Renal Developmental Anomalies

  • Felix Wang
  • Richard M. Friedenberg
Part of the Medical Radiology book series (MEDRAD)


The kidney has a complex origin and some familiarity with its development assists in understanding the numerous congenital disorders of the kidney. Two structures, the ureteric bud and the metanephric blastema, must develop and interact in a normal fashion to produce a normal kidney. The earliest precursor to the adult kidney appears at the end of the third gestational week, when a transient cellular condensation forms the pronephros. This tissue is nonfunctional and soon involutes.


Renal Pelvis Polycystic Kidney Disease Renal Agenesis Horseshoe Kidney Multicystic Dysplastic Kidney 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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  1. Abeshouse BS, Bhisitkul I (1959) Crossed renal ectopia with and without fusion. Urol Int 9:63–91PubMedGoogle Scholar
  2. Afonso DN, Oliveira AG (1988) Medullary sponge kidney, and congenital hemihypertrophy. Br J Urol 62:187–188PubMedGoogle Scholar
  3. Alexander JC, King KB, From CS (1950) Congenital solitary kidney with crossed ureter. J Urol 64:230–234PubMedGoogle Scholar
  4. Ambrose SS (1977) Unilateral multicystic renal disease in adults. Birth Defects 13:349–353PubMedGoogle Scholar
  5. Ashley DBJ, Mostofi FK (1960) Renal agenesis and dysgenesis. J Urol 83:211–230PubMedGoogle Scholar
  6. Athanasoulis CA, Brown B, Baum S (1973) Selective renal venography in differentiation between congenitally absent and small contracted kidney. Radiology 108:301–305PubMedGoogle Scholar
  7. Avni EF, Thoua Y, Van Gansbeke D, Matos C, Didier F, Droulez P, Schulman CC (1987) Development of the hypodysplastic kidney: contribution of antenatal US diagnosis. Radiology 164:123–125PubMedGoogle Scholar
  8. Beckwith JB (1974) Mesenchymal renal neoplasms of infancy revisited. J Pediatr Surg 9: 803–805PubMedGoogle Scholar
  9. Bernstein J (1987) Hepatic involvement in hereditary renal syndromes. Birth Defects 23:115–130PubMedGoogle Scholar
  10. Boatman DL, Cornell SH, Kolln CP (1971) The arterial supply of horseshoe kidneys. AJR 113:447–451Google Scholar
  11. Boatman DL, Kolln CP, Flocks RH (1972) Congenital anomalies associated with horseshoe kidney. J Urol 107:205–207PubMedGoogle Scholar
  12. Bosniak MA, Ambos MA (1975) Polycystic kidney disease. Semin Roentgenol 10:133–143PubMedGoogle Scholar
  13. Braedel HU, Schindler E, Moeller JF, Polsky MS (1976) Renal phlebography: an aid in the diagnosis of the absent or nonfunctioning kidney. J Urol 116: 703–707PubMedGoogle Scholar
  14. Brown T, Mandell J, Lebowitz RL (1987) Neonatal hydronephrosis in the era of sonography. AJR 148:959–963PubMedGoogle Scholar
  15. Buntley D (1976) Malignancy associated with horseshoe kidney. Urology 8:146–148PubMedGoogle Scholar
  16. Campbell MF (1928) Congenital absence of one kidney unilateral renal agenesis. Ann Surg 88:1039–1044PubMedGoogle Scholar
  17. Campbell MF (1963) Anomalies of the kidney. In: Campbell MF (ed.) Urology, 2nd ed., vol.2, p. 1584, WB Saunders, PhiladelphiaGoogle Scholar
  18. Castro JE, Green NA (1975) Complications of horseshoe kidney. Urology 6: 344–347PubMedGoogle Scholar
  19. Chan HS, Cheng MY, Mancer K, Payton D, Weitzman SS, Kotecha P, Daneman A (1987) Congenital mesoblastic nephroma: a clinicoradiologic study of 17 cases representing the pathologic spectrum of the disease. J Pediatr 111: 64–70PubMedGoogle Scholar
  20. Chevalier RL (1989) Obstructive nephropathy in early development. Semin Nephrol 9: 5–9PubMedGoogle Scholar
  21. Charghi A, Dessureault P, Droiun G, Gauthier GE, Perras P, Roy P, Charbonneau J (1971) Malposition of a renal lobe (lobar dysmorphism) : a condition simulating renal tumor. J Urol 105:326–329PubMedGoogle Scholar
  22. Cook WA, Stephens FD (1977) Fused kidneys: morphologic study and theory of embryogenesis. Birth Defects 13: 327–340PubMedGoogle Scholar
  23. Cope JR, Trickey SE (1982) Congenital absence of the kidney: problems in diagnosis and management. J Urol 127:10–12PubMedGoogle Scholar
  24. Craver R, Dimmick J, Johnson H, Nigro M (1986) Congenital obstructive uropathy and nodular renal blastema. J Urol 136:305–307PubMedGoogle Scholar
  25. Dacie JE (1976) The “central lucency” sign of lobar dysmorphism (pseudotumor of the kidney). Br J Radiol 49:39–42PubMedGoogle Scholar
  26. Dajani AM (1966) Horseshoe kidney: a review of 29 cases. Br J Urol 38:388–402PubMedGoogle Scholar
  27. Diard F, Le Dosseur P, Cadier L, Calabet A, Bondonny JM (1984) Multicystic dysplasia in the upper component of the complete duplex kidney. Pediatr Radiol 14: 310–313PubMedGoogle Scholar
  28. D’Alton M, Romero R, Grannum P, DePalma L, Jeanty P, Hobbins JC (1986) Antenatal diagnosis of renal anomalies with ultrasound. IV. Bilateral multicystic kidney disease. Am J Obstet Gynecol 154: 532–537PubMedGoogle Scholar
  29. Davidson AJ (1985) Radiology of the kidney. W.B. Saunders, PhiladelphiaGoogle Scholar
  30. Davies CH, Stringer DA, Whyte H, Daneman A, Mancer K (1986) Congenital hepatic fibrosis with saccular dilatation of intraphepatic bile ducts and infantile polycystic kidneys. Pediatr Radiol 16:302–305PubMedGoogle Scholar
  31. Diard F, Le Dosseur P, Cadier L, Calabet A, Bondonny JM (1984) Multicystic dysplasia in the upper component of the complete duplex kidney. Pediatr Radiol 14:310–313PubMedGoogle Scholar
  32. Dimmick JE, Johnson HW, Coleman GU, Carter M (1989) Wilms tumorlet, nodular renal blastema and multicystic renal dysplasia. J Urol 142:484–485; discussion 489PubMedGoogle Scholar
  33. Donaldson JS, Shkolnik A (1988) Pediatric renal masses. Semin Roentgenol 23: 194–204. Erratum: Semin Roentgenol (1989) 24:142PubMedGoogle Scholar
  34. Downs RA, Lane JW, Burns E (1973) Solitary pelvic kidney. Its clinical application. Urol 1: 51–56PubMedGoogle Scholar
  35. Dreter SP, Olsson C, Pfister RC (1971) The anatomic, radiologic and clinical characteristics of the pelvic kidney: an analysis of 86 cases. J Urol 105:623–627Google Scholar
  36. Elkin M (1975) Renal cystic disease — an overview. Semin Roentgenol 10:99–102PubMedGoogle Scholar
  37. Elkin M (1980) Radiology of the urinary system. Little Brown & Co, BostonGoogle Scholar
  38. Evans JA, Stranc LC (1989) Cystic renal disease and cardiovascular anomalies. Am J Med Genet 33:398–401PubMedGoogle Scholar
  39. Feldman AE, Pollack HM, Perri AJ jr, Karafin L, Kendall AR (1978) Renal pseudotumors: an anatomic-radiologic classification. J Urol 120:133–139PubMedGoogle Scholar
  40. Felson B, Cussen LJ (1975) The hydronephrotic type of unilateral congenital multicystic disease of the kidney. Semin Roentgenol 10:113–123PubMedGoogle Scholar
