Lemmels syndrome, also known as duodenal diverticulum obstructive jaundice, is a rare cause of benign obstructive jaundice that should be included in the. Lemmels syndrome is an uncommon pathology first described in 1934, 1 defined as obstructive jaundice due to a periampullary duodenal diverticulum in the absence of choledocholithiasis or neoplasm. Lemmel syndrome is defined as an obstructive jaundice caused by a periampullary duodenal diverticulum compressing the intrapancreatic common bile duct with resultant bile duct dilatation. In lemmel was the first to report the presence of juxtapapillary diverticula and hepatocholangiopancreatic disease, excluding cholelithiasis. Lemmel syndrome occurs when a duodenal diverticulum causes obstructive jaundice due to a mechanical obstruction of the common bile duct. Endoscopic sphincterotomy associated with percutaneous abscess drainage released patient from all symptoms. Down syndrome, it is estimated that the risk of having a second child with down syndrome is about one in 100. No surgical option is free of the risk for complications. A giant duodenal diverticulum causing lemmel syndrome ncbi. Lemmels syndrome is an uncommon pathology first described in 1934,1 defined. Bartter syndrome bs is a hereditary condition transmitted as an autosomal recessive bartter type 1 to 4 or dominant trait bartter type 5. Pdf a giant duodenal diverticulum causing lemmel syndrome.
For language access assistance, contact the ncats public information officer. Lemmels syndrome, also known as duodenal diverticulum obstructive jaundice, is a rare cause of benign obstructive jaundice that should be included in the differential diagnosis of biliary obstruction when pad is present, in the absence of cholelithiasis or other detectable obstacle. Lemmels syndrome, also called duodenal diverticulum obstructive jaundice syndrome was first described in 1934. Lemmel syndrome secondary to duodenal diverticulitis. Contrastenhanced axial ct of the upper abdomen pretreatment.
Lemmels syndrome, an unusual cause of abdominal pain and. A diagnosis of lemmel syndrome was made and underwent diverticulectomy. The downs syndrome handbook down syndrome, also called trisomy 21 is the most common cause of mental retardation and malformation in a newborn. Pdf 59year old female patient has been admitted to our department for a follow up after subsegmentectomy due to. Lemierre syndrome genetic and rare diseases information. Computed tomography of the abdomen showed several gallbladder stones and gallbladder wall thickening.
Lemmel syndrome is defined as an obstructive jaundice. Tokyo, japan a 63yearold male was referred for jaundice and general fatigue. Lemmels syndrome, an unusual cause of abdominal pain. Abdominal ct scan revealed stony opacity on distal cbd with cbd dilatation. Mulvihillsmith syndrome is an extremely rare disorder that, in theory, affects males and females in equal numbers. The presence of a neurovascular hamartoma within the oral cavity is truly a rare entity. Lemmel syndrome is a rare cause of obstructive jaundice and should be included in the differential diagnosis of biliary obstruction when a periampullary duodenal diverticulum is demonstrated. Duodenal diverticula are pseudodiverticula consisting of extraluminal saclike outpouchings of the duodenal mucosa which lack a. History a 70yearold man presented to the emergency department with fever, chills, rigors, and upper abdominal discomfort. Lemmel syndrome is defined that periampullary duodenal diverticulum often compressed the distal common bile duct and prevented the secretion of the bile 1,2. However, the age of the mother may also be a risk factor. Each person inherits 23 chromosomes from their mother and 23 chromosomes from their father. Lemmel syndrome occurs when a duodenal diverticulum causes obstructive jaundice due to a mechanical obstruction of the.
Lemmel syndrome is a rare cause of obstructive jaundice and should be included in the differential. If you have problems viewing pdf files, download the latest version of adobe reader. Based on analysis of ptc result before and after eswl, we diagnosed this case as lemmels syndrome obstructive jaundice, acute suppurative cholangitis and acute pancreatitis caused by a large diverticular enterolith at the peripapillary portion of the duodenum. It occurs because of the presence of an extra 21st chromosome. Syndromes two weaknesses are the ending and a weak development of the storys woman character. Meckelgruber syndrome is a rare, lethal, ciliopathic, genetic disorder, characterized by renal cystic dysplasia, central nervous system malformations occipital encephalocele, polydactyly post axial, hepatic developmental defects, and pulmonary hypoplasia due to oligohydramnios meckelgruber syndrome is named for johann meckel and georg gruber. Lemmels syndrome, an unusual cause of abdominal pain and jaundice by impacted intradiverticular enterolith. Recognition of these abnormalities is essential to prevent the development of lesions in the biliary system, as well as to avoid unnecessary surgical intervention. Mirizzis syndrome is a rare complication in which a gallstone becomes impacted in the cystic duct or neck of the gallbladder causing compression of the common hepatic duct, resulting in obstruction and jaundice. Intraduodenal diverticulum idd is a rare congenital anomaly, arising at or near the papilla of vater.
