of
the penis.
• Objective measurement of penile curvature, as identified
by artificial erection after penile degloving, is a key
element of the surgical procedure. Curvature must be
assessed and properly corrected.
• Transection of the urethral plate and/or ventral corporal
grafting are reserved for cases with curvature greater than
30 degrees after degloving and dartos dissection.
• Improperly corrected penile curvature poses a significant
morbidity to the patient, often necessitating several
procedures to achieve a straight penis.
General Considerations of Surgical Repair
Aside from the previously mentioned psychological benefits of
operating on a young infant, the literature is split on the potential
impact that age has on surgical outcomes. Hypospadias repair in
boys younger than 6 months of age has been reported as more
successful when compared with surgery on older boys (Perlmutter
et al., 2006; Yildiz et al., 2013). However, two other studies showed
review comparing outcomes in North America, Europe, and China,
including more than 16,000 patients in 113 manuscripts, identified
fewer complications in younger boys at the time of primary repair,
and recommended correction by the age of 18 months (Lu et al.,
2012). A study by Weber et al. that examined the psychological effects
of hypospadias surgery before and after 18 months of age found
that later surgery had no negative impact on the long-term emotional
impact to the patient (Weber et al., 2009). With this in mind, we
recommend beginning the surgical process with the first stage at 6
months. This will likely result in adherence to the AAP-recommended
age range of 6 to 18 months for the surgical reconstruction or
reconstructions. Hensle et al. (2001) reported that adult hypospadias
surgery, for primary and reoperative cases, is associated with a
higher than anticipated complication rate, in spite of nearly all
patients undergoing a more distal repair. This is in contrast to
subsequent studies, which suggest no increased risk of repair in
adult patients (Adayener and Akyol, 2006; Snodgrass et al., 2014b).
Antibiotics are often administered in the perioperative period,
preoperatively and as prophylaxis, because of concerns for UTI
and/or wound infection (Hsieh et al., 2011). Alternatively, prophy-
lactic antibiotics can be administered at surgery and then at the time
of stent removal. Given the shift in concern for overadministration
of antibiotics and its role in developing resistant organisms, adverse
drug reactions, and changes to the microbiome, several studies have
assessed the need for antibiotics in hypospadias repair. The first
 Chapter 45  Hypospadias 917

A B C

D E

F G

Fig. 45.11.  Management of a boy with ventral chordee without hypospadias. (A) Ventral penile curvature
is depicted with an erection. Note that the boy is already circumcised and that the urethral meatus is in
a normal position. (B) The penis is degloved, exposing the corpus spongiosum, which is normal. (C,D)
The urethra is mobilized off of the corporal bodies, and a transverse incision is made in the tunica. This
corrects the penile curvature; therefore the urethra is not divided. (E) A dermal graft is placed into the
defect in the tunica albuginea. (F) the urethra is released and the dermal graft is in place. (G) Twelve
months after repair, the penis is straight with no evidence of persistent chordee.
918 PART III  Pediatric Urology
studies elucidating the role of prophylaxis with an indwelling stent
suggested a decrease in the UTI rate, although these boys were
asymptomatic and positive cultures represented asymptomatic
bacteriuria instead of a true UTI (Shohet et al., 1983; Sugar and
Firlit, 1988). A randomized study by Meir and Livne (2004) of
patients undergoing the tubularized, incised plate (TIP) procedure
found that those who received continuous antibiotic prophylaxis
(CAP) were less likely to develop a complicated UTI (3 vs. 12, P <
.05) and complications (5/52 [9.6%] vs. 13/49 [26.5%], P < .05).
Others have found CAP to have no impact on complication develop-
ment, either surgically or infection related, whereas others have even
questioned the need for preoperative antibiotics (Baillargeon et al.,
2014; Kanaroglou et al., 2013; Smith et al., 2017; Zeiai et al., 2016).
Because postoperative UTI or wound infection is so rare after
hypospadias repair, this controversy will not likely be sorted out
without a large multicenter trial. Our approach varies by surgeon,
ranging from daily prophylaxis for the duration of the indwelling
urethral stent to a 3-day course starting the day before stent removal.
Sutures used in hypospadias repair must provide sufficient strength
while the urethroplasty heals yet should dissolve before suture tracks
can epithelialize and lead to fistulous tracts or suture sinuses.
Moreover, the suture material should elicit minimal inflammatory
response. Hypospadias-specific studies comparing absorbable sutures
are few and unfortunately inconclusive. Bartone et al. (1987) found
chromic to be superior to polydioxanone (PDS) because of concerns
for wound infection, inflammatory reaction, and poor wound healing.
In a similar study, DiSandro and Palmer (1996) found the use of
rapidly absorbing sutures such as chromic and polyglycolic acid
(Vicryl) had a significantly lower complication rate in urethroplasty
when compared with the more slowly absorbing PDS. More recent
series have also suggested a lower complication rate with Vicryl
suture as opposed to monofilament sutures (Snodgrass and Yucel,
2007; Uygur et al., 1998). Regardless of the suture used, modern
techniques require use of absorbable, microsurgical-size suture for
fine tissue approximation.
Given the complexity of hypospadias repair, even for distal
hypospadias, it is not surprising that a learning curve exists. Higher
surgeon volume has been directly correlated to lower complication
rates (Horowitz and Salzhauer, 2006; Lee et al., 2013; Titley and
Bracka, 1998). Studies comparing experienced surgeons to less
experienced surgeons have shown a decreased complication rate
for more experienced surgeons (Snyder et al., 2005a). In a similar
fashion, comparison of individual surgeon results indicates that
time from completion of fellowship training and increase in case
volume significantly reduce the incidence of surgical complications
(Horowitz and Salzhauer, 2006). A large population study of 108
surgeons performing distal hypospadias repair found that higher
yearly case volume led to a significant decrease in postop complica-
tions (Lee et al., 2013).
The use of hair-bearing skin for urethroplasty is discouraged
because postpubertal hair growth renders the neourethra suscep-
tible to stone formation and recurrent UTIs. Surgical technique is
as important a factor for outcomes as surgical approach. Gentle
tissue handling prevents crush injury and preserves blood supply
to tissues, which is critical to predictable wound healing. It is
imperative that the wounds are closed without tension. Wounds
closed under pressure reduce capillary flow and promote ischemia.
Ischemia results in fibrosis and contraction of tissues, predisposes
the wound to infection, and potentiates the breakdown of suture
lines. Tissue trauma can be minimized by the use of fine forceps, skin
hooks, and stay sutures. Intraoperative magnification facilitates this
process and helps to identify delicate blood supply and proper tissue
approximation. Flaps must be mobilized generously to minimize
tension on suture lines and to prevent ischemia. Subcuticular neo-
urethral approximation with epithelial inversion has been shown to
decrease complications when compared with full-thickness suturing,
as has dual-layer neourethral closure (Cimador et al., 2004; Ulman
et al., 1997).
Although use of healthy and well-vascularized tissue is vital,
the use of second-layer vascularized tissue coverage over the
neourethral repair further reinforces blood supply. The use of
multiple overlapping tissue layers is important in the reduction of
complications so long as the additional layers do not compromise
capillary flow. Dartos interposition flaps increasing the vascular-
ized layers superficial to the urethral closure help reduce fistula
formation after TIP repair (Savanelli et al., 2007). This can be done
in multiple ways, including the preputial de-epithelialized dartos
flap, adjacent de-epithelialized skin flaps in the absence of preputial
tissue, tunica vaginalis, and/or the use of the corpus spongiosum
for coverage (Fig. 45.12 and Video 45.3; Basavaraju and Balaji,
2017; Chatterjee et al., 2004; Churchill et al., 1996; Smith, 1973;
Snow et al., 1995). Belman (1988) initially described a technique of
harvesting a dartos flap after transposing Byars flaps around to the
ventrum (Video 45.4). The point where the excess prepuce reaches
the proximal edge of the mucosal collar is measured, and a transverse
incision is made (see Fig. 45.12). The harvested vascularized tissue
is draped over the entire neourethral repair and secured laterally in
a running fashion to offset suture lines.
Finally, careful hemostasis should be maintained with the
judicious use of pinpoint cautery and a compressive bio-occlusive
dressing. Hematoma formation can impede flap healing, resulting
in tissue ischemia, and can compromise the repair. The dressing can
be formed from an adhesive membrane such as Tegaderm, which is
applied circumferentially from the level of the glans to the penoscrotal
junction. The glans must be included in the dressing to avoid excessive
swelling. Care must be taken to provide even coverage to prevent a
constricting ring at the proximal extent of the dressing. Alternatively, a
“sandwich” dressing, which provides gentle compression for the first
72 hours postsurgery, can be equally effective (Fig. 45.13). Once the
dressing is removed, parents should be counseled that a significant
amount of edema and ecchymosis may be present. After dressing
removal, application of topical petroleum jelly may help prevent
eschar formation and trauma to the tissue as it meets the diaper to
further facilitate the healing process.
KEY POINTS
• Higher surgical volume seems to correlate with a lower
complication rate in hypospadias repair.
• Nonoverlapping tissue layers, tension-free tissue closure,
and barrier layers with well-vascularized tissue increase the
success of a repair.
Distal Hypospadias Repair
The literature on hypospadias repairs can be overwhelming, because
more than 200 techniques have been described, although the majority
are a variation of an established approach. The basic tenets of repair
include identification and correction of penile curvature, urethral
advancement either with or without urethroplasty, and glansplasty.
The skin is then closed, with or without preputioplasty. To simplify
the technical variation, urethroplasty techniques can be broadly
categorized as those using flaps of local genital skin, those using
flaps or grafts of preputial or extragenital tissue, and urethral tubu-
larization. Among the repairs outlined later, a survey of pediatric
urologists identified the TIP technique was used more than 90% of
the time for distal hypospadias repairs and 80% of the time for
midshaft repairs (Cook et al., 2005).
ADVANCEMENT PROCEDURES
Advancement procedures do not require tubularization of the urethral
plate and are typically reserved for only the most distal, glanular meatus
with minimal to no penile curvature. Urethromeatoplasty uses the
Heineke-Mikulicz principle, in which one makes a longitudinal, vertical
incision in the ectopic meatus, the edges of which are then closed in
a horizontal fashion (Fig. 45.14A). This flattens the posterior urethral
plate and achieves a cosmetically normal meatus. This technique is
particularly useful in the setting of a stenotic, distal meatus with an
associated blind ending pit in the center of a closed glans.
 Chapter 45  Hypospadias 919

C D

G
F
Fig. 45.12.  (A) Cartoon depiction of the de-epithelialized Belman flap. Redundant foreskin is incised
dorsally in the midline to the point of yielding a proper skin fit with a circumcision. Byars flaps are rotated
ventrally to the midline along either lateral side of the penis. (B–D) represent live surgical pictures depicting
the technique. (B) The lateral borders of the redundant skin are marked. (C) The epithelium of the skin is
incised and then removed, taking care to preserve the well-vascularized dartos layer. (D) The dartos is
then secured as an additional layer of coverage over the urethroplasty, with an offset suture line flanking
either side of the urethral closure. (E) Use of bilateral flaps to cover a longer urethroplasty. (F) Depiction
of securing the flap in place. (G) Tunica vaginalis flap is harvested as a barrier layer over urethroplasty.
Mobilization from the spermatic cord to the external ring prevents subsequent tethering of the testicle
and/or penis. (From Belman AB: De-epithelialized skin flap coverage in hypospadias repair. J Urol 140[5
Pt 2]:1273–1276, 1988.)
920 PART III  Pediatric Urology

A B

D E

Fig. 45.13.  (A,B) Photos of an older type of dressing that includes a Foley catheter in the urethra and
subsequently applied extensive amount of Coban to secure the penis in place. (C) A sandwich dressing,
which includes a 6-Fr Kendal stent for the urethra covered by two layers of Tegaderm. (D,E) Simple
Tegaderm dressing without a stent and another patient with a drip stent as well.

