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The yellow bird Recognizing biliary obstruction

By Habiba A. Habib, BSN, RN, and Michael Saunders, MD

As Mr. R, 66, is going about his daily activities, he experiences severe upper-abdominal pain. He describes the pain as a pressure that begins in the epigastrium and spreads in a 2.3 bandlike distribution to the left and right upper quadrants, ANCC CONTACT HOURS radiating to the upper back. It increases in intensity to 8 on a 0-to-10 scale over 1 to 2 hours. The pain is exacerbated by movement and associated with , vomiting, chills, and rigors. Mr. R is jaundiced and his urine is dark amber. His function tests (LFTs), including serum levels, are markedly elevated. Given Mr. R’s history and signs and symptoms, his healthcare provider suspects a biliary obstruction: a blockage of the flow of from the liver to the .

A RELATIVELY COMMON disorder, biliary obstruction affects approximately 5 per 1,000 people.1 (icterus) is a key sign of biliary obstruction. The word icterus derives from an ancient Greek word that also signified a yellow bird (see Recognizing the yellow bird of jaundice). This article describes the various possible causes of biliary obstruction, diagnostic studies and treatment options, and nursing considerations for patients with this disorder. NC , I What can go wrong? In biliary obstruction, the interruption of bile flow can ESEARCHERS

occur at any level within the biliary system. (See How bile R

travels to the .) Bile, the liver’s exocrine secretion, HOTO /P ARAZZI . P. M . P. R D

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Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. of jaundice:

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Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. is produced continuously by liver in the , it can be secreted by Recognizing “the yellow cells (). It contains the kidneys into urine, giving the water, cholesterol, bile salts, which bird” of jaundice urine a dark amber (“coca cola”) aid in the of fats, and Jaundice was recognized by ancient color. waste products such as bilirubin, Greeks and Romans as a sign of disease. The normal serum total bilirubin which causes bile’s familiar yellow- The word jaundice stems from the Latin concentration is less than 1 mg/dL. green color. word galbinus, which described a light An accumulation of bilirubin in the Bilirubin is formed by the break- greenish yellow color. Icterus is a Lati- bloodstream (hyperbilirubinemia) down of hemoglobin from red blood nized form of the Greek word ikteros, and subsequent deposition in the which to the ancient Greeks signified cells as they pass through the . skin and sclerae causes jaundice both jaundice and a “yellow bird.” The The process of bilirubin metabolism ancient treatment for jaundice included (icterus). Scleral icterus is generally can be affected by an alteration in the use of yellow birds as a cure, as is a more sensitive sign of hyperbiliru- the steps involving uptake, storage, described in Pliny the Elder’s Naturalis binemia than generalized jaundice. conjugation, and secretion. Historia XXX:XXVII (written in AD 77): Jaundice is a term often used inter- Unconjugated bilirubin (UCB) is “There is a bird called jaundice from its changeably with hyperbilirubine- minimally soluble in water and is color. If one with jaundice looks at it, mia, which may not be clinically transported in blood attached to they are cured of that complaint, and detected until bilirubin levels are at albumin, which limits renal excre- the bird dies.”2 least 3 mg/dL.3 tion and diffusion into tissues. UCB is cleared from the bloodstream both the small and large intestine Intrahepatic or extrahepatic? mainly by hepatocytes, which con- hydrolyze the conjugates, yielding Mr. R undergoes further evaluation to vert it to a water-soluble conju- UCB again. Colonic anaerobes then determine the origin of his signs and gated bilirubin (CB). This in turn is reduce UCB to urobilinogens that symptoms. secreted into bile. The secreted CB are eliminated mainly in the feces.2 , defined as obstruc- travels in bile down the biliary tree Bilirubin is normally absent in tion of biliary flow, can be caused by to the intestine, where bacteria in urine. But when excess CB is present mechanical factors, such as biliary

How bile travels to the duodenum1 Half of the bile produced by the liver flows directly into the duodenum via a system of ducts, including the common bile (CBD). The remaining 50% is stored in the . In response to a meal, this bile is released from the gallbladder via the , which joins the from the liver. The CBD courses through the head of the for approximately 2 cm before passing through the hepatopancreatic ampulla (ampulla of Vater) into the duodenum.

