Congenital Bladder Disorders, Campbell urology

1. URINARY BLADDER
DEVELOPMENT AND
ANOMALIES
Dr Shuah Ullah

2. • The bladder and urethra of the urinary system are ultimately derived
from the cloaca – a hindgut structure that is a common chamber for
gastrointestinal and urinary waste.

4. • In the 4th-7th weeks of
development, the cloaca is
divided into two parts by the
uro-rectal septum

5. • Urogenital sinus (anterior) – divided
into three parts:
• The upper part of the urogenital sinus
forms the bladder.
• The pelvic part forms the entire
urethra and some of the reproductive
tract in females, and the prostatic and
membranous urethra in males.
• The phallic/caudal part forms part of
the female reproductive tract, and the
spongy urethra in males.

6. • Anal canal (posterior)
• The urinary bladder is initially drained by
the allantois. However, this is obliterated
during fetal development and becomes a
fibrous cord – the urachus. A remnant of
the urachus can be found in adults; the
median umbilical ligament, which
connects the apex of the bladder to the
umbilicus.

7. As the bladder develops from the
urogenital sinus, it absorbs the
caudal parts of the mesonephric
ducts (also known as the Wolffian
ducts), becoming the trigone of the
bladder. The ureters, which have
formed as outgrowths of the
mesonephric ducts, enter the
bladder at the base of the trigone.
The final structure varies between
sexes:

9. Antenatal U.B
• An elliptical structure filled with anechoic fluid within the pelvis.
• The bladder wall thickness should not exceed 3 mm.
• The bladder can be visualized in approximately 50% of cases in the
fetal pelvis at the 10th week of gestation, concurrent with the onset
of urine production.
• The detection rate increases with fetal age to 78% at 11 weeks, 88%
at 12 weeks, and almost 100% at 13 weeks

10. • The fetal bladder empties every 15 to 20 minutes; therefore a second
ultrasound in the same setting is mandatory in case of non-
visualization of the bladder.

11. Classification :
Prenatally detected
• Dilated
• Non-dilated
Post-natally detected
• Urachal abnormalities
• Bladder diverticulum
• Bladder duplication
• Other bladder anomalies

12. Dilated Fetal Bladder
• In the first trimester, the fetal bladder is considered dilated if larger than 7 mm on
ultrasound.
• Determination of the sex of the child is very important because of the male
gender predominance of certain conditions and diseases such as posterior
urethral valves or prune belly syndrome.
• 15 patients with marked bladder dilation in utero, 8 with and 7 without
obstruction. All of the patients with obstruction presented with moderate-to-
severe oligohydramnios and a marked increase in renal echogenicity (Kaefer and
coworkers (1997)

13. Dilation Caused by Obstruction
• Urethral anomalies include congenital urethral strictures, anterior and
posterior urethral valves, and urethral atresia.
• Compression of the bladder outlet region can be due to obstructing
syringoceles, a sacrococcygeal teratoma or pelvic neuroblastoma, an
anterior sacral myelomeningocele, or rectum anomalies.

14. Dilation in Non-obstruction
• Prune Belly Syndrome
• Neurogenic Bladder Disease

15. Congenital Megacystis
• Any condition leading to a distended fetal bladder in utero, without
referring to the cause of the dilation
• Historically, congenital megacystis was thought to be caused by
bladder neck obstruction, leading to massive bilateral vesicoureteral
reflux (VUR) and a thin bladder wall.
• Surgical interventions at the bladder neck level did not change the
future outcome.

16. • Observed reflux is not an after
effect of obstruction but rather the
cause of the bladder dilation from
continuous recycling of the urine
between the upper tract and
bladder
• Defined currently as a dilated, thin
walled bladder with a wide and
poorly developed trigone.

