CC

A 54-year-old White male presents to the emergency department, complaining, “I was hit in the face, and my teeth do not meet right. It has not stopped bleeding.”

HPI

The patient was punched in the face the day before admission while intoxicated with alcohol. He denies loss of consciousness but reports the progressive development of left facial swelling, pain, difficulty eating (secondary to malocclusion), and numbness of his left lower lip. In addition, he describes persistent ooze from inside his mouth where he was hit (secondary to coagulopathy). He was subsequently diagnosed with a left mandibular angle fracture.

PMHX/PDHX/medications/allergies/SH/FH

The patient was diagnosed with alcoholic cirrhosis of the liver and associated portal hypertension 2 years ago. He has had several hospital admissions over the past year for worsening ascites (fluid in the abdomen) and one for upper gastrointestinal (GI) bleeding (secondary to esophageal varices). He has had no regular dental care. His current medications include furosemide (a loop diuretic), spironolactone (a potassium-sparing diuretic), propranolol (a nonselective beta-blocker), and omeprazole (a proton pump inhibitor [PPI]). He drinks 2 quarts of wine every other day. (Drinking more than four standard drinks per day [each standard drink is 5 fl oz of wine, 1.5 fl oz of hard liquor, or 12 fl oz of beer] would increase the risk of liver insult.)

Examination

General. Generalized muscle wasting (secondary to poor nutrition and protein catabolism) and lethargy (secondary to hepatic encephalopathy).

Vital signs. Blood pressure is 155/92 mm Hg (elevated blood pressure), heart rate is 72 bpm, respirations are 22 breaths per minute (tachypnea), and temperature is 36.2°C.

Neurologic. The patient is alert and orientated ×3 (person, place, and time) but intermittently confused, with asterixis (flapping of the hands with the arms and palms fully extended, a sign of hepatic encephalopathy).

Maxillofacial. Scleral icterus (because of hyperbilirubinemia), fetor hepaticus (caused by elevated serum ammonia level), enlarged parotid glands (caused by metabolic and nutritional derangements associated with chronic alcoholism), decreased sensation to light touch and direction of left V3, and left mandibular angle swelling and ecchymosis.

Chest and pulmonary. Bilateral crackles in the lung bases (fluid in the alveolar spaces), bilateral gynecomastia (enlarged breasts secondary to increased levels of estrogen), and hair loss over the chest.

Cardiovascular. Regular rate and rhythm, with no murmurs, gallops (S3 or S4), or rubs.

Abdominal. The abdomen is nontender and distended, with shifting dullness (caused by ascites) and splenomegaly (caused by portal hypertension secondary to liver cirrhosis). (It is very unlikely to palpate a nodule of liver on clinical examination.) Percussion on the right upper quadrant indicates hepatomegaly, and caput medusa (tortuous periumbilical veins secondary to portal hypertension) is also noted.

Extremity. Bilateral lower extremity 1+ pitting edema (secondary to hypoalbuminemia), Dupuytren contracture in the right index and middle fingers (flexion deformity of the fingers secondary to flexor tendon fibrosis), and palmar erythema.

Skin. Multiple small petechiae, spider angiomas, and testicular atrophy (all secondary to decreased hepatic metabolism of estrogen) are present.

Labs

The laboratory test in the workup of patients with liver disease can be complex and crucial to the evaluation of the extent of liver injury and the degree of dysfunction with associated systemic involvement. A complete metabolic panel includes hepatic transaminases. Elevated hepatic enzymes reflect hepatocellular dysfunction. In the current patient, both aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels are elevated. (The AST-to-ALT ratio usually is >2:1 with alcoholic hepatic damage.) However, as the liver progress from hepatitis to cirrhosis, these values return to normal. The functionality of liver is better evaluated by coagulation and albumin production. Ordered separately, elevated alkaline phosphatase (ALP) and g-glutamyl transpeptidase (GGT) levels are also seen (reflecting biliary system abnormalities). Elevated blood urea nitrogen (BUN) and creatinine levels can be seen, especially if there is associated hepatorenal syndrome. Hypokalemia and hypomagnesemia are also common with malnutrition and need to be corrected.

A complete blood count generally shows a macrocytic anemia (mean corpuscular volume >100/μm ³ ) (secondary to vitamin B ₁₂ and folate deficiency) with thrombocytopenia (secondary to hypersplenism, increased sequestration, and decreased hepatic production of thrombopoietin). An elevated prothrombin time (PT), partial thromboplastin time (PTT), and international normalized ratio (INR) are secondary to decreased synthesis of coagulation factors. The PT is often elevated first because of the shorter half-life of the vitamin K–dependent factor VII that is part of the extrinsic pathway measured best by the PT (even small decreases in factor VII result in increased PT) or the INR. High blood ammonia levels reflect the inability of the liver to convert ammonia to urea for excretion by the kidneys. Hypoalbuminemia is reflective of decreased albumin production in the liver. Finally, unconjugated hyperbilirubinemia (causing scleral icterus) is seen because of decreased bilirubin conjugation by the liver.