Duckett (1981) pioneered what was to become one of the most
widely used procedures to correct glanular hypospadias, the meatal
advancement glanuloplasty (MAGPI) (Video 45.5). The main objective
of this procedure is to advance the meatus distally without formally
tubularizing the urethra (Zaontz and Dean, 2002; see Fig. 45.14A).
We have since modified the original description (Fig. 45.14B).
Although once used for many degrees of distal hypospadias, the
best candidates have a meatus located on the glans. Application of
this technique at more proximal locations subjects the repair to
undue tension, thus increasing the risk of developing a retrusive
meatus and/or an abnormal, flattened distal glans. The evolution
of this technique from widespread use to a more selective applica-
tion reveals the critical role that anatomic assessment and proper
approach selection plays in optimizing outcomes.
In 1991 Decter developed the M inverted V glansplasty (MIV), a
repair for proximal glanular or a coronal meatus without urethral
tubularization, designed to prevent the meatal retraction and abnormal
glans shape sometimes seen in the MAGPI procedure (Decter, 1991;
Fig. 45.15; Video 45.6). This technique is used when the parameatal
skin is sufficiently compliant to allow tension-free distal mobiliza-
tion of the meatus and the glans should be of sufficient size for
glanuloplasty. In addition, the glans configuration (with ventral
medial upward angulation) should be conducive for successful
implementation of the MIV (Fig. 45.15B). The glans wings dissection
is usually, although not always, less deep than with tubularization
techniques. This procedure advances the ventral urethral lip distally
with well-apposed glanular tissue proximal to the meatus, whereas
a cosmetically pleasing mucosal collar completes the repair.
The urethral advancement procedure, or sleeve technique, incor-
porates mobilization of the distal urethra by incising the hypospadias
meatus at the glans (Koff, 1981). In some instances aggressive
mobilization is required. When done in an appropriately selected
patient, the mobilization provides increased length to advance the
urethra in a tension-free manner into the distal most glans, over
which glansplasty is performed (Fig. 45.16). Concerns for this
approach include the potential for devascularization of the
mobilized urethra with subsequent urethral stricture and possible
retraction of the urethral meatus because of tension caused by
the advancement.
TUBULARIZATION TECHNIQUES
Tubularization techniques incorporate a formal urethroplasty to
advance the meatus into a glanular location. These techniques
are used in the setting of a more proximal meatus not amenable
to advancement, particularly in the setting of curvature that is
mild or resolves after penile degloving. These procedures require
an astute assessment of the anatomy and application in the appropri-
ate setting, specifically based on the quality of the glans, urethral
plate, and periurethral tissue. These anatomic factors can have a big
impact on the success of the repair.
The glans approximation procedure (GAP) is a surgical technique
designed specifically for patients with proximal glanular/coronal
hypospadias who have a wide, deep glanular groove and a noncompli-
ant or “fish-mouth” meatus, which is often present in the MIP variant
(Zaontz, 1989; see Fig. 45.3). The pyramid procedure is essentially
the GAP with an intact prepuce (Duckett and Keating, 1989). The
glans groove is typically deep and wide enough that if one were
to pinch the glans wings together, a normal cosmetic appearance
 Chapter 45  Hypospadias 921

A
B C

a
b c
b c

D E F G

I J
H
Fig. 45.14.  The meatal advancement glanuloplasty (MAGPI) procedure. (A) Heineke-Mikulicz rearrangement
with longitudinal incision is made and then closed in a horizontal fashion. (B) Mucosal collar is marked
with holding sutures placed. (C) Penis partially degloved. (D) Holding suture placement (a, b, c). (E) Distal
retraction to provide meatal advancement (a), vs. proximal retraction (b, c). (F) Glanular spongiosum exposed
after excision of glans edges. (G,H) Alternatively marker used to inscribe V along glanular edges and
V-shaped tissue segment excised. (I) Approximation of the glans in two layers. (J) Completed repair with
mucosal collar. (From Zaontz MR, Dean GE: Glanular hypospadias repair. Urol Clin N Am 29:292, 2002.)

would result. This technique is a modification of the Thiersch-
Duplay repair originally described without the extensive mobiliza-
tion of the glans wings (Fig. 45.17). This has since been modified
to deeper glans incision and glans wing mobilization, and in our
hands, incorporates a two-layer running neourethral mobilization
and dartos flap barrier layer. As with all reconstructive procedures
for hypospadias, attention must be given to the posterior urethral
plate to determine the presence of a transverse cleft separating
the hypospadias meatus from the distal glans groove. It is critical
that the posterior urethral plate be flat to avoid urine flow deflection
as it exits the urethra.
Most operations performed in the modern era are modifications
of previously developed procedures. The Thiersch Duplay (TD) repair,
developed nearly 140 years ago by Thiersch and later Duplay, uses
the brilliant concept of urethra tubularization of local tissue distal
to the ectopic meatus (Duplay, 1874; Thiersch, 1869). They performed
922 PART III  Pediatric Urology
A B
C D E Fig. 45.16.  Urethral advancement procedure. (A) Circumferential inscription
Fig. 45.15.  M inverted V glansplasty (MIV) procedure. (A) M-shaped markings
made, including mucosal collar followed by incision. (B) Holding suture used
to retract meatal tip distally followed by proximal dissection of the ventral
tissue. The skin is typically hypoplastic on the distal penile shaft; thus care
must be taken to not violate the urethra during this dissection. If thin but not
violated, additional barrier layers can be added to reinforce the repair. (C)
Urethromeatoplasty performed followed by ventral approximation of the glans.
(D) Two-layer glans closure over the urethral advancement. (E) Completed
repair including mucosal collar. (From Zaontz MR, Dean GE: Glanular hypo-
spadias repair. Urol Clin N Am 29:292, 2002.)
their repair by creating a U-shaped incision made of vascularized
skin from the penile shaft, advancing the meatus to the coronal
margin. This was later modified for distal hypospadias by covering
the repair with a second layer of preputial skin (Allen and Spence,
1968; Belman, 1988). This technique was originally described by
Smith and involves de-epithelialization of redundant preputial skin
and securing across the repair to provide supplemental blood supply
(Smith, 1973). The natural progression was to extend these U-incisions
into the distal glans, tubularizing the glans itself over the repair,
creating a more cosmetically appealing meatus at the tip of the penis
(Firlit, 1987; King, 1970; Videos 45.7 and 45.8).
The TD procedure requires a glans of adequate width to
allow for an appropriately sized neourethral channel, at least
one covering “waterproofing” layer, and glans wings that can
loosely approximate over the repair. This technique, which we use
frequently in boys with distal hypospadias, is particularly successful
if the surgeon adheres to certain parameters (see Fig. 45.17). Parallel
incisions are made lateral to the glans groove to equal 12 Fr in
diameter; the glans wings must be deeply and widely mobilized
to allow for tension-free coverage. Neourethral reconstruction is
performed under optical magnification using a double running
subcuticular suture. A de-epithelialized pedicle flap is procured
from the preputial tissue or the more proximal shaft, should the
infant be circumcised, and placed over the entire neourethral
repair (Belman and Kass, 1982; Smith, 1973). Should the repair
A B C
D E F
of meatus and mucosal collar. (B) Degloving of penis followed by mobilization
of meatus and urethra. Care is taken to preserve well-perfused urethral tissue,
with excision of nonviable segment. (C) Excision of portion of midline spon-
giosum to allow for proper seating of the urethra in the glans. (D) Proximal
deep urethral tissue coverage with glansplasty and approximation of spon-
giosum. (E) Approximation of superficial glans tissue. (F) Completed repair
including mucosal collar. (From Zaontz MR, Dean GE: Glanular hypospadias
repair. Urol Clin N Am 29:292, 2002.)
be more proximal, a double dartos flap can be procured from the
dorsal prepuce with one flap running distal and one proximal
(see Fig. 45.17). The glans wings are approximated in two layers
(spongiosum and then epithelium), followed by the mucosal collar
to complete the circumcision defect.
The TIP procedure, a modification of the TD, is a popular
hypospadias surgical technique used worldwide (Cook et al., 2005;
Snodgrass, 1994). Rich et al. (1989) noted that the fish-mouth meatal
appearance of Mathieu hypospadias repairs or island flap/onlay
repairs could be avoided if the posterior urethral plate were incised
or hinged, thereby creating a more natural slitlike meatus. The TIP
procedure modified the classic TD procedure by incorporating the
posterior urethral plate incision as described by Rich. The midline
incision of the posterior urethral plate is designed to improve
urethral width to thus allow tubularization of the neourethra in
TD fashion, particularly when anatomy would otherwise preclude
a tension-free anastomosis with only local tissue. Compared with
the superficial incision of a dorsal urethral cleft, the TIP incision
is deep and carried to the level just superficial to the corporal
bodies, particularly in the setting of a narrow plate or flattened
glans. Surgeons must resist the urge to close the urethroplasty
too far distally, because this can increase the risk of meatal stenosis.
Snodgrass stops his urethral closure 2 to 3 mm proximal to the
intended meatal location (Fig. 45.18). In the original operative
description of the TIP procedure, parallel incisions are first made at
the lateral edges of the urethral plate followed by a midline longi-
tudinal incision to develop enough width to allow for successful
 Chapter 45  Hypospadias 923

1 2

1 2

A B
Fig. 45.17.  Modified Thiersch-Duplay repair. (A) U-shaped marking line extending down inner prepuce,
which is preserved for use in closure of mucosal collar. Inset shows narrow urethral plate, which is
conducive to TIP modification (1) incising urethral plate to determine width (2) and lateral marking lines
to achieve adequate neourethral lumen (3). (B) Tubularization of neourethra. Inset shows first running layer
(1) and Lembert second running layer (2).

A B D

E F G
Fig. 45.18.  Distal tubularized incised plate (TIP) repair. (A) Circumscribing skin incision. (B) Incision along
the visible junction of the glans wings to the urethral plate. (C) Deep, midline incision of the urethral plate
extending to the underlying corpora. (D) Tubularizing the urethral plate from distally to proximally. Note
that the first stitch is about 3 mm proximal to the end of the urethral plate, creating an oval opening.
(E) The neourethra is covered with a dartos flap. (F) Glansplasty creating the neomeatus and continuing
down to the corona. (G) Repair and circumcision completed. (Modified from Snodgrass WT: Snodgrass
technique for hypospadias repair. BJU Int 95:683–693, 2005.)
924 PART III  Pediatric Urology

A
B

C D

Fig. 45.19.  (A,B) Parameatal skin flap is marked and incised. Incisions are extending into the glans. (C)
Circumscribing incision is made. (D) Meatal flap is “flipped” onto the urethral plate, with a running suture
line running parallel to either side of the late. Once the urethra is closed, glansplasty is performed.

urethroplasty. We have found that no two urethral plates are the
same, and obtaining a consistent width throughout the urethroplasty
cannot always be achieved in our hands with the TIP. In some cases
a narrow plate increases the risk for meatal or urethral stenosis. An
experimental animal study demonstrated variable widths upon incising
the posterior urethral plate with the additional finding of contraction
of the initial incision ultimately leading to a narrower overall width
(Eassa et al., 2011). Hence we approach the TIP by first incising the
posterior urethral plate, measuring the width obtained, and then
making parallel glanular incisions to obtain 12 mm in total width
for adequate lumenal diameter. The remainder of the repair proceeds
as described for Thiersch Duplay. In our experience, we limit posterior
urethral plate incision to situations in which a transverse cleft is
present between the meatus and glanular groove to smooth out and
flatten the posterior urethral plate to avoid turbulent flow during
voiding. If we consider using the concept of the TIP repair for
distal hypospadias, we frequently add an inner preputial graft
that we believe protects against future narrowing of the recon-
structed distal urethra (Kolon and Gonzales, 2000).
The flip-flap technique (Mathieu) is a procedure in which a
parameatal-based flap proximal to the meatus is developed, advanced
(flipped) onto the deficient urethral plate to augment it, and then
closed distally (Devine and Horton, 1973; Mustarde, 1965). Initial
incisions are made parallel to the urethral plate and extended
proximally for an equal distance such that the raised flap can extend
into the distal glans (Fig. 45.19). Theoretical concerns exist regarding
the vasculature of the employed flap; if the base of the flap is not
sufficiently wide, the blood supply may be compromised, increasing
the risk of fistula and stenosis. Others have had concerns for an
abnormal, fish-mouth appearance to the meatus. This technique
has been further modified to the so-called slitlike adjusted Mathieu
(SLAM) procedure with good outcomes, including an improved
appearance to the meatus (Hadidi, 2012; Fig. 45.20).
The onlay island flap (OIF) procedure is particularly useful in
the setting of a narrow urethral plate. To augment the plate, an
island flap of dorsal inner preputial skin is harvested, transposed
ventrally, and sutured onto the plate to close the urethral defect
(Fig. 45.21). Although a popular repair in the 1990s, after introduction
of the simpler TIP procedure, this repair fell out of favor except in
a few centers. Many surgeons complain that they have difficulty
harvesting a consistent flap that will not develop a diverticulum.
We have found that a vertical incision on the ventrum of the

