Fetal Urinary Tract Anomalies

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Fetal Urinary Tract Anomalies

• Prenatal diagnosis and management

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Goal To review an interesting case, and review
US findings and management of common fetal GU
anomalies

• And to stay awake

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Case PresentationKB40 yo G1Presents for CVS at
11 4/7 weekOBGYN history negPMH NCFH NC
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Initial US
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11w 4d
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11w 4d
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Normal NT
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KB

• CVS 46 XX
• FU scans showed worsening bladder dilation and at
  14 weeks IUFD

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KB
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12w 6d
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13w 6d
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14w 5d
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DE w/o comp

• Pathology identified cystic bladder
• No renal tissue found

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? Megacystis/ microcolon
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MRI ?

• We discuss a third-trimester diagnosis of
  Megacystis-microcolon-intestinal hypoperistalsis
  syndrome (MMIHS) using magnetic resonance imaging
  (MRI) and consider the benefits of MRI as a
  noninvasive imaging technique after routine
  ultrasonography reveals genitourinary pathology
  requiring further elucidation. MMIHS is a rare
  cause of functional gastrointestinal and
  genitourinary obstruction in newborns. Because of
  the poor prognosis of MMIHS, prenatal diagnosis
  is warranted for optimal prenatal counseling and
  postnatal treatment. Although MMIHS commonly
  presents on ultrasonography, the limitations of
  ultrasonography make definitive diagnosis
  difficult. However, MRI is safe, accurate, and
  can be used for early prenatal diagnoses of
  multisystem diseases

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Antenatal Hydronephrosis

• Pyelectasis (mild renal pelvis dilation)
• Pelviectasis

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Background

• Common - 0.6-4.6 of fetuses in 2nd tri
• (.06, British study, gt118,000 fetuses, gt5mm)
  (4.5, Belgian study, gt5000 fetuses, gt 4 mm.
• Can be seen as early as 12-14 weeks
• 2X more frequent in male fetuses

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Meta analysis

• 1678 fetuses of 104,572 women (1.6 percent)
• Used various criteria

Antenatal hydronephrosis as a predictor of
postnatal outcome a meta-analysis. Lee RS
Cendron M Kinnamon DD Nguyen HT Pediatrics.
2006 Aug118(2)586-93.
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Definition

• Various measurements proposed, most based on
  renal pelvis diameter, RPD (maximum AP diameter)
• Generally accepted upper limits of normal
• 2nd trimester gt 4.0 mm
• 3rd trimester gt 7.0 mm

Woodward, M, Frank, D. Postnatal management of
antenatal hydronephrosis. BJU Int 2002 89149.
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D DX pyelectasis
UPJ obstructionVesicoureteral reflux
(VUR)Primary nonrefluxing megaureterUreterocele
Ureterovesical junction (UVJ) obstructionEctopic
ureterPosterior urethral valvesMegacystis
megaureterPhysiologic dilatationMulticystic
dysplastic kidneyAutosomal recessive polycystic
kidney diseaseExstrophyPrune belly syndrome
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Society of Fetal Urology
Grade 0 no dilation. Grade 1 renal pelvis is
only visualized. Grade 2 renal pelvis as well
as a few, but not all, calyces are visualized.
Grade 3 virtually all calyces are visualized.
Grade 4 similar to Grade 3 but, when compared
to the normal contralateral kidney, there is
parenchymal thinning.
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SFU grading system
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Management Issues

• Anxiety
• Cost
• Sensitivity/ specificity
• Aneuploidy risk
• T21 18 have pelviectasis
• Euploid fetuses 0-3 (RPD 4 mm)
• LR

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Etiology

• Transient 48 percent
• Physiologic 15 percent
• UPJ obstruction 11 percent
• VUR 9 percent
• Megaureter 4 percent
• Multicystic dysplastic kidney 2 percent
• Ureterocele 2 percent
• Posterior urethral valves 1 percent

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Risk of significant post natal disease increases
with increasing RPD

• lt 7mm 2nd tri, or lt 9mm 3rd tri- 12
• 7-10mm 2nd tri or 9-15 mm 3rd tri- 45
• gt 10mm 2nd tri or gt 15mm 3rd tri- 88

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MRI
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Severe
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Severe, bilateral, enlarged bladder with keyhole
sign
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Other factors

• Severity of hydronephrosis
• Unilateral versus bilateral involvement
• Renal parenchyma
• Bladder
• Amniotic fluid

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Recommendations isolated pelviectasis

• If RPD gt/ 4.0 mm at 18-24 weeks,
• FU at 32-34 weeks for progression, if gt 7-9mm,
  offer Peds urology consult and recommend post
  natal FU.
• Discuss amniocentesis based on adjusted risk from
  LR and age, FTS, Quad, etc.

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Recommendations cont

• By comparison, serial follow-up ultrasounds are
  indicated for fetuses with
• Moderate or severe hydronephrosis
• Bilateral involvement
• Progression and/or persistence of hydronephrosis
• Oligohydramnios

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PUV/ BOO

• Although there have been case series of antenatal
  surgery in fetuses with severe hydronephrosis and
  oligohydramnios consistent with lower urinary
  tract obstruction, this intervention has not been
  shown to improve renal outcome. There remains a
  high rate of chronic renal disease in the
  survivors necessitating renal replacement therapy
  in almost two-thirds of the cases. These
  procedures may increase the amount of amniotic
  fluid, thus potentially improving lung
  development and survival rate.

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Treatment options
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Clinical trials
PLUTO trial protocol percutaneous shunting for
lower urinary tract obstruction randomised
controlled trial. Kilby M Khan K Morris K
Daniels J Gray R Magill L Martin B Thompson
P Alfirevic Z Kenny S Bower S Sturgiss S
Anumba D Mason G Tydeman G Soothill P
Brackley K Loughna P Cameron A Kumar S Bullen
P BJOG. 2007 Jul114(7)904-5, e1-4.
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PLUTO trial
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PLUTO trial
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Fetoscopy study
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Fetoscopy study
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Before/after fetoscopic surgery
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Percutaneous urethrostomy
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Presurgical evaluation of renal function
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Cystic renal disease

• MDK unilateral/ bilateral

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MDK
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AD polycystic kidney disease
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AR PKD with severe oligo
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Ovarian cyst
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