Download Pediatric Renal Disease

Pediatric Renal Disease
Alyssa Brzenski
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.
Posterior Urethral
Valve
•
1:5,000 births
•
1:2,500 prenatal ultrasounds
•
Most common obstructive
uropathy
Long term Side Effects
• Renal Scaring
• Renal Failure
• Decreased amniotic fluid
• Pulmonary Hypoplasia
Prenatal Ultrasound
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
Work-up- Voiding
Cystourethrogram
Antenatal Intervention
Effectiveness of Antenatal
Intervention on Survival
Effectiveness of Anetnatal
Intervention on renal function
Outcomes
• Perinatal Mortality
• Renal Function at 4-6 weeks
• Serum Cr
• Renal Ultrasound
• Need for Dialysis/Transplant
• Renal Function at 12 months
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
Case 2
• 8 month old male presents to ED with fever of 102.8
and tachycardia. On initial work up a straight cath
was performed which demonstrated a UTI. How
should this child be evaluated?
Vesicoureteral Reflux
• Present in 0.5-2% of children
• May present with recurrent UTI or may be
asymptomatic
• Most resolve without treatment
Grading
Prevalence in Siblings
Prevalence with
Hydronephrosis in Utero
Current Management
Guidelines
Goals of Treatment
• Prevent recurrent UTI
• Prevent Renal Damage/scarring
• Minimize the morbidity of treatment and followup
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
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
Deflux Injections
• GA for cystoscopy
• Submucosal Injection of
Deflux (dextranomer
microspheres and
hyaluronic acid)
• 80-90% success at first
injection
Ureteral Re-implant
• GA
• Routine Monitors
• Balanced anesthetic
• Epidural or Caudal for post-op pain management
and to reduce post-op bladder spasm
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.
Causes of Pediatric Renal
Failure
From NAPRTCS Annual Report. 2010. Accessed March 25, 2013 at www.naprtcs.org
Causes of Pediatric Renal
Failure by Age
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
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
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
Surgical Approach
Anesthetic Considerations
• Intravenous Induction
• Routine Monitors, CVP +/- Aline
• Balanced Anesthetic
• Epidural for Post-op Pain Control
Anesthetic Considerations
• On release of the renal artery clamp, have CVP of
10-15 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
When should we remove a
kidney?
• Nephrectomy before transplant due to:
•
•
•
•
•
Large proteinuria
Refractory Hypertension
Recurrent UTI or urosepsis
Urolithiasis
Polyuria
Sources
•
Williams G, Fletcher J, Alexander S, Craig J. Vesicoureteral Reflex. Journal of American Society of Nephrology. May 2008; 19: 849-62.
•
Peters C, et al. Summary of the AUA Guideline on Managemnt of Primary Vesicoureteral Reflux in Children. The Journal of Urology. Sept
2010; 184:1134-44.
•
Bogaert G, Slabbaert K. Vesicoureteral Reflux. European Urology Supplements. April 2012; 11: 16-24.
•
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: 1145-51.
•
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.
2004; 22: 809-23.
•
Sharbaf F, et al. Native Nephrectomy prior to Pediatric Kidney Transplant: Biological and Clinical Aspects. Pediatric Nephrology. 2012; 27: 1179-88.
•
Dell K. The Spectrum of Polycystic Kidney Disease in Children. Adv Chronic Kidney Disease. 2011; 18: 339-47.