Blackwell Science, LtdOxford, UKIJUInternational Journal of Urology0919-81722005 Blackwell Publishing Asia Pty LtdApril 2005124419421Case ReportCongenital scaphoid megalourethraBH

Özokutan
et al.

International Journal of Urology (2005) 12, 419–421

Case Report

Congenital scaphoid megalourethra: Report of two cases

BÜLENT HAYRI ÖZOKUTAN,1 MUSTAFA KÜÇÜKAYDIN,2 HALUK CEYLAN,1
ALI GÖZÜKÜÇÜK2 AND FAHRI KARACA2
1
Department of Pediatric Surgery, Gaziantep University Medical Faculty, Gaziantep,
2
Department of Pediatric Surgery, Erciyes University Medical Faculty, Kayseri, Turkey

Abstract Congenital megalourethra is a rare congenital malformation of the penile urethra. It is defined as
the diffuse dilatation of the anterior urethra due to the absence of development of the erectile tissue
of the penis. Since the initial description, nearly 80 cases with megalourethra have been reported in
English literature. Congenital megalourethra has been classified into scaphoid and fusiform types
and is usually associated with additional urinary tract and other system anomalies, irrespective of
its type and severity. We report two rare cases of scaphoid megalourethra without any associated
anomaly.
A 13-year-old boy and an 8-month-old boy were admitted to the Department of Pediatric Surgery
with complaints of penile swelling and dysuria since birth. Physical examination and retrograde
cystourethrogram confirmed the diagnosis of congenital scaphoid megalourethra. Both patients
underwent a reduction urethroplasty. They did well postoperatively and voided with a normal stream
without any abnormality of the penile shaft.

Key words congenital urethral abnormalities, megalourethra.

Introduction ally associated with additional urinary tract and other

system anomalies irrespective of its type and severity.3,7
Congenital megalourethra is a rare congenital malfor- We report two cases of congenital scaphoid megaloure-
mation of penil urethra. It is defined as diffuse dilatation thra without any other associated malformations.
of the anterior urethra due to absence of development
of the erectile tissue of the penis.1 This anomaly affects
the anterior part of urethra, and usually cause abnormal Case reports
size and shape of the penil shaft, especially while void-
ing. Megalourethra has been classified into scaphoid Case 1
and fusiform types, according to urethrography find-
ings.2 The scaphoid form is due to poor development of A 13-year-old boy was admitted to the Department of
the corpus spongiosum in the anterior urethra whereas Pediatric Surgery with complaints of penile swelling
the fusiform type, which is more severe, is due to the during micturition and dysuria since birth. On clinical
maldevelopment of the corpus spongiosum as well as examination, there was a small swelling on the ventral
the corpora cavernosa.3–5 Although this classification is surface that ballooned markedly during micturition.
commonly accepted, intermediate forms also have been Urine dribbling from the normally-located meatus was
reported and some authors consider megalourethra as a observed after compression of the swelling. Blood
spectrum of disease.3,6 Congenital megalourethra is usu- chemistry, abdominal ultrasonography and voiding cys-
tourethrography did not reveale any abnormality. Retro-
grade cystourethrogram showed scaphoid dilatation of
Correspondence: Bülent Hayri Özokutan MD, Gaziantep the anterior urethra. After a circumcising incision and
University Medical Faculty, Department of Pediatric Sur-
gery, 27310, Gaziantep, Turkey. degloving of the skin to the base of penis, the dilated
Email: ozokutan@hotmail.com urethra was opened longitudinally. There was no ure-
Received 12 May 2004; accepted 21 September 2004. thral obstruction, but the corpus spongiosum was poorly
420 BH Özokutan et al.

developed. After the redundant portion of the urethra

was excised, a reduction urethroplasty over a catheter
was performed. The urethral catheter was removed after
10 days and the patient voided with a normal stream
without any abnormality of the penile shaft.

