Epispadias/Extrophy Flashcards
Prune belly syndrome
sex affected
triad
almost always affects boys
genetic consition
triad of
abdominal wall defects
UDT often intra abdominal may be bilat usually infertile
GU defects, dilated ureter and bladder, VUR, detrusor failure
conditions in extrophy complex 4
epispadias male and female
classic bladder exstrophy
classic cloacal exstrophy
cloacal exstrophy variants
incidence bladder exstrophy complex
1/30,000
embryological cause
Failure of migration of mesoderm to the area of the cloacal membrane, and subsequent lack of development of an intermediate layer between its inner (endodermal) and outer (ectodermal) layers, leads to rupture and exposure of an open bladder plate and urethra
features exstrophy complex
absent bladder wide pubic rami lower abdominal mass low lying umbilical cord omphalocoele lumbosacral abnormalities
initial management classic bladder exstrophy
usually few other associated abnormalities, born at term and well put to breast vitamin K cling film inside the nappy transfer specialist centre anti fungals US renal tract surgery within 48 hours
Because affected newborns are born at term and rarely have other medical problems, they can be put to the breast and require no medical intervention in the delivery suite
Vitamin K should be administered, particularly if surgery is anticipated in the next few hours
While transfer to an appropriate specialist centre is arranged, the exposed bladder plate is protected with a cling-film wrap inside the nappy.
Following transfer, the child is assessed at the specialist centre, a baseline renal ultrasound is performed and oral antifungal prophylaxis is commenced as a prelude to surgery in the first 48 hours of life
Classic bladder exstrophy associated abnormalities and hernia risk
Classic bladder exstrophy has few associated anomalies and babies are usually born at or close to term and are otherwise well. Inguinal hernias are present in up to 80% of boys and 15% of girls but may not become apparent until after primary closure.
appearance classic bladder exstrophy at birth
If not detected antenatally, classic bladder exstrophy presents at birth with a
visible bladder plate below a low-set umbilical cord.
The mucosa may become quite inflamed and polypoid, especially after a few hours’ exposure following delivery.
The shaft of the penis is usually short and thick with a good-sized glans
operation for classic bladder exstrophy
Epidural catheter
Broad spectrum antibiotics
Ureteric catheters 6Fr feeding tubes inserted
Bladder plate dissected free of skin and rectus muscles either side
Any polyps excised
Mucosal defects repaired
Hernial sacs ligated
Umbilical root excised
Close bladder neck and proximal urethra in midline
Ureteric catheters brought out through bladder closure then lateral to midline skin closure
Fine silicone stent across urethral opening
Approximate pubic rami
Legs immobilised with frog leg plaster
post op care after closure classic bladder exstrophy
Ultrasound evaluation of the upper urinary tract is performed after all catheters have been removed to assess bladder emptying and the upper tracts before discharge
Regular follow-up with ultrasound is scheduled to evaluate progress and to help plan later interventions for continence and genital reconstruction
embryology of exstrophy
Failure of migration of mesoderm to the area of the cloacal membrane, and subsequent lack of development of an intermediate layer between its inner (endodermal) and outer (ectodermal) layers, leads to rupture and exposure of an open bladder plate and urethra.
Kelly procedure at GOSH
Procedure of choice at GOSH
After successful neonatal closure of the bladder, an examination under anaesthetic is performed at 3 months to check the bladder and its outlet.
Irrespective of bladder size, the operation is performed from 6 months of age.
Initially, the bladder is reopened in the midline and both ureters reimplanted using the Cohen technique.
The soft tissues, including the urethra, penile corpora and pelvic floor, are then fully mobilised before reconstructing the bladder outlet, urethra, sphincter and penis
cloacal exstrophy associated disorder
Renal Ectopic kidney, agenesis, hydronephrosis Sacral agenesis Spinal dysraphism Club foot, hip and pelvic deformity Small bowel defects, malrotation, duodeal atresia, short gut Cyanotic heart diease
cloacal exstrophy abnormalities - 7
Bladder exstrophy and omphacele Associated with heart, spine, limbs anomalies Malrotation and absent colon Imperforate anus Severe pubic diastasis Absent urethral plate Often right and left side of penis not joined Usually one kidney is pelvic
Cloacal exstrophy is a more severe variant in which there is probably an additional failure of septation of the cloaca, so that the bladder anteriorly is not separated from the hindgut posteriorly.
