Skip to main content
SpringerLink
Log in

Imaging in Prune Belly Syndrome and Other Syndromes Affecting the Urogenital Tract

  • Chapter
  • First Online:
Pediatric Urogenital Radiology

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

  • 1160 Accesses

Abstract

The absence of the abdominal musculature, urinary tract dilatation, and bilateral undescended testis is known as prune belly syndrome (PBS) (Eagle and Barrett 1950; Greskovich and Nyberg 1988; Williams 1982). The classical syndrome is also known as triad syndrome, Eagle-Barrett syndrome, or abdominal muscular deficiency syndrome. There is a broad spectrum of malformations with severe dilatation of the urinary tract as a consequence of aplasia of the musculature. The pathogenetic mechanism is different from that of dilatation as a consequence of supra- or infravesical obstruction. Some patients with prune belly syndrome have a real obstruction, such as urethral aplasia with oligohydramnios syndrome. The prognosis of the malformations depends upon the degree of renal dysplasia (Rohrmann and Duckett 2011). There is no consensus as to the pathogenesis of this complex abnormality, although most investigators consider prune belly syndrome a distinct entity.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Bellah RD, States LJ, Duckett JW (1996) Pseudoprune-belly syndrome: imaging findings and clinical outcome. Am J Roentgenol 167:1389–1393

    Article  CAS  Google Scholar 

  • Biard JM, Johnson MP, Carr MC et al (2005) Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction. Obstet Gynecol 106:503–508

    Article  PubMed  Google Scholar 

  • Boss A, Martirosian P, Fuchs J, Obermayer F, Tsiflikas I, Schick F, Schäfer JF (2014) Dynamic MR urography in children with uropathic disease with a combined 2D and 3D acquisition protocol-comparison with MAG3 scintigraphy. Br J Radiol 87(1044):20140426. doi:10.1259/bjr.20140426, Epub 2014 Oct 1

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  • Burbige KA, Amodio J, Berdon WE et al (1987) Prune belly syndrome: 35 years of experience. J Urol 137:86–90

    Article  CAS  PubMed  Google Scholar 

  • Cazorla E, Ruiz F, Abad A, Monleon J (1997) Prune belly syndrome: early antenatal diagnosis. Eur J Obstet Gynecol Reprod Biol 72:31–33

    Article  CAS  PubMed  Google Scholar 

  • Eagle JF, Barrett GS (1950) Congenital deficiency of abdominal musculature with associated genitourinary abnormalities, a syndrome: reports of nine cases. Pediatrics 6:721–736

    PubMed  Google Scholar 

  • Fontaine E, Salomon L, Gagnandoux MF et al (1997) Longterm results of renal transplantation in children with the prune belly syndrome. J Urol 158:892–894

    Article  CAS  PubMed  Google Scholar 

  • Fusaro F, Zanon GF, Ferreli AM et al (2004) Renal transplantation in prune-belly syndrome. Transpl Int 17:549–752

    Article  PubMed  Google Scholar 

  • Grattan-Smith JD, Jones RA (2006) MR urography in children. Pediatr Radiol 36:1119–1132

    Article  PubMed  Google Scholar 

  • Greskovich FJ, Nyberg LM (1988) The prune belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol 140:707–712

    Article  PubMed  Google Scholar 

  • Hoshino T, Ihara Y, Shirane H, Ota T (1998) Prenatal diagnosis of prune belly syndrome at 12 weeks of pregnancy: case report and review of the literature. Ultrasound Obstet Gynecol 12:362–366

    Article  CAS  PubMed  Google Scholar 

  • Mandell J, Peters CA, Estroff JA, Benacerraf BR (1992) Late onset severe oligohydramnios associated with genitourinary abnormalities. J Urol 148:515–518

    Article  CAS  PubMed  Google Scholar 

  • Moore TR, Longo J, Leopold GR et al (1989) The reliability and predictive value of an amniotic fluid scoring system in severe second-trimester oligohydramnios. Obstet Gynecol 73:739–742

    PubMed  CAS  Google Scholar 

  • Nolte-Ernsting CC, Staatz G, Tacke J, Gunther RW (2003) MR urography today. Abdom Imaging 28:191–209

    Article  CAS  PubMed  Google Scholar 

  • Osler W (1901) Congenital absence of the abdominal muscles with distended and hypertrophied urinary bladder. Bull Johns Hopkins Hosp 12:331–333

