THE KIDNEYS AND URETERS 935

systems of the two moieties and the renal parenchyma separating

them are easily seen on ultrasound (Fig. 30.13). Analogous appear­
ances are seen on CT and MRI (particularly good-quality Tr
weighted images). The features of reflux nephropathy (cortical
scarring and clubbing of the calyces) may be present, most often
affecting the lower moiety. If the upper moiety ureter is severely
obstructed, the upper moiety becomes hydronephrotic and shows
diffuse cortical loss, demonstrable on ultrasound, CT or MRI
(Fig. 30.14). The upper moiety may opacify late or not at all
and the hydronephrotic pelvis may displace the lower pole moiety
inferiorly, giving rise to the so-called drooping lily sign
(Fig. 30.15). On rare occasions three or more separate ureters have
been seen on one side (Fig. 30.16).
Ureteroceles These are submucosal dilatations of the intramural
distal ureter. They often project into the bladder lumen. They may Fig. 30.16 Triplex ureters demonstrated on IVU.
become large and on occasion obstruct the other ipsilateral ureter
of a duplex system and even the urethral orifice, provoking bi­ Ureteric diverticulum This is presumed to be an abortive form of
lateral hydronephrosis. Most of them are associated with the ureteric duplication. It consists of a saccular or fusiform ureteric
upper moiety ureter of a duplex system and are therefore ectopic.
These have a strong tendency to obstruction, sometimes severe, stump tract
connected to the normal ureter. It predisposes to urinary
infection and calculi.
with marked hydroureter and hydronephrosis. There is a female
preponderance of approximately 4 to 1. Multlcystlc kidney This is a relatively common condition, which,
A minority of ureteroceles are not associated with ureteric duplica­ if not detected antenatally, usually presents as a childhood
tion and, although congenital, usually present in adults, often as inci­ abdominal mass. It is thought to be due to in utero failure of the
dental findings. They tend to be relatively small but may be associated ureteric bud to connect with the nephrons in the metanephric
with calculi and urinary tract infection. They are not usually associ­ blastema. The ureter in turn fails to develop and is atretic, while
ated with significant obstruction until complicated by calculi. the kidney becomes non-functioning. On ultrasound or CT the
On IVU the ureterocele can be seen as a contrast-filled struc­ kidney is composed of non-communicating cysts of varying size.
ture with a thin smooth radiolucent wall surrounded by contrast- A variant of this condition (hydronephrotic multicystic kidney)
containing urine in the bladder. This has been described as a cobra’s has been described in which only the upper end of the ureter is
head appearance. If the ureterocele is obstructed and the associated atretic and the cysts within the kidney are arranged around a large
kidney non-functioning, it appears as a well-defined radiolucent central cyst, with which they may communicate. It has been sug­
mass within the opacified bladder (Fig. 30.17). On ultrasound it gested that this may represent an extremely severe form of
appears as a thin-walled purely cystic structure projecting into the intrauterine pelviureteric junction obstruction. Multicystic kidney
bladder lumen at the site of ureteric insertion. Associated ureteric is associated with an increased risk of contralateral pelviureteric
calculus and dilatation and hydronephrosis can also be seen. junction obstruction.

i*.
!»

Fig. 30.17 Full length film

from an IVU series showing a
non-opacified partly obstruct­
ing ureterocele surrounded by
opacified urine in the bladder
(A). A later full length film
shows opacification of the dis­
tended upper moiety ureter
running down to the
mm opacified ureterocele (B).
936 A TEXTBOOK OF RADIOLOGY AND IMAGING

Polycystic kidneys Multiple cysts are seen in numerous congeni­

tal (mostly rare) syndromes, including Laurence-Moon-Biedl,
Noonan’s, Turner’s and the trisomy syndromes. The most fre­
quently encountered clinically important conditions in adults are
polycystic disease of kidneys, tuberous sclerosis and von
Hippel-Lindau syndrome. There are two principal types of poly­
cystic kidneys: autosomal recessive and autosomal dominant.
In autosomal recessive polycystic disease of the kidneys
(ARPCK) the renal parenchyma is replaced by numerous tiny
(1-8 mm) cysts. It has been referred to as infantile PCK but there
are four subtypes (perinatal, neonatal, infantile and juvenile), so
ARPCK is the preferred term. Most present in the neonatal period
with oligohydramnios and Potter’s syndrome, with early death from
respiratory failure. In the older subtypes renal function is better
preserved. However, there is an association with periportal fibrosis
and subsequent liver failure, which increases in frequency with age
of presentation until the juvenile subtype, when the liver disease
predominates. On ultrasound the kidneys are diffusely echogenic Fig. 30.19 Ultrasound showing relatively early polycystic disease with
rather than cystic in appearance. On IVU there is a striated nephro­ multiple simple cysts demonstrable.
gram thought to be due to contrast lying in the minority of pre­
served functioning tubules next to dilated non-opacified diseased
tubules.
In autosomal dominant polycystic disease of the kidneys
(ADPCK) numerous cysts of varying size, often becoming
extremely large, develop within the kidneys, gradually replacing
normal renal parenchyma and ultimately producing renal failure. It
usually presents between 20 and 39 years of age, although milder
forms may not present until over 60 years and lack of renal failure
has been observed in some patients up to 80 years of age.
Presentation is usually with hypertension, renal insufficiency, com­
plications of the multiple cysts (haematuria, pain and infection) or
as an abdominal mass discovered on incidental clinical or imaging
examination. There may be associated cysts in the liver (50% of
cases), pancreas, spleen and lung; 15% have associated berry Fig. 30.20 CT scan showing multiple cysts in the left kidney. The disease
aneurysms and there is an increased incidence of coarctation and in this patient is dominated by multiple hepatic cysts, the right lobe
valvular hear disease. On IVU the plain films may show some cyst containing a particularly large one.
calcification. On the contrast films the kidneys are seen to be pie bilateral (but often asymmetrical) renal cysts of varying size and
enlarged and the cysts may be visible as multiple well-defined non­ is more sensitive than IVU at the detection of small cysts in earlier
perfused areas surrounded by normal areas of renal parenchyma. disease (Fig. 30.19). In 10% of cases the disease is extremely asym­
The calyces have a classical stretched appearance due to the pres­ metrical but in virtually all cases it is bilateral and progressive.
ence of multiple cysts (Fig. 30.18). Ultrasound demonstrates multi- Initially the cysts are simple and separated by normal renal
parenchyma. Over time they increase in number to produce marked
bilateral renal enlargement, and the normal parenchyma disappears.
The cysts are prone to haemorrhage and infection with episodes of
pain, pyrexia and haematuria. As a result of this, some of the cysts
may become thick-walled, septated, calcified and contain echogenic
debris. CT and MRI will also show multiple cysts of varying size
and contents (Fig. 30.20) with wall calcification more reliably
demonstrated on CT. In patients with haematuria a small coexistent
malignancy is extremely difficult to diagnose or exclude and occa­
sionally serial imaging is required. There is a link at chromosomal
level with tuberous sclerosis and some kidneys show features of
both conditions in varying proportion (Fig. 30.21).
Tuberous sclerosis This is an autosomal dominant neuro-
cutaneous disorder of low penetrance and extremely variable
expressivity with a spectrum of central nervous system and cuta­
neous manifestations, including cerebral hamartomas, con­
Fig. 30.18 IVU demonstrating the characteristic stretching of calyces by vulsions and adenoma sebaceum. Renal manifestations include
cysts in polycystic kidneys. multiple bilateral angiomyolipomas (Fig. 30.22) and cysts. There