Download MBBS (Bachelor of Medicine, Bachelor of Surgery) 1st Year, 2nd Year, 3rd Year and Final year Pediatric Surgery 11 Exstrophy Epispadias PPT-Powerpoint Presentations and lecture notes
EXSTROPHY EPISPADIAS
Introduction
Bladder exstrophy is a rare midline defect and exists as
part of a larger spectrum of abdominal-pelvic fusion
abnormalities, known collectively as the exstrophy-
epispadias complex (EEC).
Presentation of EEC can range from isolated glanular
epispadias to cloacal exstrophy, in which several other
organ systems may be affected.
Spectrum
Epispadias
the urethra is a partial or complete open plate on the dorsal surface of
the phal us.
Classic bladder the bladder is an open plate on the low abdominal wal and the urethra
exstrophy
is epispadic.
Cloacal
the bladder and the ileocecal junction of the bowel are an open plate
exstrophy
on the low abdominal wal associated with other malformations.
Exstrophy
partial manifestations are seen of the above malformations.
variants
Anatomic pathology: Bladder exstrophy
? Brock and O'Neill described exstrophy as
? `if one blade of a pair of scissors were passed through the urethra of a normal
person; the other blade were used to cut through the skin, abdominal wall,
anterior wall of the bladder and urethra, and the symphysis pubis; and the cut
edges were then folded laterally as if the pages of a book were being
opened.'
? Presence of a layer of urothelium on the anterior abdominal wall
representing the bladder and urethra.
? At birth, the urothelium is usually normal in appearance.
? Ectopic bowel mucosa or polypoid lesions consistent with cystitis cystica
and/or glandularis may be present.
? If left untreated and without meticulous protection after birth, the exposed
urothelium will undergo squamous metaplasia in response to acute and
chronic inflammation. Other inflammatory changes such as cystitis cystica
and/or glandularis will also be seen.
? When left chronically exposed to the environment, the areas of squamous
metaplasia often undergo malignant degeneration to adenocarcinoma or
squamous cell carcinoma.
Associated anomalies
? Classic exstrophy and epispadias have a relatively low incidence of associated
anomalies.
? Patients with cloacal exstrophy have associated anomalies more often than not.
? These anomalies can affect:
? upper urinary tract
? intestines
? skeletal system
? neurologic system.
? Renal anomalies can occur with bladder
exstrophy
? Cystic dysplasia
Kidneys and
? Ureteropelvic junction (UPJ) obstruction
upper urinary
? pelvic kidney
? Megaureter
tract
? renal hypoplasia
? horseshoe kidney
? Vesicoureteral reflux (VUR) occurs almost
universally.
? In males:
? the penis is broad and shortened because
the corpora cavernosa are splayed laterally,
attached to the separated pubic bones.
Genitalia
? The penis is deflected dorsally and may have
true intrinsic dorsal chordee
? in most cases, the length of the penis and
the size of the exstrophic bladder plate are
inversely related
Penile and pelvic measurements in normal men and patients with exstrophy. ISD, intersymphyseal distance;
aCC, corpora cavernosa subtended angle; Cdiam, corpus cavernosum diameter; PCL, posterior corporeal
length; ICD, intercorporeal distance; ACL, anterior corporeal length; TCL, total corporeal length.
? The cavernosal nerves are located on the lateral aspects of the corporeal bodies.
? In the normal situation, these nerves can be found on the dorsal aspect of the penis after
they traverse the posterolateral aspects of the prostate and the membranous urethra.
? The prostate is also incompletely formed in exstrophy.
? The scrotum is usual y not affected
? increased distance between the base of the penile shaft and the scrotum
? broadening of the scrotum dependent on the degree of diastasis
? The testes may be undescended.
? Because of the underlying bladder neck anomalies, exstrophic males may have impaired
fertility.
? Retrograde ejactulation because bladder neck cannot close completely.
? In females:
? Mons pubis is absent
? Bifid clitoris
? Anterior labia are laterally displaced although they fuse in the midline
posteriorly.
? The vagina and introitus are also displaced anteriorly from their usual position.
? The vaginal opening may be stenotic.
? Internal female genital structures (uterus, cervix, fallopian tubes, and ovaries)
are unaffected in classic exstrophy.
