Bladder Exstrophy and Epispadias of the Urethra
Review literature: (Kiddoo et al, 2004) (Woodhouse et al, 2006)
Definition of Bladder Exstrophy and Epispadias
The exstrophy-epispadias complex is a serious rare malformation syndrome with bladder exstrophy, epispadias and/or cloacal exstrophy with often lifelong consequences.
- Bladder exstrophy: 1:30 000
- Epispadias: Boys 1:40 000, 1:200 000
Pathology of the Exstrophy-Epispadias Complex
Individual components of the exstrophy-epispadias complex are expressed variably:
Bladder exstrophy is the presence of bladder tissue outside the body with a deficient closure of the lower anterior abdominal wall: the bladder is open anteriorly, the trigonum of the bladder is visible. The posterior bladder wall prolapses through the abdominal wall defect. In addition, there is a diastasis of the symphysis with missing contact between the right and left pubic bone (range 2 to 8 cm).
An epispadias is a rare type of malformation of the urethra. In boys, the urethra ends in an opening on the dorsum of the penis. In girls, the urethra is usually completely open and runs from the bladder neck to the hymen. Proximal variants of epispadias are associated with urinary incontinence due to defects of the sphincter muscle.
Cloacal exstrophy is a malformation with missing closure of the ventral colon wall, there is an opening in the proximal direction to the ileum and in the distal direction to the anus. Hemibladders with one ureteral orifice are located right and left of the open colon.
Often associated anomalies:
Deformities of the spine and pelvis, hip dysplasia, abnormalities of the upper urinary tract, uterine malformations, hernia, abnormalities of the heart.
Etiology of Bladder Exstrophy and Epispadias
The missing ingrowth of mesoderm in the cloacal membrane prevents the development of the anterior abdominal wall. The fragile cloacal membrane ruptures in the course of embryonic development. The timing of the rupture and the stage of development at the time of the rupture determines the extent of the malformation: bladder exstrophy, epispadias and cloacal exstrophy. Other mechanisms or alternative explanations are defects of the genital tubercle development, an abnormal pelvic bone development or lack of development of the lateral body wall folds.
Treatment of the Exstrophy-Epispadias Complex
Early management after birth:
- Ligation of the umbilical cord to avoid the umbilical clamp
- Protection of the delicate bladder mucosa by covering with plastic wrap with regular moistening.
- Transportation of the child to a center with experience in the surgical treatment of the exstrophy-epispadias complex.
- Clinical examination to estimate the bladder capacity.
- Urogenital imaging: ultrasound of the kidneys, renal scintigraphy.
Primary closure of bladder exstrophy:
The estimated bladder capacity should be above 5 ml for a successful primary closure. The surgery is done during in the first days of life and includes the complete mobilization of the bladder plate, preparation of the urethral plate and corpora of the penis, closure of the bladder with bladder neck and proximal urethra, approximation of the symphysis ventral to the bladder neck. Some authors advocate a closure of epispadias in the same session, if good conditions are present.
Closure of epispadias:
The surgical correction of epispadias is usually performed in the 6th to 12th month of life. Phallic enlargement is obtained with testosterone treatment. Necessary operation steps are the mobilization and the tubularization of the urethral plate, mobilization and separation of the corpora cavernosa ("penile disassembly technique"), placement of the neourethra to the ventral of the penis, reunification of the corpora, and skin closure.
Surgical alternatives for bladders with small capacity:
- Delayed closure (6–12 months after birth) after growth of the bladder template.
- Delayed closure with ileocystoplasty to increase bladder capacity.
- Delayed urinary diversion with a continent stoma.
Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
Kiddoo u.a. 2004 KIDDOO, D. A. ; CARR, M. C. ;
DULCZAK, S. ; CANNING, D. A.:
- Initial management of complex urological disorders: bladder
In: Urol Clin North Am
31 (2004), Nr. 3, S. 417–26, vii-viii
Woodhouse u.a. 2006 WOODHOUSE, C. R. ; NORTH,
A. C. ; GEARHART, J. P.:
- Standing the test of time: long-term outcome of reconstruction of the
In: World J Urol