Lemmelʼs Syndrome: A Rare Cause of Obstructive Jaundice

Abstract

Periampullary diverticula (PAD) are extraluminal outpouchings of duodenal mucosa that are usually asymptomatic and incidental findings during endoscopy. When obstructive jaundice develops secondary to a PAD in the absence of choledocholithiasis or tumor, it is termed Lemmel's syndrome. Since being first described in 1934, Lemmel's syndrome represents and rare clinical entity described only infrequently through case reports. A 62 year old female with a history of diabetes and prior cholecystectomy presented with a two week history of nausea, vomiting, and abdominal pain. On presentation, the patient was afebrile with mild tachycardia with exam revealing right upper quadrant tenderness. Laboratory investigation revealed WBC 16.3, AST 415IU/L, ALT 880IU/L, alkaline phosphatase 226IU/L, bilirubin 4.7mg/dL, and lipase 4679 IU/L. Contrast CT of the abdomen showed a prominent diverticulum in the second portion of the duodenum with fat stranding and surrounding edema. Endoscopic ultrasound was performed and showed diffuse periampullary wall thickening (Figure 1), 11cm common bile duct without evidence of choledocolithiasis, and no pancreatic abnormalities. Endoscopy directly visualized a large periampullary diverticulum compressing the ampulla with impacted food and diffuse wall thickening suggestive of diverticulitis (Figure 2). Food and pus was lavaged endoscopically and clinical recovery was seen. Duodenal diverticula are outpouchings of intestinal wall and are often found in the periampullary region. Although PAD are thought to be largely asymptomatic and incidental findings, rare complications including Lemmel's syndrome have been reported. Diagnosis of Lemmel's syndrome is based on the visualization of duodenal diverticulum obstructing the ampulla in the absence of other explanation for obstructive jaundice. Imaging may be suggestive of Lemmel's syndrome, however, ERCP remains the method of choice for diagnosis. Treatment options vary from supportive care measures to endoscopic intervention. Our patient was successfully treated endoscopically by removing the food particles and draining of the infected diverticula. Lemmel's syndrome remains a rare cause of obstructive jaundice and should be included in differential diagnosis of biliary obstruction in presence of PAD.Figure 1Figure 2