renal malformations

Classification

I. renal agenesis

 unilateral renal agenesis
 bilateral renal agenesis

II. Anomalies of renal position and form

 renal ectopia
 renal malrotation
 renal cross ectopia
 renal fusion
 renal duplication (duplex kidney)
 supernumerary kidney
 renal hypoplasia
 renomegaly

III. urinary obstruction with hydronephrosis

IV. Anomalies of renal differentiation (anomalies of nephrogenesis)

 renal aplasia
 renal dysplasia

• obstructive renal dysplasia
• diffuse cystic renal dysplasia
• hypoplastic renal dysplasia
• drug-induced fetal nephropathies

 abnormal tubular differentiation

• renal tubular dysgenesis

V. renal cystic diseases

A. Renal dysplasia

 sporadic renal dysplasia

• unilateral sporadic renal dysplasia
• bilateral sporadic renal dysplasia

 hereditary renal dysplasia

• non-syndromal hereditary renal dysplasia
• syndromal hereditary renal dysplasia

B. polycystic kidney diseases (PKDs)

 autosomal recessive polycystic kidney disease (ARPKD)
 autosomal dominant polycystic kidney disease (ADPKD)

B. Medullary cysts

 medullary sponge kidney
 medullary cystic diseases

C. cortical cysts

 glomerulocystic diseases (GCKDs)
 simple cysts
 microcysts associated with malformative syndromes

D. renal cysts associated with hereditary malformative syndromes (without Renal dysplasia)

E. multilocular renal cysts

References

 Schedl A. Renal abnormalities and their developmental origin. Nat Rev Genet. 2007 Oct;8(10):791-802. PMID: 17878895

 Rodriguez MM. Developmental renal pathology: its past, present, and future. Fetal Pediatr Pathol. 2004 Jul-Aug;23(4):211-29. PMID: 16095033