Management of Hypospadias

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G.A.Manzoni, L.Reali
Varese, Italy

Correspondence

Gianantonio Manzoni, MD, FEAPU;

e-mail: gianantoniomanzoni@ospedale.varese.it
Department of Urology and Section of Pediatric Urology,
Ospedale di Circolo e Fondazione Macchi Varese Italy

Introduction

Hypospadias is one of the most common malformations of male genitalia, with an increasing
incidence [1], and may be as high as 8 in 1000 male births. Aetiologically both genetic and
environmental factors are implied and numerous theories have been proposed about both the
cause and the changing prevalence [2,3] . There is no single satisfactory way of classifying
hypospadias. Despite obvious limitations, pre-operative meatal position remains the most
commonly used criterion. By this classification, at least 70% of hypospadias is either
glandular or distal penile, 10% mid penile, and 20% more severe proximal types.
In Table 1 and Fig. 1 are reported the principal anatomical variables associated with the
spectrum of hypospadias severity, and lists the expected findings. Unfortunately hypospadias
deformities do not necessarily conform to these expectations, so this is only a broad
generalisation. The position of the meatus alone is therefore not a reliable indicator of
hypospadias severity as far as choice of appropriate surgical correction is concerned. A distal
hypospadias may in fact have severe curvature with a poorly developed urethral plate and
glans groove, whilst a proximal hypospadias may have the opposite features.
We therefore propose to determine our surgical protocol more by these other anatomical
variables, in particular the quality of the urethral plate, the glans configuration, and degree
and type of curvature. With this new approach the confusing and vast spectrum of available
repairs can be limited to a simple and logical progression of just a few related procedures [4].
Timing of surgery

Initial assessment ideally should be performed in the first few weeks of life. This can reassure
the parents and at the same time can establish an important bond between them and the

surgeon, quite important to the future management. When considering the timing of surgical
repair several factors should be considered: the local environment, the anaesthetic risk, the
penile size and the psychological implications of genital surgery.
It is recognized that after the age of 6 months the risk of anesthesia is no greater than later in
life [5] when the patient is cared by a pediatric and dedicated anesthetist in the appropriate
institution. In the first few years of development moderate penile growth occurs, therefore
penile size is not a limiting factor and there are no benefits in delaying the reconstructive
surgery. Only in the presence of a very small phallus, the use of hormonal stimulation to
achieve penile enlargement can be considered. This can be achieved either with an
intramuscular injection of testosterone enanthate (25mg) or with topical di-hydro-testosterone
cream, applied daily usually for one month before surgery [6].
Recommendations from the Section of Urology of the American Academy of Pediatrics now
suggest that the optimal time for elective reconstructive genital surgery is either in the second
six months of life or sometime later, around the fourth year of life [7,8]. Genital awareness is
starting after 18 months of age contemporary to a quite difficult and uncooperative
behavioural phase of the childs development. The worse time for hospitalisation with usually
a very low compliance and limited collaboration is between the age of 2 and 3 years. After
that the child becomes sufficiently mature to collaborate with his treatment, providing a
second window of opportunity for a hypospadias repair. This seems a more realistic and
workable option for those surgeons without specialised pediatric facilities and less prone to
undertake surgery during the first year of life.
Surgical treatment

In many countries with advanced medical and social development, the actual trend is towards
earlier intervention with ever-shorter hospitalisation. The norm in many centres is now for a
single-stage repair during the first year of life undertaken as a day-case. Early, daycase
repair may be a safe, realistic and desirable proposition when sophisticated surgery and
anaesthesia can be combined with high standards of community aftercare. It should be
practically recognised however that in many parts of the world, even in developed countries,
this ideal cannot be achieved for a variety of reasons; therefore preschool surgery and longer
periods of hospitalisation may represent a more realistic option. With constant advances in
surgical techniques and suture materials, use of optical magnification and microsurgical
instrumentation, hypospadias repair has evolved into a safe and reliable procedure with a very
high reported success rate. A further requirement is the routine use of intra-operative caudal
or penile local anaesthetic blocks as part of an effective postoperative analgesic regime.

Fig. 1. Anatomical variables in hypospadias complex (from BJUInt 2004)

A.

B.

C.

D.

E.

F.

