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Curr Urol Rep (2015) 16:69

DOI 10.1007/s11934-015-0543-5

PEDIATRIC UROLOGY (M CASTELLAN AND R GOSALBEZ, SECTION EDITORS)

Surgery for Primary Proximal Hypospadias with Ventral


Curvature >30°
Warren Snodgrass 1 & Nicol Bush 1

# Springer Science+Business Media New York 2015

Abstract This review discusses means for straightening ven- review recent publications and suggest means to improve out-
tral curvature >30°, and then subsequent urethroplasty using comes for these most severe hypospadias cases.
either one-stage flaps or two-stage flaps or grafts. The wide
variation in reported results from these various techniques
makes determination of best management difficult; however, Straightening Ventral Curvature
it is clear that complications for repairs done in major centers
can range to ≥50 %. Given the rarity of proximal hypospadias 50 % of patients with proximal shaft to perineal hypospadias
with ventral curvature >30°, which occurs in approximately have ventral curvature >30° after the penis is degloved, and
400 newborns annually in the USA, centers should refer these ventral dartos and scrotal attachments to the penile shaft are
cases to subspecialists within the group who can then develop released [1]. The next step to straighten the penis is transection
and maintain expertise. of the urethral plate. This usually corrects curvature in Asian
boys [2], although patients of European and African descent
Keywords Proximal hypospadias . Corporotomy . Prepucial most often remained bent—indicating an apparent difference
flaps . Prepucial grafts . Urethroplasty complications in etiology of ventral curvature by race (data not published).
The observation that urethral plate transection does not
straighten the penis in most patients operated in the USA led
Introduction John Duckett to explore correction while preserving the plate,
which he did by dorsal plications [3]. Although a single pli-
The best management of primary proximal hypospadias in cation does not shorten the penis, the multiple plications need-
which the urethral plate is transected during straightening re- ed to straighten bending that can exceed 90° can, which is a
mains uncertain. These comprise approximately 5 % of pri- concern in boys with proximal hypospadias since their aver-
mary hypospadias repairs, which using CDC birth data (CDC, age penile size is smaller than that of normal boys [4•]. Fur-
2014) for the USA calculates to approximately 400 per year, thermore, one report found recurrent curvature more likely
or 0.5 cases per pediatric urologist. With such low volumes, it when dorsal plication, versus ventral corporal lengthening,
is difficult for specialists to achieve and maintain expertise, was done for bending >30° [5].
and so, not surprisingly, complication rates are high. It is also Ventral lengthening can be done by either single
difficult to accumulate sufficient data to make comparisons corporotomy with corporal grafting or three transverse
between the various surgical techniques used. This article will corporotomies without grafting the defects. With single
corporotomy, incision is made from 3 to 9 o’clock through
This article is part of the Topical Collection on Pediatric Urology the point of maximum bending. The resulting defect is wid-
ened to create a diamond, and then grafted with dermis, SIS,
* Warren Snodgrass or similar material. When three incisions are made, the first is
snodgrass@parcurology.com
also done through the maximum bending but from 4 to 8
o’clock, with the second and third then made approximately
1
PARC Urology, Dallas, TX, USA 4 mm above and below the first. The incision goes all the way
69 Page 2 of 5 Curr Urol Rep (2015) 16:69

