Abstract
Renal cysts are relatively common. They may be single, multiple, or innumerable, cortical or medullary. Most renal cysts are spherical, thin-walled, epithelial-lined structures that contain fluid with features of a plasma ultrafiltrate. A clinically useful classification must include characteristics such as age of onset, family history and association with extrarenal lesions, as well as morphologic parameters. Renal dysplasia is disturbed renal histogenesis, either syndromic or non-syndromic, which usually gives rise to morphologically characteristic renal cysts. Two important and distinctive familial forms of renal polycystic disease occur. Autosomal dominant polycystic disease characteristically becomes manifest in adulthood but is becoming increasingly diagnosed in young individuals. Autosomal recessive infantile polycystic disease has complex relationships with “congenital hepatic fibrosis”. Other cystic renal lesions are less common in young patients, but their occurrence in association with other abnormalities provokes speculation regarding pathogenesis.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Gleason DG, McAlister WH, Kissane J (1967) cystic diseases of the kidney in children. Am J Roentgenol 100: 135–146
Kissane JM (1983) Congenital malformations. In: Heptinstall RH (ed) Pathology of the kidney, 3rd eds. Little Brown & Co, Boston, pp 83–140
Bernstein J (1976) A classification of renal cysts. In: Gardner KD Jr (ed) Cystic diseases of the kidney. Wiley, New York, pp 7–30
Spence HM, Singleton R (1972) Cysts and cystic disorders of the kidney: types, diagnosis, treatment. Urol Surv 22: 131–158
Potter EL (ed) (1972) Normal and abnormal development of the kidney. Year Book Medical Publishers, Chicago
Avni EF, Thoua Y, Laimand B, Didier F, Droulle P, Schulman CC (1986) Multicystic dysplastic kidney: evolving concepts. In utero diagnosis and post-natal follow-up by ultrasound. Ann Radiol (Paris) 29: 663–668
Kleiner B, Filly RA, Mack L, Callen PW (1986) Multicystic dysplastic kidney: observations of contralateral disease in the fetal population. Radiology 161: 27–29
Hartman GE (1986) The dilemma of the multicystic dysplastic kidney. Am J Dis Child 140: 925–929
Bernstein J (1986) Hepatic and renal involvement in malformation syndromes. Mt Sinai J Med 53: 421–428
Bartman I, Barraclough G (1965) Cystic dysplasia of the kidneys studied by microdissection in a case of 13–15 trisomy. J Pathol 89: 233–238
Mottet NK, Jensen H (1965) The anomalous embryonic development associated with trisomy 13–15. Am J Clin Pathol 43: 334–347
Bernstein J, Brough AJ, McAdams AJ (1974) The renal lesion in syndromes of multiple congenital malformations: cerebrohepatorenal syndrome: Jeune asphysiating thoracic dystrophy: tuberous sclerosis; Meckel syndrome. In: Bergsma D (ed) The Fifth Conference on the Clinical Delineation of Birth Defects, vol X/4. Williams and Wilkins, Baltimore, pp 34–43
Poznanski AK, Nosanchuk JS, Baublis J, Holt JF (1970) The cerebro-hepato-renal syndrome (CHRS): (Zellweger's syndrome). Am J Roentgenol 109: 313–322
Stapleton FB, Bernstein J, Koh G, Roy S, III Wilroy RS (1982) Cystic kidneys in a patient with oral-facial-digital syndrome type I. Am J Kidney Dis 1: 288–293
Tieder M, Levy M, Gubler MC, Gagnadoux MF, Broyer M (1982) Renal abnormalities in the Bardet-Biedl syndrome. Int J Pediatr Nephrol 3: 199–203
Bernstein J, Robbins TO, Kissane JM (1986) The renal lesions of tuberous sclerosis. Semin Diagn Pathol 3: 97–105
Stillwell TJ, Gomez MR, Kelalis PP (1987) Renal lesions in tuberous sclerosis. J Urol 138: 477–481
Mitnick JS, Bosniak MA, Hilton S, Raghavendra BN, Subramanyam B, Genieser NB (1983) Cystic renal disease in tuberous sclerosis. Radiology 147: 85–87
Stapleton FB, Johnson D, Kaplan GW, Griswold W (1980) The cystic renal lesion in tuberous sclerosis. J Pediatr 97: 574–579
Frimodt-Moller P, Nissen HM, Dyreborg U (1981) Polycystic kidneys as the renal lesion in Lindau's disease. J Urol 125: 868–870
Caroli J, Couinaud C, Soupault R, Porcher R, Eteve J (1958) Une affection nouvelle, sans doute congenitale, des voies biliaires. La dilatation kystique unilobaire des canaux hepatiques. Semin Hop Paris 34: 496–502
Kaplan BS, Fay J, Shah V, Dillon MJ, Barratt TM (1989) Autosomal recessive polycystic kidney disease. Pediatr Nephrol 3: 43–49