  41. Fernbach SK, Feinstein KA, Donaldson JS, Baum ES (1988) Nephroblastomatosis : comparison of CT with US and urography 166:153–156Google Scholar
  42. Fikri E, Hanrahan JB, Stept LA (1973) Renovascular hypertension in a child: Ask-Upmark kidney. J Urol 110:728–731PubMedGoogle Scholar
  43. Fridland GW, De Vries P (1975) Renal ectopia and fusion — embryologic basis. Urology 5:698–706Google Scholar
  44. Garcia CJ, Taylor KJ, Weiss RM (1987) Congenital megacalyces. Ultrasound appearance. J Ultrasound Med 6: 163–165PubMedGoogle Scholar
  45. Gary R, Cass AS, Johnson CF (1988) Computed tomography diagnosis of traumatic rupture of congenital hydrohephrotic renal pelvis. Urology 32:65–66PubMedGoogle Scholar
  46. Geyer JR, Poutasse EF (1962) Incidence of multiple renal arteries in aortography. JAMA 182:118Google Scholar
  47. Gillerot Y, Koulischer L (1988) Major malformations of the urinary tract. Anatomic and genetic aspects. Biol Neonate 53:186–196PubMedGoogle Scholar
  48. Gormely TS, Skoog SJ, Jones RV, Maybee D (1989) Cellular congenital mesoblastic nephroma: what are the options? J Urol 142:479–485; discussion 489Google Scholar
  49. Graham JM Jr, Boyle W, Troxell J, Cullity GJ, Sprague PL, Beckwith JB (1987) Cystic hamartomata of the lung and kidney : a spectrum of developmental abnormalities. Am J Med Genet 27:45–59PubMedGoogle Scholar
  50. Greene LF, Feinzaig W, Dahlin DC (1971) Multicystic dysplasia of the kidney: with special reference to the contralateral kidney. J Urol 105:482–487PubMedGoogle Scholar
  51. Griscom NT, Vawter GF, Fellers FX (1975) Pelvoinfundibular atresia: the usual form of multicystic kidney: 44 unilateral and two bilateral cases. Semin Roentgenol 10:125–131PubMedGoogle Scholar
  52. Gunn TR, Mora JD, Pease P (1988) Outcome after antenatal diagnosis of upper urinary tract dilatation by ultrasonography. Arch Dis Child 63:1240–1243PubMedGoogle Scholar
  53. Guys JM, Borella F, Monfort G (1988) Ureteropelvic junction obstructions : prenatal diagnosis and surgery in 47 cases. J Pediatr Surg 23:156–158PubMedGoogle Scholar
  54. Hanna MK, Gluck R (1988) Ureteropelvic junction obstruction during the first year of life. Urology 31:41–45PubMedGoogle Scholar
  55. Harrison JH, Gittes RF, Perlmutter AD, Stamey TA, Walsh PC (1979) Campbell’s urology, 4th edn, vol.2. W.B. Saunders, PhiladelphiaGoogle Scholar
  56. Hartman GE, Smolik LM, Shochat SJ (1986) The dilemma of the multicystic dysplastic kidney. Am J Dis Child 140:925–928PubMedGoogle Scholar
  57. Hashimoto BE, Filly RA, Callen PW (1988) Multicystic dysplastic kidney in utero: changing appearance on US. Radiology 159:107–109Google Scholar
  58. Hayden CK Jr, Swischuk LE, Smith TH, Armstrong EA (1986) Renal cystic disease in childhood. Radiographics 6: 97–116PubMedGoogle Scholar
  59. Hendren WH, Donahoe PK, Pfister RC (1976) Crossed renal ectopia in children. Urology 7:135–144PubMedGoogle Scholar
  60. Himmelfarb E, Rabinowitz JG, Parvey L, Gammill S, Arant B (1975) The Ask-Upmark kidney — roentgenographic and pathologic features. Am J Dis Child 129:1440–1444PubMedGoogle Scholar
  61. Hodson J (1972) The lobar structure of the kidney. Br J Urol 44:246–261PubMedGoogle Scholar