Lemmels syndrome as a rare cause of obstructive jaundice. The full text of this article is available in pdf format. It is defined, in the absence of cholelithiasis or other detectable obstacle, by obstructive jaundice due to juxtapapillary diverticulum, but to date very few cases have been published and fully investigated. Acg case reports journal lww journals beginning with a. Lemmels syndrome as a rare cause of obstructive jaundice should be known in order to avoid mismanagement and therapeutic delay. A diagnosis of lemmel syndrome was made by endoscopic retrograde cholangiopancreatography and upper gastrointestinal contrast studies. Be sure to verify your new user account in the next 24 hours, by checking your email and clicking the verify link. Lemmels syndrome should always be included as one of the differential diagnosis of obstructive jaundice when pad are present. Intraduodenal diverticulum associated with a double common. Lemmel syndrome is defined as an obstructive jaundice caused by a periampullary duodenal diverticulum compressing the intrapancreatic part of the common bile duct with resultant upstream dilatation of the extra and intrahepatic bile ducts. Lemmel syndrome, first described by lemmel in 1934, is defined as obstructive jaundice caused by periampullary duodenal diverticulum pad in the absence of choledocholithiasis or neoplasm. Contrast enhanced axial ct of the upper abdomen pretreatment. Additional pathophysiologic processes may also contribute to the development of lemmel syndrome.
Despite current practice of obtaining imaging studies such as ultrasound, ct, and mri, endoscopic retrograde cholangiopancreatography ercp is the. Mrcp and ercp in lemmel syndrome makiko ono, terumi kamisawa, yuyang tu, naoto egawa department of internal medicine, tokyo metropolitan komagome hospital. In particular, new findings have accumulated on the occurrence of symptoms in patients with gallstones, frequency of severe cholecystitis and cholangitis, onset of cholecystitis and. An unusual case of painless obstructive jaundice and. The most common cause is the presence of periampullary diverticula which arise within 23 cm from the ampulla of vater. The disease associates hypokalemic alkalosis with varying degrees of. While referring to the evidence adopted in the tokyo guidelines 2007 tg07 as well as subsequently obtained evidence, further discussion took place on terminology, etiology, and epidemiological data. Tg current terminology, etiology, and epidemiology of. Register with us for free to save searches, favorite articles and access email content. Lemmel syndrome is defined as an obstructive jaundice caused by a. In conclusion, lemmels syndrome is a rare cause of obstructive jaundice that should be included in the differential diagnosis of biliary obstruction when pad is present. Lemmels syndrome, juxtapapillary diverticula, periampullary duodenal in lemmel was the first to report the presence of juxtapapillary. This article has been saved into your user account, in the favorites area, under the new folder. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic cholecystitis inflammation.
As they search for a way back to their own time, grace beckettin presentday eldhjourneys to a frozen kingdom where she learns her own terrifying destiny. Case report duodenal diverticula are detected in up to 27% of patients undergoing upper gastrointestinal tract evaluation with periampullary diverticula pad being the most common type. Scarcely reported in the literature, these hamartomas. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 toll free. Lemmel syndrome is defined as an obstructive jaundice caused by a periampullary. An unusual case of painless obstructive jaundice and cholangitis. Maintaining a high index of suspicion is imperative to establish an accurate diagnosis since it can mimic other cystic or solid lesions around the pancreas head. Sulbactam sodium, cefoperazone sodium, 2g per day was administered for 5 days for attenuated acute cholangitis. The fibrous cap weakness leads to the plaque activation, plaque fissure or erosion activating a thrombotic cascade. Lemmels syndrome causes obstructive jaundice in the absence of stones or tumors. Icebreaker suvorov pdf the clinical picture in pigs is most similar to that in humans with vwd type 3.
Lemmel syndrome is secondary to a diverticulum of the second part of duodenum compressing the intrapancreatic part of common bile duct cbd with resultant upstream dilatation of the extra and intrahepatic bile ducts. Incidence of down syndrome and maternal age maternal age incidence of down syndrome 20 1 in 2000 24 1 in 0 27 1 in 1050 30 1 in 900 33 1 in 600. The patient declined further invasive treatment and was discharged 2 weeks later with the. It most commonly develops in association with a bacterial throat infection, but it may develop in association with an infection involving the ears, salivary glands parotitis, sinuses, or teeth. This page was last edited on 19 decemberat unlike most other related diseases and complications, these ulcers are not associated with normal aging. Clinical presentation patients may present with recurren.
Online resources and scientific support lemmel health. To date, there are few published cases, and the etiopathogenesis has not been completely established. The second case was a 71yearold woman, who admitted in our clinic complaining of right hypochondralgia, fever, and jaundice. Lemmel syndrome was first described in 1934, and defined as an. But beyond that, i cant rave enough about the plot concept. A wealth of data accumulated over the past 70 years has revealed that low serum testosterone levels are associated with an increased risk of cardiovascular disease, diabetes, osteoporosis. Pdf lemmel syndrome secondary to duodenal diverticulitis. Cockayne syndrome is an autosomal recessive genetic disorder. Lemmels syndrome a case report oncology, gastroenterology and hepatology reports, 2018, 7, 2, 106108. A case of lemmels syndrome caused by a large diverticular.
Double common bile duct dcbd is another rare congenital anomaly of the biliary system. Disease or syndrome t this form of acquired vwd may be more prevalent than is presently thought. Pad incidence increases with age, observed in around 1020% of patients undergoing endoscopic retrograde cholangiopancreatography ercp, with no gender predilection. Lemmels syndrome should always be included as one of the. As diagnosing lemmel syndrome is often a difficult task, it should always be considered in patients. Cowdens syndrome, bannayanrileyruvalcaba syndrome, pten. A giant duodenal diverticulum causing lemmel syndrome. This duodenal diverticulum obstructive jaundice syndrome is called lemmels syndrome. A rare pancreaticobiliary complication of duodenal diverticula is lemmel syndrome. She remains clinically and radiographically disease free after the last procedure. These include duodenal diverticula causing dysfunction of the sphincter of oddi as well as compression of the common bile duct by duodenal. Lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults.
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