Alberti, N.

Balageas, P.

Bonnet, F.

Carteret, T.

Durieux, M.

Frulio, N.

Lasserre, A.-S.

Perez, J.-T.

Petitpierre, F.

Raffray, L.

Salut, C.

Trillaud, H.

Veron, A.

Elsevier - Publisher Connector 

Diagnostic and Interventional Imaging (2013) 94,  333—335
LETTER / Cardiovascular imaging
Acute  pulmonary  embolism  revealing  Ivemark
syndrome  in  an  adult
F.  Petitpierrea,∗,  N.  Alberti a,  L.  Raffrayb,
M.  Durieuxa,  A.  Verona,  J.-T.  Pereza,  A.-S.  Lasserrea,
N.  Frulioa,  T.  Cartereta,  P.  Balageasa,  C.  Saluta,
F. Bonnetb,  H.  Trillauda
a Radiology  Department,  Saint-André  Hospital,  CHU  de  Bordeaux,  Bordeaux  Segalen
University,  1,  rue  Jean-Burguet,  33000  Bordeaux,  France
b Internal  Medicine  Department,  Saint-André  Hospital,  CHU  de  Bordeaux,  1,  rue
Jean-Burguet,  33000  Bordeaux,  France
KEYWORDS
Ivemark;
Asplenia;
Heterotaxy
Ivemark  syndrome  is  a  polymalformative  syndrome,  the  cardinal  element  of  which  is  asple-
nia,  described  for  the  ﬁrst  time  by  Björn  Ivemark  in  1955.  This  condition  is  rare  in  adults,
as  the  majority  of  children  affected  do  not  survive  beyond  their  ﬁrst  year.  We  report  the
case  of  a  21-year-old,  hitherto  asymptomatic,  male  patient,  in  whom  this  syndrome  was
diagnosed.
Observation
The  patient  was  a  21-year-old  male,  who,  suffering  from  sudden  thoracic  pain  combined
with  dyspnoea,  had  an  emergency  chest  CTA  (with  abdominal  slices).  A  proximal  bilateral
pulmonary  embolism  was  found  (Fig.  1)  combined  with  signs  of  acute  right  heart  dysfunc-
tion.  His  laboratory  results  were  normal  (including  haemostasis)  and  in  particular  did  not
indicate  thrombocytosis.
We  unexpectedly  found  a  series  of  heterotaxic  malformations:
• left  pulmonary  isomerism  (absence  of  the  middle  lobe);
• absence  of  the  retrohepatic  inferior  vena  cava  with  azygos  continuity  (direct  drainage
of  the  hepatic  veins  into  the  right  atrium,  and  the  calibre  of  the  azygos  vein  exceeding
6  mm)  (Fig.  2);• right  polysplenia  (multiple  splenic  islets  in  the  right  hypochondrium)  (Fig.  3);
• adysmorphic  pancreas  with  agenesis  of  its  corporocaudal  part  (Fig.  4);
• preduodenal  and  prepancreatic  portal  trunk;
∗ Corresponding author.
E-mail address: petitpierre.francois@gmail.com (F. Petitpierre).
2211-5684/$ — see front matter © 2012 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.
http://dx.doi.org/10.1016/j.diii.2012.11.001
334  F.  Petitpierre  et  al.
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Figure 3. Axial slice in the portal phase centred on the liver:
polysplenia in the right hypochondrium (white star) with the splenic
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that  agenesis  of  the  inferior  vena  cava  is  a  recognised  factorigure 1. Chest CTA: bilateral proximal pulmonary embolism
arrow). Dilatation of the azygos vein (star).
median  liver  (liver  symmetrical  relative  to  the  midline);
complete  common  mesentery;
minor  vascular  abnormalities  (the  presence  of  four  renal
arteries,  a  retro-aortic  left  renal  vein,  multiple  accessory
splenic  arteries  arising  directly  from  the  aorta)  (Fig.  3).
iscussion
his  patient  presented  a  polymalformative  syndrome,  the
ardinal  element  of  which  was  asplenia,  corresponding  to  a
yndrome  described  by  Ivemark  in  1955.
In  the  majority  of  cases,  major  cardiac  malformation
