Preduodenal portal vein with situs inversus and duodenal atresia

Abstract

In a 7-day-old infant referred because of bile-stained vomiting, jaundice and lack of meconium, radiological examination revealed the 'double-bubble' sign of duodenal atresia as well as dextrocardia. This infant also had a strawberry haemangioma on the right shoulder. Operation disclosed situs inversus and a preduodenal portal vein as well as duodenal atresia. A side-to-side duodeno-jejunostomy was performed successfully without damage to the anomalous vein. The history of polyhydramnion during gestation, the presence of other anomalies, the rapid onset of bile-stained vomiting and the classic 'double-bubble' sign, together appeared to indicate that the duodenal atresia was intrinsic and not due to the external pressure of the anomalous vein on the duodenum.

Situs inversus abdominus in association with congenital duodenal obstruction: a report of two cases and review of the literature

“…The coexistence of situs inversus and congenital duodenal obstruction is extremely rare, with only 18 cases reported so far in the literature [4][5][6][7][8][9][10][11][12][13][14][15][16]. This report describes two newborns with situs inversus in association with congenital duodenal obstruction.…”

“…Among the various causes of duodenal obstruction reported in association with situs inversus, annular pancreas is the most common, reported in six patients, followed by duodenal web in four patients; preduodenal portal vein, stenosis, and complete duodenal atresia have also been reported singly or jointly [4][5][6][7][8][9][10][11][12][13][14][15][16].…”

“…The association of congenital duodenal obstruction and situs inversus is extremely rare, with only 18 cases reported so far in the literature [4][5][6][7][8][9][10][11][12][13][14][15][16]. Among the various causes of duodenal obstruction reported in association with situs inversus, annular pancreas is the most common, reported in six patients, followed by duodenal web in four patients; preduodenal portal vein, stenosis, and complete duodenal atresia have also been reported singly or jointly [4][5][6][7][8][9][10][11][12][13][14][15][16].…”

Preduodenal portal vein in association with midgut malrotation and duodenal web—triple anomaly?

“…Duodenal obstruction has been noted to be the most common clinical presentation in reported cases; although in less than half of these, PDPV was thought to be directly responsible for the duodenal obstruction. Most cases had associated anomalies that led to duodenal obstruction-duodenal atresia or web, annular pancreas, or anomalies of rotation and fixation [2][3][4][5][6][7][8][9]. Thus, it is prudent to rule out an intrinsic duodenal obstruction in all cases before PDPV is labeled as a cause for duodenal obstruction.…”

“…Later in development, the caudal and the cephalad anastomosis, with the caudal part of the right vitelline vein and the cephalad part of the left vitelline vein, disappear, leaving an S-shaped portal vein passing behind the duodenum. A variation in this process, in which the middle and the cephalad anastomosis disappear together with the left vitelline vein, accounts for the PDPV [1][2][3].…”