    Google Scholar 

  • Parker RW (1895) Absence of abdominal muscle in an infant. Lancet 1:1252–1254

    Google Scholar 

  • Popek EJ, Tyson RW, Miller GJ, Caldwell SA (1991) Prostate development in prune belly syndrome (PBS) and posterior urethral valves (PUV): etiology of PBS – lower urinary tract obstruction or mesenchymal defect? Pediatr Pathol 11:1–29

    Article  CAS  PubMed  Google Scholar 

  • Potter EL (1972) Normal and abnormal development of the kidney. Year Book, Chicago

    Google Scholar 

  • Ramasamy R, Haviland M, Woodard JR, BArone JG (2005) Patterns of inheritance in familial prune belly syndrome. Urology 65:1227–1228

    Article  PubMed  Google Scholar 

  • Rascher W, Rösch W (2005) Congenital anomalies of the urinary tract. In: Davison AM, Cameron JS, Grünfeld JP, Ponticelli C, Ritz E, Winearls CG, van Ypersele C (eds) Oxford textbook of clinical nephrology, 3rd edn. Oxford University Press, Oxford, pp 2470–2494

    Google Scholar 

  • Reinberg Y, Shapiro E, Manivel JC et al (1991) Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital system. J Pediatr 118:395–398

    Article  CAS  PubMed  Google Scholar 

  • Riccabona M (2004) Pediatric MRU-its potential and its role in the diagnostic work-up of upper urinary tract dilatation in infants and children. World J Urol 22:79–87

    PubMed  Google Scholar 

  • Riccabona M, Simbrunner J, Ring E et al (2002) Feasibility of MR urography in neonates and infants with anomalies of the upper urinary tract. Eur Radiol 12:1442–1450

    Article  CAS  PubMed  Google Scholar 

  • Rohrmann D, Duckett JW (2011) Prune-belly-Syndrom. In: Stein R, Beetz R, Thüroff JW (eds) Kinderurologie in Klinik und Praxis. Thieme, Stuttgart, pp 395–405

    Google Scholar 

  • Rohrschneider WK, Haufe S, Wiesel M et al (2002) Functional and morphologic evaluation of congenital urinary tract dilatation by using combined static-dynamic MR urography: findings in kidneys with a single collecting system. Radiology 224:683–694

    Article  PubMed  Google Scholar 

  • Seidel NE, Arlen AM, Smith EA, Kirsch AJ (2015) Clinical manifestations and management of prune-belly syndrome in a large contemporary pediatric population. Urology 85:211–215. doi:10.1016/j.urology.2014.09.029

    Article  PubMed  Google Scholar 

  • Sigel A, Rösch W (1993) Prune-belly-(“Pflaumenbauch”) syndrom. In: Sigel A (ed) Kinderurologie. Springer, Berlin Heidelberg, pp 107–114

    Chapter  Google Scholar 

  • Stephens FD, Gupta D (1994) Pathogenesis of the prune belly syndrome. J Urol 152:2328–2331

    Article  CAS  PubMed  Google Scholar 

  • Wheatley JM, Stephens FD, Hutson JM (1996) Prune-belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg 5:95–106

    PubMed  CAS  Google Scholar 

  • Williams DI (1982) Prune belly syndrome. In: Williams DI, Johnston JH (eds) Paediatric urology. Butterworths, London, pp 289–297

    Google Scholar 

  • Workman SJ, Kogan BA (1990) Fetal bladder histology in posterior urethral valves and the prune belly syndrome. J Urol 144:337–339

    Article  CAS  PubMed  Google Scholar 

  • Yamamoto H, Nishikawa S, Hayashi T et al (2001) Antenatal diagnosis of prune belly syndrome at 11 weeks of gestation. J Obstet Gynaecol Res 27:37–40

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Radiology, Section of Pediatric Radiology, Medical Center of the Johannes Gutenberg University, Mainz, Germany

    Gundula Staatz MD

  2. Kinder- und Jugendklinik, Universitätsklinikum Erlangen, Loschgestrasse 15, 91054, Erlangen, Germany

    Wolfgang Rascher MD

Authors
  1. Gundula Staatz MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Wolfgang Rascher MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gundula Staatz MD .

Editor information

Editors and Affiliations

  1. Division of Pediatric Radiology, University Hospital, LKH Graz, Graz, Austria

    Michael Riccabona

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Staatz, G., Rascher, W. (2018). Imaging in Prune Belly Syndrome and Other Syndromes Affecting the Urogenital Tract. In: Riccabona, M. (eds) Pediatric Urogenital Radiology. Medical Radiology(). Springer, Cham. https://doi.org/10.1007/978-3-319-39202-8_27

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-39202-8_27

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-39200-4

  • Online ISBN: 978-3-319-39202-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation