30

THE KIDNEYS AND URETERS

Julian E. Kabala
with a contribution from Carl Roobottom

CONGENITAL LESIONS AND VARIANTS

fetal lobulation Definitive renal tissue develops in utero from
its embryological precursor (the metanephros) in response to local
stimulation by the formation of adjacent calyces, as a con­
sequence of the budding of the renal pelvis, which in turn devel­
ops from the upper end of the ureter. Renal tissue therefore
develops as a series of 8-16 lobules and the lobulated structure
remains apparent at birth. This feature gradually disappears over
the first 5 years of life as the kidney grows. In up to 5% of the Fig. 30.1 Renal ultrasound
demonstrating the characteristic
population, however, lobulation persists. This is of no clinical pattern of persistent fetal lobula­
significance but it is important not to confuse it with the scars of tion.
reflux nephropathy. Characteristically in persistent fetal lobula­
tion the parenchyma is of normal thickness (14 mm or more) with to represent normal renal tissue on ultrasound (Fig. 30.3) or CT.
smooth indentations between the calyces. Where scarring results DMSA scans have been performed to confirm normal renal tissue
from vesicoureteric reflux the scars occur over the calyces, which but this should rarely be necessary with modern imaging.
are abnormally clubbed. The characteristic outline of fetal lobula­ Renal agenesis Failure of the ureteric bud to reach the
tion and its relationship to the calyces (or pyramids) can be seen metanephros results in renal agenesis. Predictably the ipsilateral
on IVU, ultrasound, CT and MRI (Fig. 30.1). ureter and hemitrigone also fail to develop, although occasionally
Renal pseudotumours Prominent areas of normal renal tissue a blind-ending ureteric stump is present. Ipsilateral adrenal agene­
may develop and appear as mass lesions, particularly on IVU. sis is seen in 10%. Associated ipsilateral urogenital abnormalities
They are of no clinical significance but may be misdiagnosed as are common and include absence of the vas deferens, unicomuate
neoplastic masses. A column of Bertin is commonly encountered. uterus and absence or cyst of the seminal vesicle. Multiple anom­
This variant is due to a prominent column of normal renal alies in other systems may be present, particularly cardiovascular,
parenchyma, usually at the junction of the upper and middle gastrointestinal and musculoskeletal. The classical association is
thirds of the kidney. It is often bilateral. Careful study of it on seen in the VATER syndrome where developmental lesions may
ultrasound or contrast CT should demonstrate normal renal tissue include vertebral and ventricular septal anomalies, anorectal
(Fig. 30.2). A dromedary or splenic hump is a prominence of the atresia, tracheal and oesophageal lesions (fistula and atresia) and
superolateral border of the left kidney. Again it can be confirmed radial bone abnormalities.

Fig. 30.2 (A) Longitudinal renal ultrasound scan

showing a prominent column of Bertin (arrow).
(B) Transverse scan showing the same feature
(arrow).

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Fig. 30.3 Dromedary hump related to the

lateral border of the left kidney on IVU (A)
and ultrasound (B).

atrophic following a major pathological insult (particularly vesi-

coureteric reflux or infarct) and may be overlooked, particularly on
ultrasound.
Bilateral renal agenesis is rare (1/3000 live births). Oligo­
hydramnios develops as a result of the in utero failure of urine pro­
duction resulting in Potter’s syndrome, with characteristic facies
and early death from pulmonary hypoplasia.
Renal dysplasia This failure of development of normal renal
tissue is usually unilateral and affects the whole kidney.
Occasionally it is bilateral and leads to renal failure in infancy.
Rarely it affects the upper pole of one kidney only. On ultrasound
the kidney is small, with either a smooth outline or marked fetal
lobulation. There is loss of corticomedullary differentiation and
tiny cortical cysts may be visible. On IVU the kidneys excrete
contrast, which demonstrates a lack of papillary development, the
calyces appearing clubbed. There is an association with vesi-
coureteric reflux and numerous congenital syndromes including
Beckwith-Wiedemann and Laurence-Moon-Biedl (Fig. 30.5).
Renal hypoplasia This condition is thought to arise as a result of
an intrauterine insult, probably vascular, that impairs adequate
development. The kidney is normal in shape and smooth in
outline but is small, typically containing five or less calyces,
which are also otherwise normal. Similarly the renal parenchyma
is normally functioning but the global renal function is reduced in
proportion to its size.
Fig. 30.4 IVU showing left renal agenesis with bowel gas within the left
renal bed The large right kidney shows a bifid renal pelvis, the mildest form Supernumerary kidneys These are extremely rare, usually left­
of renal duplication sided, hypoplastic and caudally positioned. Ureteric drainage may
Unilateral agenesis is not uncommon, occurring in up to 0.1% of be separately into the bladder or into the normal kidney’s ureter.
live births with a ratio of three males to one female. As an isolated Rotational abnormalities Failure of normal developmental rota­
lesion it is asymptomatic but there is an increased incidence of con­ tion (malrotation) leaves the pelviureteric junction pointing ante­
tralateral renal abnormalities, including ectopia and malrotation. riorly. This relatively common, harmless anomaly produces a
Occasionally unilateral renal agenesis can be diagnosed on plain characteristic appearance on ultrasound, IVU (Fig. 30.6) and CT.
films by the absence of a renal outline and medial displacement of Rarely overrotation
the splenic (on the left) or hepatic (on the right) flexure into the pointing posteriorly. occurs, leaving the pelviureteric junction
renal bed. The contralateral kidney, when normal, commonly shows
compensatory hypertrophy and this may be visible on the plain Renal ectopia Failure of complete ascent of the kidney to the
film. These features are easier to see on ultrasound and IVU level of the second lumbar vertebra is relatively common, the
(Fig. 30.4). CT or radionuclide investigation demonstrating uni­ kidney coming to lie anywhere from the pelvis upwards.
lateral absence of renal tissue can be regarded as definitive. Failure Overascent is rare. There is usually an anomalous blood supply
to identify a kidney in the normal site may be due to agenesis, with multiple renal arteries from the aorta or iliac vessels at the
ectopia or nephrectomy. Occasionally a kidney becomes severely level of the kidney. A pelvic kidney is encountered in 1/1000 live