0022-5347 /88/1416-1407$02.

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THE JOURNAL OF UROLOGY \/ol. 141, June
Copyright© 1989 by Williams & VVilkins Printed in U.S.A.

TECHI\JICAL CHALLENGE OF THE MEGAMEATUS INTACT

PREPUCE HYPOSPADIAS VARIANT: THE PYRAMID PROCEDURE
JOHN W. DUCKETT* AND MICHAEL A. KEATING
From the Division of Urology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

ABSTRACT
An unusual variant of hypospadias is the focus of this report. This distal variant consists of a
megameatus and urethra in the presence of intact foreskin. Recognition is important, not only
because of the uncommon combination of findings, which run contrary to the classical presentation
of most hypospadias, but also because of the technical shortcomings that are encountered during
repair with standard techniques. The pyramid procedure is described, which allows for an end-on
dissection of the distal megameatus-urethra, enabling a reduction in caliber of both while facilitating
remodeling of the glans. The procedure has proved to be successful and reliable for this particular
hypospadias variant. No complications have been encountered. (J. Ural., 141: 1407-1409, 1989)

The combination of a megameatus and an intact prepuce
(MIP) represents an unusual hypospadias variant. Although
most hypospadiologists will encounter this anomaly during the
course of their practice, recognition of this combination of
findings in hypospadias as an isolated entity has been alluded
to only by a few authors. 1 • 2 Consisting of a widely splayed
"blunderbuss" meatus, widened distal urethra and deeply
clefted glanular groove, the MIP variant represents one of the
most technically challenging types of hypospadias to repair
(fig. 1). There is no chordee with the variant, which in combi-
nation with an intact prepuce frequently remains unrecognized
until after circumcision.
The majority of anterior forms of hypospadias are amenable
to conventional repairs, including meatal advancement and
glanuloplasty (MAGPI) 3 or a variety of meatal-based flaps.4
Our experience with application of these techniques to the MIP
variant has been suboptimal because of the unusual configu-
ration of the glans and distal urethra. Since the findings in the
MIP variant mirror those of balanitic epispadias, a procedure
has evolved from our experience with repair of epispadias that
has proved to be applicable to the MIP hypospadias variant in
the circumcised and uncircumcised setting. We have labeled
this repair the pyramid procedure based on the exposure that
allows for simple and safe dissection of the wide meatus and
urethra.
TECHNIQUE
A 5-zero polypropylene suture is placed in the dorsum of the
glans to facilitate handling during subsequent repair (figo
Three other traction sutures define the megameatus and base
of the pyramid. The exposure afforded by these sutures helps
to avoid inadvertent urethral injury during mobilization of the
megameatus and distal urethra. Subepithelial injection of li-
docaine 1 per cent with epinephrine (1:100,000) is performed
along the proposed lines of incision and even into the glans
itself to minimize bleeding. Intermittent use of an elastic tour-
niquet also may be needed during the initial dissection.
A tennis racket incision is made beside the glanular groove
and around the edges of the megameatus at the base of the
pyramid traction sutures. The dissection is carried proximally
below the coronal level, mobilizing the urethra (to the apex of
the pyramid) with fine iris scissors. Laterally, the edges of the
Accepted for publication November 30, 1988.
* Requests for reprints: Division of Urology, Children's Hospital of
Philadelphia, 34th SL and Civic Center Blvdo, Philadelphia, Pennsyl-
vania 19104-4399.
glanular groove are deepened to develop the glans wings from
the urethral plate. This enables glans rotational remodeling for
subsequent re-approximation in the midhne without tension or
compromise of the underlying neourethra. The distal urethral
plate is left wide (12 to 15 mm.) and intact dorsally. The
widened distal urethra is tailored in continuity with the urethral
plate by removing a small wedge of ventral tissue.
The urethra and glans strip are tubularized to form the
neourethra. A continuous 7-zero polyglycolic or polydiaxanone
suture can be used. The glans wings are re-approximated in the
midline in 2 layers using 6-zero interrupted polyglycolic sutures
in the subepithelial glans tissue and 7 -zero interrupted chromic
mattress sutures in the skin, completing the repair. A 10 or 12F
bougie-a-boule should pass easily through the neourethra.
Expression voiding while asleep will demonstrate any abnor-
mality of the stream.
Circumcision, if desired, is performed making 2 circum-
ferential incisions in the shaft skin: 1 incision 6 to 8 mm. from
the coronal edge with the prepuce retracted and 1 at a similar
level without preputial retraction. After excision of the prepuce,
sleeve reapproximation is done using fine running chromic
sutures. A 6F silicone tube is placed through the neourethra
and into the bladder. This fine catheter is sutured to the glans
with 5-zero polypropylene on a tapered needle, allowing for
continuous drainage of urine into a diapero The repair is dressed
with a simple gauze wrap that is removed the in 48
hours. The stent remains for approximately 5 to 7
RESULTS
During the last 3 years, we have performed the pyramid
procedure in 14 consecutive patients seen with the MIP variant.
Of the boys 7 had been circumcised previously. One patient
was seen after an attempted repair at another institution re-
sulted in a retrusive meatus and fistula. Patient age ranged
from 6 to 39 months. Followup ranged from 2 to 36 months.
Each patient returned for initial evaluation 3 to 4 weeks post-
operatively for assessment of the quality of the repair, and
calibration of the meatus and distal urethra using a bougie-a-
boule. Subsequent evaluations have been performed 6 to 8
weeks later and again at 1 year. The postoperative cosmetic
appearance of the penis has been uniformly excellent. Similar
functional results have been achieved, each patient voiding
with a good straight stream. There has been no evidence of
meatal stenosis, fistula, chordee or urethral strictures. To date
no patient has required a secondary procedure for correction of
a complication or improvement of cosmesis.
1408 DUCKETT AND KEATING

