CASE REPORT

Covered Exstrophy: A Rare Variant of Classical Bladder

Exstrophy
Sakti Prasad Sahoo, Ajay Narayan Gangopadhyay, Chandrasen Kumar Sinha, Dinesh Kumar Gupta
and Saroj Chooramani Gopal
From the Division of Paediatric Surgery, Department of Surgery, Institute of Medical Sciences, Banaras Hindu
University, Varanasi-221005 (U.P.), India

(Submitted October 2, 1995. Accepted for publication December 29, 1995)

Scand J Urol Nephrol 31; 103-106, 1997

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Covered exstrophy is an extremely rare variant of exstrophy-epispadias complex. It is less distressing and easier to manage
than classical exstrophy of the bladder. We report three cases of this entity, two associated with anorectal malformation and
one with unilateral renal agenesis, along with a review of the literature.
Key words: covered exstrophy, split symphysis variants, anorectal malformation, unilateral renal agenesis.

Dr A. N . Gangopadhyay, New Ll30, Hyderabad Colony, B.H.U., Varanasi-22100.5, U.P., India

Covered exstrophy is a variant of exstrophy-epispadias Case 2: A 3.1 kg female newborn was referred to our
complex. The most accepted theory that of Marshall & hospital at the age of 4 days because of apparent
Muecke (9), states that persistence of an abnormal genital abnormality and an imperforate anus. She was
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cloaca1 membrane causes a wedge defect in the
infraumbilical region and separates the midline
structures to varying degrees. A large, persistent
membrane results in exstrophy, while a more limited
membrane results in epispadias. Variants of this
complex are characterized by classical musculo-
skeletal defects with minimal bladder abnormalities.
Four types of variants have been described and include
pseudo exstrophy, superior vesical fissure, duplicate
exstrophy and covered exstrophy. We report the
largest series (3 cases) of covered exstrophy along
with a detailed review of the literature.
born at term to a gravida I1 mother by spontaneous
vaginal delivery. On physical examination there was a
bulging in the suprapubic ?cgionwhich increased on
crying. There was no anal opening but a fistula in the
vestibule was detected. Also present were extensive
defects in rectus muscles, separation of the pubic bone,
and a bifid clitoris (Fig. 2). Plain x-ray of the pelvis
and spine showed pubic diastasis of 4 c m and partial
sacral agenesis (absent S4 and S.5). Intravenous

CASE REPORTS
Case 1: A 1-day-old female neonate presented with a
history of absent anal opening. The clinical exami-
nation revealed a 2.8 kg full-term female child with a
low-set umbilicus, divaricated recti in the lower
abdomen, pubic diastasis of 2.0cm, and a subcuta-
neous bladder which bulged in between recti on
crying. The right labia majora was underdeveloped
with a tag of skin in the right groin, 6 c m from the
vulva. There was an anovestibular fistula (Fig. 1).
Anal cut-back was done. Intravenous urography
revealed a normal upper urinary tract and bladder,
Fig. I . The low-set umbilicus and bulging of the bladder through
but there was pubic diastasis of 2.0 cm. The patient did divaricated recti while crying. Also seen is the hypoplastic right
not return for subsequent follow-up. labia majora along with a skin tag (Case 1).

01997 Scandinavian University Press. ISSN 0036-5599 Scand J Urol N q h r o l 3 1

 104 S. P. Sahoo et al.
Fig. 2. Close-up view of abdomen and perineum showing bifid
clitoris, normally located umbilicus, anovestibular fistula and gross
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herniation of viscera while crying (Case 2).
urography revealed a closed bladder which was
displaced antero superiorly because of the extensive
muscular defect. Anal cut-back was done as a primary
measure for anorectal malformation. After one month,
primary reconstruction of the anterior abdominal wall
and pubic bone approximation were performed with-
out osteotomies.
Case 3: A 6-month-old male infant presented with
complaints of severe diarrhoea of 3 days’ duration and
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oliguria of 2 days’ duration. There was no significant
past history. The physical examination was not
remarkable except for moderate dehydration. Exam-
ination of the abdomen and pelvis revealed a low-set
umbilicus (about 2 cm above the symphysis pubis),
symphyseal separation and grandular epispadias with
mild dorsal chordee (Fig. 3). The anus was anteriorly
placed with perianal excoriation. There was an
inguinal hernia and cryptorchidism on the right side.
USG and intravenous urography showed agenesis of
right kidney without any other urinary tract anomalies.
On skiagram there was symphyseal diastasis of 2.5 cm.
The patient’s dehydration was managed conserva-
tively. Herniotomy and orchidopexy on the right side
were done electively after 3 weeks.
All three cases were continent.
CONCLUSION
In covered exstrophy there are classical musculo-
skeletal defects (pubic diastasis and divarications of
the recti), often with a low-set umbilicus. The bladder
is closed, but the anterior wall protrudes through
divergent recti and is covered by a varying degree of
subcutaneous tissue and skin. This is also known as a
split symphysis variant (3). Persistence of the cloaca1
membrane without later rupture explains the develop-
Scand J Urol Nephrol31
Fig. 3. Low-set umbilicus (2cm from symphysis pubis) along with
glandular epispadias. A linear scar in the right inguinal region is
from hemiotomy and orchidopexy (Case 3).
ment of covered exstrophy. Covered exstrophy was
first described by Mackenzie in 1935 (8). Since then
15 cases of covered exstrophy (including our 3 cases)
have been presented, as shown in Table I (1-3, 5-9,
11-14).
First Pattern: Sex.There was a preponderance of
female (60%) as opposed to classical exstrophy in
which males predominate, but the number of these rare
variations is hardly large enough to provide more than
a suggestion of the sexual incidence.
Second Pattern: Split symphysis and umbilicus. Split
symphysis was observed in all cases. Normally, the
intrapubic distance should not be more than 10 mm at
any age (10). A low-set umbilicus was observed in at
least 73% of cases. The characteristic musculoskeletal
defect was seen in 100% of cases.
Third Pattern: Gastrointestinal tract abnormalities.
Associated gastrointestinal anomalies were seen in 9
(60%) cases, 6 (40%) of which were anorectal
malformations and 3 (20%) of which were visceral
sequestrations. However, the incidence of associated
anorectal malformation with classical exstrophy was
only 7.5% (4). In covered exstrophy there is delayed
closure of the abdominal parities following formation
of the exstrophy. During secondary closure of the
parities, portions of the exstrophied organs may
become sequestered at the anterior abdominal surface,
although this is rare (3). The sequestered viscera is
usually colon or ileal remnant without communication
with the underlying gastrointestinal tract. However,
this seqestered viscera can also occur without any
other visible abnormality apart from classical musculo-
skeletal defects (11).
Fourth Pattern: Genitourinary tract abnormalities.
Most of the patients (80%) had genitourinary tract
abnormalities, usually minor. The genital abnor-
 Covered exstrophy 105

