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Kidney Dysplasia

Definition of Renal Dysplasia

Renal dysplasia is a congenital maldevelopment of the kidney. Disturbed metanephric differentiation usually leads to primitive structural elements in the kidney, including tubules, often accompanied by hypoplasia. Depending on the severity, renal insufficiency may develop. The following classification according to associated conditions and causes is used:

• Renal dysplasia: unilateral, bilateral, or segmental
• Renal dysplasia with normal ureteral orifices
  • With obstruction
  • Without obstruction
• Renal dysplasia with laterally displaced ureteral orifices
• Renal dysplasia with urethral (subvesical) obstruction
• Renal dysplasia in Prune belly syndrome

Etiology of Renal Dysplasia

• Impaired ureteric budding
• Hydronephrosis beginning in utero

Pathology of Renal Dysplasia

Diagnostic features of renal dysplasia are primitive tubules lined by epithelium and surrounded by concentric rings of connective tissue and smooth muscle cells. Cartilage is often found in the specimen.

References

P. M. Cuckow, M. D. Dinneen, R. A. Risdon, P. G. Ransley, and P. G. Duffy, “Long-term renal function in the posterior urethral valves, unilateral reflux and renal dysplasia syndrome,” J. Urol. vol. 158, no. 3 Pt 2, pp. 1004–7, 1997.

  Deutsche Version: Nierendysplasie

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