1. Pediatric Renal Disease
Alyssa Brzenski

2. Case #1
A 33 year old female G2P1 at 20 weeks presents for evaluation of fetal bladder distention which was found on routine prenatal ultrasound. There were no other abnormalities found. The mother wants to know what is the prognosis and what interventions she should undergo.

3. Posterior Urethral Valve
1:5,000 births
1:2,500 prenatal ultrasounds
Most common obstructive uropathy
Posterior Urethral Valves are a relatively common finding, presenting in 1:2,500 prenatal ultrasounds and in 1:5000 live births.

4. Long term Side Effects Renal Scaring Renal Failure
Decreased amniotic fluid
Pulmonary Hypoplasia

5. Prenatal Ultrasound
Prenatal Ultrasound classically show a keyhole sign with a dilated bladder and posterior urethra.

6. Theories of Etiologies
Hypertrophy of the urethral ridge
Persistence of the urogenital membrane
Abnormal development of the wolffian or mullerian duct
Fusion of the posterior urethral ridge

7. Work-up- Voiding Cystourethrogram
Post-natally the diagnostic tool of choice is VCUG. Prenatally ultrasound is the diagnostic tool of choice. When prenatal ultrasound is used bilateral hydronephrosis can be diagnostic of bladder obstruction. However, 80% of 2nd trimester hydronephrosis will resolve by term and 40% of infants with bladder outlet obstruction will not have any hydronephrosis. More common is megacystis, but 90% of megacystis will resolve by birth. Thus our screening tools prenatally are not very good.

8. Antenatal Intervention

9. Effectiveness of Antenatal Intervention on Survival
A review of the current trials available shows an overall trend towards increased survival compared to no treatment

10. Effectiveness of Anetnatal Intervention on renal function
Although the survival improves with intervention there is not necessarily improved renal function.

13. Outcomes Perinatal Mortality Renal Function at 4-6 weeks
Serum Cr
Renal Ultrasound
Need for Dialysis/Transplant
Renal Function at 12 months

14. Prune Belly Syndrome 1:40,000 births Weak abdominal muscles Weak cough
Associated with
Orthopedic defects (Congenital Hip Dislocation and Scoliosis)
GI (malrotation and volvulus)
Heart (TOF, VSD)
Trisomy 18 and 21

15. Case 2
8 month old male presents to ED with fever of and tachycardia. On initial work up a straight cath was performed which demonstrated a UTI. How should this child be evaluated?

16. Vesicoureteral Reflux
Present in 0.5-2% of children
May present with recurrent UTI or may be asymptomatic
Most resolve without treatment
VUR is a retrograde reflux of urine from the bladder into the ureter.

17. Grading
Grade I is reflux into the non-dilated ureter. Grade II is reflux in to the non-dilated renal pelvis. Grade III is mild/moderated dilation of the ureter and renal pelvis. Grade IV is reflux into the ureter and renal pelvis with moderate ureteral tortuosity. Grade V is marked dilation and tortuosity with loss of the papillary impressions.

18. Prevalence in Siblings

19. Prevalence with Hydronephrosis in Utero

20. Current Management Guidelines

21. Goals of Treatment Prevent recurrent UTI Prevent Renal Damage/scarring
Minimize the morbidity of treatment and followup

22. Antibiotic Prophylaxis
Less than 1 years old
Febrile UTI- Antibiotic prophylaxis
Afebrile UTI with Grade III-V reflux- Antibiotic Prophylaxis
Afebrile UTI with Grade I or II reflux- may offer antibiotic prophylaxis
Older than 1 years old
Febrile UTI- conservative management or antibiotic prophylaxis
Recurrent UTI- start antibiotic treatment or if on antibiotics surgical treatment

23. Who needs surgery?
Children with recurrent infections despite antibiotic prophylaxis
Children who have developed renal scaring or poor renal function
Severe reflux (Grade V or bilateral IV)
Mild to moderate reflux that persists as the patient approaches puberty

24. Deflux Injections GA for cystoscopy
Submucosal Injection of Deflux (dextranomer microspheres and hyaluronic acid)
80-90% success at first injection
Deflux Injections are a minimally invasive means to change the geometry of the ureter in hopes to prevent urinary reflux. This is done under general anesthesia with a cystoscope. A submucosal injection is given which creates a “volcano mound”.

