151 CHOLELITHIASIS, CHOLECYSTITIS, AND CHOLANGITIS
Harrison’s Manual of Medicine
CHOLELITHIASIS, CHOLECYSTITIS, AND CHOLANGITIS
Primary Sclerosing Cholangitis (PSC)
There are three major types of gallstones: cholesterol, pigment, and mixed stones. Mixed and cholesterol gallstones contain >70% cholesterol monohydrate. Pigment stones have <10% cholesterol and are composed primarily of calcium bilirubinate. In the U.S., 80% of stones are cholesterol or mixed, 20% pigment.
EPIDEMIOLOGY One million new cases of cholelithiasis per year in the U.S. Predisposing factors include demographic/genetics (increased prevalence in American Indians), obesity, weight loss, diabetes, ileal disease, pregnancy, estrogen or oral contraceptive use, type IV hyperlipidemia, and cirrhosis. Females/males = 4:1.
SYMPTOMS AND SIGNS Many gallstones are “silent,” i.e., present in asymptomatic pts. Symptoms occur when stones produce inflammation or obstruction of the cystic or common bile ducts. Major symptoms: (1) biliary colic — a severe steady ache in the RUQ or epigastrium that begins suddenly; often occurs 30–90 min after meals, lasts for several hours, and occasionally radiates to the right scapula or back; (2) nausea, vomiting. Physical exam may be normal or show epigastric or RUQ tenderness.
LABORATORY Occasionally, mild and transient elevations in bilirubin [<85 µmol/L (<5 mg/dL)] accompany biliary colic.
IMAGING Only 10% of gallstones are radioopaque. Ultrasonography is best diagnostic test. The oral cholecystogram requires a functioning gallbladder and serum bilirubin <51 µmol/L (<3 mg/dL) (Table 151-1).
Table 151-1 Radiologic and Imaging Modalities for Biliary Tract Disease
DIFFERENTIAL DIAGNOSIS Includes peptic ulcer disease (PUD), gastroesophageal reflux, irritable bowel syndrome, and hepatitis.
In asymptomatic pts, risk of developing complications requiring surgery is small. Elective cholecystectomy should be reserved for: (1) symptomatic pts (i.e., biliary colic despite low-fat diet); (2) persons with previous complications of cholelithiasis (see below); and (3) asymptomatic pts with an increased risk of complications (calcified or nonfunctioning gallbladder, cholesterolosis, adenomyomatosis). Pts with gallstones >2 cm or with an anomalous gallbladder containing stones should also be considered for surgery. Laparoscopic cholecystectomy is minimally invasive and is the procedure of choice for most pts undergoing elective cholecystectomy. Oral dissolution agents (chenodeoxycholic acid, ursodeoxycholic acid) partially or completely dissolve small radiolucent stones in 50% of selected pts but are ineffective in dissolving large, radioopaque, or pigment stones and those within a poorly opacified gallbladder following oral cholecystography. Recurrence is likely if the medication is stopped. Extracorporeal shockwave lithotripsy followed by medical litholytic therapy is effective in selected pts with solitary radiolucent gallstones.
COMPLICATIONS Cholecystitis, pancreatitis, cholangitis.
Acute inflammation of the gallbladder usually caused by cystic duct obstruction by an impacted stone.
ETIOLOGY 90% calculous; 10% acalculous; latter associated with higher complication rate and caused by prolonged acute illness (i.e.,: burns, trauma, major surgery), fasting, hyperalimentation leading to gallbladder stasis, vasculitis, carcinoma of gallbladder or common bile duct, some gallbladder infections (Leptospira, Streptococcus, parasitic etc.).
SYMPTOMS AND SIGNS (1) Attack of bilary colic (RUQ or epigastric pain) that progressively worsens; (2) nausea, vomiting, anorexia; and (3) fever. Examination typically reveals RUQ tenderness; palpable RUQ mass found in 20% of pts. Murphy’s sign is present when deep inspiration or cough during palpation of the RUQ produces increased pain or inspiratory arrest.
LABORATORY Mild leukocytosis; serum bilirubin, alkaline phosphatase, and AST may be mildly elevated.
IMAGING Ultrasonography is useful for demonstrating gallstones and occasionally a phlegmonous mass surrounding the gallbladder. Radionuclide scans (HIDA, DIDA, DISIDA, etc.) may identify cystic duct obstruction.
DIFFERENTIAL DIAGNOSIS Includes acute pancreatitis, appendicitis, pyelonephritis, PUD, hepatitis, and hepatic abscess.
