Urinary Bladder anomalies congenital

Urinary Bladder anomalies congenital

1. Dept of Urology
GovtRoyapettahHospital and KilpaukMedical College
Chennai

2. Moderators:
Professors:
Prof. Dr. G. Sivasankar, M.S., M.Ch.,
Prof. Dr. A. Senthilvel, M.S., M.Ch.,
Asst Professors:
Dr. J. Sivabalan, M.S., M.Ch.,
Dr. R. Bhargavi, M.S., M.Ch.,
Dr. S. Raju, M.S., M.Ch.,
Dr. K. Muthurathinam, M.S., M.Ch.,
Dr. D. Tamilselvan, M.S., M.Ch.,
Dr. K. Senthilkumar, M.S., M.Ch.
Dept of Urology, GRH and KMC, Chennai. 2

3. DEVELOPMENT
FORMATION OF UROGENITAL SINUS :
*At 3
rd
week of gestation a structure called
cloaca ( Latin word for drain) develops
*It drains both GIT and Urinary tract
*At about 5
th
-6
th
weeks --Partition of
cloaca into Anterior urogenital sinus and
posterior anorectal canal occurs
3Dept of Urology, GRH and KMC, Chennai.

4. The Nephric duct (wolfianduct ) fuses with the
cloaca by the 24
th
day and remains with the
urogenital sinus during seperation.
This entrance serves as a land mark
distinguishing the cephalodvesicourethral
canal and
caudal urogenital sinus
4Dept of Urology, GRH and KMC, Chennai.

5. The vesicourethralcanal gives rise to
bladder and pelvic urethra
The caudal urogenital sinus forms
phallic urethra for male and
distal vaginal vestibule in female
5Dept of Urology, GRH and KMC, Chennai.

6. FORMATION OF TRIGONE:
By day 33 of gestation
-The common excretory ducts (the portion of
nephricduct distal to the origin of ureteric bud)
dilate and become absorbed into the urogenital
sinus
-The Rtand Lt common excretory duct fuses in
the midline as a triangular area , forming
primitive trigone
6Dept of Urology, GRH and KMC, Chennai.

7. By day 37:
-The ureteric orifice exstrophicand evaginate
into the bladder
-Begins to migrate cranial and lateral
direction within the floor of the bladder
-During the process , the Nephric duct orifice
develops away from the ureteric orifice and
migrate caudally
This is the site of future veru in males and
vaginal canal in females
7Dept of Urology, GRH and KMC, Chennai.

8. 8Dept of Urology, GRH and KMC, Chennai.

9. CONGENITAL ANAMOLIES OF BLADDER:
CLASSIFICATION:
1.Anomalies in number:
a. Agenesis
b. Duplication
2.Anamolies in size:
a. Hypoplasia
b. Megacystic
3.Anomalies in form
a. Exstrophy–epispadiascomplex
b. Congenital bladder diverticula
c. Congenital bladder fistulas
9Dept of Urology, GRH and KMC, Chennai.

10. ANOMALIES IN NUMBER
a.AGENESIS
--No bladder is present
--In male :urethra and penis are absent usually
--Infant with Agenesis of bladder are generally
stillborn but all surviving Infants are female
--Ureter may open ectopically in the external
urethral meatus on each side
--Ureter may unite and open through a single
orifice
--Ureter may open directly onto the skin in the
region of natal cleft
Initial complaint is persistent incontinence
10Dept of Urology, GRH and KMC, Chennai.

11. Complication :
Infection results in chronic pyelonephritis &
hydronephrosis
Associated Anomalies:
1.Agenesis of one or both kidneys
2.Single pelvic kidney
3.Ureteral duplication
4.VATER Anomalies
Vertebral
Anorectal
TracheoEsophageal
Radial or Renal
11Dept of Urology, GRH and KMC, Chennai.

12. Diagnosis:
USG ---shows no bladder
IVP
--If ureteric opening are Identified they may
be catheterised,
Retrograde urography
--differentiate vesicalAgenesis from B/L
ureteral ectopia in which a small bladder may
be present
12Dept of Urology, GRH and KMC, Chennai.

13. b.DUPLICATION:
Three main types
Type I:
--Duplication involves the mucosa & muscle
wall
--A Peritoneal fold separates the two bladder
--sub types :1. Complete duplication
2. Incomplete duplication
Type II:
--An internal septum of mucosa only or of
mucosa &muscle divides the bladder
--groove may or may not be visible outside the
bladder 13Dept of Urology, GRH and KMC, Chennai.

14. --Sub types: 1.Sagittal septum
-complete
-incomplete
2.Frontal septum
-complete
-incomplete
3. Multiple septa
TYPE III:
--A transverse band of thick muscle divide the
bladder into 2 unequal cavities and gives it a
characteristic Hour glass shape
14Dept of Urology, GRH and KMC, Chennai.

