2Case #1A 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.
3Posterior Urethral Valve 1:5,000 births1:2,500 prenatal ultrasoundsMost common obstructive uropathyPosterior Urethral Valves are a relatively common finding, presenting in 1:2,500 prenatal ultrasounds and in 1:5000 live births.
4Long term Side Effects Renal Scaring Renal Failure Decreased amniotic fluidPulmonary Hypoplasia
5Prenatal UltrasoundPrenatal Ultrasound classically show a keyhole sign with a dilated bladder and posterior urethra.
6Theories of Etiologies Hypertrophy of the urethral ridgePersistence of the urogenital membraneAbnormal development of the wolffian or mullerian ductFusion of the posterior urethral ridge
7Work-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.
13Outcomes Perinatal Mortality Renal Function at 4-6 weeks Serum CrRenal UltrasoundNeed for Dialysis/TransplantRenal Function at 12 months
14Prune Belly Syndrome 1:40,000 births Weak abdominal muscles Weak cough Associated withOrthopedic defects (Congenital Hip Dislocation and Scoliosis)GI (malrotation and volvulus)Heart (TOF, VSD)Trisomy 18 and 21
15Case 28 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?
16Vesicoureteral Reflux Present in 0.5-2% of childrenMay present with recurrent UTI or may be asymptomaticMost resolve without treatmentVUR is a retrograde reflux of urine from the bladder into the ureter.
17GradingGrade 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.
21Goals of Treatment Prevent recurrent UTI Prevent Renal Damage/scarring Minimize the morbidity of treatment and followup
22Antibiotic Prophylaxis Less than 1 years oldFebrile UTI- Antibiotic prophylaxisAfebrile UTI with Grade III-V reflux- Antibiotic ProphylaxisAfebrile UTI with Grade I or II reflux- may offer antibiotic prophylaxisOlder than 1 years oldFebrile UTI- conservative management or antibiotic prophylaxisRecurrent UTI- start antibiotic treatment or if on antibiotics surgical treatment
23Who needs surgery?Children with recurrent infections despite antibiotic prophylaxisChildren who have developed renal scaring or poor renal functionSevere reflux (Grade V or bilateral IV)Mild to moderate reflux that persists as the patient approaches puberty
24Deflux Injections GA for cystoscopy Submucosal Injection of Deflux (dextranomer microspheres and hyaluronic acid)80-90% success at first injectionDeflux 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”.
25Ureteral Re-implant GA Routine Monitors Balanced anesthetic Epidural or Caudal for post-op pain management and to reduce post-op bladder spasm
26Case 45 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.
27Causes of Pediatric Renal Failure From NAPRTCS Annual Report Accessed March 25, 2013 at
30Polycystic Kidney Disease Autosomal Dominant- 90% of casesTypically presents in adulthood with macrocystsAutosomal Recessive- 10% of casesPresents in-utero screening or in early in infancyMicrocysts of the collecting tubules
31Autosomal Recessive Polycystic Kidney Disease AffectsKidneys- 30% progress to ESRD by 1st decade with 58% needing a renal transplant by adulthoodLiver- 50% will develop hepatic fibrosis with seqelae of portal hypertensionMay have pulmonary hypoplasia from decreased urine production in-utero
33Anesthetic Considerations Intravenous InductionRoutine Monitors, CVP +/- AlineBalanced AnestheticEpidural for Post-op Pain Control
34Anesthetic 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
35When should we remove a kidney? Nephrectomy before transplant due to:Large proteinuriaRefractory HypertensionRecurrent UTI or urosepsisUrolithiasisPolyuria
36SourcesWilliams 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: