Congenital Variants of Gastrointestinal Rotation Found at Resection of Hepatopancreatobiliary Tumors: A Case Series with Review of the Literature

Abstract

Background: Gastrointestinal malrotation arises from intrauterine events that occur early in the first trimester of gestation, and can result in a midgut volvulus that classically presents in the neonatal period with bilious emesis. Gastrointestinal malrotation can present clinically with symptoms such as chronic abdominal pain or bowel obstruction, or remain completely asymptomatic only to be discovered as an incidental finding much later in life during surgical exploration for other diseases. We sought to identify the prevalence of gastrointestinal malrotation in patients undergoing surgical exploration for hepatopancreatobiliary (HPB) malignancy and describe the operative considerations of these cases. Case Presentation: We performed a retrospective review of our prospectively acquired HPB surgery database from January 1, 2006, to December 1, 2013. We identified three cases of gastrointestinal malrotation out of a total of 1220 HPB cases reviewed, which represents 0.2%. We found two cases of gastrointestinal malrotation in the setting of pancreatic ductal adenocarcinoma and one case in the setting of cholangiocarcinoma. All three patients underwent exploratory laparotomy with resection of their respective primary tumors. We searched the English literature for cases of HPB malignancy in the setting of gastrointestinal malrotation. Conclusion: Our case series and review of the literature underscore the rarity and complexity of these cases.

A rare acute presentation of intestinal malrotation in the elderly

“…Midgut volvulus secondary to intestinal malrotation is a rare condition in the elderly with very few cases reported in literature. 3,[5][6][7][8][9][10][11][12][13][14] The first case in the elderly was probably reported in 1992 by Izes et al at the Lahey Clinic Medical Centre, USA. 15 Its rarity therefore portends a challenge in diagnosis and this mostly dependent on a high index of suspicion.…”

Duodenal Adenocarcinoma in a Patient with Partial Intestinal Malrotation

“…They found an incidence of 3 out of 1220 cases, or 0.2%. 6 In planning for these cases, the surgeon must be prepared to deviate from the classic surgical techniques utilized to ensure a safe resection.…”

“…For example, in two cases reported in Rittenhouse et al, the jejunal limb had to be brought up for the pancreaticojejunostomy and hepaticojejunostomy in a paracolic manner, as opposed to the standard retrocolic manner. 6 In our case, we were required to divide the proximal jejunum 60 cm below the ligament of Treitz to create a tension-free anastomosis. This is a much longer proximal limb than is typically required.…”