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.

Keywords: Anomalous origin of arteries; Heterotaxy; IVC abnormality; PDPV in adult; Pancreatic agenesis; Polysplenia.

Fig. 1

Anatomy that was found upon releasing the band

Fig. 2

Site of perforation

Fig. 3

Additional splenic nodule found in mesentery with left lobe of the liver in left subdiaphragmatic area

Fig. 4

Pancreatic head and a small part of the body were visible

Fig. 5

Demonstrating PDPV, Rt. sided stomach, two spleens and short pancreas

Fig. 6

IVC on the Rt. lower abdomen and continues above with prominent azygos vein

Fig. 7

The occurrence of PDPV can be explained by the embryonic development of Portal vein from the vitelline veins

Fig. 8

Distribution of associated anomalies

Fig. 9

Incidence of associated anomalies