Skip to main content
SpringerLink
Log in

The prune-belly syndrome: current insights

  • Urological Review
  • Published:
Pediatric Nephrology Aims and scope Submit manuscript

Abstract

The prune-belly syndrome comprises a constellation of well-established physical findings, yet the cause and management remain controversial. This review focuses on the current understanding of its pathogenesis and characterizes the fetal and neonatal diagnosis and management. Other associated anomalies are discussed to understand better the factors affecting treatment and prognosis as these patients grow into childhood and beyond.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Platt WB (1898) Rare case of deficiency of the abdominal muscles. Philadelphia Med J 1: 738–739

    Google Scholar 

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

    Google Scholar 

  3. Nunn IN, Stephens FD (1961) The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J Urol 86: 782–794

    Google Scholar 

  4. Ives EJ (1974) The abdominal muscle deficiency triad syndrome-experience with ten cases. Birth Defects 10: 127–135

    Google Scholar 

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

    Google Scholar 

  6. Williams DI, Burkholder GV (1967) The prune belly syndrome. J Urol 98: 244–251

    Google Scholar 

  7. Rich MA, Duckett JW (1992) The prune belly syndrome. AUA Update Series, vol XI, lesson 17, AUA Office of Education, Dallas, TX, p 130

    Google Scholar 

  8. Skoog SJ (1992) Prune belly syndrome. In: Kelalis PP, King LR, Belman BA (eds), Clinical pediatric urology, vol 2, 3rd edn. Saunders, Philadelphia, p 953

    Google Scholar 

  9. Burbige KA, Amodio J, Bernon WE, Hensle TW, Blanc W, Lattimer JK (1987) Prune belly syndrome: 35 years of experience. J Urol 137: 86–90

    Google Scholar 

  10. Snow BJ, Duckett JW (1992) Prune belly syndrome. In: Gillenwater JY (ed) Adult and pediatric urology, Mosby-Year Book, St. Louis, Mo., pp. 1921–1938

    Google Scholar 

  11. Williams DI (1982) The prune belly syndrome. In: Williams DI, Johnston JH (eds) Paediatric urology, 2nd edn. Butterworth, London, pp 289–298

    Google Scholar 

  12. Rabinowitz R, Schillinger JF (1977) Prune belly syndrome in the female subject. J Urol 118: 454–456

    Google Scholar 

  13. Reinberg Y, Shapiro E, Manivel JC, Manley CB, Pettinato G, Gonzalez R (1991) Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital systems. J Pediatr 118: 395–398

    Google Scholar 

  14. Lubinsky M, Rapoport P (1983) Transient fetal hydrops and “prune belly” in one identical female twin. N Engl J Med 308: 256–257

    Google Scholar 

  15. Aronson IA, Cremin BJ (1979) Prune belly syndrome in young females. Urol Radiol 1: 151–155

    Google Scholar 

  16. Garlinger P, Ott J (1974) Prune belly syndrome. Possible genetic implications. Birth Defects 10: 173–180

    Google Scholar 

  17. Adeyokunnu AA, Familusi JB (1982) Prune belly syndrome in two siblings and a first cousin. Possible genetic implications. Am J Dis Child 136: 23–25

    Google Scholar 

  18. Gonzalez R, Reinberg Y, Burke B, Wells T, Vernier RL (1990) Early bladder outlet obstruction in fetal lambs induces renal dysplasia and the prune-belly syndrome. J Pediatr Surg 25: 342–345

    Google Scholar 

  19. Stumme EG (1903) Über die symmetrischen kongenitalen Bauchmuskeldefekte und über die Kombination derselben mit anderen Bildungsanomalien des Rumpfes. Mitt Grennzigeb Med Chir 11: 548

    Google Scholar 

  20. Nakayama DK, Harrison MR, Chinn DH, Lorimier AA de (1984) The pathogenesis of prune belly. Am J Dis Child 138: 834–836

    Google Scholar 

  21. Palmer JM, Tesluck H (1974) Ureteral pathology in the prune belly syndrome. J Urol 111: 701–707

    Google Scholar 

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

    Google Scholar 

  23. 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 primary mesenchymal defect? Pediatr Pathol 11: 1–29

    Google Scholar 

  24. Burke EC, Shin MH, Kelalis PP (1969) Prune belly syndrome. Clinical findings and survival. Am J Dis Child 117: 668–771

    Google Scholar 

  25. Reinberg Y, Manivel JC, Pettinato G, Gonzalez R (1991) Development of renal failure in children with the prune belly syndrome. J Urol 145: 1017–1019