A B
C D E have since modified the technique from its original description (Asopa
F G
Fig. 45.20.  The slitlike adjusted Mathieu (SLAM) technique. (A) A U-shaped
incision is marked on the ventral penile shaft. Within the glans, the incisions
are made parallel to each other and long the true urethral plate to develop
proper glans wings. Proximally, the incisions diverge to create a wide-based
flap. (B,C) The flap is mobilized, taking care to preserve spongiosal tissue on
the flap. (D) The edges of the flap are sutured in place on either side of the
urethral plate. (E) V-shaped excision of the flap is performed to achieve a slitlike
meatus. (F,G) Glansplasty is performed. (From Hadidi AT: The slit-like adjusted
Mathieu technique for distal hypospadias. J Pediatr Surg 47[3]:617–623, 2012.)
penile shaft to the penoscrotal junction facilitates flattening of the
dorsal shaft skin, which makes the blood supply to the flap more
evident. The actual harvest begins dorsally at the penopubic junction
and progresses distally. This approach facilitates the procurement
of a consistent flap. Concerns for this approach compared with the
TD or TIP procedures include a fish-mouth or rounded meatus as
compared with a more cosmetic oval contour with the TD and/or
TIP unless Rich’s modification is employed (Rich et al., 1989). After
the OIF procedure, these boys are at higher risk for development of a
urethral diverticulum because of the elasticity of the inner preputial
skin present in the island flap. On the other hand, our impression
is that meatal or urethral stenosis is less common in this repair.
Proximal Hypospadias
Our appreciation for the importance of careful surgical treatment
of curvature has driven a dramatic evolution in our approach to
Chapter 45  Hypospadias 925
KEY POINT
• Many options exist for distal hypospadias repair, many of
which are technical variants of each other. Surgeons
should use a technique that they feel comfortable with
and yields a high success rate.
the management of proximal hypospadias. Concerns for recurrence
of penile curvature in severe hypospadias when managed with
dorsal plication alone have shifted the pendulum toward more
aggressive corporal lengthening procedures, and therefore, in the
hands of most surgeons, a multistage repair.
One-Stage Hypospadias Repair
In general, the one-stage proximal hypospadias repair involves
dorsal plication to correct ventral penile curvature in conjunction
with several different urethroplasty techniques. These can be
stratified according to the tissue used in the repair, specifically
preputial skin versus local skin versus buccal graft.
In 1980 Duckett harvested a preputial island flap, tubularized it
into a substitution urethroplasty, and created what is called a transverse
preputial island flap (TPIF) or the Duckett tube (Fig. 45.22). We
et al., 1971; Video 45.9). Rather than tubularizing the flap before
securing it to the penile shaft, we now anchor the left side of the
flap to the ventral surface of the penile shaft, just to the left of
midline. The flap is then closed in the midline, with interrupted
Lembert sutures to reduce the risk of exposing epithelium beneath
the repair. Both rows of sutures are carried into the midglans.
The pedicle of the flap is carefully placed over the suture line.
The glans wings are then brought over the urethroplasty and
vascular flap. This approach anchors the base of the tubularized
flap to the distal extent of the native urethra (Fig. 45.22E). This
provides a point of tension that will guide the subsequent tubulariza-
tion along the longitudinal axis of the neourethra, removing laxity
to reduce the risk of diverticulum development. The tube is closed
in a two-layer fashion, maintaining an even caliber throughout its
course (Fig. 45.22F). This approach is applicable in the circumstance
when the curvature is severe but corrected after skin dissection and
division of the urethral plate. The amount of tissue placed within
the glans can hamper glans closure and result in an oval appearance
to the meatus, as opposed to a slitlike meatus seen with other
repairs, limiting its use. A higher complication rate with the TPIF
resulted in Duckett preferentially using the OIF procedure, even for
severe hypospadias (Duckett, 1995).
The OIF procedure was described in the previous section for
distal hypospadias but evolved from Duckett’s experience with the
TPIF (see Fig. 45.22). It is a versatile technique and can be used for
more proximal variants. Indications include boys whose curvature
is corrected after penile degloving and does not require division
of a relatively thin urethral plate that otherwise could not be
tubularized. Although at one point used at CHOP in more than
90% of hypospadias repairs, it has since been replaced by other
procedures for severe hypospadias and is now most commonly
used for midshaft hypospadias repair in which release of skin
and division of urethral plate results in correction of curvature.
Technical aspects of the repair are reviewed in Figs. 45.21 and
45.22. After degloving, incisions along the urethral plate are made
in parallel through the glans (see Fig. 45.22A, Video 45.8). Dorsal
skin dissection should be in the avascular plane above Buck fascia,
preserving the vascular pedicle to the preputial skin. The prepu-
tial skin flap is marked with stay sutures (see Fig. 45.22B), then
transposed ventrally to align over and to the urethral plate with a
full-thickness suture (see Fig. 45.21C). The native urethral meatus
is spatulated (see Fig. 45.21). The combined width of the urethral
plate and flap should be 12 mm or less, and is trimmed accordingly,
in particular to narrow the proximal flap to prevent a diverticulum
at the junction of the neo and native urethral anastomosis (see
926 PART III  Pediatric Urology

A C

B D

E G

F H

Fig. 45.21.  The onlay island flap (OIF) technique. (A) Planned incisions to
harvest transverse preputial flap and urethral plate. (B) Isolation of preputial
flap on vascularized pedicle. Inset, Preparation of meatus for anastomosis,
including excision of stenotic or hypoplastic meatal tissue. (C) Initiation of
anastomosis of onlay flap and urethral plate. (D) Proximal approximation
at the native urethra. (E) Completion of the neourethra channel. (F) Applica-
I tion of second layer of barrier coverage. (G–I) Glansplasty and skin closure.

Fig. 45.21D). The remainder of the anastomosis is completed in a
running or interrupted Lembert fashion (see Fig. 45.21E).
The TIP repair was previously described in the distal hypospadias
section. Extension of this technique to more severe hypospadias was
described, particularly in the setting of a boy whose curvature is
corrected with skin dissection or plication in combination with an
adequate urethral plate (Fig. 45.23; Snodgrass and Lorenzo, 2002).
Technical modifications including urethral plate mobilization and
transverse corporotomies, in the setting of persistent ventral curvature
of greater than 30 degrees after degloving, were proposed to increase
the applicability of the TIP for proximal hypospadias (Snodgrass
and Bush, 2011). Subsequent analysis by Snodgrass et al. (2013)
suggests that aggressive mobilization of the urethral plate leads
to devascularization, increasing the risk of stricture formation,
which ultimately limits the applicability of proximal TIP in this
setting. Because of a higher risk of complications, this technique
has since fallen out of favor for other two-stage repairs, such as
the Byars flap or STAG repair.
The reasoning behind the current trend toward a staged repair
for severe hypospadias, in which the curvature is corrected at the
first stage and urethroplasty is performed at the second stage,
lies with the methods used to correct curvature and while providing
supple ventral shaft tissue for tubularization. Concerns for a higher
risk of urethroplasty failure when combined with a corporal
grafting procedure in a single-stage repair have led to this paradigm
shift (Castellan et al., 2011; Mattos et al., 2016). Alternatively, although
rarely performed, a corporal lengthening procedure may be combined
with urethral closure in a single-stage repair. This is achieved through
use of a flap corporoplasty, in contrast to a free graft, which could
theoretically prevent contracture and facilitate healing of the overlying
urethroplasty. Braga et al. (2007b) reported their approach with
simultaneous tunica vaginalis flap for corporal lengthening with
subsequent urethroplasty with TPIF to complete the repair in a single
operation.
Two-Stage Hypospadias Repair
As the management of severe ventral penile curvature has migrated
toward corporal lengthening procedures, the two-stage repair has
emerged as the approach of choice for the majority of surgeons in
management of proximal hypospadias. Although many technical
variations exist, modern two-stage approaches can be divided into
 Chapter 45  Hypospadias 927

A B C

D E F

Fig. 45.22.  Island tube hypospadias repair. (A) Preoperative appearance. The pink, shiny, ventral skin
suggests a thinning of the ventral spongiosum. In spite of the subcoronal meatus, this is a proximal variant.
Incisions are marked and made in a similar fashion according to the schematic in Fig. 45.21. (B) After
degloving the meatus is located at the penoscrotal junction and is cannulated with an 8-Fr feeding tube.
(C) Artificial erection after dorsal plication reveals no residual penile curvature. (D) The preputial island flap
is mobilized, with harvest of the supple dartos pedicle off of the shaft skin toward the base of the penis.
(E) One edge of the flap is anchored to the tunica albuginea. (F) The flap is measured to a width of 12 mm
with excision of redundant epithelium. The flap is then closed over a diversion tube, approximating the
epithelial edge to the previously anchored portion of the flap. (G) Completed repair. (From Kraft KH, Shukla
AR, Canning DA: Hypospadias. Urol Clin North Am 37[2]:167–181, 2010.)

three main categories, each separated by fundamental differences when placing a free graft directly over the corporotomies as graft
to the approach to urethroplasty (Videos 45.1 and 45.10). contracture can occur.

Two-Stage Repair With Free Graft
The Bracka two-stage repair uses a free graft, harvested from either
the inner preputial skin or buccal mucosa, as a template for ure-
throplasty (Fig. 45.24; Altarac et al., 2012; Bracka, 1995). The staged
tubularized autograft (STAG) repair is a modification of Bracka’s
original description (Pippi Salle et al., 2016; Snodgrass and Bush,
2017a; Fig. 45.25). In the first stage, penile curvature is corrected
and the urethral plate is divided. A midline incision is extended
into the glans to act as a receiving bed for the graft. A compression
dressing and quilting of the graft into place on the ventral penile
shaft can prevent hematoma formation and facilitate graft take.
The second stage is performed at least 6 months later, in which a
U-shaped incision, similar to the Thiersch-Duplay approach, is made
and the urethra is tubularized and glansplasty is performed (Bracka,
1995). Multilayer closure is performed to maintain vascular flow to
aid the healing process. This technique has since been modified
by incorporation of multiple ventral corporotomies, without
subsequent graft coverage, as a corporal lengthening procedure.
Proximal urethral mobilization and a more extensive glans dis-
section have also been used to improve the repair (Pippi Salle
et al., 2016; Snodgrass and Bush, 2017a). Caution must be taken
Two-Stage Repair With Pedicle Flap
The Byars flap procedure uses redundant dorsal preputial skin,
which is transposed ventrally with its vascular pedicle at the first
procedure, as the scaffold to form the urethra (Fig. 45.26; Byars,
1951; Retik et al., 1994). The skin can be joined in the midline on
the ventral aspect of the penis (Fig. 45.26F) or can be aligned as a
single unit as used for the STAG repair (Fig. 45.25; Video 45.11). At
the second stage the neourethra is closed by making a long U-shaped
incision with glansplasty using a standard Thiersch-Duplay technique.
Some key technical components include creation of a watertight,
two-layer closure and establishing a lumen of equal caliber
throughout the length of the urethroplasty. Multiple layers of
closure are required to ensure that the neourethra maintains
adequate blood supply. In particular, establishing a supple dartos
bed overlying the corporoplasty at the first stage will provide
sufficient vascular flow to allow safe urethroplasty at the second
stage (Video 45.10).
The third approach involves correction of the penile curvature
with simultaneous closure of the distal urethra and glans at the first
stage of the repair. An interposition flap or graft replaces the deficient
Text continued on p. 932
928 PART III  Pediatric Urology

A B

C D E
Fig. 45.23.  Proximal tubularized incised plate (TIP) repair. (A) Circumscribing incision preserved urethral
plate. (B) After degloving, the surgeon separates the glans wings from the urethral plate. Corpus spongiosum
is dissected from the cavernosal bodies and released distally from the glans wings for later spongioplasty.
Artificial erection is performed, and if curvature is less than 30 degrees and corrected with plication, TIP
procedure can be considered. (C) Midline urethral plate incision, extended to the level of the corporal
bodies. (D) Two-layer urethral plate tubularization using interrupted subepithelial 7-0 polyglactin suture.
(E) Spongioplasty approximates divergent corpus spongiosum over the neourethra before a tunica
vaginalis barrier flap is added. (Modified from Snodgrass WT: Snodgrass technique for hypospadias
repair. BJU Int 95:683–693, 2005.)
 Chapter 45  Hypospadias 929

A B C

D E F
Fig. 45.24.  Bracka two-stage free graft repair. (A,B) Inner preputial free graft incision is marked and
harvested. (C) Abnormal urethral plate is excised and penis is degloved. (D) Incision is extended into the
glans, mobilizing glans wings. (E) Preputial graft is secured into place, including quilting sutures. (F)
Compression dressing is sutured into place over the graft. (Modified from Altarac S, Papeš D, Bracka A:
Two-stage hypospadias repair with inner preputial layer Wolfe graft. BJU Int 110:460–473, 2012.)
930 PART III  Pediatric Urology

A B C D

E F G H

Fig. 45.25.  The staged tubularized autograft (STAG) repair. (A) At the time of the first stage, one to three
ventral corporotomies are made through the tunica albuginea, with the middle incision opposite the point
of maximal curvature. (B) Preputial graft is marked and harvested. (C) Preputial graft is placed on the
ventral aspect of the penile shaft and then quilted into place (D). The bottom panel displays the second
stage of the repair. (E) Urethroplasty incisions are marked and extended along the midline of the scrotum.
(F) Glans wings are developed. (G) Two-layer urethroplasty is performed and subsequently covered with
a barrier layer with tunica vaginalis. (H) Appearance after closure. (From Snodgrass W: Staged tubularized
autograft repair for primary proximal hypospadias. J Urol 198:680–686, 2017.)
 Chapter 45  Hypospadias 931

