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Radiology

Prune-Belly Syndrome

Built from Obstetric Imaging

The first 25 slides of Prune-Belly Syndrome
The first 25 slides, exactly as they appear. The full deck has 64 content slides.

What’s inside

8 sections · 64 slides

  1. 01

    Overview

    • Overview of prune-belly syndrome
    • Topics covered

    2 slides

  2. 02

    The disorder

    Definition, name, and how often it occurs

    • Definition of prune-belly syndrome
    • The classic triad
    • Typical urologic findings
    • Normal male urinary tract
    • Anomalies beyond the triad
    • Organ systems involved
    • 1:29,000
    • Prevalence and epidemiology

    8 slides

  3. 03

    Cause and mechanism

    Two theories, and the genes now under study

    • Two proposed origins
    • The obstructive uropathy theory
    • Sequence proposed by the obstruction theory
    • Limitations of the obstruction theory
    • The mesenchymal development theory
    • Environmental exposures studied
    • Evidence for a genetic contribution
    • MYOCD and the male excess
    • FLNA and the X chromosome
    • HNF1β and mesodermal transcription factors
    • Genes affecting muscle contraction
    • Findings of the genetic cohort studies
    • Recurrence risk counselling

    13 slides

  4. 04

    Manifestations of disease

    System by system, from belly wall to skeleton

    • Abdominal wall findings
    • Consequences of bladder distension
    • Bladder wall and ureteric openings
    • Urinary obstruction and retention
    • Vesicoureteric reflux on contrast cystography
    • Patent urachus
    • Significance of a patent urachus
    • Cryptorchidism in affected males
    • Presentation in affected females
    • Pulmonary consequences
    • Cardiovascular anomalies
    • Gastrointestinal anomalies
    • Musculoskeletal anomalies
    • Clubfoot in a newborn

    14 slides

  5. 05

    Prognosis

    Three groups, three very different outlooks

    • Woodard prognostic groups
    • Reading the prognostic groups

    2 slides

  6. 06

    Imaging technique and findings

    What the fetal ultrasound shows, and what mimics it

    • Timing and the leading ultrasound finding
    • Enlarged fetal bladder on transverse view
    • Hydronephrosis with a dilated right ureter
    • Interpreting the upper tract images
    • Amniotic fluid and other structures
    • Timing of a prenatal cryptorchidism diagnosis
    • Differential diagnosis from imaging findings

    7 slides

  7. 07

    Treatment options

    Before birth, after birth, and for life

    • Prenatal treatment: the vesicoamniotic shunt
    • Selecting candidates for shunting
    • Prenatal care pathway in suspected obstruction
    • Evidence from the randomised shunt trial
    • Other evidence on shunting
    • Postnatal management priorities
    • Surgical management
    • Long-term renal outcome

    8 slides

  8. 08

    Summary and sources

    What to act on, and where it comes from

    • What the referring physician needs to know
    • Key points
    • Suggested readings
    • References
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • References (continued)
    • Obstetric Imaging: Fetal Diagnosis and Care, 2nd Edition

    10 slides