Case Report
Cloacal Exstrophy Repair with Primary Closure of
Bladder Exstrophy: A Case Report and Review of Literature
George Bethell,
1
Navroop Johal,
2
and Peter Cuckow
2
1
Institute of Child Health, University of Liverpool, Liverpool L69 3BX, UK
2
Great Ormond Street Children’s NHS Trust, Great Ormond Street, London WC1N 3JH, UK
Correspondence should be addressed to George Bethell; gbethell@doctors.org.uk
Received 15 March 2016; Accepted 8 May 2016
Academic Editor: Carmelo Romeo
Copyright © 2016 George Bethell et al. Tis is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Cloacal exstrophy is the most complex congenital, ventral, abdominal wall defect. Traditionally surgery consists of a staged approach
to repair which takes place on many separate theatre visits. In this case a primary approach was undertaken resulting in a relatively
short inpatient stay and a reduced risk from multiple surgical procedures under general anaesthesia.
1. Introduction
Cloacal exstrophy (CE) is the most complex congenital,
ventral, abdominal wall defect with an incidence of less than
1 in 200,000 live births [1]. Conventionally surgical repair
consisted of a multiple staged approach; however here we
describe a primary approach in a male neonate undertaken
by two surgical specialties.
2. Case Report
A male neonate was born at 39 weeks’ gestation by normal
vaginal delivery to nonconsanguineous parents. He was diag-
nosed with CE. Antenatally, bladder exstrophy was suspected
at week 21 due to nonvisualisation of the bladder on foetal
ultrasonography and the decision was made to continue the
pregnancy. Te remainder of the gestation and labour were
unremarkable and he has a healthy brother aged 3 years old.
Immediate management consisted of dressing the defect
to minimalize fuid losses and the risk of infection. He was
then transferred, on day 1 of life, from a district general hos-
pital to a tertiary pediatric unit, where intravenous antibiotics
were given. Neurological examination and spinal ultrasound
scan did not fnd evidence of myelocystocele. On full exam-
ination we found an imperforate anus, bifd scrotum, and
pubic symphysis diastasis. Te small omphalocele was tied as
shown in Figure 1.
On day 9 of life the patient was taken to theatre where
primary closure of cloacal exstrophy with bilateral oblique
pelvic innominate osteotomies was undertaken. Firstly the
orthopaedic team performed pelvic osteotomies, followed by
the pediatric urology team closing the exstrophied caecum
with anastomosis to the hind gut, creating an end colostomy,
and closing the omphalocele with excision of the appendices.
Te bladder plates were then mobilised and sutured together
dorsally and ventrally. Te abdominal wall was then closed
ventrally with a catheter placed in the neck of the bladder and
a drain placed in each hemibladder wall. Finally orthopaedics
applied a spica cast. Overall theatre time was fve and a half
hours.
Te patient was started on total parenteral nutrition on
his return to the urology high dependency unit; urine output
was monitored closely along with the other vital signs. Te
catheters were removed; he was discharged home three weeks
following surgery and then seen in clinic when he was eight
weeks old (Figure 2). He developed no known complications.
Future surgical treatment will involve a bladder neck recon-
struction with epispadias repair and a pull through procedure
if the external anal sphincter is found to be functioning with
electrical stimulation.
Hindawi Publishing Corporation
Case Reports in Pediatrics
Volume 2016, Article ID 8538935, 3 pages
http://dx.doi.org/10.1155/2016/8538935