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Prune belly syndrome

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Abstract

The majority of paediatric surgeons will encounter a patient with prune belly syndrome (PBS) only a few times in their clinical practice. There have been many opposing views in the literature regarding the pathogenesis and management of this complex condition. A detailed review was conducted using PubMed to identify key publications involving PBS. This article discusses the evolution of our understanding of the pathogenesis and diagnosis of PBS, including its typical characteristics. We describe the management options available for bilateral intra-abdominal testes, the deficient abdominal wall, the dilated urinary system and examine the evidence base used to support the current approaches employed.

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References

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

    Google Scholar 

  2. Eagle JF Jr, Barrett GS (1950) Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: a syndrome. Report of 9 cases. Pediatrics 6:721–736

    PubMed  Google Scholar 

  3. Welch KJ, Kraney GP (1974) Abdominal musculature deficiency syndrome prune belly. J Urol 111:693–700

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  5. Smolkin T, Soudack M, Goldstein I, Sujov P, Makhoul IR (2008) Prune belly syndrome: expanding the phenotype. Clin Dysmorphol 17:133–135

    Article  PubMed  Google Scholar 

  6. Harbour R, Miller J (2001) A new system for grading recommendations in evidence based guidelines. BMJ 323:334–336

    Article  PubMed  CAS  Google Scholar 

  7. Routh JC, Huang L, Retik AB, Nelson CP (2010) Contemporary epidemiology and characterization of newborn males with prune belly syndrome. Urology 76:44–48

    Article  PubMed  Google Scholar 

  8. Druschel CM (1995) A descriptive study of prune belly in New York State, 1983 to 1989. Arch Pediatr Adolesc Med 149:70–76

    Article  PubMed  CAS  Google Scholar 

  9. Beckmann H, Rehder H, Rauskolb R (1984) Prune belly sequence associated with trisomy 13. Am J Med Genet 19:603–604

    Article  PubMed  CAS  Google Scholar 

  10. Frydman M, Magenis RE, Mohandas TK, Kaback MM (1983) Chromosome abnormalities in infants with prune belly anomaly: association with trisomy 18. Am J Med Genet 15:145–148

    Article  PubMed  CAS  Google Scholar 

  11. Baird PA, Sadovnick AD (1987) Prune belly anomaly in down syndrome. Am J Med Genet 26:747–748

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  13. Petersen DS, Fish L, Cass AS (1972) Twins with congenital deficiency of abdominal musculature. J Urol 107:670–672

    PubMed  CAS  Google Scholar 

  14. Balaji KC, Patil A, Townes PL, Primack W, Skare J, Hopkins T (2000) Concordant prune belly syndrome in monozygotic twins. Urology 55:949

    Article  PubMed  CAS  Google Scholar 

  15. Murray PJ, Thomas K, Mulgrew CJ, Ellard S, Edghill EL, Bingham C (2008) Whole gene deletion of the hepatocyte nuclear factor-1beta gene in a patient with the prune-belly syndrome. Nephrol Dial Transplant 23:2412–2415

    Article  PubMed  CAS  Google Scholar 

  16. Haeri S, Devers PL, Kaiser-Rogers KA, Moylan VJ Jr, Torchia BS, Horton AL, Wolfe HM, Aylsworth AS (2010) Deletion of hepatocyte nuclear factor-1-beta in an infant with prune belly syndrome. Am J Perinatol 27:559–563

    Article  PubMed  Google Scholar 

  17. Manivel JC, Pettinato G, Reinberg Y, Gonzalez R, Burke B, Dehner LP (1989) Prune belly syndrome: clinicopathologic study of 29 cases. Pediatr Pathol 9:691–711

    Article  PubMed  CAS  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

    Article  PubMed  CAS  Google Scholar 

  19. Hutson JM, Beasley SW (1987) Aetiology of the prune belly syndrome. Aust Paediatr J 23:309–310

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  21. Deklerk DP, Scott WW (1978) Prostatic maldevelopment in the prune belly syndrome: a defect in prostatic stromal-epithelial interaction. J Urol 120:341–344

