Prune Belly Syndrome

Katie Snider Novitski, MD, MPH, Andrei Radulescu, MD, Eric B Jelin, MD, Mitchell R. Ladd, MD, PhD, Kristen Meier, MD, Nathan M Novotny, MD
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Introduction

Prune belly syndrome (PBS) has been characterised by deficient or absent abdominal wall musculature, hypotonia, ectasia of the urinary system and bilateral intra-abdominal testes. Some of the most unusual and impressive megaureters are found in patients with prune belly syndrome - a term used by William Osler in 1901 to describe the appearance of the abdominal wall in patients with congenital deficiency of the abdominal wall musculature. The skin usually has an irregular wrinkled appearance similar to that of the prune. The condition has also been called the triad syndrome because of the three major features: deficiency of the abdominal wall muscle, hydroureteronephrosis and cryptorchidism[1][2].

PBS was initially reported by Frolich in 1839. In 1950, nine cases were reported by Eagle and Barrett who described the condition as Eagle-Barrett syndrome. Other synonyms used in the literature include triad syndrome and abdominal musculature deficiency syndrome [3][4][5].

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Introduction

Prune belly syndrome (PBS) has been characterised by deficient or absent abdominal wall musculature, hypotonia, ectasia of the urinary system and bilateral intra-abdominal testes. Some of the most unusual and impressive megaureters are found in patients with prune belly syndrome - a term used by William Osler in 1901 to describe the appearance of the abdominal wall in patients with congenital deficiency of the abdominal wall musculature. The skin usually has an irregular wrinkled appearance similar to that of the prune. The condition has also been called the triad syndrome because of the three major features: deficiency of the abdominal wall muscle, hydroureteronephrosis and cryptorchidism[1][2].

PBS was initially reported by Frolich in 1839. In 1950, nine cases were reported by Eagle and Barrett who described the condition as Eagle-Barrett syndrome. Other synonyms used in the literature include triad syndrome and abdominal musculature deficiency syndrome [3][4][5].

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Last updated: November 2, 2020