Rosen & Barkin's 5-Minute Emergency Medicine Consult (407 page)

• Icterus of sclera and tongue base (levels >2.5 mg/dL)
  
• Right upper quadrant tenderness:
  
  • Courvoisier rule:
    
    • Painless jaundice and a palpable, nontender gallbladder represent malignant common duct obstruction.
      
• Stigmata of cirrhosis:
  
  • Abdominal collateral circulation including caput medusae, hepatosplenomegaly, or hepatic atrophy
    
  • Ascites
    
  • Spider telangiectasia
    
  • Palmar erythema
    
  • Dupuytren contractures
    
  • Asterixis
    
  • Encephalopathy
    
  • Gynecomastia
    
• Palpable gallbladder
  
• Hepatomegaly
  
• Splenomegaly
  
• Abdominal mass
  
• Evidence of cachexia
  
• Excoriations (primary biliary cirrhosis, obstruction)
  
• Kayser–Fleischer rings:
  
  • Wilson disease
    

• History and physical exam, together with routine lab tests, will suggest the diagnosis in ∼80% of patients with jaundice.
  
• Bilirubin level—severity may suggest cause:
  
  • Malignancy causes highest levels (10–30 mg/dL).
    
  • Choledocholithiasis rarely exceeds 15 mg/dL.
    

• Urine dipstick is 74% sensitive for bilirubin.
  
• Alkaline phosphatase:
  
  • If no bone disease and not pregnant, then elevation suggests impaired biliary tract function.
    
  • 2X normal: Hepatitis and cirrhosis
    
  • 3X normal: Extrahepatic biliary obstruction (i.e., choledocholithiasis) and intrahepatic cholestasis (i.e., drug-induced and biliary cirrhosis)
    
• Aminotransferases—provide evidence of hepatocellular damage:
  
  • Alanine aminotransferase (ALT, SGPT): Primarily in the liver
    
  • Aspartate aminotransferase (AST, SGOT): Liver, heart, kidney, muscle, and brain
    
• γ-Glutamyl transpeptidase—throughout hepatobiliary system, pancreas, heart, kidneys, and lungs:
  
  • May be the most sensitive indicator of biliary tract disease.
    
  • Confirms hepatic origin of an elevated alkaline phosphatase.
    
• 5′-Nucleotidase—widespread tissue distribution:
  
  • Confirms hepatic origin of an elevated alkaline phosphatase level.
    
• Albumin: Decreased with severe liver disease
  
• PT: Elevation is an important prognostic indicator in patients with acute hepatitis.
  

• US: Most effective initial imaging technique:
  
  • >90% effective in identifying cholelithiasis
    
  • Ductal dilation is a reliable indicator of extrahepatic obstruction:
    
    • A dilated common bile duct (CBD) and gallbladder suggest distal obstruction, whereas dilation of the intrahepatic ducts (without CBD dilation) suggests proximal obstruction.
      
• Tumors of the liver and head of pancreas are usually well visualized.
  
• Distinguishes solid liver tumors from cystic structures.
  
• Plain radiographs:
  
  • May show evidence of hepatic and splenic enlargement or biliary calcifications
    
• Hepatic nuclear scan (hepatobiliary iminodiacetic acid scan):
  
  • Accurate method of diagnosing acute cholecystitis or cystic duct obstruction
    
  • Time consuming (usually several hours)
    
• CT:
  
  • Superior to US in detecting pancreatic and intra-abdominal tumors.
    
  • Can help differentiate fluid-containing structures.
    

Endoscopic retrograde cholangiopancreatography (ERCP):

• Diagnostic:
  
  • Stones are seen as filling defects within bile duct lumen.
    
  • Malignancies are seen as strictures.
    
• Therapeutic:
  
  • Extraction of CBD stones and insertion of stents to bypass malignant obstructions
    
  • Biopsy under direct vision
    

• Prehepatic:
  
  • Hemolysis (sickle cell, other hemoglobinopathies)
    
  • Ineffective erythropoiesis
    
  • Drugs
    
  • Gilbert syndrome: Usually benign inherited form of unconjugated hyperbilirubinemia
    
  • Crigler–Najjar syndrome
    
  • Prolonged fasting
    
• Hepatocellular:
  
  • Hepatitis (infectious, alcoholic, autoimmune, toxin, drug induced)
    
  • Cirrhosis
    
  • Postischemic
    
  • Hemochromatosis
    
• Intrahepatic cholestasis:
  
  • Idiopathic cholestasis of pregnancy
    
  • Drugs
    
  • Dubin–Johnson syndrome
    
  • Rotor syndrome
    
  • Benign recurrent cholestasia
    
  • Familial syndromes
    
  • Sepsis
    
  • Postoperative jaundice
    
  • Lymphoma
    
• Extrahepatic obstruction:
  
  • Common duct stone
    
  • Biliary stricture
    
  • Bacterial cholangitis
    
  • Sclerosing cholangitis
    
  • Carcinoma (ampulla, gallbladder, pancreas), cholangiosarcoma
    
  • Pancreatitis, pancreatic pseudocyst
    
  • Hemobilia
    
  • Duodenal diverticula
    
  • Ascariasis
    
  • Postlaparoscopic cholecystectomy complications
    
  • Congenital biliary atresia
    
  • Congenital choledochal cyst
    

Intrahepatic cholestasis:

• Cardiovascular (congenital heart disease, congestive heart failure, shock, asphyxia)
  
• Metabolic or genetic (α
  ₁
  -antitrypsin deficiency, trisomy 18 and 21, cystic fibrosis, Gaucher disease, Niemann–Pick disease, glycogen storage disease type IV)
  
• Infectious (bacterial sepsis, cytomegalovirus, enterovirus, herpes simplex virus, rubella, syphilis, TB, varicella, viral hepatitis)
  
• Hematologic (severe isoimmune hemolytic disease)
  

TREATMENT

• Isotonic IV fluid therapy if dehydrated
  
• Toxic-appearing patients:
  
  • Supplemental oxygen, cardiac monitoring
    
  • Nasogastric suction and bladder catheterization
    

• For bacterial cholangitis/sepsis, obtain blood cultures and administer parenteral antibiotics:
  
  • Ampicillin, gentamicin, and metronidazole
    or
    
  • Ticarcillin, or piperacillin, and metronidazole
    or
    
  • Cefoxitin and tobramycin
    
• Obstructive extrahepatic jaundice:
  
  • Surgical consult
    
• Choledocholithiasis:
  
  • ERCP papillotomy, balloon or basket retrieval, or open surgery
    
• Obstructive intrahepatic or nonobstructive jaundice:
  
  • Medical management:
    
    • Withdraw causative drug, ethanol
      
    • Interferon for chronic hepatitis B and C
      
    • Penicillamine and phlebotomy for Wilson disease and hemochromatosis
      
    • Corticosteroids for chronic hepatitis of autoimmune origin
      

• Exchange transfusion:
  
  • Emergent treatment of markedly elevated bilirubin (>20 mg/dL in full-term infants) and for correction of anemia caused by isoimmune hemolytic disease
    
• Phototherapy—for neonatal jaundice when bilirubin = 17 mg/dL:
  
  • Measure bilirubin once to twice daily and stop when bilirubin has been reduced by about 4–5 mg/dL.
    
• Phenobarbital: In sepsis and drug-induced causes; decreases conjugated bilirubin.
  
• Metalloporphyrins: Investigational inhibitors of heme oxygenase
  

• Ampicillin: 2 g IV q6h (peds: 25 mg/kg IV q6–8h)
  
• Cefoxitin: 2 g IV q6h (peds: 40–160 mg/kg/d div. q6–12h)
  
• Gentamicin: 5–2 mg/kg IV q8h
  
• Metronidazole: 7.5 mg/kg IV q6h (peds: Same)
  
• Piperacillin/tazobactam: 3.375 g IV q6h (peds: 300 mg/kg/d div. q6h [>2 mo of age])
  
• Ticarcillin/clavulanate: 3.1 g IV q6h (peds: 75–100 mg/kg/d div. q6h)
  
• Tobramycin: 1 mg/kg IV q6h (peds: Same)
  

FOLLOW-UP

• Bacterial cholangitis
  
• Intractable pain
  
• Intractable emesis
  
• Associated pancreatitis
  
• Elevated PT
  

• No evidence of infection (evaluate as outpatient)
  
• Tolerating liquids
  

• Addley J, Mitchell RM. Advances in the investigation of obstructive jaundice.
  Curr Gastroenterol Rep
  . 2012;14:511–519
  
• Maisels MJ, McDonagh AF. Phototherapy for neonatal jaundice.
  N Engl J Med
  . 2008;358:920–928.
  
• Roche SP, Kobos R. Jaundice in the adult patient.
  Am Fam Physician
  . 2004;69:299–304.
  
• Udell JA, Wang CS, Tinmouth J, et al. Does this patient with liver disease have cirrhosis?
  JAMA
  . 2012;307(8):832–842.
  
• Wang Q, Gurusamy KS, Lin H, et al. Preoperative biliary drainage for obstructive jaundice.
  Cochrane Database Syst Rev
  . 2008;16(3):CD005444.
  

CODES

• 277.4 Disorders of bilirubin excretion
  
• 774.6 Unspecified fetal and neonatal jaundice
  
• 782.4 Jaundice, unspecified, not of newborn