  62. Hodson CG (1974) Vesico-ureteric reflux and renal scarring with and without infection. In: Villareal H (ed.) Proceedings of the Fifth International Congress of Nephrology, Mexico. Karger, New York, Abstract p. 598Google Scholar
  63. Hodson CJ, Mariani S (1982) Large cloisons. AJR 139: 327–332PubMedGoogle Scholar
  64. Hsueh C, Hsueh W, Gonzalez-Crussi F (1987) Bilateral renal dysplasia with features of nephroblastomatosis. Pediatr Pathol 7:437–446PubMedGoogle Scholar
  65. Javadpour N, Chelouhy E, Moncada L, Rosenthal IM, Bush IM (1970) Hypertension in a child caused by a multicystic kidney. J Urol 104:918–921PubMedGoogle Scholar
  66. Javadpour N, Dellon AL, Kumpe DA (1973) Multilocular cystic disease in adults — imitator of renal cell carcinoma. Urology 1:596–599PubMedGoogle Scholar
  67. Johnson DE, Ayala AG, Medellin H, Wilbur J (1973) Multilocular renal cystic disease in children. J Urol 109: 101–103PubMedGoogle Scholar
  68. Johnson HW, Gleave M, Coleman GU, Nadel HR, Raffel J, Weckworth PF (1987) Neonatal renomegaly. J Urol 138: 1023–1027PubMedGoogle Scholar
  69. Johnston JH, Mix LW (1976) The Ask-Upmark kidney: a form of ascending pyelonephritis? Br J Urol 48:393–398PubMedGoogle Scholar
  70. Jordon D, Harpaz N, Thung SN (1989) Caroli’s disease and adult polycystic kidney disease : a rarely recognized association. Liver 9:30–35PubMedGoogle Scholar
  71. Kaariainen H, Jaaskelainen J, Kivisaari L, Koskimies O, Norio R (1988) Dominant and recessive polycystic kidney disease in children: classification by intravenous pyelography, ultrasound, and computed tomography. Pediatr Radiol 18:45–50PubMedGoogle Scholar
  72. Kaplan BS, Kaplan P, deChavarevian JP, Jequier S, O’Regan S, Russo P (1988) Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet 29: 639–647PubMedGoogle Scholar
  73. Kaplan N, Elkin M (1968) Bifid renal pelves and ureters. Radiographic and cinefluorographic observations. Br J Urol 40:235–244PubMedGoogle Scholar
  74. Kelalis PP, Malek RS, Segura JW (1973) Observations on renal ectopia and fusion in children. J Urol 110: 588–592PubMedGoogle Scholar
  75. King MC, Friedenberg RM, Tena LB (1968) Normal renal parenchyma simulating tumor. Radiol 191:217–222Google Scholar
  76. Kleiner B, Filly RA, Mack L, Callen PW (1986) Multicystic dysplastic kidney: observations of contralateral disease in the fetal population. Radiology 161: 27–29PubMedGoogle Scholar
  77. Koyle MA, Ehrlich RM (1988) Management of ureteropelvic junction obstruction in neonate. Urology 31:496–498PubMedGoogle Scholar
  78. Kunin M (1982) The abortive calyx: variations in appearance and differential diagnosis. AJR 139:931–934PubMedGoogle Scholar
  79. Lafortune M, Constantin A, Breton G, Vallee C (1986) Sonography of the hypertrophied column of Bertin. AJR 146: 53–56PubMedGoogle Scholar
  80. Lam AH (1988) Familial megacalyces with autosomal recessive inheritance. Report of 3 affected siblings. Pediatr Radiol 19:28–30PubMedGoogle Scholar
  81. Lozano RH, Rodriguez C (1975) Intrathoracic ectopic kidney. Report of case. J Urol 114:601–602PubMedGoogle Scholar
  82. Mackie GG, Stephens FD (1977) Duplex kidneys: a correlation of renal dysplasia with position of the ureteric orifice. Birth Defects 13:313–321PubMedGoogle Scholar
  83. Madewell JE, Goldman SM, Davis CJ, Hartman DS, Feigin DS, Lichtenstein JE (1983) Multilocular cystic nephroma: a radiographic-pathologic correlation of 58 patients. Radiology 146:309–321PubMedGoogle Scholar
