This enzyme is responsible for the final step in the production of cholesterol. Lemmel syndrome is defined that periampullary duodenal diverticulum often compressed the distal common bile duct and prevented the secretion of the bile 1,2. Lemmel syndrome is a rare cause of biliary obstruction. Smithlemliopitz syndrome genetics home reference nih. Lemmel syndrome was first described in 1934, and defined as an obstruction of biliary three secondary to duodenal diverticulum. 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. Rubinsteintaybi syndrome genetics home reference nih. Lemmel syndrome was first described in 1934 by lemmel as obstructive jaundice in the absence of gallstones due to a periam pullary duodenal diverticulum 1. Tokyo, japan a 63yearold male was referred for jaundice and general fatigue. Lemmels syndrome, juxtapapillary diverticula, periampullary duodenal. On ct, pads appear as thinwalled cavitary lesions, originating from the medial aspect. Patients with mirizzi syndrome can present with jaundice, fever, and right upper quadrant pain. Rubinsteintaybi syndrome is a condition characterized by short stature, moderate to severe intellectual disability, distinctive facial features, and broad thumbs and first toes.
The ct scan and mrcp are very important in lemmel s syndrome, making it possible to diagnose and illustrate the mechanism of. Lemmels syndrome, an unusual cause of abdominal pain and jaundice by impacted intradiverticular enterolith. Several reports have demonstrated an association between periampullary duodenal diverticulum and gallstone disease 2, 3. Lemmels syndrome as a rare cause of obstructive jaundice. Department of internal medicine, tokyo metropolitan. Sertolicellonly sco syndrome, also called germ cell aplasia, describes a condition of the testes in which only sertoli cells line the seminiferous tubules. Maintaining a high index of suspicion is imperative to establish an accurate lemel since it can mimic other. 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. Lemmel s syndrome, also called duodenal diverticulum obstructive jaundice syndrome was first described in 1934.
Lemmel syndrome is a rare condition that must be considered as a differential in cases of obstructive jaundice with no choledocholithiasis or tumor. Submucosal juxtapapillary duodenal diverticulum complicated with lemmel s syndrome. First described in 1934,1 lemmel syndrome is a rare complication of periampullary duodenal diverticula, which are mostly asymptomatic. Pdfelement pro the best pdf software for windows 10 pdfelement pro pdfelement pro is an exciting solution that has become extremely popular in recent years. Lemmel s syndrome, juxtapapillary diverticula, periampullary duodenal in lemmel was the first to report the presence of juxtapapillary. As diagnosing lemmel syndrome is often a difficult task, it should always be considered in patients with pad. Lemmel syndrome secondary to duodenal diverticulitis. All works by this person are still under in canada, the eu, japan, and elsewhere and are thus subject to deletion. Liangkung chen, director taipei veterans general hospital, center for geriatrics and gerontology, taiwan. Principal etiologies regarding pathogenesis in developing lemmel syndrome include direct mechanical irritation of periampullary diverticula, dysfunction of the sphincter of. The most common cause is the presence of periampullary diverticula which arise within 23 cm from the ampulla of vater. Mrcp is a noninvasive imaging modality which is useful for. Lemierre syndrome is a rare and potentially lifethreatening complication of bacterial infections that usually affects previouslyhealthy adolescents and young adults.
Department of gastroenterology, dokkyo medical university, japan. Clinical presentation patients may present with recurren. Lemmel s syndrome, an unusual cause of abdominal pain and jaundice by impacted intradiverticular enterolith. Computed tomography and mri allows diagnosis, eliminate pancreatic origin, and avoid. Lemmel syndrome occurs when a duodenal diverticulum causes obstructive jaundice due to a. Lemmel syndrome is defined as an obstructive jaundice.
Lemierre syndrome genetic and rare diseases information. Hong wang, senior research scientist, division of endocrinology, department of internal medicine university of virginia medical school, usa. Lemmel s 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. Mrcp and ercp in lemmel syndrome insight medical publishing. 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. Additional pathophysiologic processes may also contribute to the development of lemmel syndrome. Department of gastroenterology, tochigi medical center shimotsuga general hospital, japan.
Lemmels syndrome is a pancreaticobiliary disease secondary to. Lemmel syndrome corresponds to cholestatic disease secondary to compression of the main bile duct by a periampullary duodenal diverticulum. Pdf lemmel syndrome secondary to duodenal diverticulitis. The volume of pe is welldefined at 50cckg administered five times, daily or every. Patients with pathogenic breast cancer variants may receive treatment that deviates from guidelines. Contrastenhanced axial ct of the upper abdomen pre treatment. 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. Computed tomography of the abdomen showed several gallbladder stones and gallbladder wall thickening. Lemmel syndrome is a rare condition that must be considered as a differential in cases of obstructive jaundice with no choledocholithiasis or tumour. Metabolic syndrome is the medical term for a combination of diabetes, high blood pressure hypertension and obesity. It is an obstructive jaundice in the absence of gallstones primarily due to a periampullary duodenal diverticulum. Icd10 dx edit code lists this appendix lists the icd10cm diagnosis codes for the following categories associated with edits in the program.