through the tunica albuginea down to, but not into, the corpo- time-randomized trial comparing the traditional tubularized
ra. These defects are left linear rather than widened into dia- flap to the modified flap mimicking a urethral plate might
monds, and are not grafted. We find that this is most easily have highlighted the benefits, and remaining challenges, of
accomplished with a tourniquet at the base of the penis, and repair by the newer method.
we apply 1:1000 epinephrine topically if there is bothersome The difficulties in deriving evidence-based management
bleeding when it is released. and learning to reduce complications from reported hypospa-
Straightening is reliably achieved by either method [1]. We dias series are further highlighted by two reports in the past
recently reviewed our results, finding three corporotomies year concerning two-stage Byars prepucial flaps. Various ear-
successfully corrected curvature >30° with only one recur- lier publications regarding this technique reported complica-
rence in 50 consecutive patients. Over half of these boys had tions occurring in from 5 to 70 % of patients [8, 10]. The
curvature >90°, with a maximum of 120° after urethral plate lowest rate came from a series which only stated there was
transection (unpublished data). no meatal stenosis and that fistulas developed in 5 % of 58
The major difference that arises from these two methods is cases with proximal shaft to perineal hypospadias. A subse-
the resulting options for urethroplasty. Corporal grafting re- quent review from the same institution with 134 patients de-
quires flap repair, as urethroplasty grafts likely will not heal scribed fistulas in 29 % and meatal stenosis in 13 %, with up to
satisfactorily if placed onto corporal grafts. Ventral lengthen- 80 % complications overall [11•], although some patients may
ing by three corporotomies without corporal grafting preserve have had more than one. Another retrospective review pub-
the option for graft urethroplasty. lished this year similarly found 66 % of cases developed com-
plications after Byars flap repair [12•]. Conversely, a third
recent report of 128 patients stated only 12 % developed com-
Proximal Flap Urethroplasty plications [13•].
Widely divergent outcomes reported by surgeons using the
Flap repairs can be done in either one or two stages. A sys- same operation suggest selection bias for who is undergoing
tematic literature review found urethroplasty complications the procedure, and/or technical differences in performing key
reported in 38 % of tubularized prepucial flap and 32 % of steps in the operation. These three recent articles all used
Koyanagi repairs, which are one-stage options. Two-stage Byars flaps for proximal shaft to perineal hypospadias repair,
Byars flap operations had a reported 22 % complication rate although the decision in whom to transect the urethral plate
[6]. However, the authors warned the studies reviewed were potentially varied among the multiple surgeons involved. The
generally of low scientific quality, providing little evidence various steps in creating and securing the Byars flaps ventral-
basis for management. ly, tubularizing the neourethra, waterproofing it with a barrier
There have not been many recent studies published on one- flap, and performing glansplasty were only generally de-
stage tubularized prepucial flap repairs. Our review found one scribed by Stanasel et al. and McNamara et al., likely because
report with 25 primary cases, unusual in that the mean age at there was variation between the surgeons involved and by
repair was 12±11 years. The authors followed the original each surgeon over time. Yang et al. provided more technical
description of the technique, suturing the prepuce into a tube detail and reported the fewest complications, but did not con-
over a catheter which they rotated ventrally and secured to the trast their outcomes to an earlier experience, when they might
native urethra proximally and glans wings distally. have had more problems, to explain for others how to similar-
Urethroplasty complications occurred in 24 % [7]. ly improve results.
Others reported improved results when the prepucial flap One observation we have made is that flap repairs may not
was not simply tubularized, but instead transposed ventrally create normal fusion of the glans wings between the
with a portion sewn to the corpora between the native urethra neomeatus and the corona. This distance normally averages
and the glans to create a neourethral plate, to which the remain- 4.7 (±1.2)mm [14], and is needed to create a reliably compact,
ing portion was then secured. The intention was to make the straight urinary stream without spraying. Because the end of
neourethra more smooth and uniform, to resemble an onlay the flap is sewn to the glans wings, there is less glans to
flap procedure. Two series comprising 12 and 22 patients re- approximate below the neomeatus. A postoperative photo-
ported complications in 17 and 14 %, respectively [8, 9]. graph in the Yang et al. report shows the glans fused together
None of these studies described the authors’ prior results only at the corona below a large meatus. One report mention-
using one-stage tubularized prepucial flaps. Were the cases ing functional outcomes of Byars flaps stated that 37 % had
reported by Zheng et al. their first experience with this tech- abnormal voiding and urinary spraying [15], which might in-
nique, and if not, were these results better than their earlier dicate inadequate glansplasty.
outcomes? Assuming initial patients had more complications, WS initially preserved the glandular portion of the urethral
readers might have benefited from a description of technical plate when it was transected during penile straightening, and
modifications the authors made to various steps. Similarly, a used interposed Byars flaps with distal TIP. Therefore, the
Curr Urol Rep (2015) 16:69 Page 3 of 5 69

