Larkin Community Hospital South Miami, FL, United States
Alok Shrestha, DO1, Monica Multani, DO1, George Shenouda, DO1, Andrew Dam, DO2, Melissa Matheus, MD3, Andrew Shenouda, BSc4, Amit Sastry, MD3, Ilan Rzadkowolsky-Raoli, MD3, Karthik Mohan, DO1 1Larkin Community Hospital, South Miami, FL; 2Larkin Community Hospital, Hialeah, FL; 3Nova Southeastern University, Palmetto Hospital, Hialeah, FL; 4Trinity School of Medicine, Roswell, GA
Introduction: Gallbladder agenesis (GA) is a rare disorder affecting approximately 0.01 to 0.065% of live births. We present the first case of GA and anomalous pancreaticobiliary junction (APBJ) with chronic choledocholithiasis and secondary biliary cirrhosis (SBC).
Case Description/Methods: A 76 year old male with no significant medical or surgical history presented to the hospital with RUQ abdominal pain, jaundice, and weight loss for several months. Initial workup noted lipase of 1,489 U/L, total bilirubin 7.30 mg/dL, and elevated liver enzymes in a cholestatic pattern. Abdominal ultrasound showed a possible contracted gallbladder. MRCP demonstrated intra and extrahepatic ductal dilatation with multiple CBD stones and evidence of APBJ. An ERCP was unsuccessful in removing the stones. Subsequently, an open surgical CBD exploration was performed removing the proximal stones but was unable to extract a large distal stone requiring a Roux-en-Y hepaticojejunostomy. No gallbladder was present in segment 4B of the liver but a small sheet of pink tissue was found. Biopsy of this tissue was reported as liver parenchyma confirming GA. Intraoperatively, the pancreas had a diffusely firm texture and the liver appeared to have micronodular changes. ANA and AMA levels were within normal limits and all surgical specimens were negative for malignancy. However, excisional and core needle liver biopsies revealed chronic periportal inflammation with cirrhosis.
Discussion: GA is caused by an abnormal development of vessels of the gallbladder and is often asymptomatic. However, it may be symptomatic in 23% of cases with jaundice (48%), biliary stones (26%), and pancreatitis (6%). In our case, a sustained extrahepatic obstruction and cholestasis from chronic choledocolithiasis likely caused the progression to biliary cirrhosis. Although the biliary ductal dilations were likely caused by chronic obstruction, choledochal cysts cannot be completely ruled out, which may present with APBJ in 60-64% of the cases. Intraoperative findings also suggested possible chronic pancreatitis, further supporting the likelihood of longstanding choledocholithiasis with recurrent insults to the pancreas. Chronic obstruction can lead to destruction of the intrahepatic ducts and predispose to recurrent cholangitis, stone formations, and exacerbation of cholestasis. GA is associated with several biliary anomalies and increases the risk for choledocolithiasis and hepatobiliary complications including SBC.
Figure: A & B. Magnetic resonance cholangiopancreatography (MRCP) without contrast demonstrates intrahepatic and extrahepatic ductal dilatation without the presence of the gallbladder (GB). Multiple filling defects in the dilated common bile duct (CBD) consistent with choledocholithiasis and an abnormal union between the pancreatic duct (PD) and CBD with a long dilated common channel, consistent with anomalous pancreaticobiliary junction (APBJ) is seen. C. Endoscopic retrograde cholangiopancreatography (ERCP) shows multiple filling defects in the dilated CBD consistent with choledocholithiasis. PD is not visualized with contrast, likely from obstructing stone or acute angle of the PD. D. Intraoperative image shows segment 4B of the liver with a thin rim of the pink colored tissue (yellow arrow) in the gallbladder fossa.
Disclosures: Alok Shrestha indicated no relevant financial relationships. Monica Multani indicated no relevant financial relationships. George Shenouda indicated no relevant financial relationships. Andrew Dam indicated no relevant financial relationships. Melissa Matheus indicated no relevant financial relationships. Andrew Shenouda indicated no relevant financial relationships. Amit Sastry indicated no relevant financial relationships. Ilan Rzadkowolsky-Raoli indicated no relevant financial relationships. Karthik Mohan indicated no relevant financial relationships.
Alok Shrestha, DO1, Monica Multani, DO1, George Shenouda, DO1, Andrew Dam, DO2, Melissa Matheus, MD3, Andrew Shenouda, BSc4, Amit Sastry, MD3, Ilan Rzadkowolsky-Raoli, MD3, Karthik Mohan, DO1. P2173 - A Case of Gallbladder Agenesis and Anomalous Pancreaticobiliary Junction With Chronic Choledocholithiasis and Secondary Biliary Cirrhosis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.