Exstrophy results for world congress

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Exstrophy results for world congress

  1. 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. 2. 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
  3. 3. Objectives A non comparative bidirectional study in EB patients who were managed by one of the 3 mentioned reconstruction/diversion
  4. 4. 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.
  5. 5. 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.
  6. 6. 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.
  7. 7. 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
  8. 8. 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
  9. 9. Continence assessmentBy 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)
  10. 10. 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
  11. 11. 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.
  12. 12. Results
  13. 13. Group I n=21 (7 females)
  14. 14. 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
  15. 15. 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
  16. 16. Group I, ContinenceMean continence score = 7.76/15 (range 7-9, SD+ 0.76)
  17. 17. Psychosocial assessment score mean=102.9/150 (range=83-133, SD+ 11.18)
  18. 18. 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.
  19. 19. 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
  20. 20. 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
  21. 21. 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
  22. 22. Mean continence score 9.7/15 (range 6-13, SD+ 1.56)
  23. 23. Mean psychosocial assessment score 112.2/150 (range 88-127, SD+ 10.19)
  24. 24. 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.
  25. 25. 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
  26. 26. 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)
  27. 27. 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
  28. 28. Group III Psychosocial assessment Mean score 130/150 (range 114-146, SD+ 7.5)
  29. 29. Group IIIAll the patients had good quality of lifeAlmost never smell of urine
  30. 30. Results analysis Group III group not included In the continence comparison
  31. 31. Results analysis
  32. 32. 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.
  33. 33. Thank youAcknowledgements to all the affected children, parents with immense patience andsurgeons world wide who are involved in care of this disease

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