15. Chwalletheory:
--completely duplicated bladder may form if one
ureter remained obstructed at the ureterovesical
junction as a result of chwallemembrane
--this process will lead to cystic dilatation at the
lower end of the ureter
--muscles and fibrous tissue would develop
around the structure which would then be forced
over to the median line and results in bipartite
bladder
--the hourglass bladder to be a result of a
dysplastic junction between the urachusand the
bladder
15Dept of Urology, GRH and KMC, Chennai.

16. COMPLETE DUPLICATION :
Involves two completely separate bladders each
with its own wall of muscle and mucosa
separated by a peritoneal fold of varying depth
--the right and left kidney drain into right and
left bladders respectively via single ureter
--the two urethras are present one for each
bladder
16Dept of Urology, GRH and KMC, Chennai.

17. --males often have two penises ,a partially
duplicated penis or one penis with two
urethra
--commonly the scrotum is bifid
--females usually have two urethras , two
vaginas, two uteri each commonly have one
horn and fallopian tube
17Dept of Urology, GRH and KMC, Chennai.

18. Anomalies associated with complete
duplication of the bladder:
1.Renal ectopia =50%
2.Duplicated colon with an anorectal
malformation of the lateral aspect of the colon
and rectourethralfistula
3.Duplicated anus
4.Duplicated appendix
5.Duplicated ileum from meckel’sdiverticulum
to the ileocaecalvalve
6.Duplicated sacrum and coccyx
7.Wide separation of pubic rami
18Dept of Urology, GRH and KMC, Chennai.

19. Complications
--Adenocarcinoma development from
surgically defunctionalised bladder
moiety
--Accessory bladder lies in front of the
normal bladder with an accessory urethra
extending from it dorsally to a penile
epispadiacmeatus
--Neither ureter drains into accessory
bladder
19Dept of Urology, GRH and KMC, Chennai.

20. Voiding cystouretherography:
--Catheterize both the urethras and fill both
bladders
--when pts void :both bladder contract
simultaneously so the urethras are outlined
with contrast material simultaneously
DIAGNOSIS:
Antenatal Sonography : shows two bladder
IVP : shows two kidneys ,two bladder
left ureter drains into Lt bladder
right ureter drains into Rtbladder
20Dept of Urology, GRH and KMC, Chennai.

21. INCOMPLETE DUPLICATION:
involves two separate fundicomposed of
mucosa and muscle with overlying peritoneum
only a single base and only one urethra is
present
no associated anomalies
Diagnosis:
IVP
Voiding cystourethrogram/ static
cystourethrogram
shows a valentine shaped bladder
21Dept of Urology, GRH and KMC, Chennai.

22. Type II Duplication:
1a.COMPLETE SAGITTAL SEPTUM:
--A septum composed of mucosa only or mucosa
and muscle located on either side of midline,
divides the bladder completely into two unequal
portions
--one portion drained by urethra but other not
--two kidneys are present
Left drains into left portion , right drains into
opposite side
--the kidney draining into obstructed portion
which is not drained by urethra is
hydronephroticand dysplastic
22Dept of Urology, GRH and KMC, Chennai.

23. Diagnosis:
IVP shows only one functioning kidney
draining into a hemibladder
A cystogramshows half of bladder on
either right or left side
1b.INCOMPLETE SAGITTAL SEPTUM:
--similar to complete sagittalseptum
except that the septum is incomplete
inferiorly where it has a cresentriclower
edge
23Dept of Urology, GRH and KMC, Chennai.

24. 2a.COMPLETE FRONTAL SEPTUM:
--an oblique septum runs from the
posterior wall of the fundusto the base of the
bladder
--it is united with the base in front of or
behind the bladder neck and divides the
bladder into an anterosuperiorsegment and a
posteroinferiorsegment
--one or the other segment is completely
obstructed depending on whether the
septum opens of or behind the bladder neck
24Dept of Urology, GRH and KMC, Chennai.

25. --each kidney drains via single ureter into
one or other of the respective bladder
segment
--The kidney draining into the obstructed
segment is hydronephroticand dysplastic
Diagnosis
IVP: shows one functioning kidney with
contrast material in one or the other bladder
segment
25Dept of Urology, GRH and KMC, Chennai.

26. 2b.INCOMPLETE FRONTAL SEPTUM:
--similar to complete frontal septum except
that the septum is incomplete inferiorly and
has a cresentericlower edge
--Duplication of the kidney is commonly
associated
Diagnosis
IVP , cystogramor voiding cystourethrogram
shows incomplete frontal septum or a filling
defect in the bladder
26Dept of Urology, GRH and KMC, Chennai.

