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Management of Hypospadias

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

 

Correspondence

Gianantonio Manzoni, MD, FEAPU;

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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 child’s 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, day–case 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.

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

management hyposp A

A.

management hyposp B

B.

management hyposp C

C.

management hyposp D

D.

management hyposp E

E.

management hyposp F

F.

Fig. 2. Posterior form: extended Snodgrass repair
a. peno-scrotal hypospadias

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 non-pliable 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 patient’s 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].

hypospadias a

A.

hypospadias b

B.

hypospadias c

C.

hypospadias d

D.

hypospadias e

E.

hypospadias f

F.

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

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

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

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. 

hypospadias 4a

A.

hypospadias 4b

B.

hypospadias 4c

C.

hypospadias 4d

D.

Fig. 4. 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 

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.

hypospadias 5a

A.

hypospadias 5b

B.

hypospadias 5c

C.

hypospadias 5d

D.

hypospadias 5e

E.

hypospadias 5f

F.

hypospadias 5g

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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