glans closure was the same as in primary TIP repairs, which We have been reluctant to then place a tubularized graft
we are now systematically measuring and observing to be 3– onto these three incisions, which we have made in nearly
5 mm between the meatus and corona. There were 100 % 75 % of cases after transecting the urethral plate for persistent
complications in nine patients, five of which were diverticu- ventral curvature. Instead, we perform a two-stage repair,
lum despite the absence of distal stenosis. Thinking the strip allowing the smooth rectangular graft to heal for 6 months
for urethroplasty was possibly cut too widely at the second before tubularization. After harvesting the prepucial graft,
stage, he made it narrower in the next patient, resulting in a we spread it over the incised corpora from the meatus to the
stricture. tip of the glans, quilt it into place, and then apply a tie-over
We subsequently changed from Byars flaps to two-stage compression bandage to facilitate graft take. Overall graft take
prepucial graft repairs, and despite using the same tissue for was 95 %, with no difference in those placed onto smooth
urethroplasty and similar glansplasty technique, no diverticu- versus incised corporal surfaces [17].
lum have occurred in the latter patients. The dartos underlying Beginning in 2008 and continuing through 2014, we per-
Byars flaps hinders fixation to the corpora, resulting in a more formed 65 first-stage and completed 50 second-stage
tortuous neourethra with turbulent flow. Yang et al. sutured the prepucial or labial (when foreskin reconstruction was re-
midline approximation of the two halves of the Byars flaps to quested) primary proximal repairs for ventral curvature
the corpora, which may have facilitated creation of a more >30° persisting after transecting the urethral plate. The ini-
uniform, straight neourethra. tial degloving incision was made approximately 4 mm be-
low the corona to preserve most the inner prepuce for the
Two-Stage Graft Urethroplasty graft. Straightening was done, and the penoscrotal junction
was incised on each side from 6 to 2 or 3 and 9 to 10 o’clock,
The same prepucial tissues used for tubularized and Byars respectively. This frees the dorsal shaft skin and the hooded
flaps can also be harvested for either one- or two-stage graft prepuce. Stay sutures were placed into the distal corners of
urethroplasty. As mentioned above, the choice between these the unfurled prepuce and the underlying dartos was excised.
options depends in part on methods used to straighten ventral Then a horizontal line was marked on the prepuce where the
penile curvature. Before artificial erection was introduced in distal shaft skin would join the inner prepucial collar, leav-
the 1970s, the general belief was that meticulous excision of ing a graft which was mostly inner prepuce with some outer
Bchordee^ tissues between the meatus and the glans was suf- prepuce. The graft was set aside while circumcision,
ficient to correct bending. Plication might also be done if scrotoplasty, and the proximal urethrostomy were
persistent curvature was suspected, but ventral corporotomies completed.
were thought to be rarely indicated after their description by We have learned that this prepucial graft will cover defects
Devine and Horton [16]. That surgical team experimented extending from deep within the scrotum to the tip of the glans.
with both multiple corporotomies without corporal grafting It is important that the glans be opened widely, to 3 and 9
and single corporotomy with dermal corporal grafts. Of these o’clock, to place sufficient graft that meatal stenosis will not
two methods, single corporal incision with grafting became occur during second-stage tubularization. The grafts were first
most popular, and favored prepucial urethroplasty flaps to sutured to the edges of the glans wings at the coronal on either
avoid placing urethral grafts onto corporal grafts. side, and then gently stretched distally and sewn to the end of
Although a number of studies regarding one-stage the glans. We used 7–0 polyglactin, placed through the epi-
tubularized grafts report similar urethroplasty complication thelium all along the perimeter, except across the end of the
rates as with flaps, the patients discussed in this article with glans where subepithelial sutures were used to avoid marks at
transection of the urethral plate and ventral corporotomy like- the future neomeatus. Proximally, the graft was incised in the
ly were not the subject of most these reviews. midline to extend to either side of the proximal urethrostomy
Dissatisfaction with results of Byars flaps led WS to con- to 4 and 8 o’clock.
sider two-stage primary graft repairs at a time when he was After the graft perimeter was secured, additional Bquilting^
learning that technique for salvage repairs. To do so, he had to stitches of 6–0 polyglactin on a RB1 needle were sewn in the
straighten ventral curvature without incision and corporal midline and on either side at 5-mm intervals to secure the graft
grafting, which led him to revive the Devine and Horton Bfairy to the surface of the corpora. This reduces likelihood for blood
cuts^. The name is misleading, however, as these incisions or serum to accumulate under the graft during revasculariza-
extend just as deep as do single corporal incisions, which is tion postoperatively.
through the tunica albuginea avoiding injury to the enclosed 5–0 polypropylene sutures at the urethrostomy, midpenis
erectile tissues. As discussed above, our data indicate these and corona were tied over a rolled Vaseline gauze to further
three incisions without corporal grafting are as effective in immobilize the graft and promote revascularization during the
straightening the penis as are single incisions with corporal first postoperative week. We discharged infants and children
grafts. the same day as surgery.
69 Page 4 of 5 Curr Urol Rep (2015) 16:69