? Uterine prolapse as a result of deficient pelvic floor support can occur in the
older exstrophy patient and poses particular problems with pregnancy.
? Early primary bladder reconstruction with pelvic closure may decrease this risk.
? Uterine suspension procedures such as sacro-colpopexy can be employed in these
situations.
? The anus is often located anteriorly in the
exstrophy complex.
Anorectal and
? Few exstrophic patients will have insufficient
anal continence due to the underlying
intestinal
abnormalities of the pelvic floor support
structures including the levator ani and
abnormalities
puborectalis muscles.
? In untreated patients rectal prolapse can also
occur owing to insufficient pelvic floor support.
? Diastasis of the pubic symphysis occurs as part
of the exstrophy complex.
Skeletal
? This results from outward rotation of the
abnormalities
innominate bones along both sacroiliac joints.
? Outward rotation of the pubic rami at the iliac
and ischial junctions is also seen.
? Gait abnormalities in these children arise as a consequence of these bone
abnormalities.
? Many of these children learn to ambulate with a wide waddling gait. This
gait abnormality resolves as the children grow.
? Orthopedic procedures to re-approximate the pubis symphysis do not offer
any long-term benefits to these patients from an orthopedic viewpoint.
? Osteotomies to re-approximate the pubis symphysis
? increase the chance of successful primary bladder closure
? have a significant role in securing continence
? support of the pelvic diaphragm
Fascial abnormalities
? Inguinal hernias are commonly associated with exstrophy in both
male and female patients.
? Majority of these hernias occur indirectly.
? occur as a consequence of the enlarged internal and external inguinal rings
? lack of obliquity of the inguinal canal
? Rectus fascial defect
? Inferiorly, the pelvic floor support structures are also compromised.
? At the inferior portion of the fascial defect, these patients possess an
anteriorly located intersymphyseal ligament or band representing the
attenuated urogenital diaphragm.
Neurologic
? Spinal cord abnormalities
? Occult spina bifida and even myelomeningocele
system
can be seen in combination with bladder
exstrophy
Anatomic pathology: Cloacal exstrophy
? The bladder plate associated with cloacal exstrophy is divided in half
by the hindgut plate.
? the hindgut plate represents the deformation in the development of
the colon that occurs with cloacal exstrophy.
? Ileum enters and intussuscepts into the middle of the hindgut,
creating the `trunk of an elephant's face' appearance with
appendiceal appendages located laterally to give the impression of
`tusks on the face of the elephant'.
Photograph and schematic drawing of a neonate with cloacal exstrophy. BP, bladder plate; C, cecal plate; CB, corporeal
body; HG, hindgut; IL, ileum; O, omphalocele; UO, ureteral orifice
? With cloacal exstrophy, the bladder neck (internal urethral sphincter)
and external urethral sphincter are not fully developed.
? However, because the innervation to these structures is usually
intact, anatomic closure theoretically offers the possibility of
achieving urinary continence.
? The urethral plate is characteristically short as well.
Associated anomalies
? Kidneys and upper urinary tract
? Renal anomalies are much more common with cloacal exstrophy.
? They include anomalies of location such as pelvic kidneys or crossed fused
ectopia.
? Horseshoe kidneys, renal agenesis, and UPJ obstruction may also occur.
? Genitalia
? In cloacal exstrophy the penis is often separated into two hemiphalluses
owing to the wide pubic diastasis.
? Cryptorchidism is the rule with cloacal exstrophy.
? For girls, in addition to the genital pathology described with bladder
exstrophy, uterus didelphys and other fusion anomalies of the m?llerian duct
structures are seen in up to two-thirds of cloacal exstrophy patients.
? Vaginal agenesis occurs in one-third of girls with cloacal exstrophy.
? Anorectal and intestinal abnormalities
? Associated intestinal abnormalities specific to cloacal exstrophy include
? imperforate anus
? foreshortening of the midgut
? bowel duplication
? Malrotation
? Intestinal atresia, and Meckel's diverticulum.
? These are in addition to the exstrophy of hindgut, ileal intussusception, and exposed
appendices that are considered part of the primary pathology of cloacal exstrophy.
? Skeletal abnormalities
? Skeletal anomalies are seen in as many as 50% of patients with cloacal exstrophy.
? Anomalies include congenital hip dislocation, talipes equinovarus, and a variety of
limb deficiencies.
? Fascial abnormalities
? The fascial anomalies associated with cloacal exstrophy include those
described above for bladder exstrophy.
? Further, omphaloceles often occur in association with cloacal exstrophy.
? Neurologic abnormalities
? Neurologic involvement of the lower spinal cord in the cloacal exstrophy
population is reported to occur in 50?100% of patients.
? Most patients have lumbar or sacral cord involvement, but thoracic-level
myelodysplasia has been reported.
? Management of cloacal exstrophy in these situations must be coordinated
with neurosurgical plans to close the neural tube defect.
Anatomic pathology: Epispadias
? Epispadias can be considered the least severe form of exstrophy.
? It does not involve the body of the bladder.
? The urethra is represented as a plate of tissue located dorsally on the
penis.
? The genital anomalies include lateral splaying of the penis and dorsal
chordee in boys.
? In affected girls, the clitoris is bifid, the perineal body is broadened,
and the vagina is anterior to its typical position.
? Widening of the pubic diastasis occurs as well.
? Importantly, the bladder neck is also frequently involved ? it is often
wide and incompetent. This directly affects the continence
mechanism of these children and impacts the ability to achieve
urinary continence.
? In boys with epispadias, primary continence is possible if the
epispadias is located distally and the bladder neck is normally formed.
? In girls, however, continence is invariably affected to some degree
because of the associated urethral and bladder neck ectasia.
EPIDEMIOLOGY
? The incidence of bladder exstrophy is estimated at between 1 in 10,000
and 1 in 50,000 live births.
? Higher male to-female ratio of between 2.3:1 and 4:1.
? Familial recurrence is approximately 1 in 100.
? Multiple reports of bladder exstrophy among identical twins have
demonstrated variability in involvement of one or both twins.
? Subsequent siblings and the offspring of individuals with bladder exstrophy
may be at increased risk of being affected.
? However, no clear pattern of inheritance has been characterized and no
specific genetic or environmental factor that predisposes to bladder
exstrophy has yet been identified.
Embryology
? Abnormal development of the cloacal membrane, a bilaminar
structure composed of endoderm and ectoderm that overlies the
cloacal cavity at the caudal end of the germinal disk.
? In normal development, lateral ingrowth of mesoderm occurs
between the two layers of the cloacal membrane during the fourth
and fifth weeks of gestation.
? This results in formation of the lower abdominal wall and pelvis.
? Subsequent caudal growth of the urorectal septum results in its
fusion with the cloacal membrane, thus fully separating the cloaca
into the bladder anteriorly and the rectum posteriorly.
? The paired genital tubercles, which will give rise to the phallus,
migrate medially to fuse in the midline.
? Normal perforation of the cloacal membrane occurs after fusion with
the urorectal septum, at approximately the sixth week, resulting in
formation of separate urogenital and anal openings.
? Migratory failure of the lateral mesodermal folds and abnormal
overdevelopment of the cloacal membrane have both been proposed
as potential causes of the prevention of normal mesodermal ingrowth
to the cloacal membrane.
? The lack of adequate mesodermal reinforcement is thought to result
in premature rupture of the cloacal membrane, the timing of which
determines the extent of the abdominal wall defect and
degree/severity of urogenital tract involvement.
? Rupture of the cloacal membrane after fusion with the urorectal
septum results in bladder exstrophy, whereas rupture before fusion
gives rise to the more severe presentation of cloacal exstrophy.
PRENATAL DIAGNOSIS
? The use of prenatal ultrasound (US) and MRI has improved the
antenatal diagnosis of bladder exstrophy, allowing for appropriate
parental counselling and planning of postnatal management.
? The prenatal diagnosis of bladder exstrophy may be suggested on US
by failure to visualize the bladder in the presence of normal kidneys
and amniotic fluid.
SURGICAL RECONSTRUCTION
? Surgical management of classic bladder exstrophy consists of
? functional closure of the native bladder
? closure of the epispadic urethra and genitalia
? creation of a continence mechanism to allow for proper urine storage.
? Reconstruction may be accomplished in a multi- or single-stage
(complete) repair
? modern staged reconstruction of exstrophy (MSRE)
? complete primary reconstruction of exstrophy (CPRE)
MODERN STAGED RECONSTRUCTION
OF BLADDER EXSTROPHY
? A three-stage approach for the treatment of bladder exstrophy was first
pioneered by Jeffs and Cendron in the 1970s.
? Stage 1 is performed at birth to protect the upper urinary tracts and assist
later continent reconstruction.
? It consists of early closure of the bladder, posterior urethra, and abdominal wal with
or without osteotomy. The primary objective of functional closure is to convert the
bladder exstrophy into a complete epispadias.
? Stage 2 is performed in later infancy and involves repair of the epispadias,
with the goal of optimizing genital function and appearance, as wel as
increasing outlet resistance to promote bladder growth.
? Stage 3 is undertaken before school age and consists of bladder neck repair
for continence and ureteral reimplantation for vesicoureteral reflux.
INITIAL MANAGEMENT
? At birth, the umbilical cord should be ligated with a silk suture to avoid
irritation of the bladder surface from the traditional plastic clip.
? The exposed bladder mucosa should be moistened with saline and
protected with a nonadherent sheet of plastic wrap (e.g., Saran Wrap).
? A complete physical examination is performed to rule out associated
anomalies and to assess the size of the bladder plate and extent of the
genital defect.
? Renal US may be obtained to exclude hydronephrosis and/or other upper
tract abnormalities.
? Prophylactic antibiotics should be administered.
? Primary functional closure is generally undertaken in the neonatal
period, which offers several potential advantages.
? The pliability of the pelvic ring, in infants younger than 72 hours old, may
obviate the need for osteotomy.
? Early closure prevents further exposure and scarring of the bladder plate.
? Alternatively, delayed closure in combination with pelvic osteotomy
may be performed.
Stage 1
A, Completion of the dissection around the periphery of the bladder and the urethral plate.
B, Inversion of the bladder plate and approximation of the corpora as a first stage in epispadias repair.
Also note the inferior paraexstrophy incisions.
C, Further closure of the skin over the corpora and their partial freeing from the pubis.
D, Placement of a suprapubic drainage tube
E, Further closure of the skin inferiorly, with approximation to the urethral plate. Creation of the para-
exstrophy flaps is now evident.
F, The urethral plate is prepared for tubularization over a catheter.
G, The urethral plate is now tubularized, and ureteral catheters are placed bilaterally and brought out on
each side of the bladder. The bladder is also in the process of being tubularized.
H, Completion of tubularization of the bladder and urethra, and location of the various drainage
tubes.
I, After two-layer closure of the bladder and urethral plate, the bladder is reduced into the pelvis and fixed with
sutures.
J, Sutures are placed to encourage approximation of the pubic halves.
K, Drainage tubes are brought out superiorly, and fascia, subcutaneous tissue, and skin are approximated.
Approximation of the pubis helps protect the bladder closure and the abdominal wall closure.
This photograph shows a spica cast that was applied to
prevent external hip rotation and optimize pubic
apposition in the early postoperative period.
External fixator pins
are used to hold the
pelvis, and the pubic
halves are brought
together in the midline.
Stage 2
Cantwell-Ransley epispadias repair. A, The urethral plate is dissected from the corpora and is tubularized, taking
care to
preserve the lateral blood supply of the urethra and the neurovascular bundles. B, Corporotomies are created at
the midphallus, and the urethra is transposed to the ventral surface. C, The corpora cavernosa are rotated
medially and reapproximated at the corporotomy sites, pulling the corporal bodies inward and providing
coverage of the neourethra. This procedure permits further urethral lengthening, approximation of the corpora
with preservation of the blood supply, and full coverage of the urethra.
Stage 3
Young-Dees- Leadbetter procedure for bladder exstrophy repair.
The ureters are appropriately reimplanted to avoid reflux. Triangular areas at the bladder base are then denuded,
and the remaining muscle is tubularized over a catheter. This serves to lengthen the urethra and provides
sufficient pressure to encourage the development of improved bladder capacity without causing urethral
obstruction.