17. • Wide-gaping ureteral orifices are displaced very laterally, causing massive reflux.
• Bladder contractility is normal, although a majority of the urine refluxes into the
ureters with each void.
• Most patients are recognized prenatally and should be placed on prophylactic
antibiotics after birth (Mandell et al, 1992).
• Correcting the reflux often restores normal voiding dynamics and should be
performed after 6 months of age.
• Reduction cystoplasty can be performed but is usually unnecessary (Burbige et al,
1984).
•

18. • Although the bladder is large enough to accommodate the tapered ureters even
in a young infant, the operation can be very difficult because of the bladder wall’s
thinness.
• Anterior urethral valves also have been associated with megacystis, with
improvement after valve resection
• Congenital megacystis has been recognized in association with microcolon-
intestinal hypoperistalsis syndrome.

19. Non-dilated or Absent Fetal Bladder
• Cloacal and Bladder Exstrophy.
• Bladder Hypoplasia.
• Bladder Agenesis.

20. Urachal Anomalies
• The urachus is located pre-peritoneally in the center of a pyramid-shaped
space. This space is lined by the obliterated umbilical arteries, with its base
on the anterior dome of the bladder and the tip directed toward the
umbilicus.
• The urachal length varies from 3 to 10 cm. It has a diameter of 8 to 10 mm
and can connect with one or both obliterated umbilical arteries.

22. • Microscopically, three layers can be identified. An inner layer consists
of either transitional or cuboidal epithelial cells surrounded by a layer
of connective tissue. A smooth muscle layer in continuity with the
detrusor muscle composes the outer layer.
• Urachus is surrounded by the umbilicovesical fascia, disease
processes usually remain contained inside the pyramid-shaped space.

23. • Children mostly presented with umbilical drainage or on physical
examination, and 74% underwent excision.
• Adults, 66% had hematuria or pain and 90% underwent excision.
• Surgical treatment in children consisted of simple excision, whereas
over 50% of adults required partial or radical cystectomy because of
malignancy.
• Spontaneous resolution with non-operative management is likely
with remnants in patients younger than 6 months. However, if
symptoms persist or the remnant fails to resolve after age 6 months,
do excision

24. Types
1. Patent urachus (50%)
2. Umbilical-urachus sinus (15%)
3. Urachal cyst (30%)
4. Vesicourachal diverticulum (3% to 5%)

26. Patent Urachus
• 14% of patients with a patent urachus have postnatal confirmation of
in utero bladder obstruction
• Obliteration of the urachus may be independent from the level of
bladder distention.
• Retubularization, rather than primary patency, might be the cause for
urinary drainage from the umbilicus

27. • A patent urachus is suspected in the neonatal period by continuous or
intermittent drainage of fluid from the umbilicus.
• Most common organisms cultured from the umbilical drainage
include Staphylococcus aureus, Escherichia coli, Enterococcus,
Citrobacter, and, rarely, Proteus species
• Manifestations include an enlarged or edematous umbilicus and
delayed healing of the cord stump.

28. • The diagnosis is confirmed by
demonstration of the fluidfilled canal
on longitudinal ultrasound or contrast
filling on retrograde fistulogram or
VCUG .
• Computed tomography (CT) scans can
aid in the diagnosis but usually
depend on the bladder’s filling status.
• It is important to differentiate the
condition from a patent
omphalomesenteric duct.

29. Management :
• An infected urachus with abscess formation includes initial drainage
under antibiotic coverage.
• Once the infection has subsided, complete excision of the patent
urachus, including a bladder cuff, is required.
• It is important to remove all anomalous tissue.
• This avoids recurrences or stone formation and prevents the rare
event of later transformation into a malignant adenocarcinoma

30. • Patent urachus is surgically excised using a transverse or midline
infraumbilical incision.
• Before surgery, a balloon catheter is used to distend the bladder. In
infants, a small transverse subumbilical incision is often possible
because the bladder dome is still high.
• A feeding tube or small catheter is placed into the patent urachus for
better intraoperative identification.
• Alternatively, urachal remnants can be removed laparoscopically. This
can be done even in children younger than 6 months of age

31. • Three-port approach, with the camera port in the midline between
the umbilicus and the xiphoid and two working ports on either side in
the upper quadrant
• Laparoscopic techniques require an intra-abdominal approach and
pose the potential risk for spilling infected or malignant material into
the abdominal cavity.
• Robotic-assisted removal and single-port laparoscopy are other
options.