Diaphragm

Liver

Gallbladder Spleen

Cystic duct Common hepatic duct

Common Hepatopancreatic Tail of the ampulla pancreas

Duodenum Head of the pancreas

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Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. strictures, or by metabolic factors throughout the biliary tree. In Mirizzi such as due to certain syndrome, an impacted cystic duct stone medications, pregnancy, and sepsis causes inflammation and compression (due to the effects of released cyto- of the common hepatic duct and thus kines). These processes can damage biliary obstruction.1 The vast majority or impair function, of stones, however, are smaller than resulting in hyperbilirubinemia (see 1 cm and don’t obstruct the ducts. Identifying causes of cholestasis). (See Hard facts about .) Cholestasis can also have either Other causes of intraductal cho- intrahepatic or extrahepatic etiologies. lestasis include: Intrahepatic cholestasis generally • benign biliary strictures caused by occurs at the level of the hepatocyte surgical anastomosis, operative injury, or biliary canalicular membrane. The or chronic . most common causes of intrahepatic • of Oddi dysfunction or cholestasis are and .3 dyskinesia. Hepatitis is an inflammation of • PSC, a chronic disorder of unknown the liver characterized by diffuse or etiology characterized by inflamma- patchy necrosis. Causes of hepatitis Scleral icterus is generally tion, fibrosis, and stricturing of ducts include viruses, drugs, and alcohol. in the biliary tree. a more sensitive sign of • Cirrhosis is characterized by gener- hyperbilirubinemia benign and malignant neoplasms. alized disorganization of hepatic than generalized architecture with nodule formation jaundice. Assessing extraductal causes and scarring of the parenchyma. Malignant or benign neoplasms can Cirrhosis results from chronic liver also cause external compression of inflammation.2 the bile duct, resulting in obstruc- Primary biliary cirrhosis (PBC), than one million Americans are diag- tion. Causes of extraductal neoplastic another possible cause of intra- nosed with cholelithiasis (gallstones) obstruction include tumors arising hepatic cholestasis, is a chronic, auto- every year.5 Many factors increase locally from the gastrointestinal immune, granulomatous destruction the risk of developing gallstones, (GI) tract (such as pancreatic ) of the intrahepatic ducts.1 In contrast including: and metastatic from outside to patients with other types of liver • advancing age the GI tract (such as breast cancer). disease, 95% of those with PBC are • gender; gallstones are more com- Benign extraductal causes of bili- women. Most patients with PBC are mon in women5 ary obstruction include acute and asymptomatic in the early stages of • family history and genetics . disease; fatigue and pruritus are the • certain medications, such as most common presenting signs and estrogens Patient history and lab results symptoms at later stages.4 • obesity A former kitchen cabinet salesperson, Extrahepatic cholestasis can be • diet, especially a diet low in fiber Mr. R has been unemployed for the past subdivided into those that are intra- and high in saturated fats year and pays for his own insurance. ductal or extraductal. Intraductal • sedentary lifestyle. He’s proud to be with his wife of 42 causes include choledocholithiasis Gallstones can become lodged in the years. He reports drinking alcohol infre- ( in the CBD and cause complete obstruction, quently (one to three beers a month) and [CBD]), biliary strictures, primary with increased intraductal pressure denies any history of tobacco or illicit sclerosing cholangitis (PSC), dysfunction, and neoplasms 2 such as cholangiocellular carcinoma. Identifying causes of cholestasis Extraductal obstruction caused by Intrahepatic cholestasis Extrahepatic cholestasis external compression of the biliary • hepatocellular injury • choledocholithiasis ducts may be secondary to neo- • hepatitis • neoplasms plasms, such as pancreatic carcinoma, • • pancreatitis, or cystic duct stones with primary biliary cirrhosis primary sclerosing cholangitis subsequent gallbladder distension.1 • drugs and toxins • pancreatitis • sepsis • biliary strictures Exploring intraductal causes • infiltrative diseases, such as amyloidosis • parasitic infections, such as ascaris Stone disease is the most common and sarcoidosis lumbricoides cause of biliary obstruction. More www.Nursing2011.com October l Nursing2011 l 31

Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Hard facts about gallstones More than 1 million Americans are diagnosed with gallstones (cholelithiasis) each year.5 Most gallstones are smaller than 1 cm and are of two major types: cholesterol and pigment. Cholesterol gallstones account for approximately 80% of gallstones in the United States.5 Pigment gallstones differ from cho- lesterol gallstones in their composition and etiology. To form pigment gallstones, an excess of bilirubin in bile combines with other constituents, mainly calcium. These gallstones are black to dark brown and much harder than cholesterol gallstones. Most patients with gallstones are asymptomatic. When gallstones do cause problems, biliary pain (colic) is the most common symptom. It causes pain in the epigastric area and right upper quadrant, classically following the ingestion of a large fatty meal.12 Choledocholithiasis (stone in bile duct) occurs in 15% to 20% of patients with cholelithiasis. In addition, approximately 5% of patients who’ve undergone a have a retained or recurrent stone.9