27. 2c.MULTIPLE SEPTA: (Multiseptatebladder)
--fibromuscularsepta divides the bladder into 4
unequal segments
--complete bilateral ureteral duplication is
always present so one ureter drains into each
segment
--only one segment actually opens into the
urethra ,other three bladder segments are
obstructed
--only one pole of one duplex kidney is
unobstructed
Diagnosis:
IVP: demonstrates onepoleof one kidney draining
into a tiny deformed chamber
27Dept of Urology, GRH and KMC, Chennai.

28. HOURGLASS BLADDER:
--A thick transverse band of muscle
incompletely divides the bladder into 2
unequal segments
--The ureter usually opened into the lower
portion
--No associated anomalies
Diagnosis:
IVP & Voiding cystourethrogram
--Hourglass shaped bladder
28Dept of Urology, GRH and KMC, Chennai.

29. 29Dept of Urology, GRH and KMC, Chennai.

30. ANOMALIES IN SIZE
1.HYPOPLASIA:
-indicates small or tiny bladder
-usually seen in infants with sacral
agenesis
-children of diabetic mother
Occurs in
1.Hypospadias
2.B/L Renal agenesis
3.Infantile polycystic disease
30Dept of Urology, GRH and KMC, Chennai.

31. 31Dept of Urology, GRH and KMC, Chennai.

32. 2.MEGACYSTIS:
-means large bladder
-occurs in infant and children
-common cause congenital BOO
(post.urethralvalve)due to areflexiadue to
spinal dysraphism
-accompany Prunbelly syndrome
-occurs in diabetes insipidus which
produce large volume of urine
a.Congenitalmegacystis:
-congenitally huge unobstructed bladder
-large residual volume
-Trt: reduction cystoplasty 32Dept of Urology, GRH and KMC, Chennai.

33. 33Dept of Urology, GRH and KMC, Chennai.

34. b.MegaureterMegacystis
syndrome:(Inner williamssyndrome)
large smooth bladder with a
Trigone, dilated ureteral orifice and grossly
dilated ureter
when voids bladder empty completely
large volume refluxes into ureter when
voiding
when voiding ceases, ureter
contracts,urinerapidllyfills the bladder
bladder always distended forming
decompensated,dilated and thin walled
34Dept of Urology, GRH and KMC, Chennai.

35. c.Megacystis-Microcolon–hypoperistalsis
syndrome
-have huge bladder with areflux&dilated
uppertract
-a small colon,dilatedsmall bowel with
hypoperistalsis
-common in female neonate
-fatal during first years of life
35Dept of Urology, GRH and KMC, Chennai.

36. ANOMALIES IN FORM
1.EXSTROPHY-EPISPADIAS COMPLEX:
Exstrophyof bladder and epispadias
represent opposite ends of a spectrum of
anterior abdominal wall defects rather than
separate entities
--Anterior abdominal wall defects
--Separation of pubic bone >1cm at
symphysis pubis
36Dept of Urology, GRH and KMC, Chennai.

37. CLASSIFICATION:
1. Exstrophyof bladder 1 : 33,000 live births
2. Epispadias1 : 1,00,000 live births
3. Pubic umblicus
4. Covered Exstrophy
5. Covered exstrophyand vesicle sequestration
6. Superior vesicle fistula
7. Superior vesicle fissure
8. Duplicate exstrophy
9. Exstrophyof cloaca
37Dept of Urology, GRH and KMC, Chennai.

38. EMBRYOLOGY:
CLASSIC THEORY:
-Persistence of cloacalmembrane or large
cloacalmembrane
-wide pelvis
-separated two mullerianducts leads to
separate vagina and uteri
-anterior abdominal wall defect
CURRENT THEORY:
-lack of rotation of the pelvic ring
primordia
38Dept of Urology, GRH and KMC, Chennai.

39. EXSTROPHY OF BLADDER:
-lower abdominal wall and anterior wall of
the bladder is absent
-the remainder of the bladder is visible
through the resultant opening
-bladder turns inside out and protrudes
through the opening in the anterior abdominal
wall –hence the term exstrophy
39Dept of Urology, GRH and KMC, Chennai.

40. -fertility not affected
-associated with musculoskeletal,
gastrointestinal and genital tracts
Musculoskeletalanomalies:
-widelyseparatedpubicsymphysis
-theinnominatebonesarerotatedoutward
alongthesacroiliacjoint
40Dept of Urology, GRH and KMC, Chennai.

41. EXSTROPHY OF BLADDER:
-pubic bone is rotated outward at its
junction with iliumand ischium
-the iliac wings project inwardly
-the anterior segment of each iliac wing is
short
-lower part of rectus abdominus is
separated
-umblicusis low with hernia
41Dept of Urology, GRH and KMC, Chennai.