Helcyznski L, Wells Tr, Landing BH, Lipsey AI (1984) The renal lesion of congenital hepatic fibrosis: pathologic and morphometric analysis, with comparison to the renal lesion of infantile polycystic disease. Pediatr Pathol 2: 441–445
Blyth H, Ockenden BG (1971) Polycystic disease of kidneys and liver presenting in childhood. J Med Genet 8: 257–284
Kaplan BS, Kaplan P, Chadarevian J-P de, Jequier S, O'Reagan S, Russo P (1988) Variable expression of autosomal recessive polycystic kidney disease and congenital hepatic fibrosis within a family. Am J Med Genet 29: 639–647
Dalgaard OZ (1957) Bilateral polycistic disease of the kidneys: a follow-up study of 284 patients and their families. Acta Med Scand 158 [Suppl 328]: 1–255
Bricker NS, Patton JF (1957) Renal function studies in polycystic disease of the kidneys with observations on the effect of surgical decompression. N Engl J Med 256: 212–217
Kossow AD, Meek JM (1982) Unilateral adult polycystic kidney disease. J Urol 127: 297–300
Lee JKT, McClennan BL, Kissane JM (1978) Unilateral polycystic kidney disease. Am J Roentgenol 130: 1165–1167
Kaplan BS, Rabin I, Nogrady MB, Drummond KN (1977) Autosomal dominant polycystic renal disease in children. J Pediatr 90: 782–783
Sedman A, Bell P, Manco-Johnson M, Shrier R, Warady BA, Heard EO, Butler-Simon N, Gabow P (1987) Autosomal dominant polycystic kidney disease in childhood: longitudinal study. Kidney Int 31: 1000–1005
Reeders STL, Breuning MH, Corney G, Jeremiah SJ, Meera Khan P, Davies KE, Hopkinson DA, Pearson PL, Weatherall DJ (1986) Two genetic markers closely linked to adult polycystic kidney disease on chromosome 16. Br Med J 292: 851–853
Romeo G, Devoto M, Costa G, Roncuzzi L, Catizone L, Zucchelli P, Germino GG, Keith T, Weatherall DJ, Reeders ST (1988) A second genetic locus for autosomal dominant polycystic kidney disease. Lancet II: 8–11
Joshi VV, Dasznica J (1984) Clinicopathologic spectrum of glomerulocystic kidneys. Report of two cases and a brief review of literature. Pediatr Pathol 2: 171–186
Taxy JB, Filmer RB (1976) Glomerulocystic kidney. Report of a case. Arch Pathol Lab Med 100: 186–188
Carson RW, Bedi D, Cavallo T, DuBose TD Jr (1987) Familial adult glomerulocystic kidney disease. Am J Kidney Dis 9: 154–165
Rizzoni G, Loriat C, Levy M, Milanesi C, Zachello G, Mathieu H (1982) Familial hypoplastic glomerulocystic kidney. A new entity? Clin Nephrol 18: 263–268
Kuiper JJ (1976) Medullary sponge kidney. In: Gardner KD Jr (ed) Cystic diseases of the kidney. Wiley, New York, pp 151–171
Gardner KD Jr (1976) Juvenile nephronophthisis and renal medullary cystic disease. In: Gardner KD Jr (ed) Cystic diseases of the kidney. Wiley, New York, pp 173–185
Donaldson MDC, Warner AA, Trompeter RS, Haycock GB, Chantler C (1985) Familial juvenile nephronophthisis, Jeune's syndrome, and associated disorders. Arch Dis Child 60: 426–443
Boichis H, Passwell J, David R (1973) Congenital hepatic fibrosis and nephronophthisis. QJ Med 42: 221–231
Waldherr R, Lennert T, Weber H-P, Fodish HJ, Scharer K (1982) The nephronophthisis complex. A clinicopathologic study in children. Virchows Arch [A] 394: 235–254
Bernstein J, Gardner KD Jr (1983) Hereditary tubulo-interstitial nephropathies. In: Cotran RS, Brenner BM, Stein JH (eds) Contemporary issues in nephrology. Churchill Livingstone, Edinburg, London, New York, pp 335–357
Senior B (1973) Familial renal-retinal dystrophy. Am J Dis Child 125: 442–447
Cho KJ, Thornbury JR, Bernstein J, Heidelberger KP, Walter JF (1979) Localized cystic disease of the kidney: angiographic-pathologic correlation. Am J Roentgenol 132: 891–895
Dunnill MS, Millard PR, Oliver D (1977) Acquired cystic disease of the kidneys: a hazard of long-term intermittent maintenance haemodialysis. J Clin Pathol 30: 868–877
Hughson MD, Buchwald D, Fox M (1986) Renal neoplasia and acquired cystic kidney disease in patients receiving long-term dialysis. Arch Pathol Lab Med 110: 592–601
Bretan PN Jr, Busch MP, Hricak H, Williams RD (1986) Chronic renal failure: a significant risk factor in the development of acquired renal cysts and renal cell carcinoma. Case reports and review of the literature. Cancer 57: 1871–1879
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kissane, J.M. Renal cysts in pediatric patients. Pediatr Nephrol 4, 69–77 (1990). https://doi.org/10.1007/BF00858446
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00858446