Preduodenal Portal Vein Associated With Intestinal Malrotation as a Possible Cause of Intermittent Acholia.

Abstract

A 2 week old girl, born at 32 weeks gestational age and birth weight 1900 g, presented with occasional acholic stools. Abdominal examination was normal. Biochemical study revealed high-serum gamma-glutamyl transferase (GGT) levels (114 U/l [normal: 11--50 U/L]), normal alkaline phosphatase (252 U/L [150--400 U/L] and normal conjugated bilirubin (0.2 mg/dL [0--0.2 mg/dL]). Doppler ultrasound showed inversion of the superior messenteric vessels and a normal biliary system. Gastrointestinal contrast study revealed intestinal malrotation (Fig. 1). Exploratory laparoscopy confirmed a nonrotation of the midgut (malrotation type 1A) (1) and a preduodenal portal vein (PPV) causing partial obstruction (Fig. 2). After a Ladd bands procedure, laparotomy was necessary to perform an end-to-end duodenojejunal anastomosis anterior to the PPV. Stools became normal after the operation, and the GGT levels decreased to 25 U/L 3 months later.FIGURE 1: Gastrointestinal transit showing a duodenum with no rotation, which remains on the right of the midline with the rest of the small intestine.FIGURE 2: Intraoperative image showing a preduodenal portal vein (blue vessel loop) compressing the duodenum.PPV is an infrequent cause of duodenal obstruction (4%) (2). Three-fourths of them have a defective situs determination. Half of children remain asymptomatic. We, however, suspect its involvement in our patient intermittent acholia. On the other hand, intestinal malrotation has an incidence of 0.2% (3) and a PPV should be considered during its operative correction (4). Duodenoduodenostomy and duodenojejunostomy are the most described surgical treatments when this vascular anomaly leads to a complete or partial blockage (5).