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Prune Belly Syndrome
Transcript of Prune Belly Syndrome
August 12, 2012 Prune Belly
Syndrome To introduce the history and epidemiology regarding PBS
To briefly review normal urinary system development
To discuss the etiology and pathophysiology of PBS
To understand the diagnostic methods
To explore available treatment and management options Objectives Affects 1/30,000-40,000 live births Abdominal Muscle Deficiency Syndrome Congenital Absence of Abdominal Muscles Obrinsky Syndrome Prune Belly Syndrome Triad Syndrome Fröhlich Syndrome Eagle
Barrett Syndrome 1800 2012 1900 1839 Frölich describes Prune Belly Syndrome 1901 Osler publishes "Congenital Absence of the Abdominal Muscles with Distended and Hypertrophied Urinary Bladder" in the Johns Hopkins Hospital Bulletin Epidemiology Associated mortality rate is 20% (stillbirths) The Triad Abdominal Wall Deficiency Urinary Tract Anomalies Bilateral Cryptorchidism Presentation Approximately 95% of cases occur in males Contemporary Epidemiology and Characterization of Newborn Males with Prune Belly Syndrome Characterized as a genetic, congenital disorder the of the genitourinary system 30% die of renal failure or urosepsis within the first two years of life Remaining 50% have varying degrees of urinary pathology References Combined 2000, 2003, and 2006 KID databases
133 newborn male infants classified as PBS of a pooled total of 1,420,991 live male births
Adjusted incidence estimate of 3.76 PBS cases (95% CI 2.98-4.5) per 100,000 live male births Jonathan C. Routh, Lin Huang, Alan B. Retik, and Caleb P. Nelson