Polysplenia syndrome with agenesis of the dorsal pancreas and
preduodenal portal vein presenting with obstructive jaundice—a
case report and literature review
1J P LOW, BMBS,2D WILLIAMS, FRANZP, MBBS and1J R CHAGANTI, FRANZCR, MD
1Department of Radiology and2Department of Gastroenterology, St Vincent’s Hospital, Darlinghurst, NSW, Australia
ABSTRACT. Polysplenia, as part of the heterotaxy syndrome, is a rare embryological
disorder which results from failure of development of the usual left–right asymmetry of
organs. It is often associated with cardiac and biliary abnormalities, which are the usual
causes of death in early neonatal life. A congenitally short pancreas and abnormalities
with portal vein formation, gut malrotations and inferior vena cava anomalies are
known to be associated with this rare syndrome. We report a case of polysplenia in an
adult female presenting with obstructive jaundice owing to choledocholithiasis,
possibly formed by biliary stasis as a result of compression of the common bile duct by
the preduodenal portal vein, and review the literature. The patient was also found to
have complete agenesis of the dorsal pancreas on CT and endoscopic retrograde
Received 27 October 2010
Revised 15 December 2010
Accepted 22 December
’ 2011 The British Institute of
A 62-year-old woman with a medical history of breast
cancer, hypertension and paroxysmal atrial fibrillation
presented with nausea, vomiting and abdominal pain.
Blood tests revealed an obstructive pattern with raised
alkaline phosphatase and elevated conjugated bilirubin.
She underwent CT which revealed a midline liver and
multiple splenunculi to the left of the midline (Figure 1).
CT also demonstrated agenesis of the pancreatic tail and
lateral part of the body (Figure 2). The inferior vena cava
(IVC) was to the left of the midline and crossed to the
right at the level of the diaphragmatic hiatus and joined
was deemed consistent with polysplenia in heterotaxy
It was also noted that the gallbladder was significantly
dilated with sludge. There was evidence of a high
attenuating, well-circumscribed rounded lesion, which
was a suspected calculus in the suprapancreatic common
bile duct (CBD). This caused mild dilation of the
intrahepatic bile ducts.
MRI and magnetic resonance cholangiopancreatogra-
phy (MRCP) were performed to further evaluate the
cause of biliary obstruction. MRCP confirmed a calculus
measuring 11612 mm in the suprapancreatic CBD. It
also showed complete absence of the dorsal pancreatic
duct (Figure 5). Both MRI and CT demonstrated a
preduodenal portal vein which was seen to be causing
compression of the suprapancreatic segment of the CBD
with a calculus proximal to compression (Figures 6 and 7).
The patient underwent laparoscopic cholecystectomy
Polysplenia is a rare heterotaxy disorder with a
reported incidence of 1 per 250000 live births. Various
studies have attempted to classify the broad spectrum of
anomalies into asplenia and polysplenia . Classical
polysplenia results in hyparterial bronchi (both main
Address correspondence to: Dr Julia Low, Department of Radio-
logy, St Vincent’s Hospital, Victoria Street, Darlinghurst, NSW,
2010, Australia. E-mail: email@example.com
Figure 1. Axial post-contrast CT demonstrating a midline
liver (ML), small multiple splenunculi (arrows) and a dilated
common bile duct.
The British Journal of Radiology, 84 (2011), e219–e222
The British Journal of Radiology, November 2011e219
bronchi are below the pulmonary arteries) and bilobed
lungs. Asplenia results in trilobed lungs and eparterial
bronchi (both main bronchi are located superior to the
main pulmonary arteries).
The precise aetiology of polysplenia is unknown.
Embryonic, genetic and teratogenic components have
all been implicated as causative factors in polysplenia .
Although polysplenia syndrome has a wide range of
abnormalities, there is no single pathognomic abnorm-
ality that characterises this rare entity. The range of
anomalies include multiple spleens of equal volume,
visceral heterotaxia, right-sided stomach, a left-sided or
large midline liver, malrotation of the intestine, a short
pancreas and IVC anomalies .
Polysplenia has been described mainly in childhood
owing to critical anatomic malformations related to
cardiac defects or biliary atresia. Only those with mild
anatomical abnormalities survive into adulthood. Sym-
ptomatic polysplenia in adults is often caused by abnor-
mal biliary and pancreatic duct drainage, cholecystisis
and bowel obstruction . Polysplenia is usually an
incidental finding on abdominal ultrasound or CT per-
formed for other causes.
In our patient there were multiple small spleens along
the greater curvature of the stomach, a midline liver and
bilateral hyparterial bronchi. We also noted dorsal pan-
creatic agenesis and a preduodenal portal vein.
Anomalies of the pancreas have been described in
polysplenia syndrome . Normal pancreas formation
occurs from fusion of ventral and dorsal pancreatic buds.