Second-stage tubularization was done 6 months later in two The first step towards improving outcomes is for surgeons
subepithelial layers: the first with interrupted 7–0 polyglactin to learn their current results. We have consistently found they
and the second with a continuous 7–0 polydioxanone for are less favorable than we presumed, leading to practice
prepucial grafts and interrupted 6–0 polyglactin, and then con- changes. Such quality assessment and improvement can be
tinuous 6–0 polydioxanone for labial grafts. We always cov- summarized as the 3Ps: prospective data collection, periodic
ered the neourethra with tunica vaginalis, not dartos. Most of outcomes review, and practice change.
these patients had a small glans diameter, less than the 14 mm
average of healthy newborns [4•]. Consequently, we dissected Reducing the Surgical Algorithm
the glans from the corpora; first, laterally to 3 and 9 o’clock,
and then distally another 4 mm on each side before closing the Given the low number of proximal hypospadias cases, to gain
wings over the neourethra. As with TIP repair, the underlying expertise and to train others subspecialists must rely on a
end of the tubularized neourethra was not sewn to the glans limited number of surgical techniques so that the key steps
wings. This reliably created a vertical slit meatus with 3–4 mm can be learned and refined. We have found that all proximal
of glans wings fusion, closely resembling the normal glans hypospadias can be corrected using either TIP or two-stage
and meatus. grafts, depending on the extent of ventral curvature. Others
Our first 22 patients were repaired before we began the subspecializing in proximal repairs should also choose few
Bextended^ glans wings dissection discussed above. That methods that are widely applicable.
group had 45 % complications, all glans dehiscences, in one Although we believe that two-stage prepucial graft repair is
case combined with a fistula. After changing the glansplasty, superior to Byars flap repair, surgeons such as Yang et al. who
and operating as a surgical team, complications significantly can achieve similar low complication rates using flaps have no
decreased in the second group of 28 boys to 14 %, three glans reason to change their practice once they have determined
dehiscences, one with a fistula, and one recurrent curvature cosmetic and functional results are also optimal. To make
>30° [17]. the assessment of glansplasty results more objective, we are
measuring the distance from the lower lip of the neomeatus to
the corona, and encourage others to systematically do the
Progress in Proximal Hypospadias Repair same and record, and then report their results. Urinary func-
tion is more likely to be normal when glans anatomy is
3Ps normal.

We previously reported technical modifications in proximal Subspecialization


TIP repair which significantly reduced urethroplasty compli-
cations from an initial 54 to 13 % [18]. Our journey with two- It is not reasonable to expect that a surgeon who performs an
stage proximal hypospadias repair for ventral curvature >30° average of two repairs annually for the most severe forms of
and urethral plate transection began with a 100 % complica- hypospadias can either achieve or maintain expertise. While
tion rate using Byars flaps, decreased to 45 % in our initial the minimum case volume needed for these goals has not been
experience with two-stage grafts, and subsequently signifi- established in hypospadias surgery, numerous studies demon-
cantly decreased further to 14 %. This indicates that strate that better results are obtained by surgeons performing
urethroplasty complications should be considered potential higher volumes. For a condition with <500 new cases per year,
warning signs that technical changes are needed. referral to designated colleagues who subspecialize in com-
Assessment of outcomes before and after technical modi- plex hypospadias surgery is clearly needed. The reports from
fications is greatly facilitated by prospective data collection. Houston and Boston on Byars flaps referenced above both
This is especially true for uncommon operations such as prox- included multiple surgeons vying for an average of 5–7 repairs
imal hypospadias, in which a surgeon’s impression of personal per year, implying most did very few cases each year. In con-
outcomes frequently is found to be wrong because of recall trast, the experience reported by Yang et al. involved an aver-
bias, since these complications occur sporadically and are eas- age of 43 cases a year divided among three surgeons. To
ily forgotten within the overall surgical practice. accumulate data, determine current complication rates, and
For quality assurance, a simple spreadsheet listing the pa- then make changes to improve results, cases need to be con-
tient name, date of surgery, meatal location, primary versus centrated into fewer surgeons’ hands in each major institution.
reoperative repair, surgical technique, date of last follow-up,
and complications suffices to inform a surgeon regarding ac- Surgical Teams
tual results. Entering this data on the day of service requires
minimal time, and a review to determine the number of pro- All hypospadias surgery needs an assistant, which is most
cedures and complications can be done rapidly. often a trainee or a nurse. As we subspecialized within our
Curr Urol Rep (2015) 16:69 Page 5 of 5 69