    Google Scholar 

  26. Geary DE, MacLusky IB, Churchill BM, McLorie G (1986) A broader spectrum of abnormalities in the prune belly syndrome. J Urol 135: 324–326

    Google Scholar 

  27. Fallat ME, Skoog SJ, Belman AB, Eng G, Randolph JG (1989) The prune belly syndrome: a comprehensive approach to management. J Urol 142: 802–805

    Google Scholar 

  28. Ehrlich RM, Brown WJ (1977) Ultrastructural anatomic observations of the ureter in the prune belly syndrome. Birth Defects 13: 101–103

    Google Scholar 

  29. Snyder HM, Harrison NW, Whitfield HN, Williams DI (1976) Urodynamics in the prune belly syndrome. Br J Urol 48: 663–670

    Google Scholar 

  30. Kinahan TJ, Churchill BM, McLorie GA, Gilmour RE, Khoury AE (1992) The efficiency of bladder emptying in the prune belly syndrome. J Urol 148: 600–603

    Google Scholar 

  31. Bukowski TP, Perlmutter AD (1994) Reduction cystoplasty in the prune belly syndrome: a long-term followup. J Urol 152: 2113–2116

    Google Scholar 

  32. Stephens FD (1983) Triad (prune belly) syndrome. Congenital malformations of the urinary tract. Praeger, New York, pp 485–511

    Google Scholar 

  33. Shrom SH, Cromie WJ, Duckett JW Jr (1981) Megalouretha. Urology 17: 152–156

    Google Scholar 

  34. Hutson JM, Beasley SW (1987) Aetiology of prune belly syndrome. Austral Ped J 23: 309–310

    Google Scholar 

  35. Elder JS, Isaacs JT, Walsh PC (1982) Androgenic sensitivity of the gubernaculum testis: evidence for hormonal/mechanical interactions in testicular descent. J Urol 127: 170–176

    Google Scholar 

  36. Frey HL, Rajfer J (1984) Role of the gubernaculum and intraabdominal pressure in the process of testicular descent. J Urol 131: 1574–1579

    Google Scholar 

  37. Uehling DT, Zadina SP, Gilbert E (1984) Testicular histology in the triad syndrome. Urology 23: 364–366

    Google Scholar 

  38. Orvis BR, Bottles K, Kogan BA (1988) Testicular histology in fetuses with the prune belly syndrome and posterior urethral valves. J Urol 139: 335–337

    Google Scholar 

  39. Massad CA, Cohen MB, Kogan BA, Beckstead JH (1991) Morphology and histochemistry of infant testes in the prune belly syndrome. J Urol 146: 1598–1600

    Google Scholar 

  40. Woodhouse CR, Ransley PG (1983) Teratoma of the testis in the prune belly syndrome. Br J Urol 55: 580–581

    Google Scholar 

  41. Sayre R, Stephens R, Chonko AM (1986) Prune belly syndrome and retroperitoneal germ cell tumor. Am J Med 81: 895–897

    Google Scholar 

  42. Woodhouse CR, Snyder HM III (1985) Testicular and sexual function in adults with prune belly syndrome. J Urol 133: 607–609

    Google Scholar 

  43. Housden LG (1934) Congenital absence of the abdominal muscles. Arch Dis Child 9: 219–232

    Google Scholar 

  44. Mininberg DT, Montoya F, Okada K, Galioto F, Presutti R (1973) Subcellular muscle studies in the prune belly syndrome. J Urol 109: 524–526

    Google Scholar 

  45. Holder JP (1989) Pathophysiologic and anesthetic correlations of the prune-belly syndrome. AANA J 57: 137–141

    Google Scholar 

  46. Adebonojo FO (1973) Dysplasia of the anterior abdominal musculature with multiple congenital anomalies. Prune belly or triad syndrome. J Natl Med Assoc 65: 327–333

    Google Scholar 

  47. Wright JR Jr, Barth RF, Neff JC, Poe ET, Sucheston ME, Stempel LE (1986) Gastrointestinal malformations associated with prune belly syndrome: three cases and a review of the literature. Pediatr Pathol 5: 421–448

    Google Scholar 

  48. Shimizu T, Ihara Y, Yomura W, Ando N, Nishimura R (1992) Antenatal diagnosis of prune belly syndrome. Arch Gynecol Obstet 251: 211–214

    Google Scholar 

  49. Cullen MT, Athanassiadis AP, Grannum P, Green JJ, Hobbins JC (1989) In utero intravesicular pressure and the prune belly syndrome. Fetal Ther 4: 73–77

    Google Scholar 

  50. Elder JS, Duckett JW, Jr., Snyder HM (1987) Intervention for fetal obstructive uropathy: has it been effective? Lancet II: 1007–1010

