Potter classification:

• type I: autosomal recessive polycystic kidney disease, infantile
• type II: renal dysplasia
• type IIA: multicystic dysplasia, the kidney is enlarged and cystic
• type IIB: solid dysplasia, the kidney is small, cysts scarse
• type III: autosomal dominant polycystic kidney disease, adult
• type IV: cystic dysplasia with lower urinary tract obstruction
• subcapsular cysts predominate

note: many authors point out that cystic dysplasia Potter type II and IV result from a similar defect i.e. obstruction. They are seen as variants on a continuum rather then separate entities.

Minor cortical or glomerular microcysts are associated with miscellaneous sydromes and metabolic disorders e.g. trisomy 13, trisomy 18, Zellweger syndrome, several subtypes of chondrodysplasias. Renal cystic dysplasia is a cardial feature of autosomal recessive Meckel syndrome (characteristic trias: polydactyly, encephalocele, polycystic kidneys).