  84. Malek RS, Panayotis PK, Stickler GB, Burke EC (1972) Observations on ureteral ectopy in children. J Urol 107: 308–313PubMedGoogle Scholar
  85. Malone PS, Duffy PG, Ransley PG, Risdon RA, Cook T, Taylor M (1989) Congenital mesoblastic nephroma, renin production, and hypertension. J Pediatr Surg 24: 599–600PubMedGoogle Scholar
  86. Malter IJ, Stanley RJ (1972) The intrathoric kidney. With a review of the literature. J Urol 107: 538–541PubMedGoogle Scholar
  87. McAlister WH, Siegel MJ (1989) Pediatric radiology case of the day. Congenital hepatic fibrosis with saccular dilatation of the intrahepatic bile ducts and infantile polycystic kidneys. AJR 152:1329–1330PubMedGoogle Scholar
  88. McFarland WL, Wallace S, Johnson DE (1972) Renal carcinoma and polycystic disease. J Urol 107: 530–532PubMedGoogle Scholar
  89. Merklin RJ, Michels NA (1958) The variant renal and suprarenal blood supply with data on the inferior phrenic, ureteral and gonadal arteries. A statistical analysis based on 185 dissections and review of the literature. J Int Coll Surg 29:41PubMedGoogle Scholar
  90. Messing E, Kessler R, Kavaney PB (1976) Renal arteriovenous fistulas. Urology 7:101–107Google Scholar
  91. Muren C, Wikstad I (1988) Unilateral hydronephrosis with congenital absence of contralateral kidney in children. Report of six cases and review of literature. Acta Radiol 29: 679–683PubMedGoogle Scholar
  92. Ney C, Friedenberg RM (1981) Radiographic atlas of the genitourinary system, 2nd edn. Lippincott, PhiladelphiaGoogle Scholar
  93. N’Guessan G, Stephens FD (1983) Supernumerary kidney. J Urol 130:649–653PubMedGoogle Scholar
  94. Nussbaum AR, Hartman DS, Whitley N, McCauley RGK, Sanders RC (1987) Muticystic dysplasia and crossed renal ectopia. AJR 149:407–410PubMedGoogle Scholar
  95. O’Reilley PH (1989) Relationship between intermittent hydronephrosis and megacalicosis. Br J Urol 64:125–129Google Scholar
  96. Pedicelli G, Jequier S, Bowen A’D, Boisvert J (1986) Multicystic dysplastic kidneys: spontaneous regression demonstrated with US. Radiology 160: 23–26Google Scholar
  97. Peterson JE, Pinckney LE, Rutledge JC, Currarino G (1982) The solitary renal calyx and papilla in human kidneys. Radiology 144: 525–527PubMedGoogle Scholar
  98. Pettinato G, Manivel JC, Wick MR, Dehner LP (1989) Classical and cellular (atypical) congenital mesoblastic nephroma: a clinicopathologic, ultrastructural, immunohistochemical, and flow cytometric study. Hum Pathol 20: 682–690PubMedGoogle Scholar
  99. Potter EL (1972) Normal and abnormal development of the kidney. Year Book Medical Publishers, ChicagoGoogle Scholar
  100. Premkumar A, Berdon WE, Levy J, Amodio J, Abramson SJ, Newhouse JH (1988) The emergence of hepatic fibrosis and portal hypertension in infants and children with autosomal recessive polycystic kidney disease — initial and follow-up sonographic and radiographic findings. Pediatr Radiol 18: 123–129PubMedGoogle Scholar
  101. Rothermel FJ, Miller FJ Jr, Sanford E, Drago J, Rohner TJ (1977) Clinical and radiographic findings of focally infected polycystic kidneys. Urology 10: 580–585Google Scholar
  102. Sander HM 3rd, Heoyman S, Keller M, Kaplan JM, Norman ME (1987) Association of congenital megacalycosis and ipsilateral segmental megaureter. Pediatr Radiol 17: 28–33Google Scholar