A B C

D E F

G H I
Fig. 45.26.  Two-stage hypospadias repair with Byars flap. (A) First stage: incisions are marked to allow
degloving and mobilization of ventral skin. (B) The penis is degloved to the penoscrotal junction. Any
dysplastic, or chordee, tissue is removed. (C) Artificial erection to assess penile curvature. For the purpose
of the illustration the curvature has already been corrected. (D) Either midline or parallel lateral glans (inset)
incision(s) is (are) made in the glans and glans wings developed. (E) Byars flaps are developed by incising
the dorsal inner preputial skin in the midline to the mucosal collar, yielding a proper skin fit dorsally. (F)
The redundant dorsal skin is transposed ventrally and secured in place. (G) Second stage: a U-shaped
Thiersch-Duplay incision is made for tubularization. (H) Urethroplasty is performed, either in an interrupted
or running fashion, using a two-layer technique. Additional barrier layer with dartos or tunica vaginalis can
be performed. (I) Glans and shaft skin are approximated.
932 PART III  Pediatric Urology
urethra at the first-stage repair, and the patient is left with a proximal
functional meatus after the first procedure. The second stage of the
repair involves urethroplasty to close the proximal urethra, joining
it with the previously closed distal urethra. One variant of this
approach uses a TIP type of distal closure for the urethra and glans
in the first stage (Cheng et al., 2003). Alternatively, the Ulaanbaatar
repair creates a transverse island interposition tube, which is tunneled
into the distal portion of the glans (Dewan et al., 2004; Jayanthi
et al., 2017; Fig. 45.27).
Regardless of the approach, it is important to assess the quality
of graft or flap take at the second stage of the repair. In the
scenario in which there is skin deficiency or tethering precluding
safe closure, an intermediary step of a dorsal inlay buccal mucosal
graft may be used (Kolon and Gonzales, 2000) (Video 45.12). After
graft take, the urethra is then reconstructed at a later date when
all of the tissues are supple. Alternatively, a dorsal buccal graft
inlay and same-stage urethroplasty may be done at the time of the
second-stage repair (Fig. 45.28).
Regardless of the approach to proximal hypospadias repair, it
is important to confirm that penile curvature is corrected with
repeat artificial erection at the time of the second operation before
urethroplasty. If necessary a dorsal plication can be performed to
correct minor recurrent curvature or a repeat corporal lengthening
procedure if warranted. A waiting period of at least 6 months
should occur between procedures to ensure proper graft and/or
flap incorporation and neovascularization. We often allow 8 to
12 months between procedures, with interval exams to ensure satisfac-
tory graft/flap incorporation, to monitor for recurrent curvature, and
to determine if the ventral skin is supple enough to achieve satisfactory
urethroplasty.
Comments on Proximal Hypospadias Repair
Analysis of the published literature reveals a recent focus on high
complications for proximal hypospadias. Historical data likely
underestimate the complication rate by grouping mild and severe
variants, artificially decreasing the understood complication rate
for isolated proximal hypospadias (Long and Canning, 2016a).
The contemporary rate for needing additional procedures after a
planned two-stage repair ranges from 25% to 75%, and additional
procedures put these boys at further risk for further complications.
As a specialty, although we are effectively correcting 80% of boys
who have distal hypospadias, we must direct more efforts to reduce
complications in proximal hypospadias.
Why are proximal repairs harder? One reason may be the longer
urethroplasty required to repair a proximal hypospadias. This increased
length is more technically demanding, requiring precise technique
to generate a long urethra of equal caliber throughout its course.
One unanswered question is whether the neourethra, from whatever
tissue is chosen for reconstruction, is inherently narrowed as a result
of reconstruction, limiting its ability for gentle yet repeated expansion
to propel the urine bolus as a normal urethra would.
A longer neourethra may more dramatically demonstrate the physics
behind laminar flow and fluid dynamics. According to Poiseuille’s
law the resistance to flow in a cylinder is proportional to the length
of the tube but is inversely proportional to the radius to the fourth
power (Fig. 45.29). In plain terms the pressure required to push urine
through the lumen of the urethra directly increases with the length
of the tube. At the same time minor variations to the radius, either
increasing or decreasing in size, will have a much greater impact
upon intraluminal pressure. The longer the tube, the greater the risk
for stricture. The failure of the reconstructed urethra to expand with
voiding increases resistance to urine flow, ultimately resulting in
fistula and/or urethral diverticulum formation (Braga et al., 2007a).
In addition, the glans is relatively nondistensible because of densely
packed spongiosum tissue. Hence even in situations in which the
neourethral lumen is exactly the same caliber throughout, diverticulum
formation is a significant risk because of a fixed distal urethra. The
penile urethra may distend nicely, but when the urine reaches the
glans, poor distention increases turbulent flow, resulting in increased
pressure of the proximal urethra and diverticulum development.
It is also possible that the degree of tissue dysplasia, severity
of penile curvature, and relative lack of skin may compromise
wound healing, compromise vascular flow, or prevent equal tissue
growth with time as the boy ages. Given the relatively small number
of patients with proximal hypospadias across the world and the
increasing number of pediatric urologists performing these procedures,
the number of surgeries performed by most surgeons is decreasing.
Collaboration is going to be vital for our field to increase patient
numbers to provide a measurable advancement in the care of boys
with these complex challenges.
KEY POINTS
• Proximal hypospadias has a higher complication rate than
distal repairs and represents a unique challenge to the
urologist.
• The reasoning behind the current trend toward a staged
repair for severe hypospadias, in which the curvature is
corrected at the first stage and urethroplasty is performed
at the second stage, lies with the methods used to correct
curvature and while providing supple ventral shaft tissue
for tubularization. Concerns for a higher risk of
urethroplasty failure when combined with a corporal
grafting procedure in a single-stage repair have led to this
paradigm shift.
Postoperative Management
Proper postoperative care is essential. Even a well-planned and
technically excellent surgical repair can be compromised with careless
postoperative management. Options for methods of urinary diversion,
dressings, and perioperative medication administration have varied
greatly throughout the history of hypospadias management. Unfor-
tunately, quality data proving a direct influence on outcomes are
lacking. During the first 48 hours after surgery, urinary diversion
and a compressive dressing are employed by the surgeon. The dressing
is designed to minimize stress on the freshly closed neourethra,
tamponade bleeding, and minimize swelling. For complicated repairs,
urinary diversion with a soft, pliable urethral stent decreases rates
of meatal stenosis and urethrocutaneous fistula development
(Daher et al., 2015; Hakim et al., 1996). In our practice a compressive
dressing is left for a 72-hour period, after which petroleum jelly is
applied to facilitate healing. A suprapubic tube may be placed,
particularly in older children or in redo cases, to maximize urinary
diversion.
Urinary diversion is provided for a period of 3 to 21 days and
varies according to the complexity of the repair, the length of the
urethroplasty, and surgeon preference. In one study, prolonged stent
duration has been shown to decrease complication rates (Daher
et al., 2015). This finding is not universally accepted. Stent duration
must be weighed against patient discomfort and the risk for UTI,
which increases with each day of diversion. Some surgeons have
performed distal repairs of a short urethroplasty length without a
urethral stent without any change in outcomes (Steckler and Zaontz,
1997). Antibiotic prophylaxis is frequently administered, as previously
discussed. Postoperatively, bladder spasm is common and can
result in high-pressure voiding around stents and catheters, placing
undue stress on the urethroplasty. This can be alleviated with the
addition of oxybutynin until stent removal. Longer and stiffer
stents that extend well beyond the neomeatus may place undue
pressure on the ventral urethral repair, increasing the risk for
glanular dehiscence. A Foley catheter, if placed, must be secured to
the anterior abdominal wall in the midline to prevent glans separation.
Unfortunately, these catheters frequently end up with undue lateral
or ventral tension from the securing tape releasing prematurely.
Therefore many years ago we moved away from Foley catheters because
of the high glans dehiscence rate.
Most surgeons administer acetaminophen for postoperative
pain control, whereas close to half of surveyed urologists include
 Chapter 45  Hypospadias 933

A B C

D E F

Fig. 45.27.  The modified Ulaanbaatar staged hypospadias repair. (A) Initial appearance of
the penis before incision. This patient had 46,XY ovotesticular disorder of sexual differentiation,
severe ventral penile curvature, and a proximal hypospadias. Incisions are made and curvature
is corrected with small intestinal submucosa during the first stage of the operation. (B) A
transverse island tube Duckett tube is harvested for a length that will replace the glanular
and distal penile urethra. (C) The neourethra is either tunneled into the glans (if of sufficient
size) or a formal glans incision with glansplasty is performed. (D) Byars flaps are rotated
ventrally to replace the gap in the urethra between the proximal end of the Duckett tube
and the distal extent of the native urethra. A drip stent is left in the closure. (E) After an
interval 6 months, an elliptical incision is made around the urethral defect and tubularized.
(F,G) The appearance after closure and 3 months postoperatively. (From Jayanthi VR: The
G
modified Ulaanbaatar procedure. J Pediatr Urol 13(4): 353, 2017.)
934 PART III  Pediatric Urology

A B

C D E F

Fig. 45.28.  Illustration (A) and live surgery pictures (A–F) depicting a single-stage buccal inlay graft. The
penis is assessed and the urethral plate is found to be too narrow for primary tubularization. A U-shaped
incision for a Thiersch-Duplay repair is made. A midline incision is made within the future urethral plate
and a buccal graft is secured into the midline. Urethroplasty is then performed according to the width of
the edges of the initial U-incision. Additional layers of closure are added as a barrier layer and the penis
is closed. The final live surgical image represents the closed penis appearance. (Images courtesy Rafael
Gosalbez, MD.)

nonsteroidal anti-inflammatory drugs (NSAIDs) (Morrison et al.,
2014). Ibuprofen appears safe even in the immediate postoperative
period despite concerns for bleeding. A study by Schroder et al.
(2018) suggests that standing doses of acetaminophen and ibuprofen
can maximize pain control and minimize the need for supplemental
narcotic administration. Around-the-clock dosing can be tapered
within 48 to 72 hours of the procedure, after which the surgical pain
experienced decreases (Schroder et al., 2018; Wilson et al., 2017).
Complications of Hypospadias Repair
When Duckett discussed hypospadiology, he highlighted the blend
of art and science that produces a successful repair, describing it as
a humbling process, a time- and energy-consuming task that con-
founds the surgeon. Pediatric urologists with experience understand
this (Duckett, 1995). Despite continued technical modifications
and advances, successful penile reconstruction for boys with
hypospadias remains a great challenge. A combination of factors
threatens to compromise even the most meticulous repair. The
structures are delicate. The healing process is dependent upon
fragile neovascularization. Postoperative edema, the risk of infec-
tion, and the inherent variability in the healing process add to
the risk. Anyone who prepares to treat boys with hypospadias
must have a broad and deep understanding of the potential risks
and be committed to careful preoperative study, meticulous surgical
management, and zealous postoperative care.
 Chapter 45  Hypospadias 935

High resistance

8 Fr

6 Penoscrotal neourethra (TIP)

Neomeatus
A (Non-limiting) At risk for
proximal
Low resistance fistula

8 Fr

Coronal neourethra (TIP)

Neomeatus
B (Non-limiting) Urine flow

Low resistance

8 Fr during micturition (more elastic)

9
Penoscrotal neourethra (ONLAY)
Neomeatus
C (Non-limiting)

Fig. 45.29.  Comparison of the urethral resistance and flow patterns for the tubularized incised plate
(TIP) repair (A,B) vs. onlay procedure. To the left of the illustration is a corresponding uroflow. All three
diagrams assume a patent urethral meatus. (B) The short neourethral length provides a small amount of
resistance over a narrow distance, resulting in a relatively normal flow pattern. This is in contrast to (A),
where the long TIP repair provides a fixed amount of resistance across a much longer distance. This
results in a prolonged void with a dampened velocity. (C) The increased elasticity of the tissue used in
the onlay procedure decreases the resistance of the neourethra, allowing a more normal voiding pattern.
The fundamental differences between these two repairs are due to the more rigid urethra created with
the TIP repair versus the more pliable tissue used in an onlay procedure. (From Braga LP, Pippi Salle
JL, Lorenzo AJ, et al: Comparative analysis of TIP versus onlay island flap urethroplasty for penoscrotal
hypospadias. J Urol 178:1451–1457, 2007.)

Today there is a lack of standardized definitions for hypospadias
complications, leading to variability in reporting and inconsistency
in the literature. Through efforts at multiple centers across the world
this is changing, and some standard definitions are represented in
Table 45.1.
Urethrocutaneous Fistula
Urethrocutaneous fistula is the most common reported surgical
complication after hypospadias repair, with an incidence just under
10% in short-term follow-up (el-Kassaby et al., 2008; Grosos et al.,
2014; Pfistermuller et al., 2015; Snodgrass and Bush, 2011; Fig. 45.30).
A fistula can develop anywhere along the length of the urethroplasty,
although most often at the site of the original urethral meatus or
at the coronal margin. They range from a pinpoint opening with a
small urine drip to a large defect through which all urine flows. The
development of a fistula is multifactorial, with ischemia, edema,
infection, and/or hematoma contributing to improper healing of
the neourethra. Distal urethral obstruction from meatal stenosis or
urethral stricture results in high urethral pressures and turbulent
flow during voiding. This places additional stress on the suture line
and can decrease the perfusion of the tissues during wound healing.
Technical factors such as overlapping of suture lines, inadequate
inversion of the epithelium, or use of poorly absorbable suture
material may also contribute (Eardley and Whitaker, 1992). All of
these factors become even more evident as the severity of the
hypospadias worsens, further increasing the complication rate (Arlen
et al., 2015; Bush et al., 2015; Spinoit et al., 2015).
Management of urethral fistulae depends on the number, size,
location, and the presence of additional complications. Multiple
urine streams may be noticed by the parent if the child voids during
a diaper change, and most are noticed in the first 2 years after surgical
repair (Grosos et al., 2014; Spinoit et al., 2013; Wood et al., 2008).
Fistula diagnosis may be delayed until toilet training, when voiding
is more likely to be observed.
Small fistulas noted in the immediate postoperative period without
concomitant inflammation or meatal stenosis rarely close on their
own (Chandrasekharam, 2016). The majority of urethrocutaneous
fistulas require surgical repair, which is delayed until at least 6 to
12 months after the initial hypospadias repair to allow wound healing
and tissue softening.
If a fistula is suspected, a careful evaluation in the operating room
may include calibration with a bougie à boule to assess the patency
of the urethra and to identify irregularity in the urethral caliber,
which commonly accompanies the development of a fistula. If calibra-
tion suggests irregularity in the urethra, urethroscopy should be
performed. As a further test, a urethral catheter or a venocath may
be inserted and saline injected into the urethra with the proximal
and distal lumen compressed, performing a “stress test” to identify
concealed fistula tracts.
Small-caliber fistulas on the penile shaft proximal to the coronal
margin can be closed primarily, taking care to excise the epithelialized
tract to the urethra (Santangelo et al., 2003; Fig. 45.31; Video 45.13).
The urothelial edges can be closed in a subepithelial fashion. Multiple
overlapping layers are placed to prevent recurrence. Larger fistulas,
or those that are present in areas where the tissues have thinned,
require coverage with a trap-door or island flap of penile shaft skin.
Good results depend on a second layer or a vascularized flap coverage,
secured in an offset fashion (Cimador et al., 2004; Elbakry et al.,
1998; Santangelo et al., 2003).
Fistulas at the coronal margin are more challenging. Although
it is tempting to perform excision and primary closure, a fistula
936 PART III  Pediatric Urology

TABLE 45.1  Complications and Definitions

URETHROPLASTY COMPLICATIONS SKIN COMPLICATIONS

Fistula Skin surplus or deficiency with penile tethering

Glans dehiscence Penile torsion >30 degrees
Meatal stenosis Preputial fistula
Urethral stricture Preputial dehiscence
Urethral diverticulum Postpubertal phimosis
Recurrent curvature >30 degrees Lichen sclerosus

STANDARDIZED DEFINITIONS
Urethroplasty Complications: Primary Outcome
• Fistula: urethral leak anywhere below the meatus
• Glans dehiscence: complete separation of the glans wings resulting in a coronal or more proximal meatus, or complete separation
of glans wings with an intervening bridge of skin; objectively: glans fusion measurement <2 mm
• Meatal stenosis: obstructive symptoms (straining, prolonged voiding, urinary tract infection, and/or retention) and meatal calibration
<8 Fr before puberty or <12 Fr after puberty
• Urethral stricture: obstructive voiding symptoms (stranguria, prolonged voiding, urinary tract infection, and/or retention) with visual
near closure of the urethra on urethroscopy
• Urethral diverticulum: visual segmental sacculation of the urethra during voiding
• Recurrent curvature: residual ventral, dorsal, or lateral curvature >30 degrees demonstrated on erection (spontaneous or artificial)

Skin Complications: Secondary Outcome

• Skin surplus: Excess skin ≥2 cm in a circumcised patient when the suprapubic fat pad is retracted
• Penile torsion: angle between the normal and true vertical glanular plane >30 degrees from midline (specify clockwise or
counterclockwise)
• Nonretractable reconstructed preputium: cannot visualize meatus (in the absence of lichen sclerosus), acceptable before puberty,
abnormal after puberty
• Preputial dehiscence after preputial reconstruction: defect in the preputial skin recreating an incomplete prepuce
• Preputial fistula after preputial reconstruction: fistula-like defect in the preputial skin that has no contact with the urethral lumen
• Lichen sclerosus: white cicatrix, preferably with pathology diagnosis

Courtesy Warren Snodgrass, MD.