For the current patient, the following laboratory test results were obtained:

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  Chemistry: Sodium, 133 mEq/L (increased water retention caused by low renal perfusion would result in relative hyponatremia); potassium, 3.1 mEq/L; BUN, 48 mg/dL; creatinine, 1.6 mg/dL; glucose, 172 mg/dL; magnesium, 1.0 mg/dL; bilirubin, 1.3 mg/dL; ammonia, 67 mmol/L; albumin, 2.2 mg/dL

• •

  Complete blood count: white blood cells, 4500/μL; hemoglobin, 9.5 g/dL; hematocrit, 30.1%; platelets, 62,000/μL

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  Coagulation studies: PT, 17 seconds; PTT, 43 seconds; INR, 1.5

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  Liver function tests: AST, 141 U/L; ALT, 84 U/L; GTT, 45 U/L; ALP, 51 U/L

Imaging

A panoramic radiograph revealed a fracture of the left mandibular angle.

For evaluation and diagnosis of liver cirrhosis, a computed tomography–guided liver biopsy can be done as needed to demonstrate destruction of normal hepatic architecture with fibrotic changes, confirming the diagnosis of liver cirrhosis.

Assessment

Mandibular fracture complicated by hepatic dysfunction secondary to alcoholic cirrhosis.

Treatment

Preoperative preparation of patients with severe liver disease is of paramount importance to prevent perioperative complications. Preoperative management includes administration of 100 mg of thiamine (to prevent Wernicke encephalopathy, characterized by ophthalmoplegia, ataxia, and memory impairment), a nutritious diet, and multivitamins with folic acid and vitamin B ₁₂ supplementation. (Excess alcohol consumption is often associated with nutritional deficiencies.) Any coagulopathy needs to be addressed preoperatively (see the Complications section).

In the current patient, hypokalemia and hypomagnesemia were corrected with potassium chloride and magnesium sulfate infusions. Librium, a benzodiazepine, was given as a taper over 4 days to prevent life-threatening alcohol withdrawal (delirium tremens). Because of the risk of aspiration (increased in patients with alcoholism), the patient was also continued on a PPI (decreases gastroesophageal reflux and the degree of chemical pneumonitis if aspiration occurs). Because of the patient’s obvious respiratory distress as a result of the ascites, paracentesis (removal of peritoneal fluid) was performed (pleural effusion, ascites, and an elevated diaphragm lead to atelectasis); the removal of 4 L of fluid (with care taken to prevent hypotension) brought an immediate reduction in the work of breathing and the respiratory rate. The patient was started on furosemide and spironolactone to reduce the severity and frequency of recurring ascites. The hepatic encephalopathy was treated with administration of lactulose (to decrease ammonia production by enteric bacteria). The coagulopathy was treated with 6 units of fresh-frozen plasma (to overcome deficiencies of multiple coagulation factors) and 4 units of platelets (to increase the platelet numbers to >100,000 cells/μL). Subsequently, the patient underwent open reduction with internal fixation of the fracture without complications.

Complications

Complications for patients with liver disease are inherently dependent on the degree of functional impairment of the liver and concomitant preoperative systemic conditions.

Patients tend to be protein depleted, fluid overloaded, vitamin deficient, and coagulopathic, with electrolyte abnormalities, and often have an impaired ability to metabolize medications.

Adjunctive enteral feeding (nasogastric or orogastric tube) may be necessary in the perioperative period to meet caloric needs, especially in the setting of oral and maxillofacial surgery, when chewing may be difficult (e.g., intermaxillary fixation, swelling, pain). Lack of oral intake can result in dehydration; abdominal pain; and low glycogen storage, which shifts metabolism to the fat and lips. This results in a decrease in insulin and increase in counterregulatory hormones such as cortisol, glucagon, and epinephrine. The increase in lipid metabolism results in increased ketone production, which is the underlying pathophysiology for alcoholic ketoacidosis. Parenteral nutrition may also be considered but only in the setting of compromised GI function. (If the gut works, use it.) Caloric requirements should be calculated with consideration to reducing short-chain fatty acids and mercaptans acid content to prevent exacerbation of any encephalopathy. The latter is thought to relate to the blood ammonia level (however, a clear correlation between the grade of encephalopathy and ammonia does not exist), which can be further reduced with the use of lactulose (encephalopathy is graded from 0 [minimal] to 4 [coma]). Malnutrition and impaired protein synthesis impair wound healing, which can present as increased wound breakdown and delayed healing.

