A few days ago, while working with my friend and mentor Dr Luis Carvalho, he asked me about Lemmel’s Syndrome. What an interesting topic to review, I thought. Eponyms are very important because they give us the etiology, clinical presentation, diagnostic algorithm, treatment options, and prognosis of a specific entity in just one word. What is Lemmel’s Syndrome, and how to treat it?
It was first described in 1934 by Dr. Gerhard Lemmel (a German doctor). The patient suffering from Lemmel’s Syndrome has obstructive jaundice because of a duodenal periampullary diverticulum, without choledocholithiasis or a tumor. It is found incidentally in 22% of the population but is symptomatic in only 10% of cases. Surgeons must always think of this entity in a case of obstructive jaundice or acute pancreatitis in a patient without lithiasis.
Dr. Babar Khan et al. from the Department of Internal Medicine in Syracuse, USA, reported a case of a male patient diagnosed with acute pancreatitis following acute and intense epigastric pain. The patient had no cholelithiasis, no history of alcohol abuse, nor other evident causes of acute pancreatitis. Lipase and bilirubin were elevated. An MRCP diagnosed a small (1,1 cm) duodenal diverticulum adjacent to the ampulla of Vater, causing Wirsung dilation. Lemmel’s Syndrome was causing jaundice and acute pancreatitis. Medical treatment was initiated, and the patient became asymptomatic six days after. However, a recurrence occurred eight weeks later. Surgical resection of the duodenal diverticulum was conducted, achieving complete resolution of symptoms. Could they have done it differently? Is there another option aside from surgery?
According to the authors, there are two types of diverticula. The extraluminal is an acquired diverticulum due to a wall defect because of vessels, while the intraluminal is a congenital anomaly. Extraluminal diverticula usually occur in the second part of the duodenum and are found within 3 cm around the ampulla of Vater (periampullary diverticulum). Most of them are comprised only of mucosa outpouching. Although the majority is asymptomatic, the patient can present with different clinical complications.
Pancreaticobiliary complications:
Obstructive jaundice
Cholangitis
Pancreatitis (acute or chronic)
Develop gallbladder or bile duct stones.
Non-pancreaticobiliary complications:
Hemorrhage
Perforation
Enterolith formation
Lemmel’s Syndrome occurs when the periampullary diverticulum compresses the Oddi and causes obstructive jaundice. Some say it is usually associated with an impacted enterolith. Diagnosis is a challenge, and doctors be aware of its possibility in cases of obstructive jaundice without cholelithiasis. ERCP is the gold standard for diagnosis and can also be a treatment option in debilitated patients. Although a CT scan can show the diverticula, they are usually misdiagnosed with pancreatic pseudocysts, abscesses, or cystic neoplasms. MRCP can differentiate duodenal diverticula from those entities.
As Dr. Babar Khan et al. decided in the case reported, diverticulectomy is the best treatment option for Lemmel’s Syndrome. As mentioned above, endoscopic treatment (sphincterotomy or dilatation) and medical treatment are valid options for patients unsuitable for surgery.
Do you enjoy adrenal surgery? Must have this new book!
Costa Almeida CE, editor. Posterior Retroperitoneoscopic Adrenalectomy. Indications, Technical Steps and Outcomes. Switzerland: Springer; 2023.
Always remember Lemmel’s Syndrome when dealing with patients with obstructive jaundice. However, keep in mind that cholelithiasis is by far the most frequent cause. Always begin your clinical investigation searching for the common, not for the uncommon.
Finally, I have to say thank you to Dr. Luis Carvalho for always bringing the scientific discussion into the working days.
Link to article:
Dr. Carlos Eduardo Costa Almeida
General Surgeon