32. Umbilical-Urachus Sinus
• Urachus obliterates at the bladder
level but remains open at the
umbilical site, causing a
continuously draining sinus.
• Diagnosis is made by sinugram.
• The presence of a persistent
omphalomesenteric duct has to be
considered.

33. • These structures can be very difficult to differentiate from an
umbilical-urachus sinus because no connection to the bladder or
bowel can be seen on sinugram.
• The surgical approach to both anomalies requires the complete
excision of all tissue.
• Unlike urachal structures, omphalomesenteric remnants can show
gastric or small bowel mucosa on histologic examination

34. Urachal Cyst
There is no communication of the
cyst with the bladder or umbilicus.
However, the fluid-filled cyst can
drain through the umbilicus or into
the bladder intermittently.
More commonly in the distal part of
the urachus and manifest more
commonly in adults than in infants or
children.

35. • Cyst material consists of desquamated epithelial cells. These cells can
become infected; Staphylococcus aureus has been identified as the most
common organism.
• Once infected, urachal cysts can manifest as umbilical abscess formation or
bladder infections.
• Symptoms include localized lower abdominal pain, voiding symptoms, or
even a painful and palpable mass.
• Diagnosis is confirmed by ultrasound, demonstrating the localized cyst
between the anterior abdominal wall and the peritoneum.
• CT scan can clarify the anatomy and extent of disease

36. • If unrecognized, the infected cyst can perforate into the bladder or
peritoneal cavity.
• This can cause peritonitis and formation of an enteric fistula.
• Treatment consists of draining the infected cyst, followed by complete
excision of the urachal remnant structures.

39. Vesicourachal Diverticulum
• The urachus obliterates almost
completely, except at the level of
bladder apex. Here it forms a
diverticulum of varying size.
• These lesions are usually
nonsymptomatic and found
incidentally on nonrelated
radiographic workups.

40. • Although the diverticulum can enlarge in the case of urinary
obstruction, this rarely causes problems because they tend to have a
large opening and drain into the bladder well.
• Stone formation and urinary tract infections have been reported,
especially in the case of a narrowed neck causing the need for
intervention.

41. Bladder Diverticulum
• Bladder diverticula are caused by
infra-vesical obstruction, iatrogenic
after bladder surgery, or as a
congenital defect.
• The incidence is reported to be
low, with 1.7% in a selected
pediatric population of children
undergoing radiographic evaluation
for symptomatic disease.

42. • Independent from the cause, all
diverticula develop as herniation
of bladder mucosa between
defects of bladder smooth
muscle fibers.
• The neck of the resulting
diverticulum depends on the size
of the muscular defect.

43. • No any true muscle layer.
• In general there is a male
predominance for BD, which is
thought to be related to the
higher voiding pressures in
males both in utero and shortly
after birth.

44. • In his article from 1961, Hutch
describes the following two
kinds of diverticula at the
ureteral hiatus
• Primary paraureteral diverticula
are seen in smooth-walled
bladders, occur isolated with no
other diverticula, are
intermittent in manifestation,
and happen in children with no
infravesical obstruction.

45. • The 'Hutch diverticulum' refers to a primary diverticulum at or near
the ureterovesical junction (UVJ) with its orifice located above the
ureteral orifice that is found in children who are otherwise normal.
• Secondary paraureteral diverticula are found in trabeculated
bladders as one of many diverticula in the bladder, are always
present, and are caused by infravesical obstruction.

46. Primary Diverticula :
• Arise as a localized herniation of bladder mucosa through the ureteral
hiatus between the intravesical ureter and the roof of the ureteral
hiatus, also known as congenital diverticula and are most likely
caused by a congenitally deficient bladder wall.
• Some authors have implied an isolated defect in the Waldeyer’s
sheath; however, congenital diverticula often occur on one side only
and a unilateral defect seems unlikely (Stephens, 1963).

