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A Rare Cause of Congenital Duodenal Obstruction: Preduodenal Portal Vein
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Preduodenal portal vein is a rare and interesting entity which often causes duodenal obstruction. It is also associated with other congenital anomalies. We report here three cases of preduodenal portal vein associated with other anomalies causing duodenal obstruction not related to direct compression by portal vein itself
We had encountered a very rare congenital anomaly of a preduodenal portal vein while doing laparoscopic cholecystectomy. More significant in our case is that this preduodenal vein is hiding behind omental adhesions with the gallbladder. We want to raise awareness about this very rare anomaly. Recognition and knowledge of this anomaly will prevent the potential of a serious operative misadventure.
We report a rare case of 10-month-old female who underwent living donor liver transplantation (LDLT) for syndromic biliary atresia with preduodenal portal vein (PV) and its severe stricture owing to the previous Kasai portoenterostomy. Because we successfully performed “left at right liver transplantation (LAR-LT) and graft rerotation” in this case, we are present tips and pitfalls for this operation.
Preoperative computed tomography scan showed that her preduodenal PV was stenotic from the confluence of the superior mesenteric vein and splenic vein to hepatic hilum, which made us consider the necessity of ≥3 cm interposition vein graft to complete a safe PV anastomosis. To reduce a gap between donor and recipient's PV, we decided to put a left lateral section graft at the right subphrenic space called left-at-right liver transplantation. Thus, LDLT was performed with an identical lateral sectional graft from her father. After total hepatectomy, we implanted a graft in her right subphrenic space, and anastomosed the donor left hepatic vein to her inferior vena cava. Then, we anastomosed an interposition graft harvested from her left internal carotid vein to her PV.
Even after reflowing PV flow, because the duodenum compressed the interposition vein graft, PV flows were totally insufficient. Therefore, we flipped a liver graft 180° from right to left upper abdominal cavity, which could reduce the gap between PVs and acceptable PV flow was obtained.
In the present case, LAR-LT could reduce the distance of PVs. In addition, our rerotation method could be useful to alleviate tension on the PV anastomosis caused by preduodenal PV.
Preduodenal portal vein is rare, with 63 cases reported in the literature. In general, this anomaly occurs in children with associated small bowel obstruction. We report a newborn infant who presented with duodenal stenosis, mongolism, and preduodenal portal vein. Treatment consisted of a duodenoduodenal anastomosis without mobilizing the portal vein. The correlation between imaging techniques and the operative findings is discussed. Because identification of preduodenal portal vein at surgery is important, preoperative sonography may be useful in selected cases to define the position of the vein.
A preduodenal position of the portal vein is a rare anomaly reported in 52 cases in the literature. Two additional cases are reported here. In 54% of the cases of preduodenal portal vein (PPV) was combined with high intestinal obstruction and in one half of these cases PPV was considered obstructive. A critical review of these cases given the impression that obstructive PPV could well be a misinterpretation of the obstructive situation and the existence of obstructive PPV is doubted.
Preduodenal portal vein (PDPV) is a rare anomaly described in only 82 patients in the literature. In a few patients, the PDPV was the direct cause of duodenal obstruction. The authors have treated a newborn with PDPV associated with total situs inversus, intestinal malrotation and polysplenia who presented clinically with duodenal obstruction.