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renal malformations

congenital malformations of the kidney

 

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

-  abnormal tubular differentiation

V. renal cystic diseases

A. Renal dysplasia

-  sporadic renal dysplasia

-  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

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


Horseshoe kidney

Renal fusion

Streak kidney - Severe renal hypoplasia. Streak kidney along the posterior peritoneum (An urethral atresia is associated)

Unilateral renal agenesis

Pyelorenal duplication - Ectopic megaureteres, hydronephrosis of the duplicated superior kidney

Bilateral renal hypoplasia

Renal adysplasia

Renal adysplasia

Bilateral renal hypoplasia in trisomy 22 (19 weeks)

Unilateral renal hypoplasia (22 weeks)

Unilateral renal ectopia and hypoplasia (20 weeks)

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