Fm. L A, MIP variant. Prepuce is intact with no chordee and relatively innocuous appearance. B, foreskin is retracted to show megameatus.
C, less severe variant recognized after circumcision.

A B C

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Fm. 2. Pyramid technique. A, MIP variant. B, foreskin is retracted to show megaurethra. C, tennis racket incision is marked. D, periurethral
dissection to apex of pyramid. E, ventral urethral reduction by removal of wedge of tissue, neourethral closure and approximation of glans in 2
layers (deeper layer is shown).

DISCUSSION With an intact prepuce and no chordee, many patients often

Anterior hypospadias represents 65 to 70 per cent of all types
of hypospadias seen. In a series reported by Juskiewenski and
associates the preputial intact variant comprised 25 of 383 (6
per cent) cases of anterior hypospadias. 1 These figures would
place the over-all incidence of the MIP variant at approxi-
mately 3 per cent. We are suspicious that the entity is perhaps
even more common. The milder glanular variant will not cause
functional problems. The MIP variant may not have the usual
distal lip causing deflection of the stream seen with other forms
of hypospadias. The more severe MIP variants may be associ-
ated with urinary spraying and meatal irritation in the adult.
are denied surgical correction for lack of functional disability.
Nevertheless, considerable appreciation and relief are ex-
pressed by the parents after successful repair for functional
and/or cosmetic reasons. Our experience would support its
correction in most instances.
In the newborn with the MIP variant the prepuce normally
is fused to the glans around to the megameatus. Since the
prepuce is normal circumferentially, there are no clues to the
anomaly until the fusion is separated. Thus, many of these
patients undergo ~tine neonatal circumcision before detec-
tion, underscoring the'1teed for increased awareness of the MIP
variant on the part of tll:e urologist and primary care physician.
 TECHI\fICAL CI-IALLEt'sJi}E OF !v1EGA_fvfEATUS IN.TACT PREPUCE HYPOSPADIAS VARIANT
B application and potential limitations. The application of any
Fm. 3. Proposed embryology of MIP variant. A, normal develop-
ment. Glanular urethra forms from ectodermal pit at glans tip and
open end of urethral groove. Closure of groove is shown. Sagittal view
shows proximal urethra meeting glanular urethra when intervening
septum (arrow) breaks down in normal cases. B, MIP variant results
from overabundant or misdirected clefting. Adapted from data of
Sommer, J. T. and Stephens, F. D.: J. Urol., 124: 94, 1980.
Nevertheless, retention of the foreskin, important to other
hypospadias conditions, is not a factor in repair of the MIP
variant. Therefore, guilt for an inappropriate circumcision is
irrelevant.
The embryological basis of this phenomenon is unclear. The
glanular urethra forms during month 3 of gestation. It generally
is believed that the glanular urethra results from proliferation
of epithelium growing into the substance of the glans. It meets
the proximal penile urethra, which is formed by earlier closure
of the urethral groove, at the coronal sulcus. A cuff of tissue
appears at the margins of the sulcus forming the prepuce.
Failure of fusion of the urethral folds at varying levels results
in persistence of the urethral groove, arresting distal urethral
development, and formation of the prepuce. Typically, a hy-
pospadiac meatus with a dorsal hooded prepuce results. As
