P3613 - Primary Sclerosing Cholangitis in an Adolescent Presenting with Recurrent Acute Pancreatitis

Mohammed Abdulqader, MD¹, Pranav Patel, MD¹, Farrah Alarmanazi, MD², Maria Shaw, ³, Rose Barham, MD¹, David L. Diehl, MD^1
1Geisinger Health System, Danville, PA; ²St. George's University School of Medicine, Columbus, OH; ³Geisinger Commonwealth School of Medicine, Danville, PA

Introduction: Primary sclerosing cholangitis (PSC) is an idiopathic, chronic hepatobiliary disorder that rarely presents in the pediatric population. Magnetic resonance cholangiography (MRCP) can be used for diagnosis and endoscopic retrograde cholangiopancreatography (ERCP) should be considered if the MRCP is not conclusive. This case highlights a unique presentation of PSC in an adolescent with recurrent pancreatitis.

Case Description/Methods: A 17-year old male with recurrent pancreatitis (five episodes in 2 years) presented with acute epigastric pain radiating to his back similar to prior pancreatitis episodes. Labs revealed elevated lipase (1,933 U/L), GGT (412 U/L), ALT (182 U/L), AST (91 U/L), ALP (301 U/L) and total bilirubin (1.5 mg/dL). Computed tomography with contrast revealed edematous pancreatic parenchyma with surrounding free fluid, and focal intrahepatic biliary ductal dilation. MRCP showed an abrupt decrease in caliber of common bile duct at the level of entry to the pancreatic head and an irregular mildly dilated central left intrahepatic bile duct. There was no evidence of pancreas divisum ductal anatomy. ERCP showed multiple diffuse biliary strictures found in intrahepatic ducts and distal common bile duct consistent in appearance with PSC (Image 1). Two 10Fr plastic biliary stents were placed to remodel the distal common bile duct stricture. Further evaluation with autoimmune antibodies and immunoglobulin G subclasses ruled out overlap of autoimmune hepatitis, IgG 4 related cholangiopathy. A chronic pancreatitis genetic panel was also unremarkable. He is to follow-up with ERCP in 3 months for stent removal and colonoscopy is recommended to screen for inflammatory bowel disease.

Discussion: PSC is rare in the in pediatrics patient population. PSC causing distal common bile duct stricture and recurrent pancreatitis is very rare, and has not been previously reported in the pediatric age group. Serological evaluation in this patient appeared to exclude autoimmune pancreatitis and IgG4 associated cholangitis/PSC overlap. In the medical literature, autoimmune hepatitis overlap with PSC has been described, however autoimmune work up was unremarkable in this patient. PSC in pediatric age group should also be evaluated for immune deficiency syndrome, genetic diseases to exclude secondary causes of sclerosing cholangitis. This patient is potentially at risk of progression of PSC requiring young age liver transplantation.



Disclosures:


Mohammed Abdulqader, MD¹, Pranav Patel, MD¹, Farrah Alarmanazi, MD², Maria Shaw, ³, Rose Barham, MD¹, David L. Diehl, MD¹. P3613 - Primary Sclerosing Cholangitis in an Adolescent Presenting with Recurrent Acute Pancreatitis, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.