SINGLE-STAGE RECONSTRUCTION:
COMPLETE PRIMARY REPAIR OF EXSTROPHY
? Mitchell and Grady minimized the number of required operations by
combining bladder closure with epispadias repair at birth in a
technique known as complete primary repair.
? Major potential benefits of this approach include
? earlier creation of bladder outlet resistance, theoretically leading to normal
cycling and improved bladder capacity and functionality as the patient grows.
? Major principles of CPRE include total penile disassembly and division
of the intersymphyseal band, which enables posterior positioning of
the bladder, bladder neck, and urethra.
URINARY DIVERSION
? Urinary diversion, in the form of a bowel conduit or reservoir, may
ultimately be required for patients with insufficient bladder plate or
after reconstructive efforts have been unsuccessful.
OUTCOMES AND COMPLICATIONS
? The most devastating complication of bladder closure is dehiscence.
? Major contributing factors include wound infection, abdominal distension,
bladder prolapse, and loss of ureteral and/or suprapubic catheters within 6
days of closure.
? Urinary diversion, reclosure of the bladder as a urethral tube for later
augmentation, or delayed repair of the bladder may be performed.
? If not performed in the initial setting, pelvic osteotomy is frequently
necessary for successful reclosure.
? Urinary incontinence remains a significant problem for up to 30% of
bladder exstrophy patients.
? In the case of bladder neck incompetence, injectable bulking agents, bladder
neck sling or artificial urinary sphincter have all been applied.
? Bladder neck reconstruction or formal closure of the bladder neck, with the
creation of a catheterizable channel, can also be performed.
? In cases where incontinence is secondary to insufficient bladder
capacity, augmentation cystoplasty remains the most viable
treatment option.
? Following epispadias repair, the most common complication is
urethrocutaneous fistula, which ranges from 2% to 26% in modern
series.
? The incidence of adenocarcinoma of the bladder in adults with
bladder exstrophy has been estimated to be 250 times that of the
normal population and is likely due to chronic inflammation,
infection, and metaplasia of an exposed bladder.
? The development of adenocarcinoma and transitional cell carcinoma
of the bladder is also a potential risk in those patients who have
undergone augmentation cystoplasty.
? Fertility in patients with bladder exstrophy and epispadias was
studied by Shapiro and colleagues, who surveyed 2500 patients.
? Among these, 38 men had successfully fathered children and 131 women had
given birth.
? Diminished fertility rates among males may be secondary to retrograde
ejaculation, though libido and erectile function appear to be normal.
? Female patients face a significant risk of uterine prolapse.
SURGICAL RECONSTRUCTION IN CLOACAL
EXSTROPHY
? The surgical management of cloacal exstrophy is typically undertaken
in the newborn period (48 to 72 hours).
? The main goals of reconstruction include
? secure abdominal wall and bladder closure
? preservation of renal function
? prevention of short gut syndrome
? creation of functionally and cosmetically acceptable genitalia
? attainment of urinary and fecal continence.
? All approaches begin with initial separation of the intervening cecal
plate from the two bladder halves, closure of the omphalocele, and
hindgut preservation.
? Currently, after tubularization of the exstrophied cecum, it is
recommended that the hindgut segment be brought out as an end-
colostomy.
? The hemi-bladders are dissected and then reapproximated in the
midline.
? Further management based on individualised approach.
Conclusion
? For the past 20 years, survival among patients with cloacal exstrophy
has exceeded 90%.
? Death is typically related to complications related to extreme
prematurity, renal agenesis, or other complex malformations that are
incompatible with life.
? Compared with those with classic exstrophy, however, cloacal
exstrophy patients face additional challenges of achieving bowel and
bladder continence secondary to the need for anal reconstruction
and the associated defect of spinal dysraphism.
? It must be stressed that multiple operations are the rule, and these
patients will likely face significant medical, psychologic, and social
challenges throughout their lives.
? Advancements in medical and surgical management continue to
improve functional and quality of life outcomes in these patients, but
it is important that these individuals remain under the care of a
multidisciplinary team of providers who can offer medical care,
psychologic support, and lifelong follow-up.
This post was last modified on 08 April 2022