Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
948
Update Article
HYPOSPADIAS SURGERY
MANZONI
et al.
SUMMARY traditionally quoted incidence of 1 in 300 over 200 different syndromes [5]. Associated
male births. However, epidemiological malformations of the urinary tract are most
Hypospadias is repaired by paediatric evidence suggests that in developed Western common in proximal or complex hypospadias,
surgeons, paediatric urologists, adult countries the incidence is increasing [1], and but their incidence in distal forms is no
reconstructive urologists and plastic may be as high as 8 in 1000 male births. Both different from that of the general population.
surgeons. This review is unique in genetic and environmental factors are The most frequently encountered anomalies
representing all four specialities, to provide a implicated in the cause and numerous are
unified policy on the management of theories have been proposed about both the
hypospadias. The surgeon of whichever cause and the changing prevalence [2–4], but • Inguinal hernia.
speciality should have a dedicated interest in discussion of these falls outside the remit of • Undescended testis.
this challenging work, ideally having an this review. • PUJ obstruction.
annual volume of at least 40–50 cases. The • Renal agenesis.
ideal time for primary repair is at 6–12 There is no single satisfactory way of • VUR.
months old, although when this is not classifying hypospadias. Despite obvious • Persistent Müllerian structures.
practicable there is another opportunity at limitations, preoperative meatal position • Intersex states.
3–4 years old. A surgical protocol is presented remains the most commonly used criterion.
which emphasises both functional and By this classification at least 70% of It is therefore unnecessary to undertake
cosmetic refinement. Using a logical hypospadias is either glanular or distal penile, formal investigation of the urinary tract for
progression of a very few related procedures 10% mid-penile, and 20% the more severe simple distal hypospadias, unless associated
allows the reliable correction of almost any proximal types. with unexplained symptoms. However,
hypospadias deformity. A one-stage repair is proximal hypospadias requires a more
used when the urethral plate does not require Table 1 and Fig. 1 summarize the principal thorough evaluation. If one or both testes are
transection and its axial integrity can be anatomical variables associated with the impalpable, it may signify the presence of an
maintained. Occasionally, when the plate is of spectrum of hypospadias severity, and list the intersex condition such as adrenogenital
adequate width and depth, it can be expected findings. Unfortunately hypospadias syndrome or a mixed gonadal dysgenesis.
tubularized directly using the second stage of deformities do not necessarily conform to In this instance there should always be a
the two-stage repair. When (usually) the these expectations, so this is only a broad karyotype study and ultrasonography of
urethral plate is not adequately developed generalization. The position of the meatus the urinary tract and internal genital organs
and requires augmentation before it can be alone is therefore not a reliable indicator of [6].
tubularized, then that second-stage hypospadias severity as far as the choice of an
procedure is modified by adding a dorsal appropriate surgical correction is concerned. Furthermore, endoscopic examination of the
releasing incision ± a graft (alias Snodgrass Occasionally, a distal hypospadias may have urethra at the time of surgery is necessary to
and ‘Snodgraft’ procedures). The two-stage severe curvature with a poorly developed exclude the presence of a Müllerian remnant
repair offers the most reliable and refined urethral plate and glans groove, whilst a (dilated utriculus). Accurate assessment of the
solution for those patients who require proximal hypospadias may have the opposite type of hypospadias, for severity of curvature
transection of the urethral plate and a full features. and the urethral quality, is often possible only
circumferential substitution urethroplasty. with the patient under anaesthesia, and this
From available evidence this protocol Our proposed surgical protocol is determined may therefore lead to modification of the
combines excellent function and cosmesis more by these other anatomical variables, in surgical plan.
with optimum reliability. Nevertheless, it particular the quality of the urethral plate, the
would be complacent to assume that these glans configuration, and degree and type of
gratifying results will be maintained into curvature. This has allowed the confusing and THE TIMING OF SURGICAL REPAIR
adult life. We therefore recommend that there vast array of available repairs to be narrowed
is still a need for active follow-up through to to a simple and logical progression of just a Whenever possible it is helpful to assess the
genital maturity. few related procedures. problem during the first few weeks of life. This
reduces the parental guilt and ‘fear of the
unknown’ that is typically associated with
INTRODUCTION PREOPERATIVE EVALUATION congenital birth defects, whilst at the same
time establishing a bond between the parents
Hypospadias is one of the most common Hypospadias is generally an isolated anomaly and the surgeon that is instrumental to future
malformations of male genitalia, with a but it may represent one of the features of management.