Coagulopathy may be the result of decreased platelets from splenic sequestration. (Hypersplenism occurs secondary to portal hypertension and nitric oxide–induced splenic vasodilation, both secondary to liver cirrhosis and thrombopoietin reduction synthesized by the liver.) Platelet transfusion is the only treatment for thrombocytopenia. Spontaneous bleeding is seen with platelet counts less than 30,000/μL; for most minor procedures, a count greater than 50,000/μL is appropriate. Ideally, the patient should be transfused to a platelet count greater than 100,000/μL for major surgeries and procedures.

Coagulopathy may also be the result of decreased hepatic synthesis of clotting proteins, as is often the case with end-stage liver disease, or it may be the result of decreased absorption of fat-soluble vitamins (vitamins A, D, E, and K) from the GI tract. The latter is more common with cholestatic liver disease. (Decreased bile salts reduce the absorption of fat and fat-soluble vitamins.) In this situation, vitamin K can be administered, with an appropriate increase in the synthesis of vitamin K–dependent coagulation factors (factors II, VII, IX, and X). The endpoint of management is a substantial improvement in or normalization of the PT or INR. When decreased hepatic synthesis of coagulation proteins is the result of intrinsic liver disease (as in the current patient), transfusion with fresh-frozen plasma is the treatment of choice. Care must be taken to avoid worsening of the total-body fluid overload, which is typical of ascites and may precipitate pulmonary edema.

Liver failure may also be associated with hepatopulmonary syndrome (triad of vasodilation, increase in alveolar-arterial gradient, and chronic liver disease), hepatorenal syndrome (decreased renal perfusion, elevated renin and aldosterone, and exacerbated reduction in capillary perfusion, with electrolyte disturbances, improved with administration of terlipressin or noradrenaline), upper GI bleeding, and subacute bacterial peritonitis. There is a hyperdynamic circulatory system with systemic vasodilation. This is caused by increase in nitric oxide and downregulation of the sympathetic nervous system. This results in an increase in cardiac output and eventual cirrhotic cardiomyopathy. Most drugs are metabolized by the liver and as such may need to be dosed appropriately or avoided altogether. Drugs that are renally excreted are preferable to those that require hepatic metabolism.

End-stage liver disease (ESLD) can be treated with liver transplantation, although most patients die of liver disease or are not eligible for transplantation. In 2002, the Mayo Clinic began to stratify ESLD liver transplant recipients with an objective calculator to determine the severity of liver dysfunction. The Model for End-Stage Liver Disease (MELD) score can be used to predict morbidity and mortality in patients needing nonliver surgery. The MELD score is readily calculated using the patient’s INR, bilirubin, and creatinine. The formula is available at the website http://www.mayoclinic.org/meld/mayomodel5.html . Elective nonliver surgery is acceptable in a patient with a MELD score below 10; a score between 10 and 15 necessitates careful assessment of risks and benefit; and a score above 16 effectively eliminates elective procedures. Our patient, despite having a MELD score of 16, was a candidate for open reduction and internal fixation of his fractured mandible because it is not elective surgery. However, elevated MELD scores are associated with increased perioperative morbidity and mortality. Successful liver transplant recipients have functionally normal livers but are immunosuppressed to prevent graft rejection. This may result in an increase in both opportunistic and perioperative infections. The MELD score replaced the Child-Pugh classification, which is measured by albumin, hepatic encephalopathy, INR, and total bilirubin. MELD-Na (sodium level) is used now as the predictor of death for liver transplant.

Discussion

Liver disease can be the result of many insults. It is now considered the fifth most common cause of death after coronary disease, cerebrovascular accident, chest disease, and cancer. The most common causes are alcohol consumption and viral hepatitis. The disease progress from fatty liver to alcoholic hepatitis to chronic hepatitis to cirrhosis. Hepatitis C is more common than hepatitis B, with an estimated 4 million cases in the United States. As many as 90% of these cases are chronic. As the population increases in prevalence of obesity, hepatic steatosis (nonalcoholic fatty liver disease) and liver scarring is on the rise. Finally, biliary obstructive disease such as cholangiocarcinoma or pancreatic cancer can result in hepatic failure. Viral hepatitis also poses a risk for transmission to the surgeon and operating room staff from needlestick injury. Particular care should be taken to reduce this risk. The causes of liver dysfunction are many, but the consequences are often similar. Cirrhosis is the final common pathway of chronic inflammation and is irreversible. Alcoholic cirrhosis may coexist with alcoholic hepatitis. Liver dysfunction is associated with malnutrition, protein catabolism, poor wound healing, coagulopathy, portal hypertension, splenomegaly, ascites, portosystemic venous shunts (esophageal, periumbilical, retroperitoneal, and hemorrhoidal shunts), encephalopathy, and impaired drug metabolism and clearance. A proper history and physical examination, in addition to appropriate laboratory tests, are critical in the perioperative period for the surgeon. All these factors combine to make management of patients with liver disease a challenging and difficult task.