Benjamin R Biteman, MD, MS, Todd Ponksy, MD, FACS, Amelia Dorsey, MD. Akron Children’s Hospital.
Description of failure of rotation and fixation of the intestinal tract was first described in the 1920s. In 1936, Ladd and Gross wrote the article on treatment of the condition. Situs inversus is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. Its prevalence has varied but thought to be less than 1 in 10,000 people. There have been only 83 descriptions in the literature of preduodenal portal vein. Typically situs inversus is associated with malrotation. Many agree that operative intervention for malrotation in patients with situs inversus is only required for obstruction (from midgut volvulus or Ladd’s bands) or failure to thrive. We present a case of a 20 month old male with known situs inversus abdominus who presented at 12 months of life with intermittent vomiting and reflux. He was managed non-operatively for the following 8 months until he eventually had failure to thrive with 5kg weight loss. Consequently, recommendations were made to undergo a laparoscopic Ladd’s procedure and possible Nissen fundoplication, if there was no evidence of duodenal kinking. On laparoscopic operative evaluation, he was found to have a preduodenal portal vein, small bowel venous congestion and extensive peritoneal Ladd’s bands causing duodenal tortuositiy and kinking presumed to be the cause of his intermittent obstructive symptoms and failure to thrive. This video documents the Ladd’s procedure involving identification of the narrow mesenteric root, the duodenal course posterior to the preduodenal portal vein, straightening of the duodenum by lysis of Ladd’s bands, and appendectomy. The combination of situs inversus, malrotation and preduodenal portal vein make this description and video a one of a kind documentation of rarely needed intervention with unique anatomical variant.