This study evaluated outcomes for patients with bladder exstrophy managed with primary repair, augmentation, or uretero-sigmoidostomy. The study included 21 primary repair patients, 18 augmentation patients, and 14 uretero-sigmoidostomy patients. Primary repair patients had poor continence and quality of life, with a 42% incontinence rate. Augmentation patients had improved outcomes, with 88% achieving average or better continence. Uretero-sigmoidostomy patients all had good quality of life and were largely continent, with 76.9% passing urine voluntarily. The study concluded that augmentation and uretero-sigmoidostomy resulted in better continence and quality of life outcomes compared to primary repair alone.

1. Follow up results in exstrophy
bladder managed by
Primary repair,
Augmented exstrophy repair and
Uretero-sigmoidostomy
Ravi P Kanojia, Sumit A,
Prema Menon,
Ram Samujh, KLN Rao
Department of Pediatric Surgery
Post Graduate Institute of Medical
Education and Research,
Chandigarh, India

2. General protocol:
Primary bladder reconstruction if patient is early presenting (within 3months),
bladder plate is >5cm diameter, pliable and non polypoidal
Augmentation done if Bladder is of small capacity with incontinence due to
storage problem
Uretero-Sigmoidostomy done as primary for late presenting pt, poor
socioeconomic status and intractable incontinence

3. Objectives
A non comparative bidirectional
study in EB patients who were
managed by one of the 3
mentioned
reconstruction/diversion

5. Patients and methods
Five year retrospective study conducted from July 2004 to
June 2009 and one year prospective study from July 2009
to June 2010
Inclusion criteria
 All patients who have had repair of Bladder Exstrophy
irrespective of their sex and age.
 Patient should have under gone one of the three mentioned
procedures :
 Complete or incomplete Primary repair
 Secondary Salvage procedures after primary repair (Augmentation)
 Uretero-Sigmoidostomy patients (primary or secondary)
 The patient should have completed minimum 1 yr of follow
up postoperatively.

6. Patients and methods
Exclusion criteria
All patients who underwent repair for Bladder
Exstrophy outside PGIMER, Chandigarh.
Patients with incontinent epispadias
Patients who have not completed 1 year of follow up
post operatively.

7. Patients and methods
The patients are subsequently divided into 3 groups
namely
Group I= Patients with primary repair of EB done either as
staged or single staged procedure.
Group II = Patients who have undergone bowel
augmentation to achieve continence
Group III = Patients undergoing Uretero-Sigmoidostomy
either as a primary procedure or as a salvage procedure .
Once the patients are stratified in the mentioned 3 groups
they are evaluated on a separate proforma designed for
each group. Several recording parameters can be same or
different across the three groups depending on the
feasibility.

8. Physical Quality of life (PQLI) scores
PQLI will be done by means of a structured questionnaire.
KIDSCREEN -52 modified to Indian patients.
The questions asked were in the following six dimensions
representing various aspects of physical and psycho-social well
being.
Physical well being and participation in daily life activities
 School environment and performance in school and
sports
participation in group activities
 Social support and relationship with peers
Genitalia reconstruction satisfaction after surgery, and
self perception
Psychological well being

9. PQLI scores
Patients or parents were asked to indicate for each
question how they perceived quality of life as compared
to normal children with Likert scale model (never-
seldom-sometimes-often-always, represented by 1-2-3-
4-5 point scoring system).
Maximum score 150
<100 poor
101-120 average
>121 good

10. Continence assessment
By objective scoring. Score range 5-15. Higher the better
CONTINENCE 3 2 1
Ability to micturate Via natural orifice Occasional catheterization Always needs catheterization
Dry period intervals >2.5hrs during 1.5-2.5hrs during day,3- <1.5hrs during day,<3hrs
day,>4hrs during 4hrs during night during night
night
Ability to sense Always Occasional Never
bladder filling
Nocturia None Occasional(<4/month), Multiple times>4times/month
needs fluid restriction
Ability to withhold Always Occasionally Never
micturition
Continence assessment for uretero-sigmoidostomy patients was done separately
5-7 poor (incontinent), 8-11 average, >11 good(continent)

11. STATUS OF UPPER TRACTS
Upper tracts were assessed for
dilatation
pyelonephritic changes
renal function deterioration and reflux
These will be quantitively assessed by
Serum Creatinine estimation.
Renal Ultrasound
MCU
DMSA scan

12. METABOLIC COMPLICATIONS (for Group III only):
Serial Arterial Blood Gas determination + Electrolytes
determination.
SALVAGE PROCEDURES REQUIRED : Several
patients will require salvage procedures like supra-
pubic or penile fistula closure, redo bladder neck
repairs for persisting incontinence.