49. • Congenital diverticula are often found in children with generalized
connective tissue diseases such as Ehlers-Danlos, Williams syndrome (a
disorder characterized by growth and mental retardation, facial anomalies,
and aortic stenosis) , or Menkes syndrome (a neurodegenerative and
connective tissue disorder with characteristic increases in tissue copper
and metallothionein content)
• These diverticula can be resected if symptomatic; however, because of the
impaired healing in patients with connective tissue disease, recurrence and
wound healing complications are more common.

51. Secondary Para-ureteral Diverticula
• Acquired and develop as a result of existing infra-vesical obstruction.
• The resulting increased infra-vesical pressure forces the bladder
mucosa to bulge between the muscle fibers.
• These diverticula are usually just one of many pop-off mechanisms
that can occur throughout the bladder.
• These diverticula also can be caused by weakening in the bladder
muscle by infection (Barrett et al, 1976) or development of a
muscular defect after bladder surgery (Sheu et al, 1998).

52. • In both types of paraureteral diverticula, the Waldeyer sheath
eventually becomes damaged as the diverticulum expands in size.
• The increasing diverticulum pulls the intravesical ureter out of its
anchored position, causing dysfunction of the ureterovesical junction.
• Eventually, the enlarged diverticulum can become responsible for
ureteral obstruction.
• This even has been associated with renal dysplasia (Amar, 1972; Livne
and Gonzales, 1985).

54. • Paraureteral diverticula or diverticula located in the lower part of the
bladder can become so large that they compress the bladder neck or
posterior urethra.
• The resulting bladder outlet obstruction starts a vicious circle by
continuously filling and expanding the diverticulum. This increases the
obstructing and subsequently causes complete urinary retention.
• Bladder diverticula can be detected on prenatal ultrasound (Gaudet et al,
1999), but are mostly discovered during workup for infection, hematuria,
incontinence, or obstruction.

55. • They can be suspected during ultrasound examination, especially if
the bladder is viewed in different filling stages.
• The gold standard remains VCUG, which will reveal possible
accompanying VUR.
• If no VUR is present, an intravenous pyelogram (IVP) with oblique
views can help determine the relationship of the ureter with the
diverticulum if there is hydronephrosis.
• Alternatively, nuclear renal studies can be used to obtain information
concerning anatomy, kidney function, and ureteral obstruction.

56. • Diverticula are dynamic
abnormalities of the bladder that
typically appear during micturition
(voiding), when intravesical
pressures increases.
• Best views for the depiction of the
UVJ and the retrovesical portion of
the ureter are the oblique ones
(right oblique for the left VUJ and
left oblique for the right VUJ)

61. VCUG, especially in children with high grade VUR, should be performed with
careful technique and attention to details such as:
• Bladder should be completely empty before filling phase of the
examination starts (diverticula may contain non-opaque urine rendering
them undetected during the examination).
• All phases should be fluoroscopically monitored and especially voiding
because paraureteral diverticula typically appear during high pressure
micturition. Diverticula may have emptied into the bladder by the time the
next film is taken, when static VCUG is used.

62. • Oblique and lateral views should be obtained to visualize the ureterovesical
junction, near the trigone where Hutch diverticula are located.
• Post void views immediately after bladder emptying should be taken
because Hutch diverticula that enlarge during voiding become more
apparent in these views.
• Delayed post void views may be taken when needed to evaluate
narrownecked diverticula that empty later than the bladder. These
diverticula may explain aberrant micturition in these cases.