Down syndrome is by far the most common and best known chromosomal disorder in humans and the most common cause of intellectual disability. Surgical excision of the diverticulum is another treatment option. Lemmels syndrome is an uncommon pathology first described in 1934,1. 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. Lemmel syndrome is defined as an obstructive jaundice caused by a periampullary duodenal diverticulum compressing the intrapancreatic portion of the common bile duct with resultant dilatation of the extra and intrahepatic bile ducts.
Two form of hies have been described, including an autosomal. In lemmel was the first to report the presence of juxtapapillary diverticula and hepatocholangiopancreatic disease, excluding cholelithiasis. Lemmel, hartmut editor the following text was automatically transcluded from category. Makiko ono, terumi kamisawa, yuyang tu, naoto egawa. We report a new case of lemmel syndrome, caused by a periampullary duodenal diverticulum compressing the bile ducts leading to cholestasis. Lemmels syndrome causes obstructive jaundice in the absence of stones. Maintaining a high index of suspicion is imperative to establish an accurate lemel since it can mimic other cystic or solid lesions around the pancreas head. Mrcp and ercp in lemmel syndrome makiko ono, terumi kamisawa, yuyang tu, naoto egawa department of internal medicine, tokyo metropolitan komagome hospital. 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. The obstructive jaundice can be caused by direct extrinsic compression by the stone or from fibrosis caused by chronic cholecystitis inflammation. Mirizzi syndrome is defined as common hepatic duct obstruction caused by extrinsic compression from an impacted stone in the cystic duct or infundibulum of the gallbladder. Cholesterol is a waxy, fatlike substance that is produced in the body and obtained from foods that come from animals particularly egg yolks, meat, poultry.
Sulbactam sodium, cefoperazone sodium, 2g per day was administered for 5 days for attenuated acute cholangitis. A rare pancreaticobiliary complication of duodenal diverticula is lemmel syndrome. The incidence of duodenal diverticulum is about 110% and until 27% in other series, and is the second more frequent site for diverticula location only after colon. It puts you at greater risk of getting coronary heart disease, stroke and other conditions that affect the blood vessels on their own, diabetes, high blood pressure and obesity can damage your blood vessels, but having all 3 together is particularly dangerous. Smithlemliopitz syndrome is caused by mutations in the dhcr7 gene, which provides instructions for making an enzyme called 7dehydrocholesterol reductase. For the first time, doctors test an inbody dna editing therapy in an attempt to rid a 44year old man of hunter syndrome, an incurable genetic disease. Additional features of the disorder can include eye abnormalities, heart and kidney defects, dental problems, and obesity. Gene editing, in a patients body, attempted for 1st.
No bile duct stones or signs of malignancy were detected. It is characterized by intellectual disability, dysmorphic facial features, and other distinctive phenotypic traits. Lemmels syndrome, an unusual cause of abdominal pain. Despite current practice of obtaining imaging studies such as ultrasound, ct, and mri, endoscopic retrograde cholangiopancreatography ercp is the.
It is due to two mechanisms, the first by a direct mechanical irritation evolving towards the ductal fibrosis, the second would be due to a bile duct mechanical compression, as in our case. To date, there are few published cases, and the etiopathogenesis has not been completely established. Lemmel s syndrome, first described by lemmel in 1934, refers to duodenal diverticulum of second part of duodenum compressing on the common bile duct which may present as obstructive jaundice, cholangitis, pancreatitis, or intrahepatic cholangiolar abscess. 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. Lemmel advocated that periampullary duodenal diverticulum often compressed the distal common bile duct and prevented the secretion of the bile 1. Lemmels syndrome, an unusual cause of abdominal pain and. Lemmel s syndrome causes obstructive jaundice in the absence of stones or tumors.
Since ercp did not guarantee definitive treatment, the patient was referred to our. Division of gastroenterology and hepatology, department of internal medicine, korea university college of medicine, seoul, korea. Lemmel syndrome occurs when a duodenal diverticulum causes obstructive jaundice due to a mechanical obstruction of the common bile duct. These include duodenal diverticula causing dysfunction of the sphincter of oddi as well as compression of the common bile duct by duodenal. Lemmels syndrome was first described by lemmel in 1934 as a cholestatic disease secondary to the compression of the main bile duct by a periampullary duodenal diverticulum 12. We report a new case of lemmel syndrome, caused by a. Obstructive jaundice in the absence of choledocholithiasis or pancreaticobiliary or periampullary tumors is. In conclusion, lemmel s syndrome is a rare cause of obstructive jaundice that should be included in the differential diagnosis of biliary obstruction when pad is present. It is an advanced pdf editing tool that offers a wide range of features, making it easier to edit, convert, view and create pdf files.1206 1579 251 466 364 980 749 1052 777 1304 581 1506 818 1611 944 62 1350 542 1139 1655 1312 279 232 14 1561 1584 929 577 379 1417 1377 483 1150 728 1313 177 302 1039 241 378 625 806 967 376 891 158 606 479 759 1303