    Google Scholar 

  51. Clarke NW, Gough DC, Cohen SJ (1989) Neonatal urological ultrasound: diagnostic inaccuracies and pitfalls. Arch Dis Child 64: 578–580

    Google Scholar 

  52. Harrison MR, Golbus MS, Filly RA, Callen PW, Katz M, Lorimier AA de, Rosen M, Jonsen AR (1982) Fetal surgery for congenital hydronephrosis. N Engl J Med 306: 591–593

    Google Scholar 

  53. Colodny A (1987) Antenatal diagnosis and management of urinary abnormalities. Pediatr Clin North Am 34: 1365–1381

    Google Scholar 

  54. Kramer SA (1983) Current status of fetal intervention for congenital hydronephrosis. J Urol 130: 641–646

    Google Scholar 

  55. Schwartz GJ, Feld LG, Langford DJ (1984) A simple estimate of glomerular filtration rate in full-term infants during the first year of life. J Pediatr 104: 849–854

    Google Scholar 

  56. Duckett JW, Knutrud O, Hohenfellner R (1980) Prune belly syndrome. Dial Pediatr Urol 3: 1–8

    Google Scholar 

  57. Rushton HG, Majd M, Chandra R, Yim D (1988) Evaluation of 99m-technetium dimercapto-succinic acid renal scans in experimental acute pyelonephritis in piglets. J Urol 140: 1169–1174

    Google Scholar 

  58. Woodard JR, Trulock TS (1986) Prune belly syndrome. In: Walsh PC et al (eds) Campbell's urology, 5th edn. Saunders, Phia, pp 2159–2167

    Google Scholar 

  59. Randolph J, Cavett C, Eng G (1981) Abdominal wall reconstruction in the prune belly syndrome. J Pediatr Surg 16: 960–964

    Google Scholar 

  60. Woodard JR, Parrott TS (1978) Orchiopexy in the prune belly syndrome. Br J Urol 50: 348–351

    Google Scholar 

  61. Woodhouse CR, Kellett MJ, Williams DI (1979) Minimal surgical interference in the prune belly syndrome. Br J Urol 51: 475–480

    Google Scholar 

  62. Snyder HM, Harrison NW, Whitfield HG, Williams DI (1976) Urodynamics in the prune belly syndrome. Br J Urol 48: 663–670

    Google Scholar 

  63. Culp DA, Flocks RH (1954) Congenital absence of abdominal musculature. J Iowa State Med Soc 44: 155–159

    Google Scholar 

  64. Woodard JR, Parrott TS (1978) Reconstruction of the urinary tract in prune belly uropathy. J Urol 119: 824–828

    Google Scholar 

  65. Perlmutter AD (1976) Reduction cystoplasty in prune belly syndrome. J Urol 116: 356–362

    Google Scholar 

  66. Reinberg Y, Manivel JC, Fryd D, Najarian JS, Gonzalez R (1989) The outcome of renal transplantation in children with the prune belly syndrome. J Urol 142: 1541–1542

    Google Scholar 

  67. Duckett JW, Snyder HM III (1986) Continent urinary diversion: variations on the Mitrofanoff principle. J Urol 136: 58–62

    Google Scholar 

  68. Dunn BR, Anderson S, Brenner BM (1986) The hemodynamic basis of progressive renal disease. Semin Nephrol 6: 122–138

    Google Scholar 

  69. Fowler R, Stephens FD (1959) The role of testicular vascular anatomy in the salvage of high undescended testis. Aust NZ J Surg 29: 92–106

    Google Scholar 

  70. Gibbons MD, Cromie WJ, Duckett JW (1979) Management of the abdominal undescended testicle. J Urol 122: 76–79

    Google Scholar 

  71. Ehrlich RM, Lesavoy MA, Fine RN (1986) Total abdominal wall reconstruction in the prune belly syndrome. J Urol 136: 282–285

    Google Scholar 

  72. Ehrlich RM, Lesavoy MA (1993) Umbilicus preservation with total abdominal wall reconstruction in prune belly syndrome. Urology 41: 231–232

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Urology, University of California School of Medicine, Box 0738, San Francisco, California, USA

    Ronald S. Sutherland, Robert A. Mevorach & Barry A. Kogan

Authors
  1. Ronald S. Sutherland
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Robert A. Mevorach
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Barry A. Kogan
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sutherland, R.S., Mevorach, R.A. & Kogan, B.A. The prune-belly syndrome: current insights. Pediatr Nephrol 9, 770–778 (1995). https://doi.org/10.1007/BF00868740

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00868740

Key words

Search

Navigation