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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    • "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.
    Indian Journal of Radiology and Imaging 04/2014; 24(2):156-9. DOI:10.4103/0971-3026.134401
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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.
    Korean journal of radiology: official journal of the Korean Radiological Society 11/2013; 14(6):905-913. DOI:10.3348/kjr.2013.14.6.905 · 1.57 Impact Factor
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    ABSTRACT: Preduodenal portal vein (PDPV) is a rare developmental anomaly. In infants, this is often associated with duodenal obstruction or biliary atresia. It is generally asymptomatic in adults (Ooshima et al., Hepato-Biliary-Pancreat Surg 5(4):455–458, 1998). Here, we report a singular case of adult PDPV that was discovered accidentally during emergency laparotomy for peritonitis due to gastric perforation in a 38-year-old lady. A plethora of congenital anomalies was uncovered, which is consistent with the reported cases of classical polysplenia syndrome, viz., multiple spleens of equal volume, visceral heterotaxia, right (Rt.)-sided stomach, a left (Lt.)-sided or a large midline liver, malrotation of intestine, a short pancreas, PDPV and IVC abnormalities (Gayer et al., Abdom Imaging 24:178–184, 1999). In addition, abnormalities like anamolous origin of Lt. gastric and splenic arteries from the abdominal aorta with absent celiac trunk, hepatic artery arising from the superior mesenteric artery, hepatic veins draining directly to Rt. atrium, etc. along with hypertrophic and lipomatous interatrial septum have also been detected during further investigations. As of now, we have come across 29 cases of adult PDPV reported in world literature and we are reporting our unique case with a review of literature on anomalies of visceral organs associated with PDPV.
    Indian Journal of Surgery 04/2014; 76(2). DOI:10.1007/s12262-013-0812-9 · 0.26 Impact Factor
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