    PubMed  CAS  Google Scholar 

  22. Ives EJ (1974) The abdominal muscle deficiency triad syndrome—experience with ten cases. Birth Defects Orig Artic Ser 10:127–135

    PubMed  CAS  Google Scholar 

  23. Papantoniou N, Papoutsis D, Daskalakis G, Chatzipapas I, Sindos M, Papaspyrou I, Mesogitis S, Antsaklis A (2010) Prenatal diagnosis of prune-belly syndrome at 13 weeks of gestation: case report and review of literature. J Matern Fetal Neonatal Med 23:1263–1267

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  25. Shigeta M, Nagata M, Shimoyamada H, Shibata S, Okuno S, Hamada H, Watanabe T (1999) Prune-belly syndrome diagnosed at 14 weeks’ gestation with severe urethral obstruction but normal kidneys. Pediatr Nephrol 13:135–137

    Article  PubMed  CAS  Google Scholar 

  26. Chen L, Cai A, Wang X, Wang B, Li J (2010) Two- and three-dimensional prenatal sonographic diagnosis of prune-belly syndrome. J Clin Ultrasound 38:279–282

    PubMed  Google Scholar 

  27. Sutherland RS, Mevorach RA, Kogan BA (1995) The prune-belly syndrome: current insights. Pediatr Nephrol 9:770–778

    Article  PubMed  CAS  Google Scholar 

  28. KA Woodward PJ, Soahey R, Byrne JLB, Oh KY, Puchalski MD (2005) Diagnostic imaging obstetrics. Amirsys Inc, Salt Lake City

    Google Scholar 

  29. Osborne NG, Bonilla-Musoles F, Machado LE, Raga F, Bonilla F Jr, Ruiz F, Perez Guardia CM, Ahluwalia B (2011) Fetal megacystis: differential diagnosis. J Ultrasound Med 30:833–841

    PubMed  Google Scholar 

  30. Austin JC, Canning DA, Johnson MP, Flake AW, Carr MC (2001) Vesicoamniotic shunt in a female fetus with the prune belly syndrome. J Urol 166:2382

    Article  PubMed  CAS  Google Scholar 

  31. Galati V, Beeson JH, Confer SD, Frimberger D, Campbell JB, Ramji FG, Kropp BP (2008) A favorable outcome following 32 vesicocentesis and amnioinfusion procedures in a fetus with severe prune belly syndrome. J Pediatr Urol 4:170–172

    Article  PubMed  Google Scholar 

  32. Leeners B, Sauer I, Schefels J, Cotarelo CL, Funk A (2000) Prune-belly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt. J Clin Ultrasound 28:500–507

    Article  PubMed  CAS  Google Scholar 

  33. Clark TJ, Martin WL, Divakaran TG, Whittle MJ, Kilby MD, Khan KS (2003) Prenatal bladder drainage in the management of fetal lower urinary tract obstruction: a systematic review and meta-analysis. Obstet Gynecol 102:367–382

    Article  PubMed  Google Scholar 

  34. Morris RK, Kilby MD (2009) An overview of the literature on congenital lower urinary tract obstruction and introduction to the PLUTO trial: percutaneous shunting in lower urinary tract obstruction. Aust N Z J Obstet Gynaecol 49:6–10

    Article  PubMed  Google Scholar 

  35. Woodard JR (1978) The prune belly syndrome. Urol Clin North Am 5:75–93

    PubMed  CAS  Google Scholar 

  36. Smith EA (2002) Prune belly syndrome. In: Wein AJ (ed) Campbells urology. WB Saunders, Philadelphia

    Google Scholar 

  37. 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

    Article  PubMed  Google Scholar 

  38. Green NE, Lowery ER, Thomas R (1993) Orthopaedic aspects of prune belly syndrome. J Pediatr Orthop 13:496–501

    Article  PubMed  CAS  Google Scholar 

  39. 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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  44. Kroovand RL, Al-Ansari RM, Perlmutter AD (1982) Urethral and genital malformations in prune belly syndrome. J Urol 127:94–96