Posterior form: extended Snodgrass repair

a. peno-scrotal hypospadias
Fig. 2.

b. urethral plate outlined

c. ventral curvature corrected with additional dorsal TAP
d. - e. hinging of the urethral plate f. final outcome
Patient co-operation is not so crucial in very young infants and an open system with a
dripping stent and double diaper method of diversion will adequately contain the urine
drainage and also prevent the child from interfering with the operation site. A wide lumen
indwelling silicone Foley catheter is preferred in the older patient, ranging from 8F in a child
through to 12F in an adult. These days, supra-pubic diversion is seldom justified in the
pediatric population.
The choice of dressings, addition of prophylactic antibiotics and decisions about urinary
diversion are not universally agreed. These remain areas of individual surgeon preference,
influenced by the severity of the hypospadias and the type of surgical repair employed.
Modern surgical repair of hypospadias requires an experienced dedicated specialist, whether
a paediatric urologist/surgeon, a plastic surgeon, (or an adult reconstructive urologist). This is
not reconstructive surgery for the occasional operator; therefore a standard practice of at least
40 to 50 cases per year is desirable.
Selecting the surgical procedure

While in the past, functional improvement was considered a successful outcome in

hypospadias surgery, the primary goal of modern reconstruction, however, is to achieve both
functional and cosmetic normalcy. Whether this is reached by a single procedure or with a
multistaged approach it requires the creation of a straight penis, with a neo-urethra of normal
caliber and with a slit-like meatus at the apex of a naturally reconfigured glans. No attempt
should be made to underscore the complexity of hypospadias repair, and the benefits of
correction should always outweigh the potential risks. The choice of the technique is
determined by the anatomical characteristics previously described (Fig. 1). A careful
anatomical evaluation is therefore very important before any surgical decision is made. The
location of the urethral neo-meatus and its final cosmetic appearance will be determined by
the initial glans configuration and the depth of the urethral plate and groove. The shape of the
glans, such as whether it is flat, conical or grooved will condition the selection of the type of
repair. The same evaluation applies also to the urethral plate which may be flat and nonpliable or inadequately projecting to the tip of the glans.
The very distal forms, which account for the vast majority of hypospadias, is sometimes the
most challenging in terms of the decision making process, because cosmesis is often the only
real indication for treatment. Foreskin preservation and reconstruction continues to be a very
controversial issue. Reconstruction of the prepuce may well impact on the patients
perception of normality following hypospadias repair if there is a strong cultural preference
for an uncircumcised appearance. Whilst a prepuceplasty can certainly be attempted when
there is a favourable penile skin configuration [9], there are as yet no published long-term
data concerning either cosmesis or functionality in the adult. What proportion of these
reconstructed foreskins will retract normally and function sexually remains still unknown. At
the other end of the spectrum, perineal hypospadias represents the most challenging and
technically demanding surgical exercise, involving both urethral reconstruction and
correction of penile curvature and variable degrees of peno-scrotal transposition. Despite a
large variety of reconstructive techniques now available, the authors feel that a simple and

reliable protocol can be applied mostly based on the quality and development of the urethral
plate, rather than the preoperative location of the meatus.
1. stage repair - A. urethral plate tubularisation (GAP, Snodgrass); B. urethral plate
augmentation (onlay flap, Snod-graft)
2. stage repair - A. urethral plate substitution (Bracka)
Urethral plate tubularization

The urethral plate can be tubularized when its axial integrity can be maintained and there is
no need to transect it. If the plate is of adequate width and depth, according to the technique
described by Zoaonz (GAP procedure) it can be tubed directly [10]. Conversely, when the
plate is not adequately developed and requires width/depth enhancement before it can be
tabularised, the addition of a midline deep dorsal releasing incision is performed according to
the Snodgrass procedure [11]. This tubularised incised plate (TIP) repair was first described
in 1994, and has initially gained worldwide popularity as a solution for distal primary
hypospadias. It has subsequently also gained acceptance for suitable proximal forms of
hypospadias (Fig. 2) and, more recently, even for selective use in re-operations [12].

A.

B.

C.

D.

F.

E.
Mid form: onlay repair
a. poor urethral plate
Fig. 3.

b. urethra spatulated
c. dorsal preputial island flap
d. - e. OIF in situ
f. final outcome
There are still concerns regarding the potential for stricture development which have not been
substantiated, at least in the short-term, and the Snodgrass repair is currently providing
superior cosmetic and functional results compared to other techniques.
Urethral plate augmentation

In the presence of narrow and inelastic urethral plates, the potential enhancement of width
achieved by the midline releasing incision is inadequate and it is mandatory to produce a
more substantial augmentation. This situation can apply to distal hypospadias, but more
particularly to severe penile forms where the application of an extended Snodgrass procedure
may generate concerns for the long-term outcome. The onlay preputial island flap, as
popularised by Duckett [13] can be safely performed in the vast majority of these cases, with
or without penile curvature and remains for many surgeons still the ideal solution. (Fig. 3)
Table 1.

