Advertisement

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

Chapter
Part of the Medical Radiology book series (MEDRAD)

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.

References

  1. Bellah RD, States LJ, Duckett JW (1996) Pseudoprune-belly syndrome: imaging findings and clinical outcome. Am J Roentgenol 167:1389–1393CrossRefGoogle Scholar
  2. 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 106:503–508CrossRefGoogle Scholar
  3. 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 1CrossRefPubMedPubMedCentralGoogle Scholar
  4. Burbige KA, Amodio J, Berdon WE et al (1987) Prune belly syndrome: 35 years of experience. J Urol 137:86–90CrossRefGoogle Scholar
  5. Cazorla E, Ruiz F, Abad A, Monleon J (1997) Prune belly syndrome: early antenatal diagnosis. Eur J Obstet Gynecol Reprod Biol 72:31–33CrossRefGoogle Scholar
  6. Eagle JF, Barrett GS (1950) Congenital deficiency of abdominal musculature with associated genitourinary abnormalities, a syndrome: reports of nine cases. Pediatrics 6:721–736PubMedGoogle Scholar
  7. Fontaine E, Salomon L, Gagnandoux MF et al (1997) Longterm results of renal transplantation in children with the prune belly syndrome. J Urol 158:892–894CrossRefGoogle Scholar
  8. Fusaro F, Zanon GF, Ferreli AM et al (2004) Renal transplantation in prune-belly syndrome. Transpl Int 17:549–752CrossRefGoogle Scholar
  9. Grattan-Smith JD, Jones RA (2006) MR urography in children. Pediatr Radiol 36:1119–1132CrossRefGoogle Scholar
  10. Greskovich FJ, Nyberg LM (1988) The prune belly syndrome: a review of its etiology, defects, treatment and prognosis. J Urol 140:707–712CrossRefGoogle Scholar
  11. 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 12:362–366CrossRefGoogle Scholar
  12. Mandell J, Peters CA, Estroff JA, Benacerraf BR (1992) Late onset severe oligohydramnios associated with genitourinary abnormalities. J Urol 148:515–518CrossRefGoogle Scholar
  13. 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 73:739–742PubMedGoogle Scholar
  14. Nolte-Ernsting CC, Staatz G, Tacke J, Gunther RW (2003) MR urography today. Abdom Imaging 28:191–209CrossRefGoogle Scholar
  15. Osler W (1901) Congenital absence of the abdominal muscles with distended and hypertrophied urinary bladder. Bull Johns Hopkins Hosp 12:331–333Google Scholar
  16. Parker RW (1895) Absence of abdominal muscle in an infant. Lancet 1:1252–1254Google Scholar
  17. 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 11:1–29CrossRefGoogle Scholar
  18. Potter EL (1972) Normal and abnormal development of the kidney. Year Book, ChicagoGoogle Scholar
  19. Ramasamy R, Haviland M, Woodard JR, BArone JG (2005) Patterns of inheritance in familial prune belly syndrome. Urology 65:1227–1228CrossRefGoogle Scholar
  20. 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 2470–2494Google Scholar
  21. 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 118:395–398CrossRefGoogle Scholar
  22. 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 22:79–87PubMedGoogle Scholar
  23. 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 12:1442–1450CrossRefGoogle Scholar
  24. Rohrmann D, Duckett JW (2011) Prune-belly-Syndrom. In: Stein R, Beetz R, Thüroff JW (eds) Kinderurologie in Klinik und Praxis. Thieme, Stuttgart, pp 395–405Google Scholar
  25. 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 224:683–694CrossRefGoogle Scholar
  26. 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 CrossRefPubMedGoogle Scholar
  27. Sigel A, Rösch W (1993) Prune-belly-(“Pflaumenbauch”) syndrom. In: Sigel A (ed) Kinderurologie. Springer, Berlin Heidelberg, pp 107–114CrossRefGoogle Scholar
  28. Stephens FD, Gupta D (1994) Pathogenesis of the prune belly syndrome. J Urol 152:2328–2331CrossRefGoogle Scholar
  29. Wheatley JM, Stephens FD, Hutson JM (1996) Prune-belly syndrome: ongoing controversies regarding pathogenesis and management. Semin Pediatr Surg 5:95–106PubMedGoogle Scholar
  30. Williams DI (1982) Prune belly syndrome. In: Williams DI, Johnston JH (eds) Paediatric urology. Butterworths, London, pp 289–297Google Scholar
  31. Workman SJ, Kogan BA (1990) Fetal bladder histology in posterior urethral valves and the prune belly syndrome. J Urol 144:337–339CrossRefGoogle Scholar
  32. Yamamoto H, Nishikawa S, Hayashi T et al (2001) Antenatal diagnosis of prune belly syndrome at 11 weeks of gestation. J Obstet Gynaecol Res 27:37–40CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Radiology, Section of Pediatric RadiologyMedical Center of the Johannes Gutenberg UniversityMainzGermany
  2. 2.Kinder- und JugendklinikUniversitätsklinikum ErlangenErlangenGermany

Personalised recommendations