Post Cholecystectomy Bile Leak From the Duct of Luschka in a Patient With Marfanʼs Syndrome

Abstract

Marfan's syndrome is a rare connective tissue disorder and has been associated with poor post-surgical wound healing. It has been associated with increased incidence of Cholelithiasis as compared to the general population. There is no literature regarding post-cholecystectomy complications in patients with Marfan's syndrome. We present a 29 year old female with Marfan's syndrome and a history of aortic dissection with aortic valve rupture and replacement with prosthesis. She was diagnosed with gallstone pancreatitis and Choledocholithiasis. Endoscopic retrograde cholangiogram showed a 20 mm stone in the common bile duct. Multiple endoscopic procedures were needed to accomplish removal of the large stone and these included papillotomy, stenting and mechanical lithotripsy. Laparoscopic cholecystectomy was attempted which had to be converted to laparotomy due to extensive scarring around the gallbladder. Intraoperatively, a bile leak was noted which on cholangiogram was found to be from a severed accessory Duct of Luschka. The leak was closed with suturing. Two weeks post-surgery, the patient developed right upper quadrant abdominal pain. Imaging showed a fluid collection in the gallbladder fossa. Nuclear hepatobiliary (HIDA) scan did not show a bile leak. Percutaneous drainage of the fluid collection was performed and 40 cc of green colored fluid was aspirated, analysis of which did not reveal any evidence of infection. After two weeks, she presented with severe chest pain radiating to the back. Her WBC count was increased at 21,000 and CT imaging at this time showed a larger fluid collection in the gallbladder fossa. Repeat HIDA scan did not show a bile leak. She underwent repeat percutaneous drainage and 20 cc of purulent material was aspirated. She was treated with antibiotics and discharged from the hospital. Follow up CT showed significant reduction in the size of the fluid collection. To our knowledge, this is the first reported case of post cholecystectomy bile leak in a patient with Marfan's syndrome which was from injury to the Duct of Luschka. Intraoperative ligation of the duct with suturing failed to control the leak leading to a Biloma and subsequent abscess. This may have been a result of poor wound healing around the suture. In patients with connective tissue disorders such as Marfan's syndrome, Endoscopic therapy of post-cholecystectomy bile leaks with trans-papillary biliary stenting may be considered.Figure 1Figure 2