Table I. Various anomalies in covered exstrophy reported in the literature

Published series of Present series of

12 cases from 1935-94 3 cases from 1989-95

Sex Males-5 cases Males-1 case

Females-7 cases Females-2 cases
Split symphysis All 12 cases All 3 cases
Umbilicus Low-set--9 cases Low-set-2 cases
Normal-3 cases Normal-1 case
Alimentary Anorectal malformation4 cases Anorectal malformation
tract anomalies Visceral sequestration-3 cases 2 cases
Genital anomalies Bifid clitoris-5 cases Bifid clitoris-1 case
in Females Cleft labia major-1 case Hypoplastic labia
Short vagina-1 case majora-1 case
Stenosed duplicate
vagina- I case
In Males Cryptorchidism-2 cases Cryptorchidism- I case
Small penis-2 cases Epispadias-I case
Broad flat scrotum-1 case Broad flat scrotum-1 case
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Epispadias-1 case
Urinary tract Unilateral renal Unilateral renal
anomalies agenesis-1 case agensis-I case
Dysplastic kidney-1 case
Reflux-2 cases
Septate bladder-1 case
Other Omphalocele-2 cases Inguinal hernia-I case
anomalies Inguinal hernia-1 case Partial sacral
Heart disease-2 cases agenesis-1 case
Patent ductus
Botalli-1 case
Spina bifida-1 case
-

Children with covered exstrophy always have

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malities observed in females included bifid clitoris

(67%), labial cleft (11%), hypoplastic labia (11 %),
short vagina (11%) and stenosed duplicate vagina
(11%). No genital abnormalities were seen in 22% of
the female patients. 83% of the male patients had
genital abnormalities including cryptorchidism (50%),
epispadias (33%), small penis (33%) and broad flat
scrotum (33%). However, all patients were continent
except for one with complete epispadias. This implies
that pubic separation per se does not cause extrinsic
sphincter-related incontinence in covered exstrophy
unless there is complete epispadias. One of our cases
(case 3) had glandular epispadias but was continent.
Hence, reconstruction of the sphincteric complex is
not required in most of the cases. Urinary tract
abnormalities occurred in 5 out of 15 cases. These
were comprised of unilateral renal agenesis ( n = 2)
(13%), dysplastic kidney (n = 1) (6.6%),reflux (n = 2)
(13%), and septate bladder ( n = 1) (6.6%).One patient
had both dysplastic kidney and unilateral reflux.
Fifth Pattern: Other anomalies. Six patients (40%) had
other associated anomalies like omphalocele, patent
ductus Botalli, heart disease (VSD and PS), inguinal
hernia, spina bifida (thoraco cervical) and partial
sacral agenesis. The presence of these anomalies,
especially heart disease and thoracocervical spina
bifida, suggests the possibility of extensive teratogenic
action (14).
symphyseal separation and divergent rectus abdominis
muscles. Nearly all of them have minor genital
abnormalities. In the absence of severe epispadias,
all children are continent of urine and do not require
reconstruction of the sphincteric mechanism. The
incidence of associated anorectal malformation is
greater than in classical exstrophy. However, the
treatment in covered exstrophy cases is simple with
good results, and usually consists of plastic repair of
the anterior abdominal wall defect, which is far less
complex than repair of classical bladder exstrophy
with epispadias.
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vation from 80 cases. JAMA 1952; 149: 1640-1644.

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