P1823 - Localized Caroli Disease With Cholangitis: A Diagnostic Approach Using MRCP

Bala Sai Teja Nuthalapati, MBBS¹, Srinivasulu Dudyala, MBBS, MD, DM², Fnu Rukhayya, MBBS³, Arushee Rajeev Pillai, MBBS⁴, Pratyush Sachdeva, ⁵, Dayana shre Narayana Swamy, MBBS⁶, Ann Mariam Stanley, MBBS⁷, Saikumar Kokkula, MBBS⁸, Sweta Sahu, MBBS⁹, Amukta Palakurthi, ^10
1Maheshwara Medical College, Hyderabad, Telangana, India; ²Sri Sri Holistic Hospitals, Hyderabad, Telangana, India; ³Dr. VRK Women's Medical College, Hyderabad, Telangana, India; ⁴Neuroglia Health Pvt Ltd, Bangalore, Karnataka, India; ⁵Punjab Institute of Medical Sciences, Vizag, Andhra Pradesh, India; ⁶Mysore Medical College and Research Institute, Mysore, Karnataka, India; ⁷Christian Medical College, Ludhiana, Punjab, India; ⁸Guntur Medical College, Guntur, Andhra Pradesh, India; ⁹JJM Medical College, Devanagere, Karnataka, India; ¹⁰Osmania General Hospital and Medical College, Visakhapatnam, Andhra Pradesh, India

Introduction: Caroli’s disease, a fibropolycystic congenital malformation, is classified in group V of Todani's classification of biliary tract cystic diseases. Localized forms of Caroli’s disease and non-classical CT scan signs of Caroli’s disease are extremely rare and are reported less due to their limited disease setting.

Case Description/Methods: A 61-year-old male came to the emergency department with fever and abdominal pain associated with chills, vomiting, anorexia and dark coloured urine for past couple of weeks and was known diabetic. He was icteric and examination revealed tenderness in the right hypochondrium and right iliac fossa with no rebound tenderness, guarding and with no discernible organomegaly. LFT revealed increased serum bilirubin (6.26 mg/dl, Direct - 3.89 mg/dl) with increased ALP and decreased protein (5mg/dl). Testing of Scrub Typhus-IgM, Leptospira-IgM, and IgG yield negative results. In view of above findings with features of cholangitis, Contrast Enhanced Computerized Tomography (CECT) abdomen was ordered which showed multiple small inflamed diverticuli at the cecum and ascending colon, mesenteric fat stranding at the inflamed diverticulum, thrombophlebitis of the ileocecal vein with air pockets suggestive of ceco-ascending colon diverticulosis and diverticulitis which was confirmed on colonoscopy. MRCP showed an ill-defined area of subcentimeter multifocal cystic dilatation of segmental intrahepatic biliary ducts in segments VI, VII, and VIII of the liver, suggestive of Type V choledochal cysts or Caroli’s disease. The patient started with Meropenem, Cefoperazone, Sulbactam, Ursodiol for 5 days. The patient became asymptomatic clinically with a gain of appetite and the liver function tests and other parameters came back to normal values and subsequently discharged.

Discussion: Caroli’s disease, an extremely rare disorder involving intrahepatic biliary ducts, is a congenital malformation resulting from the arrest of ductal plate remodeling during embryonic life leading to abnormal ductal plate configuration, and ductal ectasias. Our case did not have typical imaging features which made it challenging. Localized disease is most commonly complicated by intrahepatic lithiasis, which is not seen in our patient. Despite presenting with cholangitis, our patient did not have typical imaging features of central dot sign, representing the enhanced central fibrovascular bundle.



Disclosures:


Bala Sai Teja Nuthalapati, MBBS¹, Srinivasulu Dudyala, MBBS, MD, DM², Fnu Rukhayya, MBBS³, Arushee Rajeev Pillai, MBBS⁴, Pratyush Sachdeva, ⁵, Dayana shre Narayana Swamy, MBBS⁶, Ann Mariam Stanley, MBBS⁷, Saikumar Kokkula, MBBS⁸, Sweta Sahu, MBBS⁹, Amukta Palakurthi, ¹⁰. P1823 - Localized Caroli Disease With Cholangitis: A Diagnostic Approach Using MRCP, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.