Polysplenia syndrome with agenesis of the dorsal pancreas and preduodenal portal vein presenting with obstructive jaundice: A case report and literature review

Department of Radiology, St Vincent's Hospital, Darlinghurst, NSW, Australia.
The British journal of radiology (Impact Factor: 2.03). 11/2011; 84(1007):e217-20. DOI: 10.1259/bjr/27680217
Source: PubMed


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 cholangiopancreatography.

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Available from: Joga Chaganti, Sep 18, 2015
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    • "In infants and children, abdominal ultrasound is a reasonable modality by which to assess splenic anatomy (Figure 3)[29]. In situations where there is inadequate visualization by ultrasound, or in adults, computed tomography is a viable alternative (Figure 4)3031. "

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    • "It has been reported to occur with very rare conditions including heterotaxy[2] and polysplenia[5] syndrome. The possible explanation for this is the close proximity of the developing pancreas with spleen in the dorsal mesogastrium.[6] It is also associated with congenital heart defects like septal defects, tetralogy of Fallot, or pulmonary artery stenosis.[7] "
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    ABSTRACT: The morphogenesis of the pancreas is a complex process having a very low frequency of anatomic variation. The congenital anomalies are rare. Complete pancreatic and ventral pancreatic agenesis are incompatible with life. Dorsal pancreatic agenesis is exceedingly rare with less than 100 cases reported in the world literature. Patients with this anomaly may be asymptomatic or may present with abdominal pain, hyperglycemia, diabetes mellitus, and acute or chronic pancreatitis. Such anomalies are rarely reported; therefore, clinical awareness of agenesis of the dorsal pancreas as a cause of these symptoms can expand the differential diagnosis and improve patient management.
    Full-text · Article · Apr 2014 · Indian Journal of Radiology and Imaging
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    • "Hypoplasia (partial agenesis) results from the absence of the ventral or dorsal pancreatic anlage. Dorsal pancreatic agenesis (short pancreas) may be an isolated finding but has also been reported as a part of heterotaxia syndromes (Fig. 14) (15). In complete dorsal agenesis, the anterior head, neck, body and tail of the pancreas, the duct of Santorini and the minor papilla are absent. "
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    ABSTRACT: Though congenital anomalies of the pancreas and pancreatic duct are relatively uncommon and they are often discovered as an incidental finding in asymptomatic patients, some of these anomalies may lead to various clinical symptoms such as recurrent abdominal pain, nausea and vomiting. Recognition of these anomalies is important because these anomalies may be a surgically correctable cause of recurrent pancreatitis or the cause of gastric outlet obstruction. An awareness of these anomalies may help in surgical planning and prevent inadvertent ductal injury. The purpose of this article is to review normal pancreatic embryology, the appearance of ductal anatomic variants and developmental anomalies of the pancreas, with emphasis on magnetic resonance cholangiopancreaticography and multidetector computed tomography.
    Full-text · Article · Nov 2013 · Korean journal of radiology: official journal of the Korean Radiological Society
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