Liver and gallbladder

Liver (left lobe) Liver (right lobe)

Gallbladder

Gallbladder

Gallstones

Cystic duct

Gallstones in common bile duct

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Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. drug use. He has no family history of bil- 52%), and platelets 222,000 cells/mm3 iary tract disorders, , pancre- (normal, 140,000 to 400,000 cells/ atitis, , or inflammatory mm3). bowel disease, and he denies any signifi- Various LFTs reflect the liver’s con- cant medical or surgical history. dition, the most common being serum Mr. R’s vital signs are: BP, 128/82; transaminases ALT, AST, bilirubin, heart rate, 71; respiratory rate, 12; ALP, albumin, and prothrombin time. temperature, 97.4° F; weight, 176 lb (See Matching assessment findings to a (80 kg). His is soft, nontender, diagnosis.) and nondistended with no masses and Regardless of the cause of cho- no hepatosplenomegaly. He’s jaundiced lestasis, serum bilirubin values are with scleral icterus. No rash is noted. usually elevated and can’t be relied The diagnostic approach to identi- upon to determine the obstruction’s fying biliary diseases begins with a etiology. Blood levels of ALP rise careful history, physical assessment, due to increased synthesis of the and screening lab studies. When tak- enzyme induced by the ing the patient’s history and perform- obstruction.7 ing a physical exam, focus on gathering The ancient treatment The degree of serum transaminase this information.1,6 elevation can occasionally help in • for jaundice included the presence or absence of pain, having patients differentiating between hepatocellu- including the onset, duration, loca- look at yellow lar and cholestatic processes. While tion, and characteristics of the pain AST and ALT values less than eight • birds as unexplained weight loss a cure. times normal may be seen in either • pruritus hepatocellular or cholestatic liver • systemic symptoms, such as anorexia disease, values greater than 25 times and malaise, which may indicate viral Review lab studies to check normal primarily develop in hepato- hepatitis liver function cellular diseases.8 • anemia Mr. R’s blood test results include an Other blood tests, such as hepati- • malignancy albumin of 3.2 (normal, 3.5 to 4.8 g/dL), tis serologies, may be indicated to • gallstone disease total bilirubin 6.4 (normal, 0.3 to differentiate viral from other causes • diabetes 1 mg/dL), conjugated (direct) bilirubin of hepatitis.1 • of recent onset 3.5 (normal, 0 to 0.2 mg/dL), aspar- • use of medications, including over- tate transaminase (AST) 128 (normal, Looking at imaging studies the-counter products and herbal and 14 to 20 U/L), alanine aminotransfer- Mr. R undergoes multiple imaging nutritional supplements ase (ALT) 403 (normal, 10 to 40 U/L), studies. Transabdominal ultrasonogra- • alcohol use (ALP) 269 (nor- phy shows a normal gallbladder with • surgeries mal, 25 to 100 U/L), prothrombin no bile duct dilation. Magnetic reso- • inherited disorders, including liver time 14.2 (normal, 11 to 13 seconds), nance cholangiopancreatography diseases and hemolytic disorders white blood cell count 11,000 cells/mm3 reveals focal intrahepatic duct dilation • HIV status (normal, 4,500 to 10,500 cells/mm3), in segments of both the right and left • recent travel hematocrit 46% (normal, 42% to lobes of the liver. • recent exposure to toxic substances • acholic (pale) stools due to the lack Matching assessment findings to a diagnosis2 of bilirubin in the intestinal tract • GI bleeding Lab test/clinical finding Intrahepatic/hepato- Extrahepatic jaundice • dark amber urine cellular jaundice • jaundice Pruritus Uncommon >75% of patients • ascites, which suggests cirrhosis Uncommon Common • fever (particularly when associated Serum bilirubin levels Increased Usually increased with chills or right upper quadrant Serum ALP levels Usually <3x normal Usually >3x normal pain, which suggests acute cholan- gitis) Serum aminotransferases Elevated, may be Mildly elevated, <300 IU, (ALT, AST) >1,000 IU but may be higher • xanthomata (fatty deposits in the skin), a sign of PBC Prothrombin time and Usually increased; Sometimes increased; • excoriations (from pruritus), which response to parenteral unresponsive to responsive to vitamin K vitamin K therapy vitamin K suggest prolonged cholestasis. www.Nursing2011.com October l Nursing2011 l 33

Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. Transabdominal ultrasonography that might be affected by the use of (US) is the procedure of choice for cautery. For women, a focused medi- the initial evaluation of cholestasis. cal history includes pregnancy status. It’s the least expensive, safest, and Perform medication reconciliation most sensitive imaging study for and assess for anticoagulant use. visualizing the gallbladder. US can Immediately before the procedure, show cholelithiasis, but it’s not as obtain baseline vital signs and confirm useful for CBD stones because bowel that the patient has given informed gas may obscure visualization.6 consent and signed the consent form. (EUS) After the procedure, be prepared to allows complete visualization of the prevent or recognize complications. biliary system, can detect small CBD The most common complications of stones, and is also highly accurate for ERCP with ES are pancreatitis (about detecting pancreatic tumors. EUS has a 5% risk), cholangitis, bleeding, and been reported to have up to a 98% perforation.10 Monitor the patient for diagnostic accuracy in patients with the following signs and symptoms: obstructive biliary disease.1 • severe abdominal pain • A computed tomography (CT) Educate the patient about a firm, distended abdomen scan is usually considered more • nausea/vomiting the disease process, • accurate than US for helping deter- including risk factors, fever or chills mine the specific cause and level of • difficulty swallowing (dysphagia) signs and symptoms of • obstruction. It also provides better biliary obstruction, a crunching feeling under the skin visualization of liver structures. The (subcutaneous emphysema) and prescribed • use of contrast media helps define management. or . vascular structures and the pancreas. Like other invasive procedures, Magnetic resonance cholangio- The extrahepatic ducts are nondilated endoscopic procedures also have pancreatography (MRCP) provides and nonstrictured but contain multiple the potential for sedation-related visualization and measurements of filling defects consistent with stones. complications. However, with the the bile and pancreatic ducts. MRCP Endoscopic sphincterotomy (ES) and judicious use of moderate sedation/ provides a diagnostic cholangiogram stone extraction is performed. analgesia and careful monitoring, in 90% to 100% of patients and ES involves cutting the deep mus- the risk can be reduced significantly. reveals the level of obstruction in cle layers of the sphincter of Oddi to These complications can range in 80% to 100% of cases.1 facilitate stone extraction.9 Biliary severity from transient oxygen Endoscopic retrograde cholangio- obstruction secondary to bile duct desaturation to life-threatening pancreatography (ERCP) combines strictures of pancreatic, biliary, or events such as respiratory arrest, endoscopic and radiologic modalities metastatic origin are treated by plac- cardiac dysrhythmias, and myocar- to visualize both the biliary and pan- ing a plastic or metal within dial infarction. The patient should creatic duct systems. It’s considered the bile duct to ensure patency and have continuous cardiac monitor- the gold standard for imaging the bil- proper drainage of bile. ing, with vital signs monitored iary tree. It’s also used therapeutically; The need for surgical interven- every 5 minutes throughout the some obstructions discovered during tion depends on the cause of biliary procedure. ECRP can be treated by performing a obstruction. For example, cholecys- Closely monitor the patient’s level sphincterotomy, removing stones, tectomy may be the treatment of of consciousness and pulmonary sta- and placing . choice in cases of symptomatic stone tus, including continuous SpO2 mon- disease. itoring and/or capnography. Treatment options is another Mr. R and his wife listen intently as their treatment option in certain patients, Education can minimize risks healthcare provider discusses diagnostic such as those with PSC. Mr. R’s ERCP is successful. After recov- and treatment strategies for his presumed Patient teaching is essential to pre- ering from the procedure and moder- biliary obstruction. After reviewing the pare the patient for ERCP. Following ate sedation/analgesia, he’s discharged risks and benefits with the healthcare facility policy, instruct the patient to home. provider, Mr. R agrees to pursue ERCP, eat nothing after midnight on the Before discharge, educate the patient which is performed in an outpatient set- day before the procedure. Assess the about signs and symptoms to report ting. The procedure reveals segmental patient for allergies, a history of adverse immediately, such as severe abdominal dilation, beading, and stricturing of the reactions to iodinated contrast media, pain, nausea, vomiting, chills or fever intrahepatic ducts consistent with PSC. and for implanted medical devices (which may indicate cholangitis),

