Abstract
A retrospective analysis of infant autopsies in the last 14 years was done with the aim of determining the frequency of associated malformations. Renal dysplasia comprised 36 of 594 autopsies done and was the commonest congenital urologic malformation (6.06% of all infant autopsies). Histologically all cases were characterised by hallmarks of disordered metanephric differentiation and organisation. Extrarenal malformations were seen in all (100%) of the cases of which other urologic anomalies were the commonest (28) followed by those of musculoskeletal (18), gastrointestinal (13), cardiovascular (10) and central nervous system (6). Syndromic association was seen in 5 (14%) cases. There were twenty two percent still births and 47% infants died perinatally. A thorough search for systemic malformations is mandatory in all cases of renal dysplasia as these may influence the survival significantly. Cystic renal dysplasia should be differentiated from other causes of cystic renal diseases.
Similar content being viewed by others
References
Bernstein J, Barness EG: Congential malformations of the kidney. In: Tischer CC and Brenner BM (eds.):Renal pathology: with clinical and functional correlations, second edition vol II. Philadelphia J. B. Lippincott Company; 1994, p 1366.
Bernstein J. The morphogenesis of renal parenchymal development (renal dysplasia).Pediatr Clin North Am 1971; 18: 395–407.
Ericsson NO and Ivemark BI. Renal dysplasia and pyelonephritis in infants and children.Arch Pathol Lab Med 1958; 115: 58–65.
Noe HN, Marshall JH, Edwards OP. Nodular renal blastema in the multicystic kidney.J Urol 1989; 142: 486–488.
Dimmick JE, Johnson HW, Coleman GV, Carter M. Wilm's tumorlet nodular renal blastema and multicystic renal dysplasia.J Urol 1989; 142 (2 pt 2): 484–485.
Milutinovic J, Agodoa LY. Potential causes and pathogenesis in autosomal dominant polycystic kidney disease.Nephron 1983: 33: 139–144.
Carone FA, Makino H, Kanwar YS. Basement membrane antigens in renal polycystic disease.Am J Pathol 1988; 130: 466–471.
Rubenstein M, Meyer, Bernstein J. Congenital abnormalities of the urinary system. A postmortem survey of developmental abnormalities and acquired congenital lesions in a children's hospital.J Pediatr 1961; 58: 356–366.
Atiyeh B, Husmann D, Baum M. Contralateral renal abnormalities in multicystic-dysplastic kidney disease.J Pediatr 1992; 121: 65–67.
Van Every MJ. In utero detection of horse shoe kidney with unilateral multicystic dysplasia.Urol 1992; 40 (5): 435–437.
Mir S, Rapola J and Koskimies O. Renal cysts in pediatric autopsy material.Nephron 1983; 33: 189–195.
Proesmans W, Moerman P, Depratere M, Van Damme B. Association of bilateral renal dysplasia and congenital hepatic fibrosis.Int J Pediatr Nephrol 1986; 7 (2): 113–116.
Fryns JP. Chromosomal anomalies and autosomal syndromes.Birth defects: original article series 1987; 23: 7–32.
Gilbert E and Opitz J. Renal involvement in genetic hereditary malformation syndromes. In: Hamburger J, Crosnier J and Grunfield JP eds.Nephrology. New York. John Wiley and Sons 1979; 893–902.
Mc Pherson E, Carey J, Kramer A, Hall JG, Pauli RM, Schimke RN and Tasin MH. Dominantly inherited renal dysplasia.Am J Med Genet 1987; 26: 863–872.
Squiers EC, Morden RS, Bernstein J. Renal multicystic dysplasia: An occassional manifestation of the hereditary renal adysplasia syndrome.Am J Med Genet (Suppl. 3) 1987; 278–284.
Greene L, Feinzaig W. and Dahlin D. Multicystioc dysplasia of the kidney: with special reference to the contralateral kidney.Urol 1971; 105: 482–487.
Chang WT, Chen HC, Peng HC. The multicystic dysplastic kidney in children.Chung-Hua-I-Hseuh-Tsa-Chih-Taipei 1993; 51 (5): 350–354.
Chaturvedi KU, Singh B. Renal dysplasia with multisystem malformation—a study of 9 cases.Ind J Pediatr 1989; 56 (3): 393–397.
Hartman GE, Smolik LM, Schochrat SJ. The dilemma of the multicystic dysplastic kidney disease.Am J Dis Child 1986; 140: 925–928.
Birken G, Kind D, Vane D, Lloyd T. Renal cell carcinoma arising in a multicystic dysplastic kidney.J Pediatr Surg 1985; 20: 619.
Peschicelli G, Jequir S, Bowen AD, Botsvert J. Multicystic dysplastic kidneys: spontaneous remission demonstrated with ultrasound.Radiology 1986; 161: 23–6.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Singh, Z.N., Dinda, A.K. Renal dysplasia: An autopsy study of associated congential malformations. Indian J Pediatr 65, 311–318 (1998). https://doi.org/10.1007/BF02752309
Issue Date:
DOI: https://doi.org/10.1007/BF02752309