Pancreatoduodenectomy with Segmental Resection of the Hepatic Artery in Distal Bile Duct Cancer with Situs Inversus Totalis

Abstract

Background: Situs inversus totalis is a rare congenital anomaly, and has reported incidence of 1 out of every 4000 to 10,000 individuals.1–6 Surgical procedures for these patients are technically demanding because of the completely reversed anatomical location and the associated potential anomalies.1–6

Materials and Methods: A 65-year-old man was admitted to our institution because of jaundice resulting from distal bile duct cancer. Preoperative image studies showed complete transposition of both thoracic and abdominal viscera and highly suspicious distal bile duct cancer. Associated anamoalies included the agenesis of ventral pancreas, anterior position of portal vein to the bile duct, preduodenal portal vein, and totally replacing hepatic artery from superior mesenteric artery. Unfortunately, some segment of the common hepatic artery was likely to be involved by the bile duct cancer.

Results: The surgeon stood on the right side of the patient as usual, and laparotomy was performed. Complete transposition of the abdominal viscera was found. After a reverse-Kocher maneuver, the portal vein and hepatic artery were identified. The ventral portion of the pancreas was absent. The gastroduodenal artery and inferior pancreaticoduodenal artery from the replacing hepatic artery were isolated and divided. The pancreas was taped above the origin of superior mesenteric artery for division. The portal vein was safely dissected from the bile duct and hepatic artery, but the common hepatic artery was noted to have been invaded by bile duct cancer. Pancreatoduodenectomy was performed with segmental resection of the hepatic artery and end-to-end anastomosis. The bile duct resection margin was reported not to be involved by malignant cells on the frozen section biopsy. After specimen delivery, pancreaticojejunostomy, hepaticojejunostomy, and duodenojejunostomy were made. The total operation time was approximately 540 minutes. The estimated amount of intraoperative bleeding was 750 mL. No blood transfusion was given. Postoperative pancreatic fistula (grade A) occurred. Postoperative follow-up axial image study showed no complication and good patency of the reconstructed hepatic artery. Pathologic examination reported an adenocarcinoma of the bile duct with moderate differentiation. The cancer extended to the pancreas and duodenal wall (pT3), but all resection margins were free from cancer cells. Twenty lymph nodes were retrieved, but not involved by malignant cells (pN0). Malignant cells have invaded the media layer of resected hepatic artery. The patient was able to go home on the 14th day postoperation. He is planning to undergo postoperative adjuvant chemoradiation therapy.

Conclusion: Pancreatoduodenectomy in patients with situs inversus totalis requires delicate surgical techniques and exact anatomic knowledge based on preoperative axial image studies due to potential associated vascular anomalies. Despite the difficulties, surgeons should avoid abandoning the goal of margin-negative oncologic surgery even in these complicated cases.