  103. Sander RC, Blakemore K (1989) Lethal fetal anomalies: sonographic demonstration. Radiology 172:1–6Google Scholar
  104. Sanders RC, Nussbaum AR, Solez K (1988) Renal dysplasia: sonographic findings. Radiology 167:623–626PubMedGoogle Scholar
  105. Shaffer SE, Norman ME (1989) Renal function and renal failure in the newborn. Clin Perinatol 16:199–218PubMedGoogle Scholar
  106. Shanbhogue LK, Gray E, Miller SS (1986) Congenital mesoblastic nephroma of infancy. Study of four cases and review of the literature. J R Coll Surg Edinb 31:171–174PubMedGoogle Scholar
  107. Stuck KJ, Koff SA, Silver TM (1982) Ultrasonic features of multicystic dysplastic kidney: expanded diagnostic criteria. Radiology 143:217–221PubMedGoogle Scholar
  108. Takehara Y, Takahashi M, Naito M, Kato T, Nishimura T, Isoda H, Kaneko M (1989) Caroli’s disease associated with polycystic kidney: its noninvasive diagnosis. Radiat Med 7: 13–15PubMedGoogle Scholar
  109. Terada T, Nakanuma Y (1988) Congenital biliary dilatation in autosomal dominant adult polycystic disease of the liver and kidneys. Arch Pathol Lab Med 112:1113–1116PubMedGoogle Scholar
  110. Thompson GJ, Pace JM (1937) Ectopic kidney: A review of 97 cases. Surg Gynecol Obstet 64:935–943Google Scholar
  111. Thompson IM, Rodriguez FR, Spence CR (1987) Medullary sponge kidney and congenital hemihypertrophy. South Med J 80:1455–1456Google Scholar
  112. Tomooka Y, Onitsuka H, Goya T et al. (1988) Congenital hemihypertrophy with adrenal adenoma and medullary sponge kidney. Br J Radiol 61: 851–853PubMedGoogle Scholar
  113. Tridenti G, Armanetti M, Flisi M, Benassi L (1988) Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis in teenagers: report of three cases. Am J Obstet Gynecol 159:882–883PubMedGoogle Scholar
  114. Vargas B, Leibowitz RL (1986) The coexistence of congenital megacalyces and primary megaureter. AJR 147:313–316PubMedGoogle Scholar
  115. Vinocur L, Slovis TL, Perlmutter AD, Watts FB Jr, Chang CH (1988) Follow-up studies of multicystic dysplastic kidneys. Radiology 167:311–315PubMedGoogle Scholar
  116. Webb JAW, Fry IK, Charlton CAC (1975) An anomalous calyx in the midkidney: an anatomical variant. Br J Radiol 48:674–677PubMedGoogle Scholar
  117. Whitaker J, Danks DM (1966) A study of the inheritance of duplication of the kidneys and ureters. J Urol 95:176–178PubMedGoogle Scholar
  118. Whitehouse GH (1975) Some urographic aspects of the horseshoe kidney anomaly — a review of 59 cases. Clin Radiol 25: 107–114Google Scholar
  119. Wilmer HA (1938) Unilateral fused kidney. A report of five cases and a review of the literature. J Urol 40: 551–571Google Scholar
  120. Worthington JL, Shackelford GD, Cole BR, Tack ED, Kissane JM (1988) Sonographically detectable cysts in polycystic kidney disease in newborn and young infants. Pediatr Radiol 18:287–293PubMedGoogle Scholar
  121. Zeier M, Geberth S, Ritz E, Jaeger T, Waldherr R (1988) Adult dominant polycystic kidney disease — clinical problems. Nephron 49:177–183PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • Felix Wang
    • 1
  • Richard M. Friedenberg
    • 1
  1. 1.Department of Radiological SciencesUniversity of California, Irvine Medical CenterOrangeUSA

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