eveals  this  condition  in  the  infant  and  is  the  reason  for  the
igh  mortality  rate  of  60%  before  reaching  1  year  of  age  [1].
nly  5  to  10%  of  patients  with  this  syndrome  reach  adulthood
2].
We  picked  out  polysplenia  of  accessory  spleens,  which
re  always  associated  in  the  normal  situation  with  a  main
pleen.The  lack  of  fusion  of  foetal  lobules  leads  to  individu-
lisation  of  multiple  splenic  nodules  [3].  They  remain  in
 posterior  location  along  the  greater  curvature  of  the
igure 2. Oblique coronal slice centred on the hepatic veins:
bsence of the retrohepatic inferior vena cava (arrow). Polysplenia
n the right hypochondrium (star).
f
f
F
l
r
prtery originating from the aorta (white arrow). Voluminous hemi-
zygos venous trunk (black arrow). Median liver (black star).
tomach  in  the  right  or  left  hypochondrium  (depending
hether  there  is  abnormal  gastric  rotation  or  not).
The  most  frequently  found  hepatic  anomaly  is  a  median
iver.  In  the  present  case,  there  was  abnormal  venous
rainage  with  four  hepatic  veins  with  no  retrohepatic  infe-
ior  vena  cava,  combined  with  azygos  continuation  [4].
The  complete  common  mesentery  consists  of  rotation  of
he  mesentery  being  stopped  at  90◦.  The  colon  remains  to
he  left;  the  small  intestine,  duodenum  and  stomach  are
laced  to  the  right  of  the  midline.  The  duodenum  does  not
ass  through  the  aorto-mesenteric  ‘‘pincer’’  [5].
The  pancreas  has  a  larger  cephalic  end  and  its  morphol-
gy  is  oval  [6].
The  pulmonary  embolism  probably  resulted  from  deep
enous  thromboses  which  could  not  be  demonstrated,  givenavouring  them  [7]  and  that  no  other  cause  of  thrombosis  was
ound  in  laboratory  tests:  hypercoagulability  factors,  JAK2
igure 4. Axial slice in the portal phase centred on the pancreas:
oop of small intestine in the left hypochondrium (white star), in
elation with the complete common mesentery. Oval head of the
ancreas without pancreatic body or tail (black star).
n  ad
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[Acute  pulmonary  embolism  revealing  Ivemark  syndrome  in  a
mutation,  and  antiphospholipid  antibodies  notably  were  all
negative.
Cases  of  pulmonary  embolism  have  previously  helped
reveal  functional  asplenia  [8]  or  a  venous  vascular  anomaly
of  agenesis  of  the  retrohepatic  inferior  vena  cava  with  azy-
gos  continuation  [9].
In  this  second  case,  haemodynamic  modiﬁcation  is
responsible  for  the  thrombotic  phenomenon.
Conclusion
This  Ivemark  syndrome  is  an  exceptional  case  because
it  has  been  revealed  by  a  massive  bilateral  prox-
imal  pulmonary  embolism.  The  malformations  classi-
cally  encountered  (pulmonary,  hepatic,  splenic,  intestinal
and  pancreatic)  are  associated  with  azygos  continu-
ity  with  agenesis  of  the  retrohepatic  inferior  vena
cava.  By  their  modifying  the  haemodynamics,  these
anatomical  variants  are  the  cause  of  the  thrombo-
sis.Disclosure of interest
The  authors  declare  that  they  have  no  conﬂicts  of  interest
concerning  this  article.
[ult  335
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Acute pulmonary embolism revealing Ivemark syndrome in an adult 

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Acute pulmonary embolism revealing Ivemark syndrome in an adult

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