Postfulguration Follow Up of PUV Patients

Postablation followup of PUV Patients

1. PUV follow up; Post fulguration Dr. Faheem Ul Hassan Fellow Pediatric Urology IGICH Banglore Dr. Vinay Jadhav Associate Professor Pediatric Surgery & Pediatric Urology IGICH Banglore

2. VBD despite successful valve ablation , intrinsic bladder dysfunction leads to deterioration of them upper urinary tracts and incontinence.

3. Causes of VBD 1. Detrusor abnormalities poor compliance, and myogenic failure 2. High-pressure voiding secondary to incomplete valve ablation Bladder neck hypertrophy 3. Detrusor-sphincter dyssynergia in which the sphincter muscle fails to relax during Voiding

4. VBD- Causes 4. Polyuria secondary to a concentrating defect 5. Thimble Bladder , which is a bladder with poor compliance resulting from fibrosis secondary to long-standing obstruction.

5. VBD- Molecular Level There is hypertrophy of smooth muscles increase in the deposition of extracellular matrix (ECM) Alteration in the detrusor blood flow resulting in ischemia and decreased perfusion There is a shift to anerobic metabolism and the nerves within the bladder wall are damaged. 

6. Prognosis At follow‑up after 18 years of age, CRF was detected in 54%, hypertension in 37.5% presence of lower urinary tract symptoms in 29%. In India , medicolegal termination in view of bad prognosis for a fetal condition is permitted as per law

7. Prognostic factors Poor prognostic factors prenatal detection at <24 weeks gestation, respiratory distress at birth, Urinary sepsis , dyselectrolytemia , nadir serum creatinine >0.8 mg/ dL , bilateral VUR, hyperechoic kidneys, and absence of pop‑off mechanism.

8. VUR in PUV VUR is present in about ⅓ to ½ of the patients of PUV. Half of these will have U/L and the other half would have B/L VUR will resolve in at least 1/3rd of the patients Remaining 2/3rd would require deflux injection or surgery . Resolution of reflux has also been seen with addition of alpha‑blockers. The incidence of ESRD before 16 years was highest in a patient with bilateral VUR (25% ), unilateral VUR (7% )

9. PUV and Bladder dysfunction 1/3 rd of the patients have persistent bladder dysfunction after PUV ablation, 50%– 70% have high PVRU due to bladder neck hypertrophy. Radiological indicators of dysfunction persistent UTD, posterior urethral dilatation, VUR, trabeculation and diverticula in bladder, and significant postvoid residual urine.

10. Goals of Follow up The principles of follow‑up for PUV are to: 1. Maximize renal function 2. Minimize urinary infections 3. Minimize renal scarring 4. Assess voiding dysfunction 5. Attaining urinary continence 6. Assess bladder growth 7. Assess need for renal replacement therapy

11. Follow up 3m, 6m, 9m, 1y, 3y, 5y, 10y, 13y, 15y RFT, CBC, electrolytes , midstream urine for analysis, USG with with BC and PVRU, uroflowmetry were noted . More than 10% of the prevoid volume is abnormal and described as significant postvoid residue

12. Indications for MCU Check MCU at 3 months postfulguration (optional) If HUN is present postfulguration on USG, repeat VCUG at 2, 5, and 10 years If deflux /ureteral reimplantation planned To study the bladder anatomy before bladder augmentation or renal transplant.

13. UDS 1. Persistent daytime urinary incontinence beyond the age of 5 years 2. Deterioration in RFT (rising creatinine or dropin GFR) with no obvious cause such as growth spurt 3. Increase in upper tract dilatation in the absence of ongoing outflow obstruction 4. Before renal transplantation to ensure a safe, compliant low‑pressure urinary tract

14. UDS 7 . Functional problems – detrusor‑sphincter discoordination or suspected dyssynergia 8. Increased PVRU.

15. Drug Treatment- Anticholinergics

16. Casey et al 3 abnormal urodynamic patterns on UDS of myogenic failure, Detrusor hyperreflexia and decreased compliance/ small capacity may develop The type of bladder dysfunction that develops after PUV ablation can be unpredictable .

17. Casey et al Initial urodynamics were performed at approximately 3 months after PUV ablation. High voiding was defined as pressure greater than 60 cm H2O), small bladder capacity was defined as less than 70% of EBC oxybutynin (0.1 mg/kg twice daily). This low dose was chosen given the young age of the patients.