A B C

Fig. 45.30.  Examples of various urethrocutaneous fistulas, each depicted with black arrows. (A) Two
fistulas are noted. (B) A lacrimal duct probe lies within the fistulous tract. (C) A midshaft fistula is present.
 Chapter 45  Hypospadias 937

A B C

D E F

Fig. 45.31.  Repair of urethrocutaneous fistula after a prior two-stage proximal hypospadias repair.
(A) Distal shaft urethrocutaneous fistula (arrow) with an intact glans and patent meatus. The distance from
the glans is a key component in the decision making for fistula management. (B) Incision is marked, first
around the fistula tract and then secondarily to raise flaps for barrier coverage. (C) Incisions are made
and the fistulous tract is isolated. The surrounding skin is undermined to sufficiently mobilize tissue for
skin closure as well as barrier layers. (D) Fistula tract is closed in a primary fashion and dartos layers are
approximated over the repair. (E) Skin is closed. (F) Six-month postoperative picture with no evidence
of recurrence.

at this site can suggest a distal narrowing or poor local tissue
that requires revision of the urethroplasty to achieve a satisfactory
repair (Video 45.14).
Despite a well-performed repair, fistulas recur about 20% of the
time (Snyder et al., 2005a; Sunay et al., 2007). This recurrence rate
may be lessened with interposition of multiple well-vascularized
nonepithelialized layers between the urethral closure and the skin.
Redundant dartos tissue, if present, or a scrotal-based tunica vaginalis
flap may also be used (Fahmy et al., 2016; Routh et al., 2008; Shankar
et al., 2002). Scrotal dartos tissue has been used in a similar fashion
(Churchill et al., 1996). A de-epithelialized flap may also be used
with success (Santangelo et al., 2003). In cases in which tissue is
scarce, supplemental coverage can be taken from adjacent de-
epithelialized shaft skin with a subsequent Cecil modification,
reviewed in more detail later in the chapter (Cecil, 1946; Ehle et al.,
2001; Weiss et al., 2018). There is no need to divert the urine for
simple repairs, but for more complex redo repairs, we routinely
divert the urine for 7 to 14 days (Holland et al., 2008; Redman,
1993; Santangelo et al., 2003).
Glans Dehiscence
Glans dehiscence is due to a combination of factors, including undue
catheter tension on the repair as noted earlier, poor technique, tension
on the glans closure, and vascular compromise (Fig. 45.32). If this
occurs, the meatus typically regresses back to the corona or subcoronal
location. Reoperation may or may not be necessary and should
be driven by the presence or absence of voiding symptoms. If
repair is indicated, an interposition inlay graft may be required
as an intermediary step for a staged repair to avoid tension on
the glans closure (Video 45.15).
Meatal Stenosis
Definitions for meatal stenosis vary considerably across studies
(Wilkinson et al., 2012). Risk of narrowing increases if the urethra
closure extends too distally or if the glans is closed with too much
tension. Prolonging the time of postoperative stenting may decrease
the risk of stenosis, although there are fewer data to substantiate
this assumption (Daher et al., 2015). In the postoperative period
the meatus should calibrate to a size no less than 8 Fr, and this
should increase with age. A narrowed meatus with symptoms of
stranguria, deflected stream, poor uroflow, or urinary retention
would suggest a clinically relevant stenosis that warrants repair.
Occasionally a stenotic meatus can be managed conservatively with
meatal dilation with a sound and/or topical betamethasone cream
(Radojicic and Perovic, 2006). Repeated dilation after hypospadias
repair is not necessary as a routine practice to decrease rates of
938 PART III  Pediatric Urology
Fig. 45.32.  Examples of glans dehiscence. All have a meatus below the corona (arrows) and lack of
fusion of the glans wings in the midline.
stenosis (Lorenzo and Snodgrass, 2002). It is not part of our practice
but has been reportedly successful when the meatus appears narrow
in the early postoperative period, and it may be diagnostic of subclini-
cal stenosis. When the stenosis is refractory to dilation, a dorsal
midline meatotomy can achieve patency in rare cases (Brannen,
1976). Otherwise a redo urethroplasty is indicated.
Meatal retrusion occurs when excessive tension on the distal urethra
or the glans dehiscence has occurred (Duckett and Snyder, 1992).
Meatal retrusion in part is due to improper surgical planning, resulting
in an immobile or poorly vascularized urethra that retracts in the
initial postoperative period because of undue tension. The conse-
quences of this complication may be cosmetic or functional. If
deflection of the stream precludes normal voiding while standing,
revision of the meatus should be considered (Marte et al., 2001).
Urethral Stricture
Urethral narrowing that extends more proximally than the meatus
is also a frequently reported complication of hypospadias repair,
with rates varying depending on urethroplasty technique and length
of urethroplasty (Duel et al., 1998; Husmann and Rathbun, 2006).
These strictures tend to form at the junction of the native and
neourethra, along the path of the neourethra, or within the glans.
The clinical picture can vary, but complaints of a diminished urine
stream force, stranguria, urinary retention, or urinary tract infection
are most common.
Several factors increase the risk of postoperative stricture. Improper
urethroplasty technique, tissue ischemia, trauma, or infection can
result in narrowing of the lumen. When clinically apparent, it is
best to characterize the stricture via cystoscopy to delineate the length,
caliber, and location of the stricture.
Initial management depends upon the severity of the stricture.
For short strictures causing minimal symptoms, urethral dilation or
endoscopic incision can be attempted. In most cases, however, this
will not provide a long-term solution. Direct visual internal ure-
throtomy in adult strictures has low success rates and risks worsening
the stricture by generating more inflammation and scar (Santucci
and Eisenberg, 2010; Tang et al., 2008). Success rates for small
strictures (less than 1 cm), even when done in conjunction with
intermittent catheterization, are poor: only 20% of boys exhibit a
normal flow rate 2 years postincision (Husmann and Rathbun, 2006).
The outcome may be better for short anastomotic strictures when
endoscopic treatment is initiated 3 months after operation, although
in our experience conservative management is rarely successful in
these patients (Duel et al., 1998).
Strictures not responding to conservative management or those
with extensive disease at diagnosis require revision urethroplasty
(Gargollo et al., 2011). The surgeon must be prepared to harvest a
local skin flap or buccal mucosa because there will likely be significant
tissue loss at the site of stricture. This often results in a multistage
repair, as discussed further in the reoperative hypospadias section.
If hair-bearing skin is used for the hypospadias repair, the patient
may notice hair extending from the meatus. This complication usually
occurs after multiple-stage procedures or complex reoperations in
which there is a shortage of non–hair-bearing skin. Hair in the
urethral lumen, if combined with urethral narrowing, acts as a nidus
for stone formation or recurrent UTIs. Laser ablation may result in
success in some cases (Cohen et al., 2007; Neal et al., 1999). Stone
debris can be endoscopically removed in the majority of cases. In
most cases in which stones, hair, and UTI coexist, the surgeon must
resect the affected portion of the urethra and replace it with new
tissue, such as a buccal graft, or in the unusual case in which redundant
local skin is present, a local graft or flap.
Recurrent UTIs should prompt evaluation for urethral diverticulum
or stricture. Occasionally, a voiding cystourethrogram or cystoscopy
demonstrates an enlarged prostatic utricle, which can promote urinary
stasis resulting in bacterial colonization. If no other source of infection
is obvious, consideration should be given to either excision or fulgura-
tion of the utricle (Ciftci et al., 1999).
Urethral Diverticulum
Symptoms of urethral diverticulum include weak urinary stream,
postvoid dribbling, UTI, or, less commonly, hematuria. The boy or
his parents may notice ballooning of the penile shaft during voiding
or report the need to “milk” residual urine from the penile urethra
to avoid soiling of the underwear. Diverticula occur more commonly
in boys undergoing preputial flap repairs, two-stage repairs, and
proximal repairs (Snyder et al., 2005b). This is in part anatomic,
because of the lack of spongiosal tissue in the neourethra, which
acts to reinforce the normal urethra during voiding. As mentioned
earlier, the extra support of the glanular neourethra after glans closure

E
can increase voiding pressure in the penile urethra, leading to
diverticulum formation (Radojicic et al., 2004).
A small, localized saccular diverticulum can be excised and reduced,
returning the urethral lumen to a uniform caliber. The more commonly
encountered extensive diverticula are repaired by excising redundant
diverticular tissue, urethral closure, and multilayered reinforcement
before skin closure (Zaontz et al., 1989; Fig. 45.33; Video 45.16).
The redundant tissue of the diverticulum is well vascularized in some
instances, making it an ideal candidate for repair of associated fistulas
and distal strictures, if present (Radojicic et al., 2004). Various flap
techniques have been employed to reconfigure redundant diverticular
tissue for this purpose (Winslow et al., 1985). Urethral plication in
an extraurethral fashion is an alternative technique that does not
violate the urethral plane (Heaton et al., 1994).
Balanitis Xerotica Obliterans
Balanitis xerotica obliterans (BXO), or lichen sclerosus, is a chronic
inflammatory dermatosis that can occcur in uncircumcised boys
with phimosis, meatal stenosis, or progressive urethral narrowing
after hypospadias repair (Fig. 45.34). Its management can prove
Chapter 45  Hypospadias 939
Fig. 45.33.  (A) Urethral diverticulum on exam with a sacular dilation
of the distal urethra (arrow). (B) Diverticulum is evident after ventral
penile incision and partial degloving. (C) Diverticulum cavity is open
and stay sutures are in place. Markings indicate the edges of urethral
closure. (D) Excess epithelial tissue is excised, leaving redundant
dartos tissue for extra layer closure over the urethra. (E) Urethral
closure before barrier layer approximation.
difficult. If limited to the urethral meatus, the initial therapy is a
course of topical steroids (betamethasone or clobetasol) or systemic
tacrolimus (Kiss et al., 2001; Snodgrass et al., 2017). In most cases,
conservative therapy is unsuccessful and the surgeon must resect
involved tissue and replace it with inner prepuce or buccal mucosa,
in multistaged operations to fully reconstruct the penis (Bracka,
2011; Dubey et al., 2005). Tissue should be sent to pathology to
confirm diagnosis. These patients must be monitored closely for
recurrence of BXO. Recurrence rates as high as 40% have been
reported with median follow-up of 26 months (and as long as
105 months) from surgery (Snodgrass et al., 2017). It is now
recognized that BXO can and does invade buccal mucosa, further
complicating treatment (Levine et al., 2007; Snodgrass et al., 2017).
Recurrent Penile Curvature
Persistent penile curvature is an unfortunate complication of hypo-
spadias repair that has severe consequences on urinary and sexual
function. Residual curvature may worsen as boys advance through
puberty, when penile growth surges with potentially differential
growth of the dorsal and ventral portion of the corporal bodies
940 PART III  Pediatric Urology

A B C

Fig. 45.34.  (A,B) Balanitis xerotica obliterans (BXO) present in the meatus as evidence by the pale, white
appearance to the tissue. Note the appearance on the penile shaft in C.

A B

Fig. 45.35.  A 14-year-old boy with a history of proximal hypospadias repair. He had undergone a single-
stage hypospadias repair as an infant, including correction of his curvature with dorsal plication. He
presented with significant ventral penile curvature, seen in (A). (B) Location of the urethral meatus after
correction of the penile curvature in the first stage operation (yellow arrow). There is a large gap between
the proximal end of the distal portion of the urethra (red arrow) and the distal end of the proximal urethra.
This boy required two additional procedures, the first of which was a buccal inlay graft with subsequent
closure at the final stage.

