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

BACKGROUND: A combination of intestinal malrotation and distal cholangiocarcinoma is considered a rare condition and poses some difficulties in surgical management. We present a case of a patient with asymptomatic nonrotation of the midgut with a concomitant distal cholangiocarcinoma who underwent successful pancreaticoduodenectomy. CASE PRESENTATION: A 52-year-old Sudanese man presented to our hospital with progressive painless jaundice associated with dark urine, pale stool, and itching for the last 2 months. He had no other complaint or significant previous medical history apart from being an ex-smoker. His clinical examination revealed a palpable gallbladder and scratch mark. His other systems were unremarkable. His blood test results showed a normal complete blood count, elevated total bilirubin (mainly direct bilirubin), elevated alkaline phosphatase, and normal cancer antigen 19-9 and carcinoembryonic antigen. Ultrasound, computed tomography of the abdomen, and magnetic resonance cholangiopancreatography showed a dilated intrahepatic and extrahepatic biliary system down to the distal part, where the lumen was obstructed by a periampullary mass measuring 2.4 by 2.1 cm. The patient’s gallbladder was distended. He had no liver metastases or ascites and few lymph nodes. Inversion of the superior mesenteric artery and superior mesenteric vein but no invasion was seen, and malrotation of the bowel was observed with the large bowel on the left side and the small bowel to the right of the abdomen. Endoscopic retrograde cholangiopancreatography showed a fleshy ampulla that was stented. Laparotomy showed malrotation, with the duodenum straight on the right side of the midline, and Ladd’s band crossed the second portion of the duodenum. The vessels were approached from the lateral side meticulously after kocherization of the duodenum and pancreas, dissection along an extended portion of the superior mesenteric artery to assure preservation of the superior mesenteric artery and branches going to the jejunum, Ladd’s procedure, division of the jejunum 10 cm below the uncinate process of pancreas, and modified pancreaticoduodenectomy were performed, and anastomoses were performed in the standard fashion. The patient had an uneventful postoperative course, started oral feeding after 5 days, and discharged to home on day 10 for regular follow-up. Histopathology confirmed distal cholangiocarcinoma, and the patient was referred for further oncological management. CONCLUSIONS: Pancreaticoduodenectomy can be safely performed in patients with intestinal malrotation with some modifications of the standard approach. Meticulous dissection after preoperative identification of vascular anomaly and a lateral approach are of great help to reduce morbidity.