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Anomalies of kidney development

 
, medical expert
Last reviewed: 04.07.2025
 
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Anomaly (anomalia; from the Greek "deviation") is a congenital deviation from the structure and/or function inherent in a given biological species. Anomalies in the development of the kidneys are diseases of the genitourinary system and are the most common and account for about 40% of congenital malformations.

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Where does it hurt?

Forms

The large number and diversity of developmental defects detected required their systematization. The first attempts to create a classification were made by I. Delmas and P. Delmas in 1910, I.Kh. Dzirne in 1914, and S.P. Fedorov in 1924. The most complete classification was proposed by E.I. Gimpelson in 1936, and in a slightly expanded form - in 1958 by R. Marton. These classifications are still used by many urologists both in our country and abroad. However, the emergence of such diagnostic methods as angiography, nephroscintigraphy, and CT at the end of the 20th century allowed N.A. Lopatkin and A.V. Lyulko to propose the following classification in 1987.

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Renal vascular anomalies

  • Quantity anomalies:
    • accessory renal artery;
    • double renal artery;
    • multiple arteries.
  • Position anomalies:
    • lumbar;
    • iliac;
    • pelvic dystopia of the renal arteries.
  • Anomalies of the shape and structure of arterial trunks:
    • renal artery aneurysms (unilateral and bilateral);
    • fibromuscular renal artery stenosis;
    • genu renal artery.
  • Congenital arteriovenous fistulas.
  • Congenital changes in the renal veins:
    • anomalies of the right renal vein (multiple veins, entry of the testicular vein into the renal vein on the right);
    • anomalies of the left renal vein (annular left renal vein, retro-aortic left renal vein, extracaval return of the left renal vein).

Kidney number anomalies

  • Aplasia.
  • Kidney doubling - complete and incomplete.
  • Additional, third kidney.

Abnormalities in kidney size

  • Hypoplasia (rudimentary, dwarf kidney)

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Anomalies of the location and shape of the kidneys

  • Renal dystopia:
    • unilateral (thoracic, lumbar, iliac, pelvic);
    • cross.
  • Fusion of kidneys:
    • unilateral (I-shaped kidney);
    • bilateral (symmetrical - horseshoe-shaped, gallet-shaped kidney; asymmetrical - L- and S-shaped kidneys).

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Anomalies of kidney structure

  • Dysplastic kidney.
  • Multicystic kidney.
  • Polycystic kidney disease:
    • adult polycystic disease;
    • polycystic disease of childhood.
  • Solitary renal cysts:
    • simple;
    • dermoid.
  • Parapelvic cyst, calyceal and renal pelvic cysts.
  • Calyceal-medullary anomalies:
    • megacalix;
    • polymegacalyx;
    • spongy kidney.

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Combined renal anomalies

  • with vesicoureteral reflux (VUR);
  • with IVO;
  • with PMR and IVO;
  • with anomalies of other organs and systems (reproductive, musculoskeletal, cardiovascular, digestive).

Research in recent years has changed some of our ideas about whether certain conditions are developmental defects, and some kidney developmental anomalies can be classified as other types.

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