Pancreaticoduodenectomy in Adults With Congenital Intestinal Rotation Disorders

Abstract

Congenital intestinal malrotation is a developmental anomaly resulting from interruption of the physiological herniation and return to the abdominal cavity of the midgut during the 6th to 10th week of embryological development. Normal vascular and anatomic relationships used as landmarks during pancreaticoduodenectomy (PD) are altered in patients with congenital malrotation. We present 3 cases of PD in adults with congenital intestinal rotation disorders. Three adult patients with congenital rotational disorders required PD. Two of these patients had bilio-pancreatic tumors, and 1 cadaveric donor underwent total pancreatectomy during pancreas allograft procurement. All patients had arterial and venous anomalies around the celiac trunk and mesenteric vessels, respectively. The midgut and hindgut in each case were shifted toward opposite sides of the abdominal cavity. Modifications to the standard approach to PD were made, and outcomes were favorable in each case. Each patient showed anatomic abnormalities with the need for identifying vascular structures through their expected (or projected) course and location before parenchymal division or ligation of any vessel. This approach becomes crucial in cases of vascular anomalies, such as ones occurring in congenital malformations, and can be used in similar situations encountered during pancreaticoduodenectomy.

Case report of intestinal non-rotation, heterotaxy, and polysplenia in a patient with pancreatic cancer

“…[ 11 ] Additionally, the hepatic artery originating from the SMA has been reported in multiple cases. [ 4 , 12 , 13 ] Suprarenal IVC interruption with azygos continuation, as observed in our patient, is also a common abnormality. [ 14 ] The presence of a preduodenal portal vein is associated with polysplenia.…”

Intestinal malrotation associated with colon cancer in an adult: Report of a case

“…11,20 Mateo et al reported one case of intestinal malrotation with an ampullary villous adenoma. 21 It is not single in this case. Gardner-Thorpe et al also reported a case of 66-year-old woman with congenital rotational abnormalities accompanied with a 9-cm ampullary villous adenoma.…”

“…Although there is no direct evidence for the relationship between a congenital abnormality and carcinogenesis, several studies may provide valuable clues to support the speculation that intestinal malrotation in adults is a possible underlying cause of pancreatitis and pancreatic tumors listed in Table 3. [21][22][23] Midgut volvulus in adults usually results in vascular compromise, and chronic intestinal ischemia and infl ammation. 14 Chronic infl ammation is one of factors associated with the formation of several malignant tumors.…”

Pancreaticoduodenectomy in a patient with intestinal malrotation and distal cholangiocarcinoma: a case report and review of the literature

“…Intestinal malrotation occurs during the sixth to tenth weeks of embryological development. It affects the position and peritoneal attachments of the bowel with alteration in vascular and anatomic relationships [1,2], and it has three stages: nonrotation (absent), incomplete rotation, and malfixation [2,3]. Cholangiocarcinoma (CCA) is a heterogeneous group of tumors derived from cells of the biliary tree that represent the second most frequent primary liver tumor [4].…”