NEPHROLOGY CONFERENCE - Jamaica Kidney Kids...

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


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)


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


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.

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