The ventral pancreatic bud gives rise to the uncinate
process and the head, while the dorsal pancreatic bud
gives rise to the body and tail. The development of both
dorsal pancreatic bud and spleen occur in the dorsal
mesogastrium. Consequently, anomalies in both these
organs can be expected in patients with polysplenia
Clinical significance of dorsal pancreatic agenesis is
the development of pancreatitis owing to poor drainage
from the remnant ventral duct. CT demonstration of a
short pancreas is not synonymous with agenesis and is
a pitfall well avoided. Fat replacement in the distal
pancreas can mimic agenesis. Similarly partial vs com-
plete dorsal duct agenesis can only be differentiated by
pancreatic duct studies using either MRCP or endoscopic
Presence of preduodenal portal vein was seen in 12
out of 15 patients in 1 large review . First described
by Knight in 1921, the preduodenal portal vein is a
congenital anomaly that involves the portal vein passing
in front of the duodenum . Preduodenal portal vein
can be associated with duodenal atresia, stenosis, annular
pancreas and malrotation. Surgery may be required for
these conditions. A preduodenal portal vein anomaly
occurs owing to loss of vitelline veins in their cranial and
middle communication during 9 mm stage of embryonic
Figure 3 (a) Axial post-contrast CT demonstrating preduodenal portal vein (white arrow), a dilated common bile duct proximal
to the preduodenal portal vein (black arrow) and left-sided inferior vena cava (IVC). (b) Axial maximum intensity projections of a
normal control volunteer showing the portal vein (black arrow) relationship to the first part of the duodenum (*). Note the
portal vein traversing posterior to the first part of duodenum to enter the porta hepatis.
Figure 2. Axial post-contrast CT at a lower level demonstrat-
ing absence of pancreatic body and tail, right–sided stomach
and left–sided inferior vena cava (IVC). AO, aorta; PV, portal
vein; *absence of pancreatic body.
J P Low, D Williams and J R Chaganti
e220 The British Journal of Radiology, November 2011
development. In normal development, the loss of caudal
and cranial communications result in the development of
a normal ‘S’ shaped portal vein.
We postulate that formation of calculus was caused by
stasis of bile in the CBD owing to distal compression of
the CBD by the preduodenal portal vein. Similar opinion
was also expressed by Seo et al . When surgery is
required care should be exercised in patients with pre-
duodenal portal vein. Lack of awareness may lead to
unwanted ramifications such as severe haemorrhage and
other complications related to portal vein ligation.
The diagnosis of polysplenia in adults is usually
made during investigation for unrelated causes. Biliary
drainage abnormalities could result owing to the altered
course of the portal vein in these syndromes. Increased
awareness of such anatomical anomalies would prevent
serious complications during abdominal surgery. Pan-
creatic duct anomalies have been described but are rare
in polysplenia syndromes. We have reported one such
Figure 5. Three-dimensional magnetic resonance cholangio-
pancreatography reconstruction study demonstrating a
calculus (*) in the proximal common bile duct with absence
of the dorsal pancreatic duct.
Figure 6. T2 fat saturated axial MR study demonstrating
compression of the common bile duct (CBD) (white arrow) by
the preduodenal portal vein (arrow head). Note the calculus
is posterior to the CBD and cannot be visualised.
Figure 7. Coronal reformatted CT demonstrating preduo-
denal portal vein (black arrow) causing compression of the
suprapancreatic common bile duct (white arrow).
Figure 4. Coronal reformatted CT demonstrating bilateral
morphological left lungs. Note the bilateral hyparterial
bronchi—both of the main bronchi (black arrows) are below
the pulmonary arteries (PA).
Case report: Polysplenia syndrome with agenesis of the dorsal pancreas
The British Journal of Radiology, November 2011e221
unusual presentation of polysplenia syndrome present- Download full-text
ing in older age with obstructive jaundice and both
preduodenal portal vein and dorsal pancreatic agenesis.
1. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs
revisited: imaging of the heterotaxy syndrome. Radio-
graphics 1999;19:837–52; discussion 853–4.
2. De la Monte SM, Hutchins GM. Sisters with polysplenia. Am
J Med Genet 1985;21:171–6.
3. Gayer G, Apter S, Jonas T, Amitai M, Zissin R, Sella T, et al.
Polysplenia syndrome detected in adulthood: report of eight
cases and review of the literature. Abdom Imaging 1999;
splenia/heterotaxy syndrome: a novel association with aortic
coarctation and a review of the literature. JOP 2007;8:433–7.
5. Maier M, Wiesner W, Mengiardi B. Annular pancreas and
agenesis of the dorsal pancreas in a patient with polysplenia
syndrome. AJR Am J Roentgenol 2007;188:W150–3.
6. Knight HO. An anomalous portal vein with its surgical
dangers. Ann Surg 1921;74:697–9.
7. Seo HI, Jeon TY, Sim MS, Kim S. Polysplenia syndrome
with preduodenal portal vein detected in adults. World
J Gastroenterol 2008;14:6418–20.
J P Low, D Williams and J R Chaganti
e222 The British Journal of Radiology, November 2011