Gastrointestinal System

{"ops":[{"insert":"Diagnosis and reasoning"},{"insert":"\n","attributes":{"header":1}},{"insert":"Jaundice is a very concerning presenting complaint that often heralds serious underlying disease. Key to the workup of these individuals is determination of jaundice is medical (i.e., pre- or intrahepatic in origin) or surgical (i.e., extrahepatic) in nature. In this respect, the dark urine suggests at conjugated hyperbilirubinemia, can be caused by both intrahepatic and extrahepatic disease. However, the presence of upper abdominal pain favors the latter.\n\nConsidering his age and gender, key extrahepatic etiologies to consider include gallstone disease (choledocolithiasis), acute cholecystitis and acute pancreatitis. It should also be kept in mind that tumors of the biliary tract (cholangiocarcinoma and gallbladder carcinoma) or of the pancreas can also present in this manner.\n\nUnfortunately, the examination is only significant for tenderness in the right upper quadrant, a nonspecific finding. \n\nA liver profile and ultrasound scan of the abdomen are essential first steps in his further evaluation. The former confirms the presence of direct (i.e., conjugated) hyperbilirubinemia, with the elevated levels of alkaline phosphatase (ALP) supporting the clinical assumption of an extrahepatic etiology.\n\nThe ultrasound scan reveals an unexpected finding: a contracted gallbladder with multiple gallstones impacted in the neck, along with dilation of the common hepatic duct and proximal biliary tree; unusually, the common bile duct (CBD) appears to be normal. These findings are suggestive of Mirizzi Syndrome (MS), a rare complication of cholelithiasis.\n\nFollow up imaging is advisable, with magnetic resonance cholangiopancreatography (MRCP) being a suitable choice. This confirms the presence of MS, while also showing no evidence of cholecysto-biliary or cholecysto-enteric fistula formation; in a nutshell, this appears to be Type I MS.\n\nAn interesting point here is the absence of previous episodes of upper abdominal pain or jaundice in the past, even through the gallbladder is contracted (indicating chronic inflammation); however, this is not unheard of.\n\nA cholecystectomy is a key element of his management; while this has traditionally been performed via open surgery, recent evidence suggests that a laparoscopic approach may be safely used in Type I MS. \n\nEndoscopic retrograde cholangiopancreatography (ERCP) and stenting is only a temporary measure; given the potential morbidity associated with the procedure, it is probably best avoided. While ursodeoxycholic acid can be used for the medical management of gallstones, it is also a suboptimal choice in this specific situation. Given the absence of pruritus, cholestyramine is not indicated.\nDiscussion"},{"insert":"\n","attributes":{"header":1}},{"insert":"Mirizzi Syndrome (MS) is a rare complication of gallstone disease, where impaction of a calculus (or calculi) in the cystic duct or Hartmann\u0027s pouch results in obstruction of the hepatic duct, via extrinsic compression.\n\nMS occurs in 1% to 4% of individuals with cholelithiasis. there is a marked female preponderance (70%), with the incidence increasing with age; this parallels the demographics of gallstone disease in general.\n\nAs mentioned earlier, the precipitating event is impaction of a single large gallstone or multiple smaller stones in Hartmann\u0027s pouch or the cystic duct; this results in recurrent episodes of acute cholecystitis. Subsequently, the walls of the inflamed gallbladder impinge on those of bile duct, resulting in in their fusion by the edematous inflammatory tissue; obstruction of the hepatic duct results due to fibrotic changes in the fused walls, as well the direct pressure effect of the impacted stone(s). These patients may also subsequently develop a cholecysto-biliary fistula; this is believed to be secondary to pressure necrosis of the intervening walls. In certain cases, the gallstones may also give rise to other pressure ulcers, resulting in formation of a cholecystoenteric fistula.\n\nAs might be apparent from the above, the presence of anatomical anomalies such as a low insertion of cystic duct into the bile duct, or a long cystic duct which lies parallel to the bile duct predispose to MS.\n\nThe clinical diagnosis of MS is extremely challenging, given the absence of unique clinical features; these patients can present both acutely, or with chronic symptoms. Between 60% to 100% of individuals develop obstructive jaundice, often accompanied by pain over the right upper quadrant of the abdomen. Other presentations include acute cholecystitis and acute cholangitis; MS in the setting of acute pancreatitis and gallstone ileus has also been reported.