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  46. Woodard JR, Zucker I (1990) Current management of the dilated urinary tract in prune belly syndrome. Urol Clin North Am 17:407–418

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  49. McMullin N, Hutson J, Kelly J (1988) Minimal surgery in the prune belly syndrome. Pediatr Surg Int 3:51–54

    Article  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  51. Denes FT, Arap MA, Giron AM, Silva FA, Arap S (2004) Comprehensive surgical treatment of prune belly syndrome: 17 years’ experience with 32 patients. Urology 64:789–793 discussion 793–784

    Article  PubMed  Google Scholar 

  52. Caldamine A, Woodard JR (2011) Campbell-Walsh Urology, 10th edn, vol 4. Prune Belly Syndrome. Elsevier Saunders, Philadelphia

  53. Singh-Grewal D, Macdessi J, Craig J (2005) Circumcision for the prevention of urinary tract infection in boys: a systematic review of randomised trials and observational studies. Arch Dis Child 90:853–858

    Article  PubMed  CAS  Google Scholar 

  54. Chen IL, Huang HC, Lee SY, Liu CA, Tain YL, Ou-Yang MC, Chao PH (2011) Urachal catheter provides new choice for long-term urinary diversion in prune belly syndrome. Urology 77:466–468

    Article  PubMed  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  56. Afifi AK, Rebeiz J, Mire J, Andonian J, VMd Kaloustian (1972) The myopathology of the Prune belly syndrome. J Neurol Sci 15:153–165

    Article  PubMed  CAS  Google Scholar 

  57. Smith CA, Smith EA, Parrott TS, Broecker BH, Woodard JR (1998) Voiding function in patients with the prune-belly syndrome after Monfort abdominoplasty. J Urol 159:1675–1679

    Article  PubMed  CAS  Google Scholar 

  58. Randolph J, Cavett C, Eng G (1981) Surgical correction and rehabilitation for children with “Prune-belly” syndrome. Ann Surg 193:757–762

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  60. Monfort G, Guys JM, Bocciardi A, Coquet M, Chevallier D (1991) A novel technique for reconstruction of the abdominal wall in the prune belly syndrome. J Urol 146:639–640

    PubMed  CAS  Google Scholar 

  61. Furness PD III, Cheng EY, Franco I, Firlit CF (1998) The prune-belly syndrome: a new and simplified technique of abdominal wall reconstruction. J Urol 160:1195–1197 discussion 1216

    Article  PubMed  Google Scholar 

  62. Franco I (2005) Laparoscopic assisted modification of the firlit abdominal wall plication. J Urol 174:280–283

    Article  PubMed  Google Scholar 

  63. Crompton CH, MacLusky IB, Geary DF (1993) Respiratory function in the prune-belly syndrome. Arch Dis Child 68:505–506

    Article  PubMed  CAS  Google Scholar 

  64. Woodard JR (2003) Prune-belly syndrome: a personal learning experience. BJU Int 92(Suppl 1):10–11

    Article  PubMed  Google Scholar 

  65. Woodhouse CR, Ransley PG, Innes-Williams D (1982) Prune belly syndrome–report of 47 cases. Arch Dis Child 57:856–859

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  67. 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

    PubMed  CAS  Google Scholar 

  68. 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

    PubMed  CAS  Google Scholar 

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

    PubMed  CAS  Google Scholar 

  70. Elyas R, Guerra LA, Pike J, DeCarli C, Betolli M, Bass J, Chou S, Sweeney B, Rubin S, Barrowman N, Moher D, Leonard M (2010) Is staging beneficial for Fowler-Stephens orchiopexy? A systematic review. The Journal of urology 183:2012–2018