(Barbagli et al., 2006). Persistent or recurrent curvature occurs when
curvature is underestimated or is repaired incompletely (Braga et al.,
2008). If significant concern is present and intervention is indicated,
the penis must be fully degloved and artificial erection performed.
Curvature may be present at the base of the penis, which can be
obscured by the presence of a tourniquet. To properly assess this,
artificial erection is performed with compression of the corporal
bodies against the pubis. Alternatively, prior preservation of a
tethering urethral plate or ventral skin contraction can tether the
penis ventrally.
When curvature recurs, parents should be counseled that several
procedures may be required (Fig. 45.35). The first stage assesses the
cause of curvature, with particular attention to corporal disproportion
if a dorsal plication was performed previously (Snodgrass, 2008).
If a graft is used for corporoplasty, a healthy dartos layer should
cover this area to act as a recipient for a buccal mucosa graft at the
next stage of reconstruction. In many cases, a buccal graft to the
ventral penile shaft is required for full penile reconstruction, unless
a sufficient amount of skin is present to provide tension-free coverage.
If shaft skin is deficient, we perform a Cecil modification during the

third stage, which provides supple tissue for skin closure at the
fourth stage (as outlined later) (Ehle et al., 2001).
Skin Complications
Although often perceived as minor, several skin complications may
occur after primary repair. If penile concealment and poor skin fixation
are present, a buried penis with shortening may occur. This condition
may be avoided at the original surgery with penile shaft degloving
and well-placed anchoring sutures that superficially attach Buck fascia
to the corresponding dermis of the abdominal wall/shaft skin juncture.
Suture sinus tracts can occur from suture reaction of the skin, par-
ticularly if thin ventral shaft skin is present or if full-thickness sutures
are placed. In addition, tightly closed sutures may lead to ischemia
and foster suture tracts. Patients with proximal hypospadias and/or
penoscrotal transposition, penoscrotal webbing, or poor definition
of the penoscrotal junction can result in an abnormal clinical appear-
ance after repair. A scrotoplasty may be used in these settings to
prevent postoperative penile concealment.
HYPOSPADIAS OUTCOMES
We have elected to combine the reported outcomes for distal and
proximal hypospadias in a single section. As we have mentioned
throughout this chapter, there are significant concerns about the
quality of the literature in its current form. Although thousands
of publications can be found with a simple PubMed search, most
if not all are limited to some extent by their retrospective nature,
small cohort sizes, variations in technique, poorly defined out-
comes, lack of independent outcome assessment, and patient loss
to follow-up. This is recognized across our specialty and efforts
to improve publication standards and quality collaboration across
institutions are being made (Braga et al., 2016).
In the largest and most comprehensive outcome analysis, a
meta-analysis by Pfistermuller et al. in 2014 included 49 studies
with 4675 boys having undergone a TIP repair (Pfistermuller et al.,
2015). They identified an overall complication rate of 10.6%, with
a reoperation rate of 4.5%, at a mean follow-up of 16.1 months.
Of the complications assessed, fistulae (5.7%) were most common,
followed by meatal stenosis (3.6%), and urethral strictures (1.3%).
The authors highlighted the high degree of variability in assessment
of outcomes, limiting their ability to compare studies. Outcomes
were improved with use of an additional layer of dartos coverage, but
proximal and reoperative cases had a significantly higher complication
rate (Pfistermuller et al., 2015). In a similar meta-analysis comparing
1872 TIP repairs with 1496 Mathieu repairs, the overall complica-
tion rate was similar, occurring in 129 (6.9%) and 88 (6.7%) of
boys (Wilkinson et al., 2012). Urethrocutaneous fistulas occurred
equally in both repairs, although meatal stenosis was much more
common after the TIP repair. These authors also highlighted the
difficulty in comparing studies, specifically the poor quality of the
data, including surgical indications, lack of follow-up, and lack of
clarity in defining outcomes.
Recurrent UTIs are uncommon after hypospadias repair, occurring
in 43/2249 (1.91%) of boys in a large study from the group in
Toronto in the postoperative period after urethral stent removal
(Wehbi et al., 2014). In this population, diagnostic imaging identified
several anomalies: distal urethral narrowing in 17/43 (40%), VUR
in 14/43 (33%), urethral diverticulum in 5/43 (12%), and an enlarged
utricle in 5/43 (12%). Comparing boys who underwent TIP versus
OIF, the TIP group was more likely to have an elevated PVR, whereas
the OIF group was more likely to develop a diverticulum and/or
fistula. Based on these findings, surgeons should perform anatomic
imaging for any boy with recurrent UTIs after repair.
Prat et al. (2012) shared their findings from a single-institution
study of 707 boys with distal penile to glanular hypospadias operated
on over a 31-year period. Patients were included only if they had
more than 1 year of follow-up. A variety of techniques were used
throughout the inclusion period, including MAGPI, TIP, Mathieu
flap, onlay flap, and a tubularized flap. The overall complication
Chapter 45  Hypospadias 941
rate was 24% and worsened with increasing severity of the hypo-
spadias. In a similar study of 366 distal hypospadias repairs at a
median follow-up of 23 months, the reoperation rate for TIP, MAGPI,
or OIF repairs was 21.3% (Spinoit et al., 2013). The complication
rate was significantly higher for patients who had longer duration
of follow-up and for those with a more proximal meatus.
A single-institution study of 578 patients undergoing TD distal
hypospadias repair reported a complication in 17.4% of patients
(Grosos et al., 2014). The median follow-up was 25.6 months, with
urethrocutaneous fistula and glans dehiscence occurring more com-
monly in the first year, whereas urethral stricture occurred more
commonly as a late complication. Successful outcomes in distal
hypospadias is high, ranging from 83% to 95% (Perlmutter et al.,
2006; Pfistermuller et al., 2015; Rushton and Belman, 1998).
Unfortunately, the overall complication rate for proximal hypo-
spadias is much higher, ranging from 23% to 68% when reviewing
papers that report only on proximal hypospadias and more mild
forms being excluded (Castagnetti et al., 2013; Gorduza et al., 2011;
Long et al., 2017; McNamara et al., 2015; Pippi Salle et al., 2016;
Stanasel et al., 2015; Snodgrass and Bush, 2017a). As with distal
hypospadias repair, urethrocutaneous fistula is the most common
complication, occurring in 3% to 45% of boys (Gong and Cheng,
2017). This is followed by glans dehiscence and meatal stenosis.
Urethral diverticula occur more frequently in the setting of a
Byars flap repair or onlay procedure versus a two-stage graft repair
(STAG or Bracka repair).
Regardless of technique, the rate of recurrent penile curvature for
two-stage repair of proximal hypospadias is relatively low, ranging
from 0 to 10% of patients (Gong and Cheng, 2017; Long et al.,
2017; McNamara et al., 2015; Pippi Salle et al., 2016; Snodgrass and
Bush, 2017a; Stanasel et al., 2015). Two-stage repair with corporoplasty
was associated with increased penile length and improved cosmetic
results (Castagnetti et al., 2013). Penile length is an important variable,
particularly with proximal hypospadias, as a survey of adult patients
after infant repair reveals concerns primarily for shortened penile
length (Andersson et al., 2018; Moriya et al., 2016).
The two-stage graft repair (STAG) has an overall reported
complication rate of less than 25% (Ferro et al., 2002; Castagnetti
et al., 2013; Snodgrass and Bush, 2017a). The most common
complication encountered was glans dehiscence and/or urethro-
cutaneous fistula. The rate of recurrent penile curvature is low.
Additional layers of closure and increasing the local blood supply
have been shown to decrease the complication rate (Telfer et al.,
1998). A comparison of four approaches to proximal hypospadias
repair, including TIP, preputial island onlay, island flap tube, and a
two-stage Bracka repair, found that repair approach did not affect
complication rates (Castagnetti et al., 2013). Concerns for a skin
graft incorporation over a corporal graft can be circumvented with
dorsal plication techniques combined with proximal urethral
mobilization to correct the majority of curvature (Warwick et al.,
1997). Otherwise, if severe curvature persists a corporal graft should
be performed and this would increase the risk for skin graft loss.
Several reports have indicated a high complication rate
associated with the Byars flap two-stage repair (Long et al., 2017;
McNamara et al., 2015; Pippi Salle et al., 2016; Stanasel et al., 2015).
Complication rates ranged from 30% to 70%, with urethrocutane-
ous fistula representing the most common complication noted,
followed by glans dehiscence. Urethral diverticulum is also a potential
complication, thought to occur as a result from a combination of a
lack of spongiosum and lack of fixation of the flap onto the ventral
penile shaft at the time of the urethroplasty, as mentioned previously.
The group from Texas Children’s Hospital shared their 11-year
experience with 56 boys with median follow-up of 34 months
(Stanasel et al., 2015). The surgeons used a two-stage repair with
at least 6 months between stages. Their overall complication rate,
defined as any additional procedures planned beyond the initial two-
stage repair, was 68%. In a similar report, the Boston Children’s
Hospital team reported their results over 20 years for 134 boys
undergoing a staged repair for proximal hypospadias. They reported
a complication rate of 49% with a median follow-up of 46 months,
including fistula, diverticulum, meatal stenosis, and glans dehiscence
942 PART III  Pediatric Urology
(McNamara et al., 2015). Pippi Salle et al. (2016) from Toronto
compared their experience with 3 separate techniques on 140 boys
with proximal hypospadias: a long TIP, dorsal inlay graft, and a
staged repair for proximal hypospadias. With mean follow-up ranging
from 30 to 48 months, the complication rate was highest for a long
single-stage TIP (53%) and lowest for the staged repair (32%). Our
experience with proximal hypospadias repair at CHOP is similar.
Of 167 consecutive boys with proximal hypospadias operated on
from 2006 to 2014, 86 underwent a single-stage repair and 81 a
planned two-stage repair. Median follow-up was 29 and 31 months,
respectively. The complication rate was higher for the single-stage
repairs compared with staged repair (62% vs. 49%, P = .11) (Long
et al., 2017). Although mostly represented by case series, the two-
stage approach incorporating distal urethra closure and correction
of curvature at the first stage has reportedly favorable outcomes
(Cheng et al., 2003; Dewan et al., 2004).
Castagnetti et al. (2013) sent a questionnaire as well as the penile
perception score to 93 patients who underwent proximal hypospadias
repair. These patients on initial exam all had ventral penile curvature
that was reconstructed with an onlay island flap, onlay island flap
on albuginea (a technical modification in which the onlay flap is
anchored in the midline to the albuginea of the corpora cavernosa),
TIP, or two-stage repair. Their significant findings include the achieve-
ment of a longer penis in the staged repair, improved cosmetic
outcomes (as per the PPS) for the two-stage repair, and that the
complication rate (23%) and urinary complaints were similar across
all four surgical approaches.
To our knowledge no direct comparison of these three approaches
mentioned earlier has been performed that would determine the
ideal approach to proximal hypospadias with severe ventral penile
curvature. Regardless of the approach taken, long-term outcomes
data are lacking and raise concerns that more complications will be
reported as these patients are followed over time (Grosos et al.,
2014; Spinoit et al., 2013).
Outcome Assessment
In some cases, complications after hypospadias repair are not obvious.
A urethrocutaneous fistula with multiple streams is easy to identify.
However, subtle changes heralding stricture formation may be hard
to appreciate. Visualization or measurement of the urinary stream
in the office or on a video captured by family can be indicative of
stricture. Urethral calibration in the office is not an easy task. If a
high degree of concern is present for a stricture or meatal stenosis,
then exam under anesthesia is warranted. Uroflow can be performed
to further characterize a slowed urinary stream, although exact
definitions of a poor flow after a hypospadias repair are not well
defined and difficult to obtain in the pre–toilet-trained population.
It can also be difficult to predict which child needs surgical
attention because improved flow rates with aging have been
reported (Andersson et al., 2015).
Some argue that the majority of complications are identified in
the first year postsurgery (Snodgrass et al., 2014c). However, longer
follow-up universally yields higher complication rates with only
50% of postoperative complications noted in the first postoperative
year (Grosos et al., 2014; Spinoit et al., 2013; Wood et al., 2008).
Spinoit et al. (2013) examined 474 primary hypospadias repairs
and identified only 54 of 114 (47%) of their complications within
1 year of surgery. By 36 months of follow-up, 88 of 114 (77%)
had undergone repair of complications. In a similar study, Grosos
et al. (2014) reviewed results following the Thiersch-Duplay
technique and reported that only 57% of their complications
were discovered during the first year of follow-up. The type of
complication varied according to the time to presentation, with
fistulas occurring more commonly in the first year, whereas urethral
stricture was more common beyond this time point. These findings
suggest that although a majority of boys have complications identified
early in the first 3 years of surgical follow-up, there is a large number
that develop later complications.
Our practice has evolved to include a minimum standard of
follow-up of our surgical patients through puberty, particularly for
our proximal hypospadias patients. All patients follow up after toilet
training to allow an assessment of the urinary stream. Extension of
follow-up through puberty allows further evaluation of the voiding
velocity, curvature, and sexual function at the completion of penile
development.
Patient-Reported Outcomes
Much of the discussion in the chapter has focused on surgical
procedures and their outcomes as observed by the surgeon. Although
surgeon assessment, for fistula and glans dehiscence for example,
is important, without patient input, the process is incomplete.
In spite of what surgeons may determine a successful repair,
parental and patient perception of their outcomes may differ
(Lorenzo et al., 2014; Mureau et al., 1996). Analyzing issues that
contribute to parental decisional regret include the development
of complications, parental hesitancy regarding the potential surgery,
and the desire for circumcision while improved parental education
and understanding of hypospadias repair decreased conflict about
repair (Lorenzo et al., 2012, 2014). Further refinement of the patient
experience can help stem some of the parental and patient disap-
pointment, which can occur even in the setting of a successful repair.
To capture PRO, several hypospadias specific scoring systems have
been developed. The Hypospadias Objective Scoring Evaluation
(HOSE), Pediatric Penile Perception Score (PPS), Genital perception
Score (GPS), and the Hypospadias Objective Penile Evaluation
(HOPE) score are scoring systems that have been generated and
validated in children (Holland et al., 2001; Mureau et al., 1995; van
der Toorn et al., 2013; Weber et al., 2008; Fig. 45.36 and Table 45.2).
Each instrument incorporates similar components to varying
degrees, including the meatus, the presence of a fistula, the quality
of the urinary stream, penile length, skin appearance, and general
penile appearance. The HOPE score is unique in that a standard
set of postsurgery pictures are obtained and scored by independent
panels consisting of parents, patients and pediatric urologists,
including reference pictures ranging from normal to severely
abnormal, resulting in a high degree of interobserver reliability
for surgical outcomes (Fig. 45.37). A recent review by Sullivan et al.
(2017) examines these scoring systems in depth, highlighting the
lack of assessment of sexual function and psychosocial factors related
to the repair. The authors also note a strong bias toward surgical
outcomes, such as the location and appearance of the urethral meatus,
Interview child
We will talk about several aspects of your penis. Please tell me how
satisfied you are with these. There are four possible answers: Very
satisfied, satisfied, dissatisfied, very dissatisfied. Please tell me
which one is the most appropriate for you
Very Very
satisfied Satisfied Dissatisfied dissatisfied
a Length of your penis (3) (2) (1) (0)
b Position and shape (3) (2) (1) (0)
of your urethral opening
c Shape of your glans (3) (2) (1) (0)
d Shape of your penile (3) (2) (1) (0)
skin
e Penile axis (straightness (3) (2) (1) (0)
upon erection
f General appearance (3) (2) (1) (0)
of your penis
Fig. 45.36.  Pediatric Penile Perception Score instrument is administered to
the patient and parents for assessment after hypospadias repair. Considerations
include cosmetic satisfaction with the length of the penis, meatus, glans,
penile skin, and straightness of the penis. (From Weber DM, Schonbucher
VB, Landolt MA, et al.: The Pediatric Penile Perception Score: an instrument
for patient self-assessment and surgeon evaluation after hypospadias repair.
J Urol 180(3):1080–1084, 2008.)

