NEPHROLOGY CONFERENCE - Jamaica Kidney Kids …kidneykidsja.com/wp-content/uploads/ANTENATAL-HYDRONEPHROSIS.pdfNEPHROLOGY CONFERENCE Jamaica ... anterioposterior!diameter!of!the!renal!pelvis

1st JAMAICAN PAEDIATRIC NEPHROLOGY CONFERENCE

Jamaica Conference Centre

Kingston Jamaica October 4th 2014  

in association with

Antenatal  Hydronephrosis-­‐    When  To  Act  

Rulan  S.  Parekh    Professor  of  Pediatrics  and  Medicine  

Hospital  for  Sick  Children    University  of  Toronto  

Disclosures    

•  None  

Learning  Objec<ves  

At  the  end  of  this  session  you  will  be  able  to:  

1.  Understand  the  significance  of  antenatal  hydronephrosis  (ANH)  

2.  Understand  the  management  of  a  newborn  with  ANH  

3.  Understand  some  management  issues  of  newborns  with  ANH  

Outline  

•  Antenatal  Hydronephrosis  •  DefiniKons  •  Measurement  •  Epidemiology  

•  Postnatal  Hydronephrosis  •  DefiniKons  •  Measurement  •  Epidemiology  •  EKology  •  Management  

•  Future  Risk  

Congenital  Anomalies  of  the  Kidney  and  Urinary  Tract  -­‐  CAKUT  Broad  range  of  anomalies  •  MalformaKons  of  the  renal  parenchyma  •  AbnormaliKes  of  embryonic  migraKon  •  AbnormaliKes  of  the  developing  urinary  collecKon  system  

–  Vesicoureteral  reflux  (VUR)  –  Ureteropelvic  juncKon  obstrucKon  (UPJO)  –  Ureterovesical  juncKon  obstrucKon  (UVJO)  –  Posterior  urethral  valves  (PUVs)  –  Prune  Belly  syndrome  (males)  –  Ureterocele  

Variables involved in urine flow impairment

Geary  and  Schaefer  (eds.),  Comprehensive  Pediatric  Nephrology,  Chapter  37  

Consequence of congenital urethral obstruction

Geary  and  Schaefer  (eds.),  Comprehensive  Pediatric  Nephrology,  Chapter  37  

Antenatal  hydronephrosis  •  DefiniKons  by  ultrasound:  

–  Society  of  Fetal  Urology  (SFU)  based  on  •  Pelvic  dilataKon  •  Number  of  calyces  seen  •  Presence  and  severity  of  parenchymal  atrophy  

–  Maximum  renal  pelvic  diameter  measured  from  the  anterioposterior  diameter  of  the  renal  pelvis  

•  Mild  >  4-­‐10  mm  •  Moderate  >10  mm  •  Severe  or  at  greatest  risk  >15  mm  

–  AddiKonal  factors  •  Unilateral  or  bilateral  •  Size  of  kidneys/parenchymal  •  Bladder  abnormaliKes  •  AmnioKc  fluid  •  Other  issues-­‐  ?  Down  syndrome  or  other  chromosomal  anomalies  

Timing  of  antenatal  ultrasound  

•  12-­‐15th  week  of  gestaKon:  Fetal  hydronephrosis  (kidney/bladder)  can  be  detected  

•  16-­‐20th  week  of  gestaKon:  OpKmal  Kme  window  for  screening  ultrasound  (before  18-­‐24th  week  may  fail  detecKng  significant  disease)  

•  28-­‐34th  week  of  gestaKon  (third  trimester):  Repeat  US  to  idenKfy  fetuses  requiring  postnatal  intervenKon  

Classifica<on  of    antenatal  hydronephrosis  

Antenatal  hydronephrosis  (ANH)  •  Epidemiology:  

–  Incidence  •  ProspecKve  studies  >4-­‐5  mm    in  2nd  trimester  

–  0.6-­‐4.5%    in  over  25,000  women  in  Europe  

•  Meta-­‐analyses  of  17  studies  and  various  definiKons  –  1.6%  in    over  100,000  women  

– Majority  are  transient  over  65%  – More  common  among  males  – Antenatal  hydro  severity  predicts  postnatal  degree  of  hydronephrosis  BUT  risk  of  vesicoureteral  reflux  varies  (measurement  error?)  