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Copyright © 2011 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. difficulty swallowing or neck stiff- But with advancements in current http://www.uptodate.com/contents/enzymatic- measures-of-cholestasis-eg-alkaline-phosphatase-5- ness, and signs of bleeding, such as diagnostic modalities and treatments, nucleotidase-gamma-glutamyl-transpeptidase. vomiting blood or dark tarry stools. complications of biliary obstruction 8. Kaplan MM. Approach to the patient with abnor- ■ mal . UpToDate. 2011:1-20.http:// The patient should also be instructed can be prevented. www.uptodate.com/patients/content/topic. to avoid alcohol and drugs that may do?topicKey=~d9TdAFObj4HGCV. interfere with normal coagulation, REFERENCES 9. Raijman I. Endoscopic management of bile duct 1. Bonheur JL, Ells, PF. Biliary obstruction. eMedi- stones: standard techniques and mechanical litho- such as aspirin and ibuprofen, as cine . 2009:1-20. http://emedicine. tripsy. UpToDate. 2010:1-10. http://www.uptodate. instructed by the healthcare pro- medscape.com/article/187001-overview. com/patients/content/topic.do?topicKey=~mp5ZXed WCD6DPA. vider.11 Ensure that a responsible 2. Ostrow J, Saunders M, Silverstein B. The Gut Course Syllabus, Human Biology 551. 41st ed. Univer- 10. Carr-Locke D. Endoscopic retrograde cholangio- adult drives the patient home after sity of Washington Division of Gastroenterology. pancreatography (ERCP). AccessMedicine. 2010:1-6. 2010:112-171. http://www.accessmedicine.com.offcampus.lib. discharge. washington.edu/content.aspx?aID=6203895/. 3. Roy-Chowdhury N, Roy-Chowdhury J. Classifi- In addition, discharge planning cation and causes of jaundice or asymptomatic 11. Bruesehoff MP. ERCP: much ado about block- includes educating the patient about hyperbilirubinemia. UpToDate. 2011:1-11. http:// ages. Nursing. 2010;40(9):46-50. www.uptodate.com/patients/content/topic.do? 12. Zakko S. Uncomplicated gallstone disease. the disease process, including risk topicKey=~OqyqJgCSG93Ec. UpToDate. 2010:1-16. http://www.uptodate.com/ contents/uncomplicated-gallstone-disease?source= factors, signs and symptoms of bili- 4. Kaplan MM. Clinical manifestations, diagnosis, search_result&selectedTitle=2~150. ary obstruction, and prescribed and natural history of primary biliary cirrhosis. UpToDate. 2010:1-12. http://www.uptodate.com/ management. For example, discuss patients/content/topic.do?topicKey=~zgfICtNw8rsAg. RESOURCE recommended dietary changes and 5. Zakko SF. Patient information: Gallstones. Pfadt E, Carlson DS. Spotlight on sphincter of Oddi review medications to identify any UpToDate. 2010:1-6. http://www.uptodate.com/ dysfunction. Nursing. 2011;41(8):42-45. contents/patient-information-gallstones?source= that may increase the risk of cho- search_result&selectedTitle=2~150. Habiba A. Habib is a staff nurse at the University of lestasis. Encourage the patient to Washington Medical Center, Digestive Disease 6. Roy-Chowdhury N, Roy-Chowdhury J. Diag- Center, Seattle, Wash. Michael Saunders is a Clinical lose weight if indicated, and explain nostic approach to the patient with jaundice or Professor and Director of the Digestive Disease asymptomatic hyperbilirubinemia. UpToDate. Center at the University of Washington Medical how a sedentary lifestyle can 2010:1-7. http://www.uptodate.com/patients/ Center, Seattle, Wash. content/topic.do?topicKey=~Z_vlV7O4fQHgw. increase risks. The authors have disclosed that they have no financial 7. Kaplan MM. Enzymatic measures of cholestasis relationships relating to this article. Unlike the ancients, we don’t have (eg, alkaline phosphatase, 5´-nucleotidase, gamma- a “yellow bird” to cure our ailments. glutamyl transpeptidase). UpToDate. 2011:1-12. DOI-10.1097/01.NURSE.0000405102.68864.c2

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