(from BJUInt 2004) Anatomical variables in hypospadias

well /poorly developed
absent
shallow + conical
Glans and groove configuration
deep + well developed
well developed / hypoplastic
Urethral plate
broad / narrow
Penile size
normal / reduced
Curvature
present / absent
Meatal position
normal / hypoplastic
Scrotum
bifid / transposed
Foreskin

However, as an alternative, there is a more recently developed and increasingly popular

concept: the Snod-graft repair. [14] This represents a logical progression of the original
Snodgrass principle, wherein a free graft (with prepuce if available or buccal mucosa) is
quilted into the dorsal defect rather than leaving it to epithelialise. This is an excellent and
useful procedure when the glans configuration is more conical, with a minimal groove and
lacking the usual external rotation of the glans wings. To achieve an apical meatus would in
this instance necessitate extension of the Snodgrass dorsal releasing incision beyond the distal
limit of the glans groove and thereby invite a meatal stricture, unless the defect is grafted.
The novel Snod-graft concept is usually less indicated for primary repairs but is
particularly useful for redo salvage cases (Fig. 4).
Urethral plate substitution

With severe proximal forms, in the presence of significant ventral curvature, urethral plate
transection becomes inevitable, and a total substitution urethroplasty is then required. Single
stage tubularised repairs, the most popular being the Duckett TPIF [15], have been largely
rejected because of their prohibitive long-term complication rate. Over the last few years the
forced concept of a single-stage repair has been abandoned in favour of a 2-stage procedure
such as described by Bracka [16] which is now regarded by many as a better option. When
still available (primary cases) the inner preputial skin layer is used as a free full thickness
(Wolfe) graft (Fig. 5). Conversely when the prepuce is poorly developed or absent because of
circumcision, then buccal mucosa or non-genital skin can be used either in addition to
prepuce or as an alternative to it.

A.

B.

C.

D.

Hypospadias failure: "Snod-graft" repair with buccal mucosa free graft

a. midline incision of scarred urethral plate
b. dorsal urethral defect
c. buccal mucosa free graft quilted into the dorsal defect
d. final outcome
Fig. 4.

This approach allows for an excellent release of ventral chordee tissue and maximizes penile
length preservation. Remaining inherent corporeal disproportion may however still require
correction by a dorsal procedure (Nesbit, TAP). Consideration may be given to ventral tunica
release and lengthening with dermal or tunica vaginalis grafts in the presence of unacceptable
shortening of an already hypoplastic organ [17,18]. Longterm published data are still lacking
however and some caution is required, because erectile dysfunction is a well recognised
complication in adults who undergo tunicagrafting procedures for curvature correction.

A.

B.

C.

D.

E.

F.

G.
Fig. 5. Severe form : two-stage Bracka repair
a. scrotal hypospadias with severe curvature
b. 1st stage (dorsal prepuce free-graft)
c. 6 months post-op
d. - f. 2nd stage closure
g. final outcome (modified from BJUInt 2004)
Follow-up protocol

It is stressed the absolute importance of an adequate long-term follow in all hypospadias

patients. It has been assumed that patients will themselves seek review for the few problems
that may subsequently, if ever, arise. Therefore early discharge has also been justified on the
grounds that it is best to let the patient forget that he had a genital abnormality; as repeatedly
bringing the fact to his attention might actually generate psychological concerns. Traditional
thinking has been that any significant complications will most likely have presented within
the first two years after surgery and therefore follow-up beyond this time is not cost effective.
Conversely strong evidence from adult studies [19, 20], clearly refutes these wrong
assumptions and shows that early discharge is just a convenient way to underestimate the true
complication rate.
An ideal protocol should include an early evaluation within 3 months of surgery, followed by
a review at 1or 2 years, and again at 4 or 5 years. The quality of micturition should be
assessed subjectively, and when possible confirmed objectively with uroflowmetry and
perhaps a bladder pre and post-micturition ultrasound evaluation. With the onset of rapid
growth at puberty there is potential for new problems to arise. The patient should therefore be
reassessed at puberty and again at around mid-teens, by which time genital maturation will be
at, or near completion and the patient is able to comment about social and sexual aspects of
his penile surgery. [21,22]
Conclusions

A simple and reliable protocol for the correction of almost all primary (and redo) hypospadias
is presented by using only a very few logically related surgical procedures. Once again it is
confirmed that there is nothing new in hypospadias surgery and mainly this protocol is
based on the quality and development of the urethral plate, rather than the pre-operative
location of the urethral meatus.

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