Professional Documents
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current management
and postoperative imaging
(Ketsia Pierre & Joseph Borer & Andrew Phelps & Jeanne S. Chow)
20 January 2014
Journal Reading
Oleh
dr.Mahendra
Abstract
Bladder exstrophy is a rare malformation
Incomplete closure of the bladder with
mucosa continuous with the abdominal wall,
epispadias, and alterations in the pelvic
bones and muscles.
More common in boys
Embryology
Anatomical defects
The pelvic floor anatomy is also altered. The puborectal sling is flatter
(compared to its more normal conical shape) and wider with divergence of
the levator ani muscles. Compared to the normally centered position of the
rectum within the levator muscles, the rectum is positioned in the anterior
third of the pelvic floor (Fig. 5)
The ureters insert low into the bladder and have a J-shape configuration
because of the enlarged pouch of Douglas, which forces the distal ureters
downward and laterally. These children invariably have vesicoureteral reflux
following exstrophy closure unless ureteral reimplantation is performed (Fig.
6)
Stage I
Stage II
Stage III
Iliac osteotomy
Several types of pelvic osteotomies have
been developed. The first described was the
bilateral posterior iliac osteotomy.
Pelvic osteotomies reduce pubic diastasis,
which has many benefits.
In some cases iliac osteotomy is not
performed, typically in infants younger than
72 h with pubic diastasis less than 4 cm
Augmentation cystoplasty
Catheterizable conduits
Mitrofanof
Appendicovesicostomy
YangMonti ileovesicostomy
ileovesicostomy
Fluoroscopy
Osseous structures
Ultrasound
Kidneys
Bladder
Augmented bladder
MRI
Fluoroscopy
Urethrocutaneous fistula
Urethral stricture
Bladder rupture/perforation
Complications of bladder augmentation
Ultrasound
Renal scarring
Stone formation
Miscellaneous complications
Bladder malignancy
Bladder malignancy, most commonly
adenocarcinoma, in children with bladder
exstrophy occurs rarely in the present day of
early primary repair. Squamous cell carcinoma
is the second most common pathology and is
thought to occur on a background of squamous
metaplasia.
Conclusion
Bladder exstrophy is a rare congenital
abnormality with a complex surgical repair
regardless of the approach.
Imaging plays a key role in the follow-up of
these patients.
Therefore an understanding of the surgical
techniques and the norma postoperative
appearances of bladder exstrophy repair and
familiarity with the imaging appearance of
complications are essential for the pediatric
radiologist and pediatric urologist.
Thank You