demonstrated by the MIP variant, preputial development ap-
pears to be independent of formation of the glanular urethra.
It might be postulated that the folding process of proximal
urethral development to the level of the corona was completed
with normal circumferential preputial fusion" Maldevelopment
of glanular epithelial infolding would appear to be the abnormal
process responsible for MIP formation. Overabundant or mis-
directed clefting might split the glans and proceed down the
already fused distal urethra, creating the megameatus and
widened urethra seen while keeping the foreskin intact (fig. 3).
Most anterior hypospadias can be repaired using standard
including the MAGPI or the meatal-based flap
1409
(Mathieu).4 Each of these urethropiasties has its particular
favorite repair to an unsuitable candidate invites complication
regardless of the expertise of the surgeon. This caveat proves
especially true in the MIP variant. The ideal candidates for
performance of the MAG PI technique are boys with subcoronal
hypospadias without fibrous chordee when the glans is broad;
although the meatal caliber may be stenotic the urethra is
normal. As noted by Gibbons and Gonzalez, unsuitable candi-
dates include those with a widened meatus and severe dysplastic
ventral skin, and in whom the urethra is immobile.' Meatal-
based flaps should not be used in patients with proximal ventral
skin deficiency or when the length of flap needed to form the
neourethra exceeds a 2:1 ratio. In the application of meatal-
based flaps to this particular variant the surgeon encounters
an inability to tailor the megaurethra, and difficulty in readying
the glans and urethral plate in preparation for neourethral flap
and glans remodeling.
The use of periurethral skin tubularization to form a neoure-
thra in hypospadias repairs is not a new principle. King" and
Duplay 7 a century earlier both described its use. The shortcom-
ings of their procedures, as reported, lie in an inability to
extend the neourethra onto the tip of the glans" Extrapolating
our experience with the repair of glanular epispadias, the pyr-
amid procedure permits a more cosmetic glans reconstruction,
and can be applied to the MIP variant in the circumcised and
uncircumcised states. The pyramid exposure optimizes a safe
and simple dissection of the megameatus-urethra, and allows
for subsequent reduction and caliber of both with excellent
functional and cosmetic results.
REFERENCES
1. Juskiewenski, S., Vaysee, P., Guitard, J. and Moscovici, J.: Traite-
ment des hypospadias anterieurs. Place de la balanoplastie. Chir.
Ped., 24: 75, 1983.
2. Palma, P. C. R., Ikari, 0. and Netto, N. R., Jr.: Approach for
treatment of subcoronal meatus with excessively deep glanular
groove. Urology, 31: 135, 1988.
3. Duckett, J. W.: MAGPI (meatoplasty and glanuloplasty). A pro-
cedure for subcoronal hypospadias. Urol. Clin. N. Amer., 8: 513,
1981.
4. Duckett, J. W.: Hypospadias. In: Adult and Pediatric Urology.
Edited by J. Y. Gillenwater, J. T. Grayhack, S. S. Howards and
J. W. Duckett. Chicago: Year Book Medical Publishers, vol. 2,
chapt. 57,pp. 1880-1915, 1987.
5. Gibbons, M. D. and Gonzales, E. T., Jr": The subcoronal meatus.
J. UroL, 130: 739, 1983.
6. King, L. R.: Hypospadias~a one-stage repair without skin graft
based on a new principle: chordee is sometimes produced by the
skin alone. J. Urol., 103: 660, 1970.
7. Duplay, S.: Sur le traitement chinfgical de l'hypospadias et de
l'epispadias. Arch. Gen. Med., 145: 257, 1880.