Meta-­‐analysis  of  antenatal  hydronephrosis  

Degree  of  antenatal  hydronephrosis  

 Hydronephrosis  ANH  CAKUT  

 Mild  <  7  mm  (2nd)      <  9  mm  (3rd)  12%  

 Moderate  7-­‐10  mm  (2nd)      9-­‐15  mm  (3rd)  45%  

 Severe  >  10  mm  (2nd)      >  15  mm  (3rd)  88%  

Lee  et  al,  Pediatrics  2006  

Congenital  Anomalies  of  the  Kidney  and  Urinary  Tract  -­‐  CAKUT  Broad range of anomalies •  Malformations of the renal parenchyma •  Abnormalities of embryonic migration •  Abnormalities of the developing urinary collection system

–  Vesicoureteral reflux (VUR) –  Ureteropelvic junction obstruction (UPJO) –  Ureterovesical junction obstruction (UVJO) –  Posterior urethral valves (PUVs)

Prune Belly syndrome (males) –  Ureterocele

Vesicoureteral  reflux  (VUR)  

Outcome  of  antenatal  hydronephrosis  

•  Transient/physiologic        63%  •  Uteropelvic  juncKon  obstrucKon  11%  •  Vesicoureteral  reflux      9%  •  Megaureter          4%  •  Ureterocele          2%  •  Posterior  Urethral  valves      1%  •  Others            10%  

Vesicoureteral  reflux  (VUR)  

Geary  and  Schaefer  (eds.),  Comprehensive  Pediatric  Nephrology,  Chapter  37  

Obstruc<ons  of  upper  urinary  tract  

1.  Upper  calyx  syndrome  2.  Ureteropelvic  juncKon  obstrucKon  (UPJO)  3.  Ureterovesical  juncKon  obstrucKon  (UVJO)  4.  Ureterocele    

Ureteropelvic  junc<on  obstruc<on  (UPJ)  

UPJ  –  pre  /  post  surgery  

Geary  and  Schaefer  (eds.),  Comprehensive  Pediatric  Nephrology,  Chapter  37  

Management  of  antenatal  hydronephrosis  •  Ultrasound  aier  48  hours  of  life  

–  PotenKally  volume  depleted  –  Increasing  renal  flow  in  first  48  hours  of  life  –  Thus,  increasing  glomerular  filtraKon  and  increased  urine  output  

•  Prior  to  48  hours  of  life  –  If  concern  of  significant  renal  impairment  

•  Renal  parenchymal  disease  •  Bilateral    •  Bladder  involvement  •  Other  congenital  anomalies  

–  If  negaKve,  cannot  rule  out  persistent  hydronephrosis  

Antenatal  Hydronephrosis:  Current  Protocol  at  Sick  Kids  

•  All  children  with  prior  history  of  ANH  are  referred  for  ultrasound  –  Most  visits  occur  within  1-­‐2  months  aier  birth  –  Most  on  anKbioKc  prophylaxis  –  Detailed  physical  exam  to  rule  out  syndromic  disease  –  Normal,  low  risk  or  high  risk  

•  High  risk:  If  postnatal  ultrasound  renal  pelvis  diameter  >12  mm  or  caliectasis  or  another  renal,  bladder  or  urinary  tract  anomaly  

–  Voiding  cystourethrogram  (VCUG)  to  rule  out  VUR  –  Simultaneously  MAG-­‐3  scan  (  or  when  VCUG  is  normal)  to  rule  out  UPJ  –  Remain  on  anKbioKc  prophylaxis    

•  Low  risk:  If  postnatal  ultrasound  renal  pelvis  diameter  7-­‐12  mm    –  Followed  at  3-­‐12  months  with  US  depending  on  the  degree  of  AP  diameter    –  AnKbioKc  prophylaxis  for  at  least  2-­‐3  months  and  longer  depends  on  follow-­‐up  –  Follow-­‐up  ultrasound  and  DMSA  to  evaluate  for  potenKal  renal  scarring  

•  Children  with  UTI  will  undergo  VCUG  and  DMSA  irrespecKve  of  US  

Why  not  do  a  VCUG/MAG-­‐3    in  every  child?  