18. Casey et al UDM was performed six monthly upto toilet training Oxybutinin was stopped at toilet training Mean age for PUV fulguration was 17 days Oxybutinin was started on mean age of 3 months

19. Casey et al Initial voiding pressures were high (defined as greater than 60 cm H2O) in 17 of 18 Among the 17 patients with initially high voiding pressures 15 demonstrated improvement to a mean voiding pressure of 49.9 cm after 12 months

20. Casey et al Poor bladder compliance was defined as a PSBV at 25 cm H2O of less than 90% bladder capacity all demonstrated improvements in bladder compliance while on oxybutynin,

21. Casey et al Low bladder capacity was defined as less than 70% EBC, normal bladder capacity as 75% to 200% EBC and high bladder capacity as greater than 200% EBC. All the patients experienced significant improvement in capacity with OXB However 2 patients out of 7 had abnormal increase in bladder capacity (MF)

22. Casey et al 4 patients out of 17 needed discontinuation of OXB due to Inappropriate increase in BC MF Increased PVRU

23. M K Abraham

24. Abraham et al Forty-two children with significant PVRU after valves ablation were studied placed on Terazosin ranging from 0.25 to 2 mg . Post void urine at follow up was monitored with abdominal ultrasound

25. Abraham et al PVRU significantly reduced in 95% who were put on Terazosin. Mean pretreatment PVR was 15.7 ml and mean PVR at the last follow up was 2.4 ml (P = 0.000). Mean follow up was 17 months

26. Abraham et al

27. PUV-Role of Nephrologist The nephrologist should be involved earlier in cases of deranged renal parameter. They take care of timely institution of renal replacement therapy including Vitamin D , soda bicarbonate, calcium, erythropoietin , Proteinuria depicts early renal damage.

28. PUV-Timed Voiding The children with dilated upper tracts should be encouraged to do double or triple voiding. Constipation should be avoided . Adequate bladder emptying can be achieved by timed voiding

29. PUV- CIC Bladder may not be able to empty completely due to Myogenic failure Overdose of OXB Hyperactive sphincter (DSD) CIC should be instituted with raised PVR It increases bladder complicance Improves renal function and GFR Decreases the grades of reflux

30. CUA

31. CUA CIC was instituted in < 4 year children when indicated

32. CUA

33. PUV-NTD

34. PUV-NTD Overnight drainage in conjunction with daytime CIC can be appropriate management in children with poorly compliant bladders, especially in the early stages of renal compromise

35. ` PUV-Biofeedback Therapy Children with urinary tract dysfunction biofeedback therapy and Home pelvic floor exercises, with an overall consistent good response in 70 %. Ansari MS, Srivastava A, Kapoor R, Dubey D, Mandani A, Kumar A. Biofeedback therapy and home pelvic floor exercises for lower urinary tract dysfunction after posterior urethral valve ablation. The Journal of urology. 2008 Feb 1;179(2):708-11

36. PUV-Bladder neck ablation Secondary bladder neck obstruction has been over diagnosed in patients with PUV The practice has not been adopted universally

37. PUV-Bladder neck ablation No Improvement was found

38. PUV-Bladder Augmentation Augmentation may be required in valve bladder when medical management fails to prevent the deterioration of renal function or the bladder is very small with thickened wall and High Grade VUR with recurrent UTI Renal transplant has been planned.

39. PUV-Bladder Augmentation

40. PUV-Bladder Augmentation Treatment of  VBD with  clean  CIC  and NTD via a Mitrofanoff stoma can achieve significant improvements in hydronephrosis and bladder dysfunction urodynamic parameters . However , it does not prevent renal deterioration.

41. Renal transplantation As up to 50% of PUV patients can end in ESRD, renal transplantation may be required . This may require bladder augmentation to increase the bladder capacity

42. Controversy about pretransplant augmentation Pretransplant augmentation raises the risk of febrile UTIs significantly and may lead to malfunction of the graft

43. Controversy about pretransplant augmentation

44. Controversy about pretransplant augmentation

45. PUV-VUR

46. PUV-VUR The incidence of VUR at diagnosis of PUV is 64% Spontaneous resolution of reflux is seen in 50% of ureters within 2 years (67% within 3 years) after valve ablation, occurring more rapidly in unilateral than bilateral Kidneys with refluxing ureters have worse primary function VUR is frequently bilateral

47. PUV-VUR The refluxing nonfunctioning kidney was removed in 18% of our patients, although the possible benefit remains undetermined

48. PUV- Minivesicostomy

49. PUV- Minivesicostomy

50. Minivesicostomy The mini- vesicostomy allows bladder cycling as the stoma is very small and keeps the storage function of the bladder intact, provides an access for CIC. This may decrease the need for future bladder augmentation in these patients

51. Urethral Index

52. Urethral Index MCU to be done at 3 months post ablation

53. SWRD Score Incontinence is found in 20 % of patients with treated PUV on longterm follow-up It is caused by sphincter weakness postablation Interestingly , most patients (up to 90%) will have spontaneous improvement by puberty OXB, CIC and AC are the treatment options

54. SWRD Score Compliant bladder Score 1, Non Compliant bladder Score 3

55. Thank you