11 8 8 © 2 0 0 4 B J U I N T E R N A T I O N A L | 9 4 , 11 8 8 – 11 9 5 | doi:10.1111/j.1464-410X.2004.05128.x
HYPOSPADIAS SURGERY
SURGICAL TREATMENT
FIG. 3. Primary Bracka two-stage repair for proximal hypospadias: A,B, first stage with inner preputial layer concerns. Evidence from adult studies, cited
free graft; C,D, second stage after 6 months. below, clearly refute these assumptions and
show that early discharge is just a convenient
way to ‘sweep problems under the carpet’ and
allow for flattering ‘re-operation rates’ which
bear little relation to the true complication
rate.
actively follow their patients. Instead, the FIG. 4. Retrograde and voiding cysto-urethrogram: A, stricture and diverticulum of the neourethra; B, an
responsibility is placed with the family to unrecognized and unrelated proximal stricture; C, enlarged prostatic utriculus; D, a hairy urethra.
request a review appointment if and when
problems arise, or when the boy reaches
maturity. The large adult review study by
Bracka [25] shows this to be a naive policy.
About half the patients in that study took the
opportunity to have their hypospadias repairs
surgically revised, even though they had
originally been discharged in childhood with
an apparently satisfactory outcome. Had it
not been for the request to return for review,
few of these young adults would have sought
help by their own initiative, either because of
embarrassment, resignation or ignorance that
further improvement was possible.
FIG. 5. A ‘Snod-graft’ repair with buccal mucosal free graft; A–C, diagrammatic outline; D, midline dorsal incision of the urethral plate; E, a free graft quilted into the
dorsal defect; F, initial tubularization of the augmented neourethra.
A B C
D E F
medical profession. Spending quality time trauma can be reduced by using perioperative dartos fascia ‘waterproofing’ flap between the
addressing their socio-sexual concerns and antiandrogen therapy or topical cold sprays urethra and skin, or using advancement or
aspirations, in addition to providing adequate [20]. rotation of skin flaps to offset the skin
clinical information about the salvage surgical closure.
options, is an essential part of the trust- ONE-STAGE REPEAT SURGERY
building and rehabilitation process. Some Whilst the Snodgrass TIP repair has
surgeons may delegate part of this process to As with primary hypospadias repair, when revolutionized the primary correction
a sympathetic body-image counsellor or the axial integrity of the neourethra can of hypospadias, it is less well suited to
clinical psychologist. be maintained for at least part of its repeat urethral augmentation. First, the
circumference (e.g. fistula repair, urethral surgeon is likely to be incising into previously
The principles of salvage surgery are not reduction or augmentation stricture-plasty), operated, less vascular tissues that will
dissimilar to those already described for then repair can be safely effected in a single therefore have a greater propensity to heal
primary repair, but faced with already scarred, operation. by contraction and scarring. Furthermore,
less vascular and perhaps deficient tissues, whilst the size of the dorsal wall defect
this surgery is often a greater technical Simple fistula repair is usually successful may be proportionately the same as for
challenge and not surprisingly carries a provided that there is no associated problem primary repair, in absolute terms the size
somewhat higher complication rate. with the calibre of the urethra, and that of defect that is required to re-epithelialize
Postoperative erections can be distressing attention is paid to separating suture lines. will be much larger in an adult penis. The
or disruptive in the older patient, and this This can be achieved either by interposing a problem is overcome by quilting a free
graft of buccal mucosa (or inner preputial FIG. 6. The Bracka two-stage repeat repair for BXO/urethral stricture in hypospadias failure. A, the urethral
skin if still available) into the dorsal stricture clearly visible; B, buccal mucosal free graft after 6 months, ready for second-stage closure; C, the
defect [26–29], thereby creating a graft- final outcome with glanular reconstruction completed; D, a closer view of the meatal appearance.
augmented Snodgrass or ‘Snod-graft’ repair
(Fig. 5).
Assessing the effects of endocrine 12 Podesta E, Buffa P, Di Rovasenda E, for the correction of chordee. J Urol 1985;
disruptors in the National Children’s Scarsi P. La ricostruzione del prepuzio 134: 311–4
Study. Environ Health Perspect 2003; nelle ipospadie distali. Proposta di una 23 Bracka A. Sexuality after hypospadias
111: 1678–82 modifica alla tecnica di Righini. Rass It repair. BJU Int 1999; 83 (Suppl. 3):
3 Aaronson IA, Murat AC, Key LL. Defect Chir Ped 1985; 27: 273–6 29–33
of the testosterone biosynthetic pathway 13 Frey P, Cohen SJ. Reconstruction of 24 Mureau MA, Slijper FM, Nijman RJ, van
in boys with hypospadias. J Urol 1997; foreskin in distal hypospadias repair. der Meulen JC, Verhulst FC, Slob AK.