•  VCUG  –  For  a  child  5-­‐10  years  old,  the  effecKve  radiaKon  dose  

•  1.6  mSv    •  average  person  receives  from  natural  background  radiaKon  in  6  months    

–  For  an  infant,  the  effecKve  radiaKon  dose    •  0.8  mSv    •  average  person  receives  from  natural  background  radiaKon  in  3  months  

•  MAG-­‐3/DMSA  –  ~1mSv  

Children's  (Pediatric)  Voiding  Cystourethrogram  Copyright©  2010,  RadiologyInfo.org  

Remember…..    

•  Over  65%  will  go  on  and  have  no  long  term  issues!  

Risk    •  CAKUT  

–  Familial  –  Can’t  jusKfy  screening  asymptomaKc  family  members  

•  Urinary  tract  infecKons(UTIs)  –  Females  – DysfuncKonal  voiding  

•  Progression  –  Scarring  can  be  independent  of  UTIs  and  prophylaxis  –  Scarring  is  common  cause  of  hypertension  in  adolescents  

–  Slower  rate  of  progression  to  chronic  kidney  disease  compared  to  renal  parenchymal  disorders  

Pedigree  Structure  

Weng  et  al.,  JASN,  2009  

Gene<c  loci  and  CAKUT/  VUR  

•  Familial  based  studies  –  Genome  wide  linkage  analyses  –  Among  Ashkenazi  families,  recessive  locus  on  12p11-­‐q13  –  Among  a  Somalian  family,  recessive  locus  on  8q24  –  Among  European  Americans,  SNPs  idenKfied  at  mulKple  loci  –  Among  Europeans,  3  SNPs  idenKfied  in  one  group  but  not  validated  in  a  separate  group  

•  No  case-­‐control  genome  wide  associaKon  study    •  Issues    

–  Sample  size  –  Phenotypic  variability-­‐  extreme  phenotypes/syndromes  –  Long-­‐term  outcome  is  mild  in  the  majority  of  VUR  

Discussions  with  Families    

•  Provide  informaKon  on  – Hydronephrosis  –  IndicaKons  for  severity  of  disease  – Ask  about  family  history  – Discuss  need  for  careful  monitoring  and  follow-­‐up  – Guarded  about  making  comments  on  prognosis  – Provide  all  expectant  families  with  reading  material  on  tests  and  anKbioKcs  

Time  to  first  recurrent  febrile  episode  or  symptoma<c  UTI  in  RIVUR  

Summary  •  Antenatal  hydronephrosis  occurs  in    <5%  of  pregnancies  

•  Over  65%  will  have  no  long-­‐term  sequelae  •  Postnatal  ultrasound  should  be  done  aier  48  hours  of  life    

•  Family  history  is  important  risk  factor  •  Overall  long-­‐term  outcome  is  very  good  •  AnKbioKc  prophylaxis  is  currently  recommended  for  even  mild  disease  

•  Management  depends  iniKal  findings  (pracKce  is  evolving)  

QuesKons?  

Reviews  •  Sidhu,  G,  Beyene,  J,  Rosenblum,  ND.  Outcome  of  isolated  antenatal  hydronephrosis:  a  systemaKc  review  and  meta-­‐analysis.  Pediatr  Nephrol  2006;  21:218.    

•  Lee  RS;  Cendron  M;  Kinnamon  DD;  Nguyen  HT,  Antenatal  hydronephrosis  as  a  predictor  of  postnatal  outcome:  a  meta-­‐analysis.  Pediatrics.  2006  Aug;118(2):586-­‐93.  

•  Woodward,  M,  Frank,  D.  Postnatal  management  of  antenatal  hydronephrosis.  BJU  Int  2002;  89:149.