25. Ureteral Re-implant GA Routine Monitors Balanced anesthetic
Epidural or Caudal for post-op pain management and to reduce post-op bladder spasm

26. Case 4
5 year old, 15kg, female with chronic renal failure, secondary to polycystic kidney disease, is admitted for a kidney transplant. She is currently on peritoneal dialysis and was last dialyzed yesterday. She has limited exercize tolerance. Labs including potassium are all normal. ECHO was normal.

27. Causes of Pediatric Renal Failure
From NAPRTCS Annual Report Accessed March 25, 2013 at

28. Causes of Pediatric Renal Failure by Age

29. Pediatric Chronic Renal Disease
CV- HTN, LV thickening, CHF, Volume Overload
Pulm-Volume Overload
GI- Delayed gastric emptying
Heme- anemia, dysfunctional platelets
Endo/Metabolic- Hyperkalemia, hypercalcemia
Growth- Delayed growth

30. Polycystic Kidney Disease
Autosomal Dominant- 90% of cases
Typically presents in adulthood with macrocysts
Autosomal Recessive- 10% of cases
Presents in-utero screening or in early in infancy
Microcysts of the collecting tubules

31. Autosomal Recessive Polycystic Kidney Disease
Affects
Kidneys- 30% progress to ESRD by 1st decade with 58% needing a renal transplant by adulthood
Liver- 50% will develop hepatic fibrosis with seqelae of portal hypertension
May have pulmonary hypoplasia from decreased urine production in-utero

32. Surgical Approach

33. Anesthetic Considerations
Intravenous Induction
Routine Monitors, CVP +/- Aline
Balanced Anesthetic
Epidural for Post-op Pain Control

34. Anesthetic Considerations
On release of the renal artery clamp, have CVP of with blood pressure at baseline or 10% higher (may need pRBC or dopamine)
Adult kidney in a small child or infant will require a significant portion of total blood flow, leading to potential hypotension (volume load prior)
Small infants or very sick children should remain intubated, but most children can be extubated in the OR

35. When should we remove a kidney?
Nephrectomy before transplant due to:
Large proteinuria
Refractory Hypertension
Recurrent UTI or urosepsis
Urolithiasis
Polyuria

36. Sources
Williams G, Fletcher J, Alexander S, Craig J. Vesicoureteral Reflex. Journal of American Society of Nephrology. May 2008; 19:
Peters C, et al. Summary of the AUA Guideline on Managemnt of Primary Vesicoureteral Reflux in Children. The Journal of Urology. Sept 2010; 184:
Bogaert G, Slabbaert K. Vesicoureteral Reflux. European Urology Supplements. April 2012; 11:
Skoog S, et al. Pediatric Vesicoureteral Reflux Guidelines Panel Summary Report: Clinical Practice Guidelines for Screening Children with Vesicoureteral Reflux and Neonates/Infants with Prenatal Hydronephrosis. The Journal of Urology.Sept 2010; 184:
Holmes N, Harrison M, Baskin C. Fetal Surgery for Posterior Urethral Valves: Long-Term Postnatal Outcomes. Pediatrics. 2001; 108: 1-7.
Casella D, Tomaszewski J, Ost M. Posterior Urethral Valves: Renal Failure and Prenatal Treatment. Internation Nephrology. 2012; 1-4.
Uejima T. Anesthetic Management of the Pediatric Patient Undergoing Solid Organ Transplantation. Anesthesiology Clinics of North America ; 22:
Sharbaf F, et al. Native Nephrectomy prior to Pediatric Kidney Transplant: Biological and Clinical Aspects. Pediatric Nephrology. 2012; 27:
Dell K. The Spectrum of Polycystic Kidney Disease in Children. Adv Chronic Kidney Disease. 2011; 18: