Methodological Instructions for Students
Theme: Congenital anomalies of the urinary tract
Aim: To teach students to diagnose cngenital anomalies of the urinary tract and principles
of treatment of this disease.
Professional Motivation:
Congenital anomalies of the upper urinary tract comprise a diversity of abnormalities,
ranging from complete absence to aberrant location, orientation, and shape of the kidney as well
as aberrations of the collecting system and blood supply. This wide range of anomalies results
from a multiplicity of factors that interact to influence renal development in a sequential and
orderly manner. Abnormal maturation or inappropriate timing of these processes at critical points
in development can produce any number of deviations in the development of the kidney and
ureter.
Basic Level:
1. Anatomy, physiology of kidneys.
2. Development of urinary system/
3. X-ray, instrumental, laboratory, functional and endoscopic methods of investigation
used to diagnose pathology of kidneys.
Student's Independent Study Program
I. Objectives for Students' Independent Studies.
The classification of renal and ureteral anomalies:
Anomalies of number
 Agenesis
a. Bilateral
b. Unilateral
 Supernumerary kidney
1. Anomalies of volume and structure
 Hypoplasia
 Multicystic kidney
 Polycystic kidney
o Infantile
o Adult
 Other cystic disease
 Medullary cystic disease
2.
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Anomalies of ascent
Simple ectopia
Cephalad ectopia
Thoracic kidney
3.
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Anomalies of form and fusion
Crossed ectopia with and without fusion
Unilateral fused kidney (inferior ectopia)
Sigmoid or S-shaped kidney
Lump kidney
L-shaped kidney
Disc kidney
Unilateral fused kidney (superior ectopia)
Horseshoe kidney
4.
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Anomalies of rotation
Incomplete
Excessive
Reverse
5.
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Anomalies of renal vasculature
Aberrant, accessory, or multiple vessels
Renal artery aneurysm
Arteriovenous fistula
6.
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Anomalies of the collecting system
Calyx and infundibulum
Calyceal diverticulum
Hydrocalyx
Megacalycosis
Unipapillary kidney
Extrarenal calyces
Anomalous calyx (pseudotumor of the kidney)
Infundibulopelvic dysgenesis
Pelvis
Extrarenal pelvis
Bifid pelvis
Embryology
Complete differentiation of the metanephric blastema into adult renal parenchyma requires the
presence and orderly branching of a ureteral bud. This occurs normally between the 5th and 7th
weeks of gestation, after the ureteral bud arises from the mesonephric or wolffian duct. It is
theorized that induction of ureteral branching into major and minor calyces depends on the
presence of a normal metanephric blastema (Davidson and Ross, 1954).The absence of a
nephrogenic ridge on the dorsolateral aspect of the celomic cavity or the failure of a ureteral bud
to develop from the wolffian duct will lead to agenesis of the kidney. The absence of both
kidneys, therefore, requires a common factor causing renal or ureteral maldevelopment on both
sides of the midline.
Simple Renal Ectopia
When the mature kidney fails to reach its normal location in the "renal" fossa, the condition is
known as renal ectopia. The term is derived from the Greek words ek ("out") and topos ("place")
and literally means "out of place." It is to be differentiated from renal ptosis, in which the kidney
initially is located in its proper place (and has normal vascularity) but moves downward in
relation to body position. The ectopic kidney has never resided in the appropriate location.
An ectopic kidney can be found in one of the following positions: pelvic, iliac, abdominal,
thoracic, and contralateral or crossed ( Fig. 55–8). Only the ipsilateral retroperitoneal location of
the ectopic kidney is discussed here. Thoracic kidney and crossed renal ectopia (with and
without fusion) are described later.
With the increasing use of radiography, ultrasonography, and radionuclide scanning to visualize
the urinary tract, the incidence of fortuitous discovery of an asymptomatic ectopic kidney is also
increasing. The steady rise in reported cases in recent years attests to this fact.
Most ectopic kidneys are clinically asymptomatic. Vague abdominal complaints of frank ureteral
colic secondary to an obstructing stone is still the most frequent symptom leading to discovery of
the misplaced kidney. The abnormal position of the kidney results in a pattern of direct and
referred pain that is atypical for colic and may be misdiagnosed as acute appendicitis or as pelvic
organ inflammatory disease in female patients. It is rare to find symptoms of compression from
organs adjacent to the ectopic kidney. Patients with renal ectopia may also present initially with
a urinary infection or a palpable abdominal mass. Several cases of a rare association of renal and
ureteral ectopia causing urinary incontinence have been reported (Borer et al, 1993, 1998). The
difficulty in diagnosing this condition is related to the poor function of the ectopic kidney.
Dimercaptosuccinic acid (DMSA) scintigraphy or computed tomography with contrast will
delineate these unusual cases (Borer et al, 1998; Leitha, 1998).
Horseshoe Kidney
The horseshoe kidney is probably the most common of all renal fusion anomalies. It should not
be confused with asymmetric or off-center fused kidneys, which may give the impression of
being horseshoe-shaped. The anomaly consists of two distinct renal masses lying vertically on
either side of the midline and connected at their respective lower poles by a parenchymatous or
fibrous isthmus that crosses the midplane of the body. It was first recognized during an autopsy
by DeCarpi in 1521, but Botallo in 1564 presented the first extensive description and illustration
of a horseshoe kidney (Benjamin and Schullian, 1950). In 1820 Morgagni described the first
diseased horseshoe kidney and since then more has been written about this condition than about
any other renal anomaly. Almost every renal disease has been described in the horseshoe kidney.
Generally, the isthmus is bulky and consists of parenchymatous tissue with its own blood supply
(Glenn, 1959; Love and Wasserman, 1975). Occasionally it is just a flimsy midline structure
composed of fibrous tissue that tends to draw the renal masses close together.It is located
adjacent to the L3 or L4 vertebra just below the origin of the inferior mesenteric artery from the
aorta. As a result, the paired kidneys tend to be somewhat lower than normal in the
retroperitoneum. In some instances, the anomalous kidneys are very low, anterior to the sacral
promontory or even in the true pelvis behind the bladder (Campbell, 1970).The isthmus most
often lies anterior to the aorta and vena cava, but it is not unheard of for it to pass between the
inferior vena cava and the aorta or even behind both great vessels (Jarmin, 1938; Meek and
Wadsworth, 1940; Dajani, 1966).
Symptoms
Almost one third of all patients with horseshoe kidney remain asymptomatic (Glenn, 1959; Kolln
et al, 1972). In most instances, the anomaly is an incidental finding at autopsy (Pitts and Muecke,
1975).When symptoms are present, however, they are related to hydronephrosis, infection, or
calculus formation. The most common symptom that reflects these conditions is vague
abdominal pain that may radiate to the lower lumbar region. Gastrointestinal complaints may be
present as well. The so-called Rovsing sign—abdominal pain, nausea, and vomiting on
hyperextension of the spine—has been infrequently observed. Signs and symptoms of urinary
tract infection occur in 30% of patients, and calculi have been noted in 20% to 80% (Glenn,
1959; Kolln et al, 1972; Pitts and Muecke, 1975; Evans and Resnick, 1981; Sharma and Bapna,
1986). Five percent to 10% of horseshoe kidneys are detected after palpation of an abdominal
mass (Glenn, 1959; Kolln et al, 1972). Horseshoe kidneys have been detected after angiography
for evaluation of an abdominal aortic aneurysm (Huber et al, 1990; deBrito et al, 1991).
UPJ obstruction causing significant hydronephrosis occurs in as many as one third of individuals
(Whitehouse, 1975; Das and Amar, 1984). The high insertion of the ureter into the renal pelvis,
its abnormal course anterior to the isthmus, and the anomalous blood supply to the kidney may
individually or collectively contribute to this obstruction.
Anomalies of Rotation
The adult kidney, as it assumes its final position in the "renal" fossa, orients itself so that the
calyces point laterally and the pelvis faces medially. When this alignment is not exact, the
condition is known as malrotation.Most often, this inappropriate orientation is found in
conjunction with another renal anomaly, such as ectopia with or without fusion or horseshoe
kidney. This discussion centers on malrotation as an isolated renal entity. It must be
differentiated from other conditions that mimic it and are caused by extraneous forces such as an
abnormal retroperitoneal mass.
It is thought that medial rotation of the collecting system occurs simultaneously with renal
migration. The kidney starts to turn during the 6th week, just when it is leaving the true pelvis,
and it completes this process, having rotated 90 degrees toward the midline, by the time ascent is
complete, at the end of the 9th week of gestation.
The kidney and renal pelvis normally rotate 90 degrees ventromedially during ascent. Weyrauch
(1939), in an exhaustive and detailed study, outlined the various abnormal phases of medial and
reverse rotation and labeled each according to the position of the renal pelvis ( Fig. 55–23).
Rotation anomalies per se do not produce any specific symptoms. The excessive amount of
fibrous tissue encasing the pelvis, UPJ, and upper ureter, however, may lead to a relative or
actual obstruction of the upper collecting system. Vascular compression from an accessory or
main renal artery or distortion of the upper ureter or UPJ may contribute to impaired drainage.
Symptoms of hydronephrosis (namely, dull, aching flank pain) may be experienced during
periods of increased urine production. This is the most frequent cause of symptoms. Hematuria,
which occurs occasionally within a hydronephrotic collecting system from jostling of sidewalls,
may be noted as well. Infection and calculus formation, each with its attendant symptoms, may
also occur secondary to poor urinary drainage.
II. Tests and Assignments for Self-assessment
Multiple choice. Choose the correct answer/statement:
1. First change in urine which will prove acute hematogenic first pyelonephritis:
A. Bacteriuria.
B. Leukocyturia.
C. Proteinuria.
D. Haematuria.
E. Hyaline casts.
2. What should we have to do firstly to diagnose pyelonephritis of pregnant women?
1. Retrograde urography.
2. Excretory urography.
3. Cystoscopy.
4. Radiologic examination.
5. Chromocystoscopy.
Real life situation to be solved:
1. In cabinet of urology came a women with clinical findings of acute pyelonephritis on
right side (body temperature is 38 ° C-40 °С; there are proteins less, leucocytes in analysis of
urine). What methods of diagnostic we should provide? What are tactics of treatment?
2. Patient K., at the age of 42, complaints of pain in left side back at the region of kidney
with temperature of body is 39,2°C; he's shivering and weak. He is suffering from stone in left
kidney during six years.
What complication of disease appears in this patient? What methods of examination we
should do to the patient? What are tactics of treatment?
III. Answers to the self-assessment.
The correct answers to the tests:
1. A.
2. E.
The correct answers to the real life situations:
l. We must do chromocystoscopy. In case when kidney won't function we should do
catherization of kidney.
2. Acute chronic pyelonephritis (the complication apostematous nephritis). We must
provide ultrasound screening, retrograde and excretory urography. An operative treatment.
Visual Aids and Material Tools:
1. Slides (chronic pyelonephritis, x-ray of pyelonephritis).
2. Excretory pyelogram.
3. Retrograde pyelogram.
Students' Practical Activities:
Students must know:
1. Pyelonephritis (aetiology, pathogenesis).
2. Classification of pyelonephritis.
3. Primary acute pyelonephritis (clinical signs, diagnosis).
4. Treatment of primary acute pyelonephritis.
5. Differential diagnosis of the primary acute pyelonephritis from tuberculosis of urinary
system, from acute diseases of organs of abdominal cavity.
6. Secondary acute pyelonephritis (clinical signs, diagnosis).
7. Treatment of secondary acute pyelonephritis.
8. Pyelonephritis in pregnant women.
9. Treatment of pyelonephritis in pregnant women.
10. Forms of acute pyelonephritis (clinical signs, diagnosis, treatment).
11. Apostematous pyelonephritis (clinical signs, diagnosis, treatment).
12. Abscess and carbuncle of "kidney (clinical signs, diagnosis, treatment).
Students should be able to:
1. Suspect acute pyelonephritis with the complaints of a patient and anamnesis (life
history).
2. Make plan of investigation.
3. Make differential diagnosis.
4. Plan of treatment the patient suffering from acute pyelonephritis.
5. Estimate analysis of blood and urine biochemical analysis of blood, x-ray, radioisotopical and vascular examination.