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
General protocol:Primary bladder reconstruction if patient is early presenting (within 3months),bladder plate is >5cm diameter, pliable and non polypoidalAugmentation done if Bladder is of small capacity with incontinence due tostorage problemUretero-Sigmoidostomy done as primary for late presenting pt, poorsocioeconomic status and intractable incontinence
Objectives A non comparative bidirectional study in EB patients who were managed by one of the 3 mentioned reconstruction/diversion
Patients and methodsFive year retrospective study conducted from July 2004 to June 2009 and one year prospective study from July 2009 to June 2010Inclusion 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.
Patients and methodsExclusion criteriaAll patients who underwent repair for Bladder Exstrophy outside PGIMER, Chandigarh.Patients with incontinent epispadiasPatients who have not completed 1 year of follow up post operatively.
Patients and methodsThe patients are subsequently divided into 3 groups namelyGroup 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 continenceGroup 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.
Physical Quality of life (PQLI) scoresPQLI 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 sportsparticipation in group activities Social support and relationship with peersGenitalia reconstruction satisfaction after surgery, and self perceptionPsychological well being
PQLI scoresPatients 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 poor101-120 average>121 good
Continence assessmentBy objective scoring. Score range 5-15. Higher the better CONTINENCE 3 2 1Ability to micturate Via natural orifice Occasional catheterization Always needs catheterizationDry 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 restrictionAbility 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)
STATUS OF UPPER TRACTSUpper tracts were assessed for dilatation pyelonephritic changes renal function deterioration and reflux These will be quantitively assessed bySerum Creatinine estimation.Renal UltrasoundMCUDMSA scan
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.
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.
Group II N=1817 males 1 femaleMean 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 10Reason for augmentation – small capacity bladder with no hold up of urine
Group IIType of augmentation Colocystoplasty 13 (4 with mitrafannoff) Ileocystoplasty 3 Gastrocystoplasty 2Complications UCF 7 Wound infection 3 Bladder calculus 7 Incisional hernia 1
Group IIRenal 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
Mean continence score 9.7/15 (range 6-13, SD+ 1.56)
Mean psychosocial assessment score 112.2/150 (range 88-127, SD+ 10.19)
Group II analysis88% (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 ---- plasty88% (16/18) of the patients had good quality of life with a score of >100/150.
Group IIIUretero-sigmoidostomy group14 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
Group IIIRenal 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)
Group IIIContinence 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
Group III Psychosocial assessment Mean score 130/150 (range 114-146, SD+ 7.5)
Group IIIAll the patients had good quality of lifeAlmost never smell of urine
Results analysis Group III group not included In the continence comparison
ConclusionsAchieving 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 diversionThe 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.
Thank youAcknowledgements to all the affected children, parents with immense patience andsurgeons world wide who are involved in care of this disease