multisystem manifestations of haemolytic uremic syndrome ......wall thickening was the most common...
TRANSCRIPT
LOU
ISE
HA
RTL
EY1
SAN
DR
A B
UTL
ER1
EMIL
Y ST
ENH
OU
SE1
IAN
RA
MA
GE
2B
EN R
EYN
OLD
S2
1-
PAED
IATR
IC R
AD
IOLO
GY
2-
PAED
IATR
IC N
EPH
RO
LOG
Y, R
OYA
L H
OSP
ITA
L FO
R C
HIL
DR
EN, 1
34
5 G
OV
AN
RO
AD
, GLA
SGO
W, U
NIT
ED K
ING
DO
M, G
51
4TF
INT
RO
DU
CT
ION
Wepresentacomprehensivepictorialcase
seriesillustratingthe
diversemultisystem
radiologicalmanifestationsofShigatoxin-
producingEscherichiacoliHaemolyticUremicSyndrome(STEC-
HUS).
MA
TER
IAL A
ND
MET
HO
DS
STEC-HUS
ischaracterised
by
haemolytic
anaemia,
thrombocytopeniaandacute
renaldysfunctionwithsignificant
morbidity
and
mortality.
We
retrospectively
reviewed
the
imaging
findingsof148
casesofSTEC-HUS
inanational
paediatricnephrologycentreovera13yearperiod
.
RES
ULT
SR
en
al:
Renalultrasoundwasperformedin
18.2%
ofcases,of
whichahyperechoicrenalcortexwasthemostcommonfinding
(19/27).
Threecasesofrenalcorticalnecrosisandonecase
of
acquired
cystic
kidney
disease
were
diagnosed
on
serial
ultrasoundandMRIimaging.
Gast
roin
test
inal:
Abdominalradiographywasperformedin
14.2%
ofcases,ofwhichcolonicwallthickeningwasthemost
commonfinding(14/21).
Onabdominalultrasoundcolonic
thickening(12/27)andfreefluid(10/27)werethemostcommon
findings.OnecaseofbowelischaemiawasdiagnosedonMRI.
Neu
rolo
gic
al:Neuroimagingwasperformedin8.1%ofcases,of
whichbilaterallentiform
nucleiand/orthalamicinfarctionswere
themostcommonabnormality(5/12).
Twopatientshadmulti-
territorialinfarctsincludingthefirstreportedcase
oflargevessel
arterialthrombosisinHUSonimaging.
Hep
ato
bil
iary
:Two
casesofhepatomegaly
were
seen
on
abdominalultrasound.
OnAXRbiliary
excretionofcontrast
media
due
torenal
failure
was
reported
inone
case.Haemodialysis-relatedsecondary
haemochromatosiswas
diagnosedonfollow-upMRIintwocases.
Resp
irato
ry:
Chest
radiographywasperformedin
21.6%
of
cases,
ofwhichpulmonary
oedemawasthemost
common
abnormality(7/32).
Ocu
lar:
Eyeultrasounddemonstratedvitreoushaemorrhagesin
onecase.
. CO
NC
LU
SIO
NS
This
pictorialcase
seriesillustratestheimagingfindingsof
common,
rare
and
previously
unreported
multisystem
manifestationsofSTEC-HUS.Awarenessoftheroleofradiology
willaid
earlydiagnosisandsubsequentmanagementin
this
complexdisease.
REFER
EN
CES
1.D
un
das
S e
t al.
Th
e C
entr
al Sco
tlan
d E
sch
eri
chia
co
li O
157:H
7 O
utb
reak: R
isk F
act
ors
fo
r th
e H
aem
oly
tic
Ure
mic
Syn
dro
me a
nd
Death
am
ong
Ho
spitaliz
ed
Patients
. C
linic
al In
fect
ious
Dis
ease
s. 2
001;
33:9
23–31.
2.
Gallo
G e
t al.
Ext
ra-r
enal In
volv
em
ent
in D
iarr
ho
ea-a
sso
ciate
d H
aem
oly
tic
Ure
mic
Syn
dro
me. Paed
iatr
ic N
ep
hro
log
y. 1
995; 9:1
17-1
19.
3.
htt
ps:
//ra
dio
paed
ia.o
rg/c
ase
s/p
an
creatic-
necr
osi
s-se
cond
ary
-to
-haem
oly
tic-
ura
em
ic-s
ynd
rom
e
ab
cd
Fig
ure
1.Lo
ng
itu
din
al
ren
al
ultra
sou
nd
ina
4ye
ar
fem
ale
(a)
sho
ws
incr
ease
d
cort
icalech
og
en
icity
of
the
rig
ht
kid
ney
com
pare
dto
no
rmalim
ag
e(b
).C
oro
nal
T1
po
st-c
on
trast
imag
ein
a1
year
old
male
(c)
sho
ws
bila
tera
lre
nal
cort
ical
necr
osi
s.P
re-r
en
al
tran
spla
nt
coro
nal
T2
imag
e(d
)fo
ur
years
late
rsh
ow
sre
nal
atr
op
hy
and
small
cyst
sco
nsi
sten
tw
ith
acq
uired
cyst
ickid
ney
dis
ease
.Lo
w
hep
atic
T2
sig
nald
ue
toir
on
ove
rlo
ad
seco
nd
ary
toh
aem
od
ialy
sis.