TABLE 45.2  Hypospadias Objective Penile Evaluation
(HOPE) Score
1. Position of meatus: Assess the position of the meatus.
See HOPE score reference pictures (see Fig. 45.37).
□ Position 1 (10 points)
□ Position 2 (8 points)
□ Position 3 (5 points)
□ Position 4 (3 points)
□ Position 5 (1 point)
2. Shape of meatus: What is the shape of the meatus?
See HOPE score reference pictures (see Fig. 45.37).
□ Normal (10 points)
□ Slightly abnormal (7 points)
□ Moderately abnormal (4 points)
□ Severely abnormal (1 point)
3. Shape of glans: What is the shape of the glans?
See HOPE score reference pictures (see Fig. 45.37).
□ Normal (10 points)
□ Slightly abnormal (7 points)
□ Moderately abnormal (4 points)
□ Severely abnormal (1 point)
4. Shape of skin: What is the shape of the penile skin?
See HOPE score reference pictures (see Fig. 45.37).
□ Normal (10 points)
□ Slightly abnormal (7 points)
□ Moderately abnormal (4 points)
□ Severely abnormal (1 point)
5.1 Torsion: Is there a torsion of the penis?
See HOPE score reference pictures (see Fig. 45.37).
□ 0–30 degrees (10 points)
□ 30–50 degrees (7 points)
□ 50–70 degrees (4 points)
□ >70 degrees (1 point)
5.2 Curvature in penile erection: Is there a curvature of the
penis in erection?
See HOPE score reference pictures (see Fig. 45.37).
□ No erection observed (5.2 does not account for the
HOPE score)
□ 0–30 degrees (10 points)
□ 30–50 degrees (7 points)
□ 50–70 degrees (4 points)
□ >70 degrees (1 point)
Hypospadias Objective Penile Evaluation (HOPE) score = mean number
of points question 1–5.
which may prove to be less important for patient and parent satisfac-
tion. Finally, they recognize that in spite of recognition of the need
for PRO, universal incorporation into outcome analysis has been
poor in the literature.
Although PRO regarding surgical and functional status is limited,
they become more evident when examining sexual and psychosocial
impacts. Although there has been a recent trend toward a focus on
longer-term follow-up through puberty, the majority of hypospadias
patients are still lost to follow-up. Assessment of 500 previously
undiagnosed men with hypospadias revealed 65 (13%) with glanular
to subcoronal hypospadias and 160 (32%) with anterior (proximal
glanular) displacement of the meatus (Fichtner et al., 1995). The
patients with previously undiagnosed hypospadias—all mild, distal
variants with no penile curvature—had minimal sexual, fertility, or
voiding concerns, with a number of men unaware that they even
had hypospadias. Another study identified 56 men (mean 53 years
of age) with hypospadias, 9 of whom had undergone previous repair
but had a persistent defect (Dodds et al., 2008). Of this group, 20
Chapter 45  Hypospadias 943
patients who had spraying or angulation to their urinary stream did
not elect to pursue repair. Eighteen of 56 men were unaware they
had an anomaly, 53 were sexually active without concern, and 41
of 43 attempted and fathered a child. Both of these studies suggest
that when the defect is mild, the patients readily adapt and can
compensate for the defect. On the contrary, men with more severe
forms of hypospadias, such as a more proximal location and
ventral curvature, do report worse sexual and functional outcomes
(Schlomer et al., 2014).
In boys who have undergone surgery, some studies have identified
psychological concerns. Parental survey of boys aged 6 to 10 years
of age identified behavior problems and poorer school performance,
which was significant when compared with normal controls but
also increased in boys with more severe defects (Sandberg et al.,
1989). In a series of 34 men who underwent hypospadias correction
at an average age of 5 years, in spite of normal sexual development
by age the men having undergone surgery were delayed in initiating
their first sexual encounter and were less sexually active in their
current relationships (Berg et al., 1981). A more contemporary series
comparing 167 men with repaired hypospadias with 169 age-
matched controls found that both groups had equal reported
fertility, sexual satisfaction, and age of initiation of sexual activity
(Ortqvist et al., 2017). Subgroup analysis did reveal a lower rate
of sexual satisfaction, worse fertility, and decreased sensation in
patients with proximal hypospadias compared with distal variants
and controls. An analysis of 55 men (ages 14 to 25) with a history
of proximal hypospadias repair found that these individuals had
concern about penile growth but little issue with the location of the
urethral meatus (Andersson et al., 2018). Although the majority of
these men did initiate sexual activity, a significant number of them
were hesitant about sexual physical contact compared with both
controls and those with distal hypospadias.
In general, men with a history of hypospadias repair do not
manifest major psychological problems. However, they tend to be
less satisfied with penile appearance, have more inhibitions in seeking
sexual contacts as a result of embarrassment, and have a more negative
genital appraisal. These men tend to manifest more anxiety, hostility,
and lower self-esteem, some of which may be ameliorated by operating
at an earlier age (Berg and Berg, 1983). What remains unclear is if
the dissatisfaction with the genitalia is due to the physical appearance,
the surgical procedure, development of complications, or limitations
in sexual relationships. A survey of individuals unaware of hypospadias
was conducted to determine their ability to identify a difference in
appearance between circumcised males and variable forms of
hypospadias (Ruppen-Greeff et al., 2016). They concluded that men
with distal hypospadias were perceived in a similar fashion to controls,
whereas those with proximal hypospadias did trend toward a perceiv-
able difference, although this effect was minor. Several studies
corroborate these findings, suggesting that the sexual and psychological
effects can occur but differ only minimally from controls for distal
variants but worsen with increasing severity of the defect, although
large-scale studies that incorporate validated PRO that encompass
all elements of the patient experience will be able to assess these
outcomes (Chertin et al., 2013; Jiao et al., 2011; Rynja et al., 2011).
Hypospadias Reoperations
Although some complications such as a simple fistula or skin
redundancy can be managed in a single intervention, others may
require a multistaged repair with a well-thought-out surgical plan.
The tissue available for reconstruction is variable. Its quality decreases
with each trip to the operating room. Each successive procedure
leaves the penis progressively scarred, changes the established blood
supply, and decreases tissue options for reconstruction, all of which
result in increasing complication rates with each unplanned operation
(Snodgrass and Bush, 2017b; Snodgrass et al., 2009). This can result
in a boy or man who has undergone repeated unsuccessful repairs,
remaining with a significant defect with poor function, a so-called
hypospadias cripple.
Devine reasoned that “once complications have occurred in
hypospadias repairs, the surgeon has a tendency to attempt small
944 PART III  Pediatric Urology

3. Shape of Glans:

Normal Slightly abnormal Moderately abnormal Severely abnormal

Normal Slightly abnormal Moderately abnormal Severely abnormal

Normal Slightly abnormal Moderately abnormal Severely abnormal

Normal Slightly abnormal Moderately abnormal Severely abnormal

Normal Slightly abnormal Moderately abnormal Severely abnormal

Fig. 45.37.  Reference pictures administered in conjunction with the HOPE scoring system (see Table
45.2) depicting various degrees of appearance ranging from normal to grossly abnormal. Anatomic
assessment includes the position and shape of the meatus, the shape of the glans, the penile skin, and
if any penile torsion and/or curvature is present. A numerical score for each of the 5 criteria ranges from
1 to 10, with a higher score given for a more normal appearance. (From van der Toorn F, de Jong TP,
de Gier RP, et al.: Introducing the HOPE [Hypospadias Objective Penile Evaluation]-score: a validation
study of an objective scoring system for evaluating cosmetic appearance in hypospadias patients. J
Pediatr Urol 9[6 Pt B]:1006–1016, 2013.)

KEY POINTS
• Sixty percent of urethroplasty complications occur within
1 year after surgery. Complications such as urethral
stricture or penile curvature can occur in a delayed fashion.
Thus boys, particularly those with proximal hypospadias,
should be monitored into puberty.
• Potential risk factors for complications after urethroplasty
include a proximal meatus location, glans width less than
14 mm, a lack of a barrier flap over the neourethra, and
having a prior hypospadias operation.
• Urethrocutaneous fistula is the most common
complication after hypospadias repair. If there is supple
tissue present, a primary closure can be attempted, but if
thin glans tissue is present, redo urethroplasty and glans
closure should be performed.
• Meatotomy or skin flap repair of meatal stenosis most
often fails in the presence of BXO, whereas excision of all
tissues affected by BXO with staged buccal grafting is
considered most likely to succeed without recurrent
stenosis. These patients have up to a 40% failure rate;
therefore they must be closely monitored.
• Long-term follow-up of patients who have had a
hypospadias repair indicates that they are more likely to
have ejaculatory problems, are less satisfied with sexual
function, and are more likely to be dissatisfied with the
appearance of their penis than controls. Patient-reported
outcomes are an underrepresented component of
follow-up and must be a focus of future studies.
operations, hoping to convert failure with a minimum of effort.
This generally makes matters worse. In these cases, extensive resection
of scarring, major shifts of tissues and meticulous reconstruction is
necessary to transform a difficult problem into a therapeutic success”
(Devine, 1973). Given the complexity of reoperative hypospadias
repair and its high complication rate, this quote serves as an important
guide to achieve the ultimate goal of a cosmetic and functional penis
(Myers et al., 2012).
The assessment begins in the office. A careful history identifies
any voiding, skin, and erectile concerns. The timing of previous
repairs and operative reports, if available, provide very important
information such as the previous use of tunica vaginalis flaps or
grafts, the method used for urethroplasty and correction of penile
curvature. In the operating room the surgeon should err on the side
of obtaining more information as opposed to less, having a low
threshold to perform a cystoscopy to fully delineate the anatomy.
The skin and available tissues must be carefully assessed for possible
usage, and an artificial erection should always be performed, regardless
of the type of repair performed, because if this goes undiagnosed,
it will have severe long-term consequences (Nozohoor Ekmark et al.,
2015; Safwat et al., 2013; Snodgrass et al., 2009). Because of the
variable nature of the non-virgin tissue, there is less certainty and
uniformity in the blood supply to flaps and local skin. Thus every
effort should be taken to add additional layers of coverage, such as
tunica vaginalis, to increase the chance of tissue survival.
Depending upon the initial approach to repair, tubularization
techniques can be used to redo urethroplasty or for fistula closure.
Mathieu, Thiersch Duplay, and redo TIP have been described in
instances when deemed appropriate by the surgeon with a reasonable
degree of success (Bar-Yosef et al., 2005; Borer et al., 2001; Nozohoor
Ekmark et al., 2015; Simmons et al., 1999). Alternatively, placement
of an inlay graft after incision of the urethral plate can improve
outcomes in reoperative hypospadias, although surgeons must use
caution when using tissue that has already failed closure in the past
and now has a more variable blood supply (Kolon and Gonzales,
2000; Snodgrass et al., 2009; White and Hanna, 2018).
Salvage hypospadias for a focal issue (fistula, diverticulum) in the
absence of penile curvature can be performed using local or remote
Chapter 45  Hypospadias 945
skin flaps, which can be used as an onlay flap or for multilayer
vascular flaps (Nozohoor Ekmark et al., 2015; Patel et al., 2005;
Snodgrass et al., 2009). We typically take this approach when
redundant penile shaft skin is present from the original repair
as it provides a source of non–hair-bearing skin. Our experience
with the split onlay skin (SOS) flap is such that even a small
piece of skin on a vascularized pedicle can achieve its intended
effect (Fig. 45.38). Once harvested, the flap can be used in its
entirety as an onlay to augment the urethral plate for closure or
split to provide additional skin coverage for shaft skin closure
or de-epithelialized for additional supplemental layers over the
urethroplasty.
Pharmacotherapy has been used to augment neovascularization
or to improve local blood flow, potentially increasing flap and graft
uptake. Topical nitroglycerin ointment can be applied after surgical
reconstruction, typically for a period of 24 hours immediately after
the repair (White and Hanna, 2018). Nitroglycerin ointment exerts
its effect on the arterial and venous systems, with a larger impact
on the latter, and has been shown to increase blood flow to skin
flaps as evidenced by fluorescein imaging, promoting improved
survival (Scheuer and Hanna, 1986). It has been used in complex
hypospadias reconstruction and may provide benefit to ischemic
tissues, although it has yet to be compared in a controlled, random-
ized fashion to determine its definitive impact (Fam and Hanna,
2017; White and Hanna, 2018).
Hyperbaric oxygen (HO) has established clinical benefits in the
setting of gas gangrene, radiation-induced necrosis, and diabetic foot
ulcers, and has been proposed as a potential adjunct to promote
neovascularization in the use of ischemic skin flaps and grafts (Fried-
man et al., 2006). HO promotes new vessel growth because of an
exaggerated oxygen gradient and a reduction in tissue bacterial content
(Park et al., 1992; Sander et al., 2009; Shaw et al., 2014). Its role in
reoperative hypospadias is not yet established. Anecdotal reports are
beginning to emerge about its potential benefits in graft take and
improved vascularization (Kara et al., 2017; White and Hanna, 2018).
Subcutaneous tissue expansion allows the surgeon to augment
autologous, non–hair-bearing skin for penile reconstruction in salvage
procedures when scar tissue or lack of available skin threatens to
compromise the reconstruction (Kajbafzadeh et al., 2007; Mir et al.,
2011; Rochlin et al., 2014). A subcutaneous pocket is created under
the penile shaft skin and a commercial, expandable device is inserted
and inflated with saline. Additional saline is injected via a subinguinal
injection port at 1- to 2-week intervals to gently dilate the tissue
until sufficient tissue is developed. At the time of reconstruction,
the device is removed and flaps of the expanded skin are rotated
ventrally for penile shaft skin coverage or de-epithelialized to provide
additional vascularized layers over the urethroplasty. One potential
benefit proposed by authors is the preservation of androgen receptor–
bearing shaft skin, which could prove a key factor in longitudinal
growth during puberty (Celayir et al., 2007; Kajbafzadeh et al., 2007;
Rochlin et al., 2014). In the largest study, Rochlin et al. (2014)
operated on 42 patients with hypospadias. Although complications
developed in 41%, including a 28% complication rate related specifi-
cally to the tissue expander, tissue expansion provided sufficient
skin for reconstruction in 95% of these patients.
Extragenital skin harvest is another option in the setting of salvage
hypospadias repair. A graft is harvested and placed in a vascular bed
on the ventrum of the penis, to augment or completely replace the
urethral tissue. Several donor sites exist and have been used with
variable success. Single-stage repairs with full-thickness skin grafts
have frequently failed secondary to graft shrinkage, scarring, and
stricture formation, limiting their use (Amukele et al., 2005; Webster
et al., 1984).
Bladder mucosal grafts can be harvested and used to replace the
urethral plate in cases where skin is damaged or scarce (Ehrlich et al.,
1989; King, 1994; Memmelaar, 1947). Bladder mucosa contracts when
exposed to air, which typically leads to narrowing of the neomeatus.
Prolapse of the graft resulting in a cauliflower-type deformity is
reported in up to 38% of patients. Meatal stenosis and stricture at
the junction with the native urethra occurs frequently (Amukele
et al., 2005; Hendren and Reda, 1986; Keating et al., 1990; Kinkead
946 PART III  Pediatric Urology