13. Results

14. Group I n=21 (7 females)

15. Group I
Mean age at completion of all stages in staged repair 3 yrs
5 patients had u/l inguinal hernia 1 pt with b/l hernia
Surgical complications
 Ucf =5
 Wound infection 3
 Intestinal obstruction 1
Secondary operative procedures
 Fistula closure 5
 Herniotomy in 6
 Laparotomy for perf 1

16. Group I
Renal function status
Mean serum creatinine 0.57 (range 0.2-0.8 SD + 0.13)
Persistent Hydronephrosis with impaired fun = 3
b/l HDN 1
VUR 11 (7 bilateral)
Small capacity bladder 11

17. Group I, Continence
Mean continence score = 7.76/15 (range 7-9, SD+ 0.76)

18. Psychosocial assessment score mean=102.9/150
(range=83-133, SD+ 11.18)

19. Group I analysis
None of these patients were satisfactorily continent (ie
continent score >11.15)
42% (9/21) were incontinent even after complete repair
38% (8/21) had poor quality of life
Small capacity bladder was a probable cause of incontinence
in 50% of these patients.
57% (12/21) were with average continence
57% (12/21) had an average quality of life.

20. Group II N=18
17 males 1 female
Mean age 11.52 yrs (range 3.5-17, SD+ 6.05)
Mean age at augmentation 7.3 yrs (range 3-18 SD+ 4.34)
Mean duration of follow up 4.13 years (range 1-13, SD+
3.55)
Initial operative treatment
 One stage 8
 Staged repair 10
Reason for augmentation – small capacity bladder with no
hold up of urine

21. Group II
Type of augmentation
Colocystoplasty 13 (4 with mitrafannoff)
Ileocystoplasty 3
Gastrocystoplasty 2
Complications
UCF 7
Wound infection 3
Bladder calculus 7
Incisional hernia 1

22. Group II
Renal status
Mean sr creat 0.6 (range 0.4-1.4 SD+ 0.21)
Hydronephrosis 3 (2 bilateral)
Renal scarring 5 (2 bilateral)
Reflux bilateral gr II=7, gr I =4, gr III= 5
Metabolic derangements – none

23. Mean continence score 9.7/15 (range 6-13, SD+ 1.56)

24. Mean psychosocial assessment score 112.2/150
(range 88-127, SD+ 10.19)

25. Group II analysis
88% (16/18) had average continence (ie continence
score 7-10)
Only one patient was incontinence.
---- palsty faired better and had a higher mean
continence score as compared to ---- plasty
88% (16/18) of the patients had good quality of life
with a score of >100/150.

26. Group III
Uretero-sigmoidostomy group
14 patients (4 females)
Mean age 8.4 years (range 14 – 3.5 yrs (SD+ 3.6)
USG as primary procedure in 8 , 4 after staged bladder
repair, 2 after bladder turn in.
Mean age at USG 4.7 (range 1-11, SD+ 2.85)
Mean duration of follow up 3.6 yrs (1– 10yrs, SD+ 3.21)
Indications for USG
 Bladder dehiscence + incontinence 2
 Incontinence 3
 Small fibrosed bladder 9

27. Group III
Renal status
Normal in 11
Bilateral HUN 1
Unilateral HUN 1
Renal scans normal in all except 1 having severe
impaired cortical fun with b/l scar
Mean sr cr 0.6 (range 0.3-0.7, SD+ 0.16)

28. Group III
Continence status
76.9% (10) patients passed urine at will
23% pt had occasional wetting
66% patients did not have any urinary leak while
passing flatus
23% had urine leak only during straining
92% patients did not smell of urine

29. Group III
Psychosocial assessment
Mean score 130/150 (range 114-146, SD+ 7.5)

30. Group III
All the patients had good quality of life
Almost never smell of urine

31. Results analysis
Group III group not included In the continence comparison

32. Results analysis

33. Conclusions
Achieving continence with primary repair is evolving
our incontinence rate is 48%, and these patients have
poor quality of life.
Majority of these patients have small capacity bladder
hence they are heading for either augmentation or
diversion
The augmented patients do fairly better than primary
repair group the continence rate achieved is 88%.
Uretero-Sigmoidostomy patients since do not leak
urine by virtue of diversion are dry and have almost
normal social life and the PQLI of these patients is
good.
Contrary to general opinion metabolic problems were
not seen in any of these groups.

34. Thank you
Acknowledgements to all the affected children, parents with immense patience and
surgeons world wide who are involved in care of this disease