Extrahepatic intraductal biliary cystadenomas: Solving the mystery of “Idiopathic biliary obstruction”? A tertiary care institute experience

Abstract

Introduction: Biliary cystadenoma is a rare benign neoplasm of the liver with less than 200 cases being reported all over the world. The greatest challenge in the management of biliary cystadenoma lies in the pre-operative diagnosis, since it is most often misdiagnosed as simple liver cyst or hydatid cyst. We report a series of 12 cases highlighting the radiological findings and problems related to its management with special focus on intrahepatic biliary cystadenomas; a very unusual benign cause of obstructive jaundice and their management. Case Series: Records of Sugi R. V. Subramaniam1, Sugumar Chidambaranathan2, Senthil Kumar3, Prabhakaran Raju4, Jeswanth Sathyanesan5, Ravichandran Palaniappan6 Affiliations: 1Post Graduate Resident, Institute of Surgical Gastroenterology & Liver Transplant Govt. Stanley Medical College, Chennai, Tamil Nadu, India; 2Professor, Institute of Surgical Gastroenterology & Liver Transplant, Govt. Stanley Medical College, Chennai, Tamil Nadu, India; 3Assistant Professor, Institute of Surgical Gastroenterology & Liver Transplant, Govt. Stanley Medical College, Chennai, Tamil Nadu, India; 4Assistant Professor, Institute of Surgical Gastroenterology & Liver Transplant, Chennai, Tamil Nadu, India; 5Professor, Institute of Surgical Gastroenterology & Liver Transplant, Govt. Stanley Medical College, Chennai, Tamil Nadu, India; 6Professor& Head of Department, Institute of Surgical Gastroenterology & Liver Transplant, Govt. Stanley Medical College, Chennai, Tamil Nadu, India. Corresponding Author: Dr. Sugi R. V. Subramaniam, 601, Old Jail Road, Institute of Surgical Gastroenterology & Liver Transplant, Govt. Stanley Medical College, Chennai, Royapuram, Tamil Nadu 600001, India; Email:drrvsugi@ gmail.com Received: 23 May 2018 Accepted: 22 June 2018 Published: 19 July 2018 12 patients who underwent surgery for biliary cystadenomas, between 2013 and 2016, were reviewed and analysed retrospectively. Of the 12 patients with biliary cystadenomas, three patients had features of obstructive jaundice in the absence of any other recognized cause of biliary obstruction. Majority of the patients were females. The most frequent symptom was abdominal pain (92%); with obstructive jaundice seen in three patients (25%). All patients were subjected to routine blood investigations with Liver function tests and in addition Serum CA 19-9 was done. We had three patients who had protruding mass like lesion into the biliary tract causing obstruction to bile flow. MRI with MRCP was done in all patients before subjecting them to surgical exploration; who were managed with extrication of the growth along with enucleation/ resection. There has been no recurrence during the follow-up period ranging from six months to three years. All 12 patients underwent surgical management which included enucleation in four patients and non-anatomical liver resection in eight patients and some in addition required T-tube drainage of the bile duct. Totally 25 cases of Biliary Cystadenomas have been managed in our Institute till date with only three cases of Obstructive jaundice. Conclusion: In patients with obstructive jaundice, especially middle aged women, with the background of normal CA 19-9 levels and a polypodal mass projecting into the bile duct lumen intraductal biliary cystadenomas should be considered as a possible diagnosis. If feasible limited resection of the cyst with extrication of this benign lesion with close follow up and frequent imaging of the liver is advisable to locate early recurrences if any.