university group, we also realized that a team approach using a 5. Braga LHP, Lorenzo AJ, Bagli DJ, Dave S, Eeg K, Forhat WA,
et al. Ventral penile lengthening versus dorsal plication for severe
senior and junior faculty surgeon might improve intraopera-
ventral curvature in children with proximal hypospadias. J Urol.
tive decision-making, following the example of many centers 2008;180:1743.
closing bladder exstrophy. Furthermore, a junior surgeon has 6. Castagnetti M, El-Ghoneimi. Surgical management of primary se-
to gain experience to eventually assume the role of a senior vere hypospadias in children: systematic 20-year review. J Urol.
2010;184:1469.
hypospadias surgeon. The reduction in complications we
7. Zheng DC, Wang H, Lu MJ, Chen Q, Chen YB, Ren XM, et al. A
achieved in two-stage graft repairs occurred in part due to a comparative study of the use of a transverse prepucial island flap
technical change in glansplasty, but also in part due to working (the Duckett technique) to treat primary and secondary hypospadias
as a surgical team. in older Chinese patients with severe chordee. World J Urol.
2013;31:965.
8. Shukla AR, Patel RP, Canning DA. The 2-stage hypospadias repair:
Compliance with Ethics Guidelines
is it a misnomer? J Urol. 2004;172:1714.
9. Aoki K, Fujimoto K, Yoshida K, Hirao Y, Ueoka K. One-stage
repair of severe hypospadias using modified tubularized transverse
Conflict of Interest Warren Snodgrass and Nicol Bush each declare no preputial island flap with V-incision suture. J Pediatr Urol. 2008;4:
potential conflicts of interest. 438.
10. Retik AB, Bauer SB, Mandell J, Peters CA, Colodny A, Atala A.
Human and Animal Rights and Informed Consent This article does Management of severe hypospadias with a 2-stage repair. J Urol.
not contain any studies with human or animal subjects performed by any 1994;152:749.
of the authors. 11.• McNamara ER, Schaeffer AJ, Seager CM, Rosoklija I, Retik AB,
Diamond DA et al. Management of proximal hypospadias with 2-
stage repair: 20 year experience. J Urol. 2015. e-pub. 2-stage Byars
flaps repair resulted in complications in somewhere between
References 80% (total complications) and 49% (reoperations).
12.• Stanasel I, Le HK, Bilgutay A, Roth DA, Gonzales ET Jr, Janzen N
et al. Complications following staged hypospadias repair using
Papers of particular interest, published recently, have been transposed prepucial skin flaps. J Urol. 2015. e-pub.
highlighted as: Complications occurred in 68 % of patients after Byars flaps
• Of importance repair.
13.• Yang T, Zou Y, Zhang L, Su C, Li Z, Wen Y. Byars two-stage
procedure for hypospadias after urethral plate transection. J
Pediatr Urol. 2014;10:1133. This large series reported only
1. Snodgrass W, Prieto J. Straightening ventral curvature while pre- 12% complications after Byars flap repair.
serving the urethral plate in proximal hypospadias repair. J Urol. 14. Hutton KAR, Babu R. Normal anatomy of the external urethral
2009;182:1720. meatus in boys: implications for hypospadias repair. BJU Int.
2. Yoshino K. Personal communication 2007;100:161.
3. Hollowell JG, Keating MA, Snyder III HM, Duckett JW. 15. Gershbaum MD, Stock JA, Hanna MK. A case for 2-stage repair of
Preservation of the urethral plate in hypospadias repair: extended perineoscrotal hypospadias with severe chordee. J Urol. 2002;168:
applications and further experience with the onlay island flap 1727.
urethroplasty. J Urol. 1990;143:98. 16. Devine Jr CJ. Chordee and hypospadias. In: Glenn J, Boyce W,
4.• Bush NC, DaJusta D, Snodgrass W. Glans penis width in patients editors. Urologic surgery. Philadelphia: JP Lippincott; 1983. p. 775.
with hypospadias compared to healthy controls. J Pediatr Urol. 17. Snodgrass W, Bush N. Outcomes of 2-stage graft repair for proxi-
2013;9:1188. This study measured maximum glans width in mal hypospadias with >30° ventral curvature: modified glansplasty
infants with distal and proximal hypospadias at median age 9 reduces glans dehiscence. ESPU abstract. 2015.
months and compared them to controls undergoing elective 18. Snodgrass W, Bush N. Tubularized incised plate proximal hypospa-
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14.8, 12.9 and 14.3 mm. Pediatr Urol. 2011;7:2.

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