157: 1884–8 Progr Pediatr Surg 1989; 23: 192–4 Psychosexual adjustment of children and
4 Baskin LS. Anatomical studies of the 14 Erdenetsetseg G, Dewan PA. adolescents after different types of
fetal genitalia: surgical reconstructive Reconstruction of the hypospadiac hypospadias surgery: a norm-related
implications. Adv Exp Med Biol 2002; 511: hooded prepuce. J Urol 2003; 169: 1822– study. J Urol 1995; 154: 1902–8
239–49 4 25 Bracka A. A long-term view of
5 Moreno-Garcia M, Miranda EB. 15 Zaontz MR. The GAP (glans hypospadias. Br J Plast Surg 1989; 42:
Chromosomal anomalies in approximation procedure) for glanular/ 251–5
cryptorchidism and hypospadias. coronal hypospadias. J Urol 1989; 141: 26 Hayes MC, Malone PS. The use of a
J Urol 2002; 168: 2170–2 359–61 dorsal buccal mucosal graft with urethral
6 Kaefer M, Diamond D, Hendren WH 16 Snodgrass W. Tubularized, incised plate plate incision (Snodgrass) for hypospadias
et al. The incidence of intersexuality urethroplasty for distal hypospadias. salvage. BJU Int 1999; 83: 508–9
in children with cryptorchidism and J Urol 1994; 151: 464–5 27 Barbagli G, Palminteri E, Lazzeri M,
hypospadias: stratification based on 17 Snodgrass W, Nguyen MT. Current Bracka A. Penile and bulbar urethroplasty
gonadal palpability and meatal position. technique of tubularized incised plate using dorsal onlay techniques. Atlas Urol
J Urol 1999; 162: 1003–6 hypospadias repair. Urology 2002; 60: Clin 2003; 11: 29–41
7 Betts EK. Anesthesia in the neonate and 157–62 28 Barbagli G, Palminteri E, Lazzeri M,
young infant. Dialogues Pediatric Urol 18 Hollowell JG, Keating MA, Snyder HM, Guazzoni G. Anterior urethral strictures.
1981; 4: 3 Duckett JW et al. Preservation of the BJU Int 2003; 92: 497–505
8 Schonfeld WA, Beebe GW. Normal urethral plate in hypospadias repair: 29 Barbagli G, Palminteri E, Bracka A,
growth and variation in the male genitalia extended applications and further Caparros Sariol J. Penile urethral
from birth to maturity. J Urol 1942; 48: experience with the onlay island flap reconstruction: concepts and concerns.
759–61 urethroplasty. J Urol 1990; 143: 98–101 Arch Esp Urol 2003; 56: 594–6
9 Tsur H, Shafir R, Shachar J. Microphallic 19 Duckett JW. The island flap technique for 30 Depasquale I, Park AJ, Bracka A. The
hypospadias: testosterone therapy prior hypospadias repair. Urol Clin North Am treatment of balanitis xerotica obliterans.
to surgical repair. Br J Plast Surg 1983; 36: 1980; 8: 503–11 BJU Int 2000; 86: 459–4
398–400 20 Bracka A. Hypospadias repair: the two-
10 Kelalis P, Bunge R, Barkin M et al. The stage alternative. Br J Urol 1995; 76 Correspondence: Giantonio Manzoni,
timing of elective surgery on the genitalia (Suppl. 3): 31–41 Department of Urology and Section of
of male children with particular reference 21 Pope JC, Kropp BP, McLaughlin KP et al. Paediatric Urology, Ospedale di Circolo,
to undescended testes and hypospadias. Penile orthoplasty using dermal grafts in Varese, Italy.
Pediatrics 1975; 56: 479–83 the outpatient setting. Urology 1996; 48: e-mail: gianantoniomanzoni@virgilio.it
11 Lepore AG, Kesler RW. Behaviour of 124–7
children undergoing hypospadias repair. 22 Perlmutter AD, Montgomery BT, Abbreviations: TIP, tubularized incised-plate;
J Urol 1979; 122: 68–71 Steinhardt GF. Tunica vaginalis free graft BXO, balanitis xerotica obliterans.