Case 2

An 8-month-old boy was referred for difficulty in void-

ing and deformed penis. His family noticed the swelling
in the penoscrotal region during the neonatal period.
The patient’s history revealed that a misdiagnosis of
hydrocele was made by a primary care physician and
surgery was recommended if the hydrocele persisted
after 1 year of age. Physical examination showed a
penile swelling extending into the scrotum and rotation
of penis counter-clockwise (Fig. 1). Both testes were
located in the scrotum and hydrocele was not detected.
Examination of other systems, including abdominal
musculature, did not disclose any abnormality. Retro-
grade cystourethrogram revealed a large scaphoid dila-
tation of the anterior urethra (Fig. 2). Blood chemistry,
renal ultrasonography and voiding cystourethrography
were normal. Reduction urethroplasty was performed as
in case 1 (Figs 3 and 4) and a suprapubic catheter was
placed. The patient did well postoperatively. The cathe-
ter was removed after 12 days and the patient voided
normally. Fig. 2 Retrograde cystourethrogram demonstrating a
large scaphoid dilatation of the anterior urethra.

Discussion

Congenital megalourethra was first described and suc-

cessfully repaired by Nesbitt in 1955.1 Since this initial

Fig. 1 Congenital scaphoid megalourethra presenting as Fig. 3 Appearance of urethral dilatation after degloving
a penoscrotal mass. of penil skin.

Fig. 4 Longitudinally opened urethral cavity.
description, nearly 80 cases with megalourethra have
been reported in English literature.7 The etiology of
megalourethra is not fully understood. The erectile tis-
sue of the corpora cavernosa and corpus spongiosum
arise from differentiation of mesodermal columns in the
urethral folds. It is presumed that failure of the meso-
dermal columns to differentiate and provide support to
the urethral epithelium results in megalourethra.8
Stephens et al. proposed that delayed canalization of the
glans urethra results in transient urethral obstruction and
the severity and the duration of the urethral anomaly
determines the type of megalourethra.4
A true anatomical obstruction has not been identified
in either form of magalourethra, but in the scaphoid
type, poor development of the corpus spongiosum
causes functional obstruction and stasis of urine. Some-
times, differential diagnosis of malformations that affect
the anterior urethra and cause abnormal size and shape
of penile shaft may be difficult. Some authors suggest
that megalourethra and diverticula have to be considered
as a single malformation with a spectrum of presenta-
tions.9 To the contrary, Appel et al. have stated that
congenital urethral diverticula are different from mega-
lourethra, since they have narrow orifices and cause
obstruction by luminal compression upon filling,
whereas the megalourethra never presents a true distal
anatomic obstruction.3
Megalourethra is usually associated with severe
upper urinary tract and anterior abdominal wall defects.
In nearly 85% of the reported cases, coexisting congen-
ital anomalies were identified.7 The most commonly
reported associated malformations include renal dyspla-
sia/hypoplasia, hydronephrosis, hydroureter, posterior
urethral valves, vesicoureteral reflux, prune–belly syn-
Congenital scaphoid megalourethra 421
drome, undescended testis, hypospadias and anorectal
malformation.3,5,7–9 Although it is generally accepted
that associated anomalies are more frequent and more
severe in the fusiform variant,7 some authors disagree
that a relationship exists between the type of malforma-
tion and coexisting diseases.3 It has been stated that even
with milder forms of urethral abnormality, there may be
coexistent and often severe associated upper urinary
tract anomalies,3 but we did not identify any associated
anomaly in either of the present cases.
Despite the presence of prenatally detected cases,10
congenital megalourethra is usually diagnosed in neo-
natal period or early infancy. Case 1, a 13-year-old boy,
presented with penile swelling during micturation and
dysuria since birth, but this long-term functional
obstruction did not affect upper urinary tract. Unusually,
our second case initially had the misdiagnosis of hydro-
cele, which delayed the treatment. It is essential to con-
sider megalourethra in the differential diagnosis of all
children with penile or penoscrotal swelling.
Congenital megalourethra is a rare and surgically
correctible malformation of the anterior urethra. It
should be considered in the differential diagnosis of
penile swelling. The presence and severity of associated
anomalies in patients with megalourethra determines
the quality of life.
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