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Imaging Case Book

Prune belly syndrome

Journal of Perinatology volume 29, pages 69–71 (2009)Cite this article

The abdominal wall, renal, bladder and urethral abnormalities in this infant are those of prune belly syndrome (PBS). PBS is characterized by a triad of findings (and is sometimes therefore called triad syndrome). These include the following: (a) deficient abdominal musculature, (b) urinary tract anomalies with megacystis and megaureters and (c) cryptorchidism.1 The disease occurs in approximately 1 in 50 000 live births and 95% of patients are male. Most cases are sporadic, although rarely familial cases have been described. The skin of the anterior abdominal wall is wrinkled, thin and lax, which accounts for the prune-like appearance and the name of the syndrome. The anterior abdominal wall muscles are absent in 30% of cases, replaced by dense collagenous tissue and hypoplastic with areas of absence in 70%.2 The genitourinary anomalies consist primarily of renal hypoplasia, dysplasia, ureteral dilatation, megacystis, dilated prostatic urethra and prostatic hypoplasia.1 Variable degrees of renal dysplasia occur ranging from severe dysplasia with cartilage metaplasia to mild cystic dysplasia. The ureters are usually dilated, ectatic and thick-walled. There may be focal areas of ureteral stenosis and gigantism. The ureterovesical junction is usually widely patent and vesicoureteral reflux occurs in 75% of patients.1 The urinary bladder is enlarged, often thickened, but non-trabeculated. Between 25 and 50% of patients have either a patent urachus or urachal diverticulum. The prostatic urethra is dilated with a small verumontanum, and urethral obstruction is an associated finding reported in 0–100% of patients. Cryptorchidism is present in 95% of patients. Obviously, the entire triad is not present in female patients. In reviews of female patients with PBS, all patients have had urethral atresia,2, 3 which has increased interest that urethral obstructions are the underlying etiology of PBS. Calcifications occur in the bladder dome and urachus in some patients with PBS, which are dystrophic, possibly related to bladder distention.4

Although, over the years, many theories have been suggested to explain PBS, including persistent abnormal bladder fixation at the umbilicus, aganglionosis and a genetic cause—x-linked or sex-modified autosomal-recessive condition1—these theories have not been confirmed by histological examination, fetal sonography or twin studies. Currently, the most frequently discussed etiologies of PBS include urethral obstruction, mesodermal defect and abnormalities of the allantois. The latter theory is not widely held but suggests that the allantois is abnormally large and abnormally incorporated into the abdominal wall. This theory may be supported, however, by the very slight increased frequency of omphalocele in PBS patients.5 The mesodermal defect theory implies that a noxious event to the mesoderm prevents normal development of the abdominal muscles and urinary tract muscles. The theory of urethral obstruction is now most widely held.1, 6 This theory suggests that urethral obstruction at a certain period of fetal life leads to distention of the urinary system, urinary ascites and abdominal distention with degeneration of the abdominal muscles.1 The dilated bladder presents normal testicular descent. It is most widely believed that bilateral fetal obstructive uropathy leads to PBS, especially owing to urethral obstruction at the junction of the anterior and posterior urethra,4 but which may have been relieved by the time of delivery. This relief of presumably high-pressure urinary tract obstruction in utero explains why low pressure, non-obstructed urinary tract systems are often found in newborns with PBS.1 Fetal sonographic examinations have provided additional evidence that patients with PBS had ascites and abdominal distention in utero.7

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  1. Department of Radiology, Mallinckrodt Institute of Radiology, St Louis Children's Hospital, Washington University School of Medicine, St Louis, MO, USA

    T E Herman & M J Siegel

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  1. T E Herman
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Correspondence to T E Herman.

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Herman, T., Siegel, M. Prune belly syndrome. J Perinatol 29, 69–71 (2009). https://doi.org/10.1038/jp.2008.88

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