    Article  PubMed  CAS  Google Scholar 

  71. Baker LA, Docimo SG, Surer I, Peters C, Cisek L, Diamond DA, Caldamone A, Koyle M, Strand W, Moore R, Mevorach R, Brady J, Jordan G, Erhard M, Franco I (2001) A multi-institutional analysis of laparoscopic orchidopexy. BJU Int 87:484–489

    Article  PubMed  CAS  Google Scholar 

  72. Kirsch AJ, Escala J, Duckett JW, Smith GH, Zderic SA, Canning DA, Snyder HM 3rd (1998) Surgical management of the nonpalpable testis: the Children’s Hospital of Philadelphia experience. J Urol 159:1340–1343

    Article  PubMed  CAS  Google Scholar 

  73. Abolyosr A (2006) Laparoscopic versus open orchiopexy in the management of abdominal testis: a descriptive study. Int J Urol 13:1421–1424

    Article  PubMed  Google Scholar 

  74. Lindgren BW, Franco I, Blick S, Levitt SB, Brock WA, Palmer LS, Friedman SC, Reda EF (1999) Laparoscopic Fowler-Stephens orchiopexy for the high abdominal testis. The Journal of urology 162:990–993 discussion 994

    Article  PubMed  CAS  Google Scholar 

  75. Patil KK, Duffy PG, Woodhouse CR, Ransley PG (2004) Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome. J Urol 171:1666–1669

    Article  PubMed  CAS  Google Scholar 

  76. Saxena AK, Brinkmann OA (2007) Unique features of prune belly syndrome in laparoscopic surgery. J Am Coll Surg 205:217–221

    Article  PubMed  Google Scholar 

  77. Philip J, Mullassery D, Craigie RJ, Manikandan R, Kenny SE (2011) Laparoscopic orchidopexy in boys with prune belly syndrome-outcome and technical considerations. J Endourol 25:1115–1117

    Article  PubMed  Google Scholar 

  78. Docimo SG, Moore RG, Kavoussi LR (1995) Laparoscopic orchidopexy in the prune belly syndrome: a case report and review of the literature. Urology 45:679–681

    Article  PubMed  CAS  Google Scholar 

  79. Hutson JM, Balic A, Nation T, Southwell B (2010) Cryptorchidism. Semin Pediatr Surg 19:215–224

    Article  PubMed  Google Scholar 

  80. Noh PH, Cooper CS, Winkler AC, Zderic SA, Snyder HM 3rd, Canning DA (1999) Prognostic factors for long-term renal function in boys with the prune-belly syndrome. J Urol 162:1399–1401

    Article  PubMed  CAS  Google Scholar 

  81. 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

    PubMed  CAS  Google Scholar 

  82. 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

    PubMed  CAS  Google Scholar 

  83. Fusaro F, Zanon GF, Ferreli AM, Giuliani S, Zacchello G, Passerini-Glazel G, Rigamonti W (2004) Renal transplantation in prune-belly syndrome. Transpl Int 17:549–552

    Article  PubMed  Google Scholar 

  84. Kamel MH, Thomas AA, Al-Mufarrej FM, O’Kelly P, Hickey DP (2007) Deceased-donor kidney transplantation in prune belly syndrome. Urology 69:666–669

    Article  PubMed  Google Scholar 

  85. Fontaine E, Salomon L, Gagnadoux MF, Niaudet P, Broyer M, Beurton D (1997) Long-term results of renal transplantation in children with the prune-belly syndrome. J Urol 158:892–894

    Article  PubMed  CAS  Google Scholar 

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Hassett, S., Smith, G.H.H. & Holland, A.J.A. Prune belly syndrome. Pediatr Surg Int 28, 219–228 (2012). https://doi.org/10.1007/s00383-011-3046-6

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