Fig
ure
2.
AX
Rin
a1
year
old
male
(a)
sho
ws
thu
mb
pri
ntin
go
fth
etr
an
svers
e
colo
n.
Peri
ton
eal
dia
lysi
s,n
aso
gast
ric
an
dce
ntr
al
ven
ou
slin
es
insi
tu.
Lon
gitu
din
al
an
dtr
an
svers
eu
ltra
sou
nd
ina
9ye
ar
old
male
(b)
an
d(c
)sh
ow
s
mark
ed
colo
nic
wall
thic
ken
ing
an
dh
ypera
em
ia.
Axi
al
po
st-c
on
trast
fat-
sat
T1
imag
ein
a1
year
male
(d)
sho
ws
no
n-e
nh
an
cin
gsm
all
bo
wel
loo
ps
inth
eri
gh
t
low
er
qu
ad
ran
tco
nsi
sten
tw
ith
bo
welis
chaem
ia.
bc
da
Fig
ure
3. 1
year
old
male
deve
lop
ed
in
crease
d t
on
e a
nd
hyp
err
eflexi
a. C
T h
ead
exa
min
atio
n w
as
un
rem
ark
ab
le. A
xial T2 im
ag
e (
a)
sho
ws
hig
h s
ign
al in
bila
tera
l p
ost
eri
or
len
tifo
rmn
ucl
ei w
ith
DW
I
hyp
eri
nte
nsi
ty(b
) an
d r
ed
uce
d A
DC
(c)
. F
ollo
w-u
p c
oro
nal FLA
IR im
ag
e (
d)
six
mo
nth
s la
ter
sho
ws
resi
du
al le
ntifo
rmn
ucl
ei g
liosi
s co
nsi
sten
t w
ith
est
ab
lish
ed
in
farc
tio
n.
a ab b
c gd
Fig
ure
4. 3 y
ear
old
fem
ale
deve
lop
ed
part
ial se
izu
res.
A
xial T2 im
ag
e (
a)
an
d D
WI (b
) sh
ow
mass
ive left
an
teri
or
an
d m
idd
le c
ere
bra
l art
ery
in
farc
tio
n a
nd
less
mark
ed
rig
ht
mid
dle
cere
bra
l art
ery
in
farc
tio
n. Tim
e
of
flig
ht
an
gio
gra
ph
y (
c) r
eve
als
th
rom
bo
tic
occ
lusi
on
of
the s
up
racl
ino
idan
d t
erm
inal se
gm
en
ts o
f th
e left
inte
rnal ca
rotid
art
ery
. A
xial u
nen
han
ced
CT im
ag
e (
d)
sho
ws
po
st-o
pera
tive
deco
mp
ress
ive c
ran
iect
om
y.
Fig
ure
7.
1ye
ar
old
intu
bate
dm
ale
deve
lop
ed
ari
gh
tin
trao
rbital
mass
.
Ultra
sou
nd
of
the
rig
ht
glo
be
(a)
sho
ws
larg
e
sph
eri
cal
mix
ed
ech
og
en
ic
lesi
on
inth
ep
ost
eri
or
cham
ber
with
no
inte
rnal
vasc
ula
rity
an
dsm
all
volu
me
ech
og
en
icm
ate
rial
inth
ele
ftg
lob
e(b
).
Ap
peara
nce
sin
keep
ing
with
bila
tera
lvi
treo
us
haem
orr
hag
es.
Mu
ltis
yste
m M
an
ifest
atio
ns
of
Haem
oly
tic
Ure
mic
Syn
dro
me: A
Pic
tori
al C
ase
Seri
es
Gast
roin
test
inal
Hep
ato
bil
iary
Neu
rolo
gic
al
Ocu
lar
b
dc
a
ab
Resp
irato
ry
Ren
al
Fig
ure
5.
AX
Rin
a1
year
old
fem
ale
for
ass
ess
men
to
fp
eri
ton
eal
cath
ete
rp
osi
tio
n
(a)
sho
ws
foca
lo
paci
fica
tio
nin
the
rig
ht
up
per
qu
ad
ran
tre
flect
ing
hep
ato
bili
ary
exc
retio
no
fp
revi
ou
sly
ad
min
iste
red
con
trast
du
eto
ren
al
failu
re,
inad
ditio
nto
tran
svers
eco
lon
thu
mb
pri
ntin
g.
Po
rtalve
no
us
CT
ab
do
men
ina
2ye
ar
old
male
(b)
sho
ws
diffu
sep
an
creatic
oed
em
aan
dh
ypo
perf
usi
on
sug
gest
ive
of
necr
osi
s.
No
rmalp
an
creatic
pare
nch
yma
on
follo
w-u
pim
ag
ing
³.
Fig
ure
6.
CX
Rin
a4
year
old
fem
ale
(a)
sho
ws
alv
eo
lar
pu
lmo
nary
oed
em
aan
d
mo
dera
teri
gh
tp
leu
raleff
usi
on
.C
XR
ina
3ye
ar
fem
ale
(b)
sho
ws
rig
ht
up
per
lob
e
colla
pse
.A
xial
T2
fat-
sat
imag
ein
a1
year
old
male
(c)
sho
ws
bila
tera
lp
leu
ral
eff
usi
on
san
db
ibasa
lco
nso
lidatio
n.
ba
c
ab
ab