42. Classic bladder exstrophy
42Dept of Urology, GRH and KMC, Chennai.

43. Classic bladder exstrophy
43Dept of Urology, GRH and KMC, Chennai.

44. EXSTROPHY OF THE BLADDER:
OEIS COMPLEX:
Omphalocele
Exstrophyof the cloaca
Imperforate anus
Spinal defects
44Dept of Urology, GRH and KMC, Chennai.

45. EXSTROPHY OF THE BLADDER:
Diagnosis:
IVP:uppertractsnormalin65%or
hydronephrosis
MRI&CT:
pubicboneseperation
corporeallengthassessed
PRENATALSONOGRAPHY:reveals
Lowerabdominalwall
absentbladder
malformationofexternalgenitalia
normalkidneys
normalamnioticfluidvolume 45Dept of Urology, GRH and KMC, Chennai.

46. EPISPADIAS
-congenital fissure in the upper wall of
female urethra or
-a congenital defect in male urethra
Incidence:
In females :0.2/ 100000 population
In male :0.9/100000 population
46Dept of Urology, GRH and KMC, Chennai.

47. EPISPADIAS:
In females
-clitoris is divided
-labium minoraill developed
-bladder is small and thin walled
-bladder neck absent
-urethra short and wide
-pts is incontinent
IVP:wideningof pubic symphysis
funnel shaped bladder base
upper tract –normal
low grade reflux -90%
47Dept of Urology, GRH and KMC, Chennai.

48. EPISPADIAS
In males:
-roof of distal end of urethra is absent
-proximal urethra opens anywhere from
base of penis to the glans
-penis bows upwards
-2/3rd of pts are incontinent
48Dept of Urology, GRH and KMC, Chennai.

49. Epispadias
49Dept of Urology, GRH and KMC, Chennai.

50. PUBIC UMBLICUS:
-umblicusis extremely low
-symphysis pubis is widely separated
SUPERIOR VESICLE FISTULA:
-tiny fistulous tract running from the
top of the bladder to a small area of
transitional epithelium on the anterior
abdominal wall
-symphysis pubis is wide
-usually continent
50Dept of Urology, GRH and KMC, Chennai.

51. Superior vesicalfissure
51Dept of Urology, GRH and KMC, Chennai.

52. CONGENITAL BLADDER DIVERTICULA:
-diverticula measures >2cm in diameter in
IVU or voiding cystourethrogram
-sacculesmeasures <2cm in diameter
-herniation of bladder mucosa through
the detrusor muscle
-98% congenital diverticula occurs in male
-most common in bladder base
-most frequently in the region of ureteral
hiatus known as Hutch diverticulum
-gives rise to obstruction or reflux
52Dept of Urology, GRH and KMC, Chennai.

53. Diverticula in fundusis –rare
bilateral &symmetrical ,gives rise to Micky
mouse appearance
MENKE’S syndrome(kinky hair syndrome)
-a disease of copper metabolism have
associated bladder diverticulum
Congenital bladder diverticulum occurs in
-Williams disease
-Diamond –black fan disease
-Fetal alcohol disease
-Prune –belly syndrome
-Ehler–danlostype 9 syndrome 53Dept of Urology, GRH and KMC, Chennai.

54. 54Dept of Urology, GRH and KMC, Chennai.

55. Associated anomalies :
-Ipsilateral Renal agenesis
-Ipsilateral undescendedtestis
Voiding cystourethrogram:
-diverticula are best shown
USG,CT,MRI: visible
55Dept of Urology, GRH and KMC, Chennai.

56. 56Dept of Urology, GRH and KMC, Chennai.

57. INDICATIONS:
for surgical treatment of bladder diverticulum is dictated by the
presence of
persistent high-grade vesicoureteralreflux,
and/or recurrent urinary tract infections,
or documented obstruction of either the distal
ureter or the bladder neck.
In cases of functional bladder outlet obstruction due to a very
large diverticulum extending underneath the bladder neck,
excision of the diverticulum is recommended.
For patients with recurrent urinary tract infection and
persistent vesicoureteralreflux, diverticulectomy is
recommended and should be accompanied by reimplantation
of the ureter
57Dept of Urology, GRH and KMC, Chennai.

58. The surgical approach can be either intravesicalor
extravesical.
The key surgical principle is to reinforce
the muscular hiatus of the bladder to prevent
reformation of the bladder diverticulum.
If upper tract evaluation reveal a non-functioning
kidney, consideration should be given to
nephroureterectomywith diverticulectomy.
Extravesicalapproaches and
laparoscopic techniques can be used
58Dept of Urology, GRH and KMC, Chennai.

59. THANK YOU
59Dept of Urology, GRH and KMC, Chennai.