An Unexpected Cause of Obstructive Jaundice: A Case of Lemmelʼs Syndrome

Abstract

An 83 years old female was evaluated at the ED presenting with abdominal pain for two weeks, anorexia, nausea, fatigue, dark urine and yellowing of the skin. Her past medical history included hypertension, dyslipidemia, major depressive disorder and prior cholecystectomy. Her medication profile included losartan 50mg/HCTZ 12.5mg and Sertraline 50mg. She had a family history of pancreatic and breast adenocarcinoma in a sibling. Physical examination was remarkable for jaundice, scleral icterus, transverse right upper quadrant abdominal surgical scar, with mild epigastric tenderness upon palpation. Laboratory work-up revealed WBC 6.28, AST 725IU/L, ALT 1059IU/L, alkaline phosphatase 130IU/L, amylase 53IU/L, lipase 23IU/L and bilirubin 30mg/dL. Contrast enhanced abdominal CT revealed an outpouching along the second portion of the duodenum, compatible with a periampullary duodenal diverticulum, resulting in biliary dilatation with extensive perihepatic edema. Endoscopic retrograde cholangiopancreatography(fig.1) confirmed a large, diverticulum, 0.5cm from the ampulla of Vater with debris(fig.2), without evidence of choledocholithiasis or pancreatic abnormalities. Endoscopic sphincterectomy was performed and clinical recovery was observed. Diverticula of the gastrointestinal tract are outpouchings of all or part of the intestinal wall. The duodenum is the second most common site of diverticula in the gastrointestinal tract. Among duodenal diverticula, periampullary diverticula (PAD) are the most common type comprising about 70% to 75% of all duodenal diverticula. PAD develops within the radius of 2 to 3 cm from the ampulla of Vater. Most PAD are asymptomatic but complications can occur in about 5% of cases. Rarely, duodenal diverticula may present with obstructive jaundice, in the absence of cholelithiasis or other detectable cause, known as Lemmel's syndrome. Imaging studies such as contrast enhanced abdominal CT and MRCP may be suggestive of Lemmel's syndrome; however, direct visualization by ERCP remains the gold standard for diagnosis. Diverticulectomy is standard of care for treatment, nonetheless there are additional options such as papillary balloon dilatation, conservative medical management or endoscopic sphincterectomy, as in our case. Knowledge of such condition is important, since misdiagnosis could lead to delay in treatment. Lemmel's syndrome should always be included in the differential diagnosis of obstructive jaundice in a patient with PAD.Figure: ERCP showing biliary dilatation.Figure: ERCP showing periampullary diverticula with debris.