True duplicate bladder exstrophy: a rare lesion.
ABSTRACT A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.
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ABSTRACT: To describe a unique approach to the management of duplicate bladder exstrophy combining initial bladder closure and epispadias repair. Bladder exstrophy has been successfully managed by staged surgical repair with early bladder closure, subsequent epispadias repair, and, finally, bladder neck reconstruction. Duplicate bladder exstrophy is a rare variant of the exstrophy complex with fewer than 20 cases reported. A male newborn presented with the appearance of both bladder exstrophy and a ruptured omphalocele. Repair of the omphalocele and bilateral orchiopexy was performed shortly after birth, but bladder closure was delayed until there was complete healing of the omphalocele defect, and the investigators believed the infant was ready for abdominal wall and bladder exstrophy closure. At age 8 months, bladder closure was performed with the intraoperative finding of a duplicate bladder lying posterior to the exstrophied bladder. The left ureter drained on the exstrophic bladder plate, and the right ureter drained into the posterior (internalized) duplicate bladder. After anterior innominate and vertical iliac osteotomies, the left ureter was reimplanted into the posterior bladder. A portion of the exstrophied bladder was then tubularized to construct a neourethra of the epispadic penis. The child has done well with an excellent cosmetic appearance of the abdominal wall and a straight phallus. The bladder subsequently required a Mitrofanoff-type continent stoma along with bladder augmentation, which was performed at the time of his Young-Dees-Leadbetter bladder neck reconstruction (age 3.5). The child is currently continent but requires intermittent clean catheterization through his appendicovesicostomy. The investigators report a unique and unexpected variant of bladder exstrophy and its successful management.Urology 03/1999; 53(2):401-4. · 2.42 Impact Factor
Article: Duplicate exstrophy of the bladder.[Show abstract] [Hide abstract]
ABSTRACT: Duplicate exstrophy of the bladder is a rare, distinct congenital anomaly without additional major anomalies of the urinary tract. A 15-year-old boy with duplicate exstrophy is reported on, and the possible role of bladder sequestration in the development of classical musculoskeletal deformities in this anomaly is discussed.Journal of Pediatric Surgery 05/1999; 34(4):626-7. · 1.38 Impact Factor
- Plastic & Reconstructive Surgery 03/1963; 31(4):396. · 3.54 Impact Factor
Correspondence and Reprint requests : Dr. Basant Kumar,
Room-189, Resident Doctors‘ Hostel, JLN Marg, S.M.S. Medical
College, Jaipur – 302004, Rajasthan, India.
[Received May 22, 2008; Accepted August 26, 2008]
Exstrophy bladder is an exceedingly rare congenital
anomaly and can present as a spectrum of anomalies.1
The incidence of exstrophy–epispadias complex ranges
from 1 / 10,000 to 1 / 50,000 live births and most of
these are classical bladder exstrophy.1,2 Rare variants of
bladder exstrophy accunt only 10 % of cases.2 Duplicate
exstrophy is one of the rarest variant and only 23 cases
reported in literature so far.2 True duplicate exstrophy
as defined by Marshall and Muecke.3 is further an
extremely rare variant of exstrophy bladder and only 7
cases reported in world literature.4 Here, we describe a
2-day-old male child with true duplicate bladder
exstrophy and briefly review the literature.
REPORT OF CASE
A 2-day-old, normally delivered, full term male child
weighing 2500 kg was admitted in our institute as a case
of bladder exstrophy. On examination, the rectus muscles
were widely separated in the suprapubic region and the
dry bladder mucosa protruded through the abdominal
wall defect. Penile length was normal and directed
upward. It had normal urethral opening at the tip of glans
with a dorsal groove on the corona glandis (Fig. 1). Both
testicles were palpable in respective scrotum. Anal
opening was normally placed and was adequate. No
other congenital anomaly was present. Child was passing
urine normally through the meatal opening; present at the
tip of glans (Fig. 2). X-ray pelvis showed a widened
symphysis pubis. Bilateral kidney was normal on
True Duplicate Bladder Exstrophy: A Rare Lesion
Basant Kumar, Chetan Sharma and Devendra D. Sinha
Department of Pediatric Surgery, Sir Padampat Mother and Child Health Institute, (JayKayLon Hospital)
S.M.S. Medical College, Jaipur, Rajasthan, India
A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true
duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe
another one and briefly review the literature. [Indian J Pediatr 2009; 76 (8) : 852-853] E-mail : drbkpg75@ rediff.com,
Key words : Duplicate exstrophy; Bladder exstrophy; Exstrophy variants
ultrasonography; serum creatinine and blood urea were
within normal limits. Cystourethroscopy showed
normal lower urinary tract. Under general anesthesia,
the redundant exstrophied mucosa was excised and the
Fig. 1. Photograph showing true duplicate exstrophy.
Fig. 2. Photograph showing dorsal groove over corona.
852 Indian Journal of Pediatrics, Volume 76—August, 2009
Tru Duplicate Blaldder Exstrophy: A Rare Lesion
Indian Journal of Pediatrics, Volume 76—August, 2009 853
abdominal wall defect was closed. No attempt was
made to correct the dorsal chordee. Post-operative
period was uneventful.
True duplicate exstrophy as defined by Marshall and
Muecke3 refers to a suprapubic exstrophic mucosal
plate that is associated with a subjacent covered
bladder and a relatively well formed phallus. The
exstrophic plate did not receive ureters and there was no
epispadias.3 Embryologically, it represents part of the
spectrum of superior vesical fissure in which the
underlying fissure has fused, leaving only an overlying
bladder-mucosal plate.3,4 True duplicate bladder
exstrophy is an extremely rare variant of bladder
exstrophy and only 7 cases reported in world
Sheldon et al5 believe that duplicate bladder
exstrophy can be divided into two categories. The first
is associated with a suprapubic exstrophic mucosal
plate and a covered bladder. The phallus is relatively
well formed in this setting. The second type is true
duplication with the classic findings of exstrophy
complex.5 Exstrophy variants have been classified into
five main subgroups by Chadha et al6 namely
pseudoexstrophy or covered exstrophy; superior vesical
fistula; superior vesical fissure; duplicate exstrophy and
covered exstrophy with visceral sequestration.3,6,7,8,9
Marshall and Muecke3 have suggested that an
overdeveloped cloacal membrane prevents the lateral
mesoderm from migrating medially, preventing its
insertion between the ectodermal and endodermal
layers. Thus, numerous variations in the exstrophy
complex can develop. The superior vesical fissure
represents one such variation in which the cloacal
membrane opens only in the upper portion.3 If the
superior vesical fissure fuses, embryologic remnants of
bladder may persist externally, leading to duplication
of the bladder.3 Patients with an exstrophy variant
associated with complete vesical duplication may have
associated duplication of uterus and vagina, bifid
clitoris, bifid caecum, double appendix and abnormal
mesentery.6 An associated low anorectal malformation
or ectopic anus and spinal dysraphism were also
reported with it.6
Our case resembles to those of Ellis7 and Marshall
and Muecke3 and identical to the patient described by
Mathur P et al4. The operative management for our
patient was relatively simple. Excision of the redundant
exstrophied mucosa and closer of the abdominal wall
defect gave excellent result.
Basant Kumar - Concepts, Design, Literature search, clinical studies,
data acquisition, manuscript preparation and manuscript editing;
Chetan Sharma – Concepts, design, definition of intellectual
content, clinical studies, manuscript editing and manuscript review;
Devendra D Sinha - Conepts, design, definition of intellectual
content, clinical studies, manuscript editing and manuscript review.
Conflict of Interest : None
Funding Source : None
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