No oral intake, nasogastric suction, IV fluids and electrolytes, analgesia (meperidine or NSAIDS), and antibiotics (ampicillin, cephalosporins; consider combination with aminoglycosides in diabetic pt or others with signs of gram- negative sepsis). Acute symptoms will resolve in 75% of pts. Surgery is definitive and should be performed as soon as feasible (within 24–48 h of admission). Delayed surgery is reserved for pts with high risk of emergent surgery and where the diagnosis is in doubt.
COMPLICATIONS Empyema, hydrops, gangrene, perforation, fistulization, gallstone ileus, porcelain gallbladder.
ETIOLOGY Chronic inflammation of the gallbladder; almost always associated with gallstones. Results from repeated acute/subacute cholecystitis or prolonged mechanical irritation of gallbladder wall.
SYMPTOMS AND SIGNS Often nonspecific; include dyspepsia, fatty food intolerance, and abdominal pain.
LABORATORY Tests are usually normal.
IMAGING Ultrasonography preferred; usually shows gallstones within a contracted gallbladder (Table 151-1).
DIFFERENTIAL DIAGNOSIS PUD, esophagitis, irritable bowel syndrome.
Surgery indicated if pt is symptomatic.
ETIOLOGY In pts with cholelithiasis, passage of gallstones into common bile duct occurs in 10–15%; increases with age. At cholecystectomy, undetected stones are left behind in 1–5% of pts.
SYMPTOMS AND SIGNS Choledocholithiasis may present as an incidental finding, biliary colic, obstructive jaundice, cholangitis, or pancreatitis. Cholangitis usually presents as fever, RUQ pain, and jaundice (Charcot’s triad).
LABORATORY Elevations in serum bilirubin, alkaline phosphatase, and aminotransferases. Leukocytosis usually accompanies cholangitis; blood cultures are frequently positive. Amylase is elevated in 15% of cases.
IMAGING Diagnosis usually made by cholangiography either preoperatively by endoscopic retrograde cholangiopancreatography (ERCP) or intraoperatively at the time of cholecystectomy. Ultrasonography may reveal dilated bile ducts but is not sensitive for detecting common duct stones (Table 151-1).
DIFFERENTIAL DIAGNOSIS Acute cholecystitis, renal colic, perforated viscus, pancreatitis.
Laparoscopic cholecystectomy and ERCP have decreased the need for choledocholithotomy and T-tube drainage of the bile ducts. Endoscopic biliary sphincterotomy followed by spontaneous passage or stone extraction is the treatment of choice in the management of pts with common duct stones, especially in elderly or high-risk pts. Preoperative ERCP is indicated in gallstone pts with (1) history of jaundice or pancreatitis, (2) abnormal LFT, and (3) ultrasound evidence of a dilated common bile duct or stones in the duct. Cholangitis treated like acute cholecystitis; no oral intake, hydration, and analgesia are the mainstays; stones should be removed surgically or endoscopically.
COMPLICATIONS Cholangitis, obstructive jaundice, gallstone-induced pancreatitis, and secondary biliary cirrhosis.
PRIMARY SCLEROSING CHOLANGITIS (PSC)
PSC is a sclerosing inflammatory process involving the biliary tree.
ETIOLOGY Males outnumber females and most patients are 25–45 years old. Associations: inflammatory bowel disease (ulcerative colitis—70% of cases of PSC), AIDS, rarely retroperitoneal fibrosis.
SYMPTOMS AND SIGNS Pruritus, RUQ pain, jaundice, fever, weight loss, and malaise. May progress to cirrhosis with portal hypertension.
LABORATORY Evidence of cholestasis (elevated bilirubin and alkaline phosphatase) common.
RADIOLOGY/ENDOSCOPY Transhepatic or endoscopic cholangiograms reveal stenosis and dilation of the intra- and extrahepatic bile ducts.
DIFFERENTIAL DIAGNOSIS Cholangiocarcinoma, Caroli’s disease (cystic dilation of bile ducts), Fasciola hepatica infection, echinococcosis, and ascariasis.
No satisfactory therapy. Cholangitis should be treated as outlined above. Cholestyramine may control pruritus. Supplemental vitamin D and calcium may retard bone loss. Glucocorticoids, methotrexate, and cyclosporine have not been shown to be effective. Urodeoxycholic acid improves liver tests but has not been shown to affect survival. Surgical relief of biliary obstruction may be appropriate but has a high complication rate. The efficacy of colectomy for pts with ulcerative colitis is uncertain. Liver transplantation should be considered in pts with endstage cirrhosis.
For a more detailed discussion, see Greenberger NJ, Paumgartner G: Diseases of the Gallbladder and Bile Ducts, Chap. 302, p. 1776, in HPIM-15.