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Anatomy Atlases: Illustrated Encyclopedia of Human Anatomic Variation: Opus IV: Organ Systems: Kidneys, Ureters, Bladder, and Urethra

Illustrated Encyclopedia of Human Anatomic Variation: Opus IV: Organ Systems: Urinary System

Kidneys, Ureters, Bladder, and Urethra

Ronald A. Bergman, PhD
Adel K. Afifi, MD, MS
Ryosuke Miyauchi, MD

Peer Review Status: Internally Peer Reviewed

According to Gutierrez, 40% of pathologic conditions of the urinary system are due to variations. Urologic variations are found in 10% of vertebral variations, in 15% of meningocele or myleomeningocele, in 25% of double anus, 42% of duplication of colon, 90% of duplication of genitalia (Cernak, 1989).

The kidneys sometimes vary from their normal form, being either longer and narrower or shorter and more rounded.

The characteristic fetal lobulation may persist in the adult (7%).

Occasionally, one kidney is very small, while the other is proportionally enlarged.

Numerous cases are recorded of absence of one kidney, most frequently the right. Congenital absence of a kidney has a reported frequency of 0.24% (6,500 autopsies) or in another study of 52,198 cases the incidence of agenesis was 1/1000 or 0.10%.

Ectopic kidney has a reported frequency of 1:500 to 1:110; ectopic thoracic kidney, 1:13,000 (solitary kidney, 1:1,000; solitary pelvic kidney, 1:22,000; one normal and one pelvic kidney, 1:3,000, and crossed renal ectopia, 1:7,000.

The single kidney is usually enlarged, but not invariably. The left kidney is usually larger than the right.

Berengario da Carpi (1523) was the first to write on fused kidneys with horseshoe configuration. They were first described and illustrated however by Botallo in 1564. When it is elongate it may lie, in part, behind the esophagus.

On average, in males the right is 12.7 X 6.3 cm while the left is 13.2 X 6.4 cm. In females, the right is 12.4 X 5.9 cm and the left is 12.8 X 6.1 cm. These figures come from radiologic studies. The occurrence of an additional kidney is rare. In one case, the right kidney was normal, but on the left side there were two kidneys (an upper and lower) separated from one another by a distance of 3 cm. The ureter from the upper kidney was joined by that from the lower, so that there were only two uteric openings into the bladder.

The two kidneys may be joined by their upper and lower poles or even in the middle of the organs. They cross in front of the great vessels and vertebral column. The conjoined organ usually has the form of a horseshoe, with the convexity downward due to fusion of the caudal ends of the two kidneys. Horseshoe kidney was found in 0.21% of 6,500 autopsies in one study and in 0.12% in 68,000 autopsies (1:850) in another. Cumulative data from 192 authors (202,777 autopsies and x-ray studies) indicated a total of 378 horseshoe kidneys. The incidence therefore is about 1:536 or 0.19%; 84% of cases in males and about 17% in females. In one study of 2424 cases, 15 horseshoe kidneys were found in males and three in females.

Sometimes two united kidneys are situated in the lumbar region on one side of the vertebral column, or more rarely in the cavity of the pelvis. Pelvic kidney was found in 0.04% of 6,500 autopsies.

Dumbbell- shaped organs or two disk-shaped kidneys joined together at their midpoint have also been described.

The kidneys may be mobile, owing to the laxity of its areolar and adipose capsule. These conditions occur much more frequently with the right kidney. The perinephric fatty tissue is continuous across the mid-line from one kidney to the other.

  1. Anomalies of Number
    1. Absence of both kidneys
    2. Absence of one kidney (solitary kidney)
    3. Double or multiple kidney (supernumerary kidney)
      • Unilateral or Bilateral
  2. Variations in Size
    1. Hypoplastic kidney.
      • True renal hypoplasia.
      • Renal aplasia.
    2. Hypertrophic kidney.
      • Lobulated.
      • Compensated.
  3. Variations of Form.
    1. Long kidney.
    2. Short kidney.
    3. Broad kidney.
    4. Lobulated kidney.
    5. Cystic kidney.
    6. Polycystic kidney.
      • Unilateral.
      • Bilateral.
    7. Fused kidney.
  4. Variation of Position.
    1. Movable or floating kidney.
    2. Ectopic kidney.
      • Simple unilateral.
      • Simple bilateral.
      • Bilateral with fusion (horseshoe kidney).
      • Crossed with or without fusion.
      • Any of these variations of position may be Lumbar, Iliac, Plevic or Median.

Variations of Rotation.

  1. Deficient rotation.
  2. Excessive rotation.

The Excertory Apparatus of the Kidney (Calices, Pelvis and Ureter):

  1. Calices
  2. Renal Pelvis
  3. Ureter

Modified from Gutierrez, 1936.

The calices minores may vary in number from six to 14, the smaller number indicating that some of the calices minores embrace the apices of several pyramids. Variations in the calices minores and pelvis are more marked.

The calices majores may pass downward for some distance beyond the hilum, and end by joining to form the ureter without undergoing and obvious expansion. In such cases, the pelvis is absent; if the calices dilate, one or two pelves may be present.

Unilateral renal agenesis associated with genital anomalies have been reported to occur in 12% of men and 40% of women. Anomalies of the seminal vesicle are uncommon but when they do occur, in 68% of such cases, unilateral renal agenesis is also found.

Bilateral anephrogenesis has an incidence of 0.0017%.

Heterotopic renal tissue has been reported in the heart and in the suprarenal gland.

The principal variation in the ureter is a more or less complete division into two. As a rule, the two ureters unite a little above the bladder, so that there is only one vesical orifice. When the division is complete there are two separate openings into the bladder. In one study of 26,480 autopsies, 136 double or bifid ureters were found (0.5%).

In rare cases, three or four ureters may be found. Several instances are recorded in which a supernumerary ureter, proceeding from the upper part of the kidney, opened directly into the urethra. Ureters have also been reported to open into the vagina, the seminal vesicle, or the prostatic urethra.

The right ureter has been found passing behind the inferior vena cava (postcaval or retrocaval ureter) between that vessel and the aorta.

Urinary bladders of variable shape (e.g., hourglass bladder) have been reported. The bladder may be doubled. Agenesis has a reported incidence of 1:600,000 (0.00017%).

The urethra may be doubled.

Horseshoe Kidney

Kidney - Horseshoe

Horseshoe Kidney

Horseshoe Kidney with Three Ureters

Pelvic Kidney

Supernumerary Third Kidney

Bilateral Duplication of the Ureter

Types of Double Urethra

Unilateral, Incompletely Bifid Ureter

Sigmoid Kidney

Bilateral Bifid Ureters

Fused Kidneys

Fused Horseshoe Kidneys

Horseshoe Kidneys

Types of Triplicate Ureter

Patterns of Pelvis and Calyces

Congenital S-shaped kidney


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