\n\nLaboratory investigations are also not particularly helpful, typically only being suggestive of biliary obstruction. Imaging studies are of more value; abdominal ultrasound may reveal a contracted gallbladder, with stones impacted in the infundibular region, and dilation of the common hepatic duct (with a normal common bile duct). Unfortunately, the sensitivity is low, reportingly ranging from 8.3% to 27%.\n\nComputed tomography (CT) of the abdomen can also detect biliary dilatation and gallbladder contraction; however, the imaging findings are nonspecific, with periductal inflammation potentially being misinterpreted as cancer of the gallbladder.\n\nEndoscopic retrograde cholangiopancreatography (ERCP) allows confirmation of the presence of MS, with a diagnostic accuracy ranging from 55% to 90%; it also affords the opportunity for therapeutic measures such as stone retrieval and placement of stents. However, ERCP is invasive, with potential for significant complications; in addition, failure rates range from 5% to 10%. Magnetic resonance cholangiopancreatography (MRCP) has been proposed as an alternative; while this has the advantage of being non-invasive, current studies have reported a diagnostic accuracy of only ~50%.\n\nNote that in recent literature, a combination of two or more of the above techniques has been proposed as a means of increasing the diagnostic yield.\n\nAs of the time of writing, MS is classified into five types, based on the work of Csendes et al. Type I MS is identified by external compression of the hepatic duct by an impacted gallstone in the infundibulum or cystic duct; types II to IV consist of varying degrees of cholecysto-biliary fistula, while type V is defined by the presence of a cholecysto-enteric fistula.\n\nUnfortunately, the preoperative diagnosis of MS can only be made in 8% to 62.5% of patients; thus, a high index of suspicion at the time of operation is essential. Key surgical characteristics suggestive of MS include a shrunken gallbladder with distorted anatomy or a dilated gallbladder with thick walls and a large stone; multiple gallstones impacted at the gallbladder neck or infundibulum; an obliterated Calot\u0027s triangle; a dense fibrotic mass at the Calot\u0027s triangle; and dense adhesions in the subhepatic space. In such cases, intraoperative ultrasonography or cholangiography may help confirm the diagnosis, and delineate the anatomy of the biliary tree.\n\nThe management of MS often presents a formidable challenge, particularly as condition is often misdiagnosed prior to surgery, resulting in an unpleasant surprise for the operating surgeon.\n\nType I MS is typically managed by open cholecystectomy, although recent studies have suggested subtotal cholecystectomy as a valid alternative. In cases of Type II to Type IV MS, subtotal cholecystectomy is usually performed, along with either reconstruction of the common hepatic duct with T-tube insertion, or bilioenteric anastomosis with a Roux-en-Y hepaticojejunostomy (for the more severe grades). For type V MS (cholecystoenteric fistula), subtotal cholecystectomy with division and suturing of the bilioenteric communication is typically the treatment of choice.\n\nOpen cholecystectomy is typically preferred over laparoscopic techniques, as the anatomy of Calot\u0027s triangle is often distorted due to inflammation, tissue edema, and the presence of adhesions. However, in recent literature, laparoscopic cholecystectomy has been suggested for the management of type I MS, and in selected cases of type II MS. Regardless of the technique employed, intraoperative biopsies should be obtained (particularly in type III and IV disease); this is because between 6% to 27% of cases of MS eventually turn out to be associated with gallbladder carcinoma.\nTake home messages"},{"insert":"\n","attributes":{"header":1}},{"insert":"Mirizzi syndrome (MS) is a rare complication of gallstone disease, occurring in less than 4% of such patients."},{"insert":"\n","attributes":{"list":"ordered"}},{"insert":"There are no signs or symptoms specific for MS, and most investigations have a low diagnostic yield, resulting in over half of cases being diagnosed only intra-operatively."},{"insert":"\n","attributes":{"list":"ordered"}},{"insert":"MS is classified into five different types; the management involves surgical cholecystectomy and reconstruction, with the exact details depending on the type."},{"insert":"\n","attributes":{"list":"ordered"}},{"insert":"As MS may be associated with carcinoma of the gallbladder, intra-operative biopsies should always be obtained."},{"insert":"\n","attributes":{"list":"ordered"}},{"insert":"References"},{"insert":"\n","attributes":{"header":1}},{"insert":"AL-AKEELY MH, ALAM MK, BISMAR HA, KHALID K, AL-TEIMI I, AL-DOSSARY NF. 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