This has the effect of bringing both bladder and bowel components to the surface, with a central bowel field that lies in the midline between two separated halves of the bladder.
The anus is imperforate and the sacrum is shortened with a high incidence of myelomeningocoele and spinal dysraphism
The pubic diastasis in these patients may be so great that the penile corpora fail to meet in the midline and are completely separated. In addition, the testes are often undescended and absent from the scrotum or hemiscrotum. Superiorly there is usually an associated exomphalos
initial management cloacal exstrophy
It is usual to delay primary closure while other medical problems affecting the gastrointestinal tract and cardiorespiratory systems are evaluated and treated if necessary.
Occasionally, surgical reconstruction may have to be delayed for several months.
During this time the exstrophied bladder/bowel plate is protected with cling film and barrier creams applied to the surrounding skin.
surgery for cloacal extrophy
Routine preoperative evaluation includes urinary tract and spinal cord ultrasound examinations.
Iliac osteotomies are routinely performed at the time of closure because of the surgical delay and because of the wider diastasis invariably present in these patients.
The use of osteotomies usually permits a tension-free abdominal wall closure but when it is not possible to reduce the abdominal contents and close the abdominal wall, a silo is attached over the upper abdomen to reduce tension.
The bladder/bowel plate is dissected free from the skin and rectus muscles superolaterally and the abdominal cavity is entered.
The proximal and distal bowel loops of bowel joining the midline bowel plate are identified and separated from the two hemibladders.
A tubularised distal hindgut tube is created which is usually brought out in the left iliac fossa as an end colostomy (in preference to an incontinent anal canal).
The hemibladders are joined and closed, reconstructing a bladder outlet and proximal urethra
primary epispadias
The anomaly classically presents in childhood with a failure of potty training or a history of dribbling or stress incontinence. In some cases there is a long history of attendance at wetting clinics.
epispadias repair cantwell ransley
The Cantwell–Ransley epispadias repair is favoured by many surgeons and can be performed in the first year of life.
In this procedure the urethral plate is fully mobilised off the penile corpora from the proximal urinary outlet and corporeal bodies but is left attached to the glans.
The urethral plate is then tubularised and brought to a ventral position, below the corpora cavernosa, which are then approximated dorsal to the urethra
incontinence post epispadias repair
difficult to reconstruct bladder neck
see on cystoscopy veru close to bladder neck or even in bladder
may need artificial sphincter
or mitrofanoff
Epispadias is commonly associated with incontinence and on cystoscopy there is demonstrable weakness of the bladder outlet with the verumontanum lying close to the bladder neck or even within the bladder.
Patients who have only undergone penile reconstruction (the majority) will require additional bladder neck/sphincter surgery if their continence is impaired.
The surgical options include a bladder neck repair or artificial urinary sphincter.
In practice, it is difficult to reproduce a physiological bladder neck and sphincter mechanism and for some patients it may be necessary to progress to bladder augmentation and a Mitrofanoff conduit
types of urachal abnormalities
patent urachus
primary epispadias in girls
Underlying deficiencies of the bladder neck and proximal urethra and striated sphincter complex determine the degree of associated incontinence. As a general rule, however, the severity of incontinence correlates with the position of the urethral opening, being most severe in proximal epispadias
treatment epispadias in girls
Since the majority of girls with epispadias are incontinent, reconstructive surgery must be directed to correction of the incontinence as well as the genital abnormality.
Good results have been reported for distal urethral reconstruction, but more proximal bladder neck surgery may also be required.
The Kelly procedure offers good prospects of continence coupled with an improved external cosmetic appearance
