mehul a. shah · • most serious and fortunately less common of the antenatal renal anomalies-~30%...

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Mehul A. Shah M.B.,B.S. - Seth G. S. Medical College & KEM Hospital, Bombay, 1988 M.D. (Ped) & DCH – Wadia Children’s Hospital, Bombay, 1993 M.D. (Ped) Univ of Connecticut, USA, 1996 Fellowship in Ped Nephrology at Southwestern Medical Center, Dallas, Texas, USA, 1999 American Board Certified in Pediatrics & Pediatric Nephrology Consultant Pediatric Nephrologist in Apollo Hospitals & Little Stars Children’s Hospital, Hyderabad since past 19 years Special interest Renal Tubular disorders

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Mehul A. Shah

• M.B.,B.S. - Seth G. S. Medical College & KEM Hospital, Bombay, 1988

• M.D. (Ped) & DCH – Wadia Children’s Hospital, Bombay, 1993

• M.D. (Ped) – Univ of Connecticut, USA, 1996

• Fellowship in Ped Nephrology at Southwestern Medical Center, Dallas, Texas,

USA, 1999

• American Board Certified in Pediatrics & Pediatric Nephrology

• Consultant Pediatric Nephrologist in Apollo Hospitals & Little Stars Children’s

Hospital, Hyderabad since past 19 years

• Special interest – Renal Tubular disorders

Antenatal Renal Anomalies:What do you need to know to counsel?

Mehul A. ShahM.D.(Ped.), DCH (Bom), M.D.(USA), DABPN (USA)

Consultant Pediatric NephrologistApollo Health City, &

Little Stars Children’s Hospital

Hyderabad

NEOCON, Hyderabad, 13 Dec 2019

Outline

1) Introduction

2) Hydronephrosis grading system

3) What is the outcome of AN HN?

4) Can we predict outcome?

5) When to worry?

6) When NOT to worry

IntroductionCAKUT: Congenital Anomalies of the Kidney & Urinary Tract

• CAKUT - 1970, Garrett et al.

[Garrett WJ, Aust NZ J Obstet Gynaecol 1970; 10: 7 – 9.]

• 0.2 – 1.7% of pregnancies

[Livera LN, BMJ 1989; 298: 1421.]

[Sanghvi KP, J Trop Pediatr 1998; 44: 235.]

[Shah MA, unpublished, FH, 2008]

• 20-30% all congenital anomalies

[Dugoff L, Prenat Diagn 2002; 22: 316.]

CAKUT

Dysgenesis of kidney Abn. in shape / position

1] Renal Agenesis 1] Ectopic 1] Hydronephrosis

2] Renal hypo-dysplasia* 2] Fusion anomalies 2] Hydroureteronephrosis*

3] MCDK - Horseshoe 3] Bladder (PUV)*

4] ARPKD* - Crossed fused 4] Patent Urachus

5] PTD*

* Risk for ESRD

Abn. Collecting system

Introduction

• Dilatation === Obstruction

• Dilatation -

• Dilatation – Does not specify function

Reflux

PUJO /

VUJO / PUV

Benign

Introduction

• Transient dilatation of the fetal urinary tract

– Delay in maturation

– Absence of fat

– Bladder distension

Antenatal Renal Anomalies

• Disadvantages:

» Over-workup

» Excessive parental anxiety

(75% anomalies are benign)

(25% significant)

• 2 risks should be avoided:

– Excessive treatment

– Delayed treatment

• The challenge is to identify the small group (25%) of babies who have

significant nephro-uropathies with out subjecting the majority (75%)

to unnecessary investigations and antibiotics

Significant nephro-uropathies

Significant nephro-uropathies

• Surgery and / or long term medical follow-up

• Morbidity:

- UTI, renal stones, hypertension, proteinuria, & CKD

• 36% of ESRD – CAKUT

[NAPRTCS Annual Report 2014]

Significant nephro-uropathies:2 major concerns

• ARPKD

• PUV (especially if

oligohydramnios)

How do we define hydronephrosis?

Hydronephrosis Grading SystemsQualitative: SFU (Ped Radiol 1993; 23: 478-480.)

Hydronephrosis Grading Systems:Blachar’s Quantitaive (Pediatr Nephrol 1994; 8: 30-35.)

• Based on renal pelvis AP diameter [RPAPD]:

– Normal, 0-4 mm

– Mild HN, 5-9 mm

– Moderate HN, 10-14 mm

– Severe HN, > 15 mm

• Limitations:

– Does not consider calyceal dilatation and parenchymal changes

(which reflects severe hydronephrosis)

Antenatal Hydronephrosis

• ANH is present when APRPD is > 5 mm at 20 weeks of gestation

and > 7 mm at 32 weeks

[Duncan KA. Clinical Radiology 2007; 62: 134 - 139.]

• Presence of calyceal dilatation at any stage is significant

[Grignon A. Radiology 1986; 160: 645 – 647.]

Post natal hydronephrosis

• Threshold renal pelvis APD value that separates normal from

abnormal does not exist

– Most accept up to 5mm

Normal

– Some, up to 7 mm

What is the post-natal outcome of antenatal renal

anomalies?

