Abstract
Prune belly syndrome is an extremely rare congenital condition in both sexes, particularly in females, with less than 50 cases having been reported; by comparison, the condition is at most uncommon in males. There has been much debate over the pathogenesis of the syndrome, in particular between two main theories. The first implicates a failure of mesodermal cell migration as the primary cause, whereas the second pinpoints urinary distention as the initial event. We describe a case of prune belly syndrome in a female that backs up the second of these theories. The features and the alternative theories as to the aetiology of this syndrome are reviewed, along with an explanation for the marked sex difference.
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References
Adeyokunnu AA, Familusi JB (1982) Prune belly syndrome in two siblings and a first cousin. Am J Dis Child 136: 23–25
Beasley SW, Bettanay F, Hutson JM (1988) The anterior urethra provides clues to the etiology of prune belly syndrome. Pediatr Surg Int 3: 169–172
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
Eagle JF, Barrett GS (1950) Congenital deficiency of abdominal musculature with associated genitourinary anomalies: a syndrome. Report of 9 cases. Paediatrics 6: 721
Frohlich F (1839) Der Mangel der Muskeln, insbesondere der Seitenbauchmuskeln. CA Zurn, Würzburg, cited by Housden LG (1934). Arch Dis Child 9: 219
Garlinger P, Ott J (1974) Prune belly syndrome — possible genetic implications. Birth Defects 10: 173
Geary DF, MacLusky IB, Churchill BM, McLone G (1986) A broader spectrum of abnormalities in the prune belly syndrome. J Urol 135: 324–326
Kroovand RL, Al-Ansari RM, Perlmutter AD (1982) Urethral and genital malformations in prune belly syndrome. J Urol 127: 94–96
Kuruvilla A, Kester K, Williams J, McLee MJ (1987) Congenital cystic adenomatoid malformation of the lung associated with prune belly syndrome. J Pediatr Surg 22: 370–371
Lattimer JK (1958) Congenital deficiency of the abdominal musculature and associated genitourinary anomalies. A report of 22 cases. J Urol 79: 343
Lubinsky MS (1980) Female pseudohermaphroditism and associated anomalies. Am J Med Genet 6: 123–136
Lubinsky M, Doyle K, Trunca C (1980) The association of “prune belly” with Turner's syndrome. Am J Dis Child 134: 1171–1172
Lubinsky M, Rapoport P (1983) Transient foetal hydrops and “prune belly” in one identical female twin. N Engl J Med 308: 256–257
McGovern JH, Marshall VF (1959) Congenital deficiency of the abdominal musculature and obstructive uropathy. Surg Gynecol Obstet 108: 289
McMullin ND, Hutson JM, Kelly JH (1988) Minimal surgery in the prune belly syndrome. Pediatr Surg Int 3: 51–54
Nunn N, Stephens FD (1961) The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J Urol 86: 782
Pagon RA, Smith DW, Shepard TH (1979) Urethral obstruction malformation complex: a cause of abdominal muscle deficiency and the “prune belly.” J Pediatr 94 (6): 900–906
Parker RW (1895) Absence of abdominal muscles in an infant. Lancet I: 1252
Rabinowitz R, Schillinger JF (1977) Prune belly syndrome in the female subject. J Urol 118: 454–456
Rogers LW, Ostrow PT (1973) The prune belly syndrome. J Pediatr 83: 786–793
Silverman FM, Huang N (1950) Congenital absence of the abdominal muscles associated with malformation of the genitourinary and alimentary tracts. Report of cases and review of the literature. Am J Dis Child 80: 91
Snyder HM, Harrison NW, Whitfield HN, Williams DI (1976) Urodynamics in the prune belly syndrome. Br J Urol 48: 663–676
Stephens FD (1983) Congenital malformation of the urinary tract. Praeger, New York, pp 497–498
Straub E, Spranger J (1981) Etiology and pathogenesis of prune belly syndrome. Kidney Int 20: 695–699
Williams DDI, Burkholder GV (1967) The prune belly syndrome. J Urol 98: 244–251
Woodard JR, Trulock TS (1986) Prune belly syndrome. In: Cann C (ed) Campbell's urology, Saunders, Philadelphia, pp 2159–2178
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Shaw, R.A., Smyth, J. & Pringle, K. The prune belly syndrome in a female. Pediatr Surg Int 5, 202–207 (1990). https://doi.org/10.1007/BF00179665
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DOI: https://doi.org/10.1007/BF00179665