Renal Hypoplasia, From Grossly Insufficient to Not Quite Enough: Consideration for Expanded Concepts Based Upon the Author’s Perspective With Historical Review

Hypoplasia is defined in the Merriman-Webster dictionary as “a condition of arrested development in which an organ, or part, remains below the normal size, or in an immature state.” The degree of reduced size is not definitional. Renal hypoplasia, however, has historically been defined as a more marked reduction in renal mass such that presentation in childhood is the norm. There are 3 commonly recognized types of renal hypoplasia, simple hypoplasia, oligomeganephronic hypoplasia (oligomeganephronia) and segmental hypoplasia (Ask-Upmark kidney). They have in common a reduction in the number of renal lobes. A fourth type, not widely recognized, is cortical hypoplasia where nephrogenesis is normal but there is a reduction in the number of nephron generations. Recently there has been great interest in milder degrees of reduced nephron mass, known as oligonephronia because of its association with risk of adult-onset hypertension and chronic kidney disease. Since the last pathology review of this topic was published by Jay Bernstein in 1968, an update of the renal pathology findings in renal hypoplasia is provided with a review of 18 new cases. The renal hypoplasias are then framed within the modern concept of oligonephronia, its diverse causes and prognostic implications.