A B C

D E
Fig. 45.38.  Split onlay skin flap hypospadias repair technique. (A) A fistula is identified on the ventrum of
the penis. (B) A skin flap approximately 1 cm in length is isolated and harvested, preserving its vascular
pedicle. The flap can be harvested from any location with skin redundancy, either on the penile shaft or
along the circumcision line. (C) Flap is divided into two segments, one to be used as an onlay for the urethral
closure and the second either as a barrier layer or to facilitate skin closure. (D) Island onlay procedure is
performed, closing the fistula. (E) Residual penile skin is approximated dorsally and the second half of the
island flap is interposed to close the penile shaft skin. (From Patel RP, Shukla AR, Leone NT, et al: Split
onlay skin flap for the salvage hypospadias repair. J Urol 173:1718–1720, 2005.)

et al., 1994). Although technical modifications may decrease the
complication rate, extending its applicability, more recent success
with buccal mucosa grafts has limited use of bladder mucosa
(Ehrlich et al., 1989).
Buccal mucosa is now the graft of choice in complex penile
reconstruction (Brock, 1994; Bracka, 1995; Duckett et al., 1995).
The tissue is ideally suited for urethral repair because it is well adapted
to contact with fluid and air; it readily neovascularizes and is hairless;
contraction is minimal; and harvest results in little damage at the
donor site (Castagnetti et al., 2008). Contrary to other grafts, it
retains its favorable histologic properties after placement (Mokhless
et al., 2007; Soave et al., 2014). It also grows well in proportion to
the phallus through puberty (Figueroa et al., 2014). Because of its
elasticity and good tensile strength, buccal mucosa can be used to
reconstruct the urethra in a single or multistage fashion, although
a more conservative approach across two stages, allowing the graft
to establish a blood supply before tubularization, is optimal (Faure
et al., 2016; Ye et al., 2008). Long-term observation into puberty
shows that if the graft is placed on a well-vascularized bed, the buccal
graft is pliable and will not contract with age, an important aspect
of management as we aim to avoid ventral penile curvature, which
can result from a shortened, tethered urethra (Figueroa et al., 2014).
Buccal mucosa can be harvested from the cheek, inner lip, or both.
Reported local complications in men who have had buccal harvests
include tightness of the mouth, transient numbness, motor deficits,
scarring, and salivary duct obstruction (Wood et al., 2004).
Although single-stage buccal mucosa graft and tubularization
can be performed and has been reported, the staged repair results
in a higher success rate with improved graft incorporation (Hensle
et al., 2002; White and Hanna, 2018). A dorsal inlay graft, in which
the dorsal portion of the urethra is incised and the graft sutured in
place, is a better alternative to either an onlay or a complete buccal
tube, especially in the setting in redo repairs in which blood supply
may be compromised (see Fig. 45.28).
Buccal mucosa taken from the upper or lower lip or tongue is
thinner than that from the cheek and outcomes suggest that these
grafts are better suited for urethral reconstruction, particularly in
the glans. Local complications at the harvest site are no different for
a lip or cheek location (Maarouf et al., 2013).
There are several important technical points when harvesting a
buccal mucosa graft (Eppley et al., 1997). The graft should be
harvested 10% larger than the measured receiving bed to allow
for graft contraction as it incorporates. Closure of the donor site
may result in worsened postoperative pain with little objective
benefit, because the sites heal by secondary intention (Alwaal
et al., 2015; Rourke et al., 2012). Although both are viable options
for reconstruction, a labial graft appears to have fewer complica-
tions when compared with the inner cheek buccal mucosa, par-
ticularly an increased risk for glans dehiscence with cheek mucosa
(Lumen et al., 2016; Maarouf et al., 2013; Snodgrass et al., 2009).
For longer defects in complex cases or in older children, buccal
mucosa can be used because it allows for a larger graft size, although
multiple sites can be combined to maximize tissue harvest (Morey
and McAninch, 1996). Strict attention to de-fatting and removing
all nonmucosal tissue is important to maximize neovascularization.
The graft can be fenestrated with an 18- or 21-gauge needle and
placed in saline until the recipient site is prepared. Fenestration is
thought to decrease hematoma formation, which can impede
graft take. The recipient site is prepared by making an incision
ventrally from the urethra through the glans to the site of the
intended neomeatus (Mokhless et al., 2007). Care should be taken
to develop the recipient bed to preserve the underlying dartos,
 Chapter 45  Hypospadias 947

as this acts as the blood supply for graft uptake. The graft is secured
along its axis, taking care not to narrow the graft, which could lead
to insufficient tissue for urethroplasty. Quilting stitches are typically
placed to prevent hematoma formation. The graft should be given
at least 6 months to heal before proceeding with urethroplasty
and skin closure, although it is our practice to wait 12 months
before the next stage in reconstruction. Regardless, periodic examina-
tion in the office must be performed to ensure proper graft take and
to monitor for contraction. Topical steroids can be considered in
the setting of some contraction in the postoperative period, waiting
at least 1 month after placement. Outcomes after completion of the
repair range from 30% to 75% success rate, with meatal stenosis
the most common complication followed by stricture and breakdown
of repair (Leslie et al., 2011; Metro et al., 2001).
The scrotum provides a rich vascular bed that can be taken
advantage of in salvage hypospadias repairs. Originally described in
1946 by Cecil and modified by Culp (1951), it includes urethroplasty
followed by midline scrotal incision and recession of the repair
within the supple dartos bed. The penis is released at a second stage,
originally reported by Cecil at 6 to 8 weeks, with the urethroplasty
having parasitized the local blood supply, which aids the healing
process of the urethra. Although this repair fell out of favor with
the emergence of single-stage repair and alternative techniques,
it has re-emerged in the setting of difficult salvage procedures
(Fig. 45.39; Ehle et al., 2001; Fam and Hanna, 2017; Weiss et al., 2018).
Concerns with this approach include the potential incorporation of
hair-bearing scrotal skin on the penile shaft and the need for multiple
procedures. Weiss et al. (2018) have modified the approach by
delaying penis release from the scrotum for a period of 9 to 12
months, increasing the transfer of secondary vascularized layers to
the shaft, which in most cases eliminates the need for utilization
of scrotal skin after the release of the scrotal flap.

A B C

D E F

Fig. 45.39.  Cecil-Culp repair for hypospadias fistula. (A) Initial appearance of a boy with a history of what
was originally a penoscrotal hypospadias. He underwent a failed two-stage repair followed by buccal
graft and re-ubularization. He came to us with two coronal fistulae and an irregular glans. (B) Intraoperative
appearance after closure of fistulae and glans but a ventral skin defect (*) was present, precluding tension-
free skin closure. A midline incision was made in the scrotum as the receiving site for the distal penile
shaft anastomosis (white arrow). (C) Attachment of the penile shaft skin to the scrotal skin to cover the
defect. (D) Approximately 1 year after Cecil-Culp placement, scar has softened and penile shaft skin has
stretched. (E) Appearance after Cecil-Culp release, with midline anastomosis of penile shaft skin. (F) Three
months postoperation from Cecil-Culp release. Glans is closed and there is no evidence of recurrent
fistula. (From Weiss DA, Long CJ, Frazier JR, et al: Back to the future: Cecil-Culp technique for salvage
penile reconstructive procedures. J Pediatr Urol 14[4]:328.e1–328.e7, 2018.)
948 PART III  Pediatric Urology

KEY POINTS
• For buccal mucosal reconstruction, lip tissue placement is
preferable within the glans because it is thinner than cheek
tissue and results in a lower complication rate.
• Buccal mucosa is now the graft of choice in complex
penile reconstruction. The tissue is ideally suited for
urethral repair because it is well adapted to contact with
fluid and air, it readily neovascularizes and is hairless,
contraction is minimal, and harvest results in little damage
at the donor site. Contrary to other grafts, it retains its
favorable histologic properties after placement.
• The Cecil modification provides an attractive alternative
for skin vascularization in the setting of multiple prior
repairs.

FUTURE
As we have outlined in this chapter, there is room for improvement,
the first step of which will depend on continued honest report-
ing of our results. Reduction of complication rates will occur
through standardization, allowing us to increase comparison
across institutions with larger volume series and longer follow-
up and incorporation of patient-reported outcomes. To facilitate
comparison within institutions (among surgeons) and across institu-
tions, standardization of pre- intra-, and postoperative assessments
according to exam, measurements, and outcome definitions must
be agreed upon and accepted.
This should lead to establishment of multi-institutional collabora-
tive efforts focused on identifying potential sources of complications
that we are currently underpowered to assess with single-institution
efforts (Joshi et al., 2018).
The fundamental aspect of this improvement process will be
coaching and teamwork. Coaching is used in athletics to maximize
performance and perfection of technique, and we feel strongly that
this approach can be applied to the more rare or difficult disease
processes to improve our approach and outcomes, particularly for
proximal hypospadias (Borer et al., 2015; Gawande, 2011).
Much work remains to be completed in the advancement of
surgical reconstruction of severe hypospadias (Long and Canning,
2016b). These efforts must focus on improving the relatively high
complication rate, extending follow-up into adulthood, and using
long-term analysis to guide patient and parental expectations of
the repair.
Clinical questions such as what is the ideal location of urethral
meatus, what degree of penile curvature is cosmetically and function-
ally acceptable, and to what extent are boys willing to undergo painful
and complicated procedures to achieve an ideal result still await an
answer. This patient, with a history of proximal hypospadias repair
left with a coronal meatus yet with good voiding parameters and
an otherwise straight penis, has a satisfactory quality of life in spite
of the meatus location (Fig. 45.40). Is that the case for all such
patients? Other questions similarly await further study, such as how
best to study the long-term psychological challenges of living with
the birth defect and the ensuing penile reconstruction with the
potential for multiple repairs. Perhaps another may be most important
of all: How can we maximize our patient and parental education
so that they are prepared for the road ahead?
As we clamor to standardize our surgical interventions, we are
limited until we can objectify our outcome measures across
institutions. To this end, technology will play an increasing role.
Preoperative, intraoperative, and postoperative videography with
the potential for “crowd sourcing” can aid in severity assessment
and long-term assessment of outcomes. Recording and cataloging
of videotaped procedures, combined with artificial intelligence
or machine learning, allows what was once a single surgeon’s
subjective assessment of a boy’s outcome to be measured across
thousands of patients. As personalized medicine continues to evolve
and it becomes easier to perform routine genetic, proteomic, and
microbiome analysis, one can imagine the potential for a refined
Fig. 45.40.  Boy with a history of a two-stage repair resulting from a proximal
hypospadias and severe ventral penile curvature. His meatus is located at the
coronal margin, but his penis is straight and he voids without any spraying.
physical and risk assessment for the boy while planning surgery.
Those who take on these challenges will be the hypospadiologists
of the future.
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