Preduodenal Portal Vein in Adult with Polysplenia Syndrome Revisited with a Case Report

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.

Left Isomerism (Polysplenia Syndrome)

“…Polysplenia (IS) can lead to pathogenic abnormalities of internal organs, in some cases a large number of accessory spleens and splenic nodules being reported, with apparently inexplicable clinical presentation and pseudo-chaotic association of semiological phenomena (4). This fact can be a factor of unpredictability in the case of emergency surgery, especially due to the association of gastric dextroposition, a liver located medially or in the left hypochondrium; at the same time, the agenesis of the celiac trunk, the origin of the hepatic artery at the level of the superior mesenteric artery, the swelling of the hepatic veins at the level of the right atrium, but also cardiac abnormalities such as hypertrophy and lipomatosis of the interatrial septum can predispose these patients to organ pathologies at an early age (5). Usually, most cases (40%) are discovered by adulthood, they manage to pass the age of 2 years this majority dying before 5 years.…”

Asymptomatic left isomerism with preduodenal portal vein: computed tomography appearance

“…There is no single pathognomonic abnormality that characterizes LI [ 1 ]. In the abdomen, polysplenia, a short pancreas, abnormalities of the portal vein formation, various types of gut malrotations and inferior vena cava anomalies are the most common associated anomalies [ 1 , 2 , 7 – 9 ] apart from the specific thoracic variations: Polysplenia is characterized by the presence of several spleens, usually between two and six. They are about of equal size, associated with the main spleen in the left hypochondrium and vascularized by an artery with normal birth.…”

“…Polysplenia must be differentiated from supernumerary spleens (which generally measure only 1–2 cm) and from splenosis [ 3 ]. Congenital preduodenal portal vein (PDPV), often present (75%) in LI, is characterized by a portal vein passing in front of the duodenum [ 3 , 7 – 11 ]. PDPV is rare as an isolated variation (25%) [ 4 , 11 ].…”

“…In childhood, PDPV may be associated with features of intestinal obstruction caused by coexisting congenital anomalies such as duodenal atresia, duodenal web, annular pancreas, intestinal malformation, biliary atresia, etc. [ 7 ]. Surgery may be required for these obstructive conditions.…”