63. • Small, asymptomatic congenital diverticula detected during unrelated
workups can be treated conservatively with regular observation.
• Many surgeons tend to recommend excision of paraureteral
diverticula if they are accompanied by VUR.
• Girls will show spontaneous resolution of their diverticula in
association with VUR more frequently than boys

64. • In acquired bladder diverticula, the infravesical obstruction has to be
eliminated first.
• After bladder outlet resistance is normalized, the bladder can
reshape and diverticulectomy might become unnecessary.
• If symptomatic, the diverticulum should be excised.
• The ipsilateral ureter should be reimplanted if it is near or included in
the diverticulum.
• This traditionally has been performed intravesically; however, the
procedure can be safely performed by an extravesical approach

65. • Laparoscopic excision also has been successfully performed in a 6-
year-old child (Kok et al, 2000).
• Endoscopic subureteral injection of dextranomer/ hyaluronic acid
(Deflux) has been used for the correction of VUR, even in the
presence of a primary paraureteral diverticulum (PerezBrayfield et al,
2004).
• Robotic-assisted diverticulectomy in 14 patients was reported and
described as a safe alternative to open surgery (Christmas and Casale,
2012).

73. Bladder Duplication
• Duplication of the bladder and urethra can be complete or incomplete.
• It can occur in either the coronal or sagittal plane.
• Abrahamson (1961) attempted to classify the various bladder duplication
anomalies and found complete duplication in the sagittal plane the most
common.
• In incomplete duplications, the two bladder halves communicate and are
usually drained by a single urethra.

75. • In complete duplications, the
two bladders are fully separated
entities with normal mucosa and
a full-thickness musculature wall
divided by a peritoneal fold.

76. • Although the size and quality of each entity can be different, they are
usually supplied with their own ureter and are drained by an
individual urethra and external meatus (Esham and Holt, 1980).
• In rare cases, one bladder can lack a urethra. This leads to ipsilateral
renal dysplasia via complete obstruction (Cheng and Maizels, 1996).
• Both bladders may possess a sufficient continence mechanism, or one
side may be compromised, causing incontinent episodes.

77. • Associated duplication anomalies of the external genitalia have been
reported in up to 90% of cases; associated duplication anomalies of
the lower GI tract have been reported in up to 42% of cases (Kossow
and Morales, 1973).
• Duplicated vaginas can be connected to a separate unicornuated
uterus.
• Duplicated penises are supplied with an individual urethra.
• Additional urologic abnormalities such as VUR, renal ectopia, or
dysplasia are commonly found.

78. • Association with other nonurologic congenital anomalies are more
frequent in sagittal than coronal duplications.
• Multiple manifestations have been described, including GI
malformations, duplications of the spine, spina bifida conditions,
and various fistula formation between the urogenital and GI tract
(Berrocal et al, 1999).
• In duplication variations of the classic cloacal-bladder exstrophy
complex, patients present with an exstrophic bladder and urethra in
addition to a closed regular intra-abdominal bladder (Perren and Frey,
1998).

80. • Complete preoperative diagnostic evaluations with karyotype,
ultrasound, IVP, videourodynamic studies, genitogram, and GI tract
imaging are useful to determine the anatomic situation.
• VCUG and nuclear renal scans can supply additional information
regarding VUR and renal function.
• Complete understanding of the various anomalies can be very
difficult.
• Often the final treatment plan has to be deferred until the time of
endoscopic and surgical exploration of the malformation.

81. • Initial treatment is directed toward renal preservation and prevention of
infections by relieving possibly obstructed genitourinary tracts.
• Long-term goals include achieving continence and reconstructing the internal and
external genitalia.
• Incomplete duplications may not require surgical procedures if both bladder
halves are sufficiently drained by a common urethra.
• In complete duplications, the two bladders can be combined into one. If both
sphincter complexes are competent, the distal urethras are connected. If one is
incompetent, the corresponding bladder neck can be closed and the connected

82. • Duplicated vaginas are combined in the midline, and a vulvoplasty is
performed.
• The urogenital duplications also can be left uncorrected if the patient is
asymptomatic; Gastol and associates (2000) reported two successful
pregnancies in a 26-year-old woman.
• Because of the rarity of the disease and the large variety of manifestations,
the surgeries must be individualized and should be performed in centers
experienced in complex urogenital reconstruction

87. Thank you !