Antenatal Renal anomalies:Postnatal Outcome, Summary

• Isolated / benign / transient hydronephrosis ~ 70%

• Significant nephro-uropathies ~ 30%

– PUJO

– VUR

– PUV

– MCDK

– ARPKD

– Ectopic / Horseshoe kidney

» Kitagawa, Fetal Diagn & Therap, Jan 1998

» Khalid Ismaili, J of Ped, June 2004

Fernandez Hospital Experience2008-2009

• Antenatal renal anomalies: 160 / 9500 (~ 1.7%)

• Isolated / Benign hydronephrosis: 60%

• Significant nephro-uropathies: 40%

• PUJO – 18%

• VUR – 10%

• PUV – 10%

• MCDK – 2%

• UTI: 3/160 (1.5%) - PUV

Can we predict the outcome based on antenatal

hydronephrosis?

Can we predict the outcome based on antenatal

hydronephrosis?

• Mild Hydronephrosis (RPAPD 5-9 mm):

– Isolated / benign in majority (80%)

– VUR (15%)

– PUV / PUJO / Others (< 5%)

• Moderate-Severe HN (RPAPD > 10mm):

– PUJO (25%)

– VUR (10%) 45-80% (higher with > 15 mm)

– PUV (10%)

» Richard S. Lee, Pediatrics, Aug 2006

» Bernardica Valent-Moric, Acta Clin Croat 2011; 50: 451-455

Antenatal renal anomalies: When to worry?

AN renal anomalies:When to worry?

1) Bilateral, enlarged, and echogenic kidneys s/o ARPKD / Infantile Nephronopthisis

2) Bilateral hydro-uretero-nephrosis or bilateral severe hydronephrosis (s/o PUV in boys)

3) Oligohydramnios

4) Other organ anomalies

~ 10-15% of

all cases

Autosomal Recessive Polycystic Kidney Disease:enlarged and echogenic kidneys, oligohydramnios

Posterior Urethral

Valves (PUV)

• Most serious and fortunately less common of the antenatal renal anomalies-

~30% risk of chronic kidney disease / failure

• During the antenatal period, PUV can be suggested by

• Bilateral severe HN / HUN

• Echogenic renal cortex

• Distended bladder

• Decreased amniotic fluid

• Amniotic fluid index remains one of the most important predictor of long term renal outcome in PUV

[decreased AFI ie < 5 cms = 90% risk of postnatal CKD]

[Zaccara A. J Urol 2005; 174: 1657 – 1660.]

Antenatal renal anomalies: When NOT to worry?

AN renal anomalies:When NOT to worry?

• Transient / benign HN (RPAPD <10 mm)

• Single kidney status

– Congenital absence of one kidney

– Unilateral multicystic dysplastic kidney

• PUJO (25-40% require surgery)

• VUR (25% require surgery)

~ 85% of

all cases

Case 1:

• 20 weeks antenatal scan

• Bilateral hydronephrosis, RPAPD 6 and 10 mm

• AF adequate

• Outcome:

Benign / Isolated – 75%

VUR – 10-15%

PUJO <10%

• Plan:

Reassure

Repeat antenatal scan at 32-34 weeks

Case 2:

• 28 weeks antenatal scan

• Right fetal severe hydronephrosis of 52 mm (normal < 5 mm)

• AF adequate

• Outcome: Likely right PUJO

• Plan:

Reassure

Post natal – serial USG at 1 week and 2 months

- EC or DTPA scan at 2-3 months,

- Pyeloplasty

• Prognosis: Good

Case 3:

• 20 weeks antenatal scan

• Bilateral enlarged and echogenic kidneys, 3.5 and 3.7 cms, No HN

• AF 10 cms

• Possibilities:

ARPKD

Proximal Tubular Dysgenesis

• Outcome:

Risk of progressive CKD high

• Plan:

Repeat antenatal scan after 2 weeks – MTP / continue pregnancy

Case 4:

• 32 weeks antenatal scan

• Bilateral hydro-ureteronephrosis (mild & severe)

• Possibilities: PUV, VUR, VUJO

• 3 scenarios:

a) Bladder thick walled with adequate AF – PUV (good outcome 80-90%)

b) Bladder thick walled with decreased AF (4 cms) – PUV (Poor outcome 90%)

c) Normal bladder and adequate AF – VUR (good outcome)

• Post natal – serial RFT monitoring, USG and MCUG in first 1-2 days (boys)

Case 5:

• TIFFA scan at 22 weeks

• Unilateral multi-cystic kidney with echogenic cortex

• Other kidney is normal and AF adequate

• Diagnosis: Unilateral multicystic dysplastic kidney (MCDK)

• Outcome: Excellent

• Long term prognosis: Normal quality & quantity of life

Summary

• Goals of screening are to identify babies with significant nephro-uropathies (25%) with out over-investigating the other babies (75%)

• AN Hydronephrosis – renal pelvis APD > 5 mm at 20 weeks and > 7 mm at 32 weeks

• 5-9 mm Mild; 10-14 mm Moderate; and > 15 mm Severe HN

• RPAPD 5-15 mm (10%), and >10 mm (45-80% significant uropathies)

• Majority of babies (90-95%) have good outcome –– transient / benign HN, single kidney, MCDK, ectopic kidney, PUJO, VUR

• The 2 major conditions that causes significant morbidity and mortality are – ARPKD and ~ 30% of boys with PUV (Low AF) – < 5% of all cases

Thank you