NURS 6560 Final Exam Study Guide Liver Biliary Tract, Pancreas, Spleen Completed for 2022/2023

NURS 6560 Final Exam Study Guide Liver Biliary Tract, Pancreas, Spleen  Evaluation, differential diagnosis, diagnostic studies, and treatment options 1.Resection of the liver and regeneration and follow up labs pp 54 ...

NURS 6560 Final Exam Study Guide Liver Biliary Tract, Pancreas, Spleen  Evaluation, differential diagnosis, diagnostic studies, and treatment options 1.Resection of the liver and regeneration and follow up labs pp 546-548 -commonly indicated for primary and secondary malignant tumors and symptomatic benign tumors -May also but indicated in traumatic injury, infection/abscesses, and living donor transplantation -after resection, liver function will be impaired for several wks -Regeneration starts within 24hr with hepatic cell replication and has considerable regeneration within 10 days and completed regeneration by 4-5wks. -growth consists of formation of new lobules and expansion of residual lobules -Resection is contraindicated in cirrhosis due to the liver not being able to meet metabolic demand for regeneration of tissue. -Child-Pugh classification: based on amount of ascites, degree of encephalopathy, albumin, total bilirubin and PTT/PT levels; this predicts mortality in pts with cirrhosis after hepatic resection -Child-Pugh A and selective B pts are candidates for resection -up to 80% of liver can -MELD score: used to improve allocation of liver transplant to cirrhosis pts with highest risk of death; also used to assess liver function in pts undergoing resection2 Types of Hepatic Resections: anatomical (based on segmental liver anatomy) and nonanatomical -Anatomical resections are proffered due to less blood loss, and lower incidence of positive resection margins with malignancy -major resections are done in accordance with segmental anatomy -Two step resection may benefit pts with multiple metastases- allowing time for the liver to regenerate and compensate for the second resection. -operation entails removal of lobe or segment and it’s afferent and efferent vessels while avoiding remnant tissue-most elective resections done with abdominal incision (open approach) and is standard of care, more are being done laparoscopically. -If the pt has a very large right lobe tumor then incision should be throacoabdominal approach. -Perioperative goal is to minimize blood flow by: achieving vascular inflow and outflow control, careful division of the liver with control of vascular structures, inking low central venous pressure anesthesia (which reduces hepatic venous blood loss). -Pringle maneuver: (temporary occlusion of the hepatic artery and portal vein) clamping portal inflow pedicle for 10-15 min to decrease blood loss -preoperative portal vein embolization may improve safety of major resection -Close monitoring is need for major resections for several days. -Major post-operative concern is bleeding -Pts without cirrhosis may have some metabolic changes but normalize and can be discharged within 7-8 days post op. Follow Up Labs after Resection: -T.Bili: increases after surgery but returns to normal as regeneration progresses; persistent rise may indicate liver failure and perihepatic fluid collection(biloma)-Albumin: decreases -PTT/PT: increases : give FFP when INR>2.0 -Ascites: give diuretics -Hypoglycemia(due to glycogen stores in the liver): 5% Dextrose solutions; profound hypoglycemia concern for liver failure -Phos/Mg/K+: decreases and must be replaced -AST/ALT: increases in the first few days pos op then normalizes -ALK PHOS: initially normal then rises and remains high for days to weeks Complications post op resection: include perihepatic fluid collectiaons which may require drainage, hepatic insufficiency (hyperbilirubinemia, ascites, coagulopathy) common but resolves, pleural effusions, atelectasis, and pneumonia (tx with aggressive pulmonary toilet post op). Hepatic Trauma: -Blunt or penetrating trauma -Penetrating: more than 1/2 of cases and include projectiles such as bullets, shrapnel or knives. -In civilian practice most of these trends tend to be clean wounds that are dangerous because of increased intra-abdominal bleeding. -Military weapons: high velocity projectiles and that energy is transferred to the abdominal viscera and can shatter the parenchyma even if the projectile does not enter the liver. -Blunt trauma: direct blow to the upper abdomen or lower right rib cage -Causes of blunt trauma include: far from great height, (sudden deceleration), MVC (can produce explosive bursting wounds or linear lacerations of the hepatic surface with parenchymal destruction), shearing forces (tear the hepatic veins leading to retrohepatic exsanguination injury which is hard to expose and repair during surgery). -Stellate bursting type injury affects the posterior and superior aspect pot the right liver (segment VI, VII, and VIII). -Damage to the left liver is less common -Imaging includes: CT scan which allows staging of injury-Clinical manifestations: hypovolemic shock, hypertension, decreased urinary output, low central venous pressure, abdominal distention, hemodynamic instability, rich upper quadrant abdominal pain, -Labs: leukocytosis greater than 15,000 following rupture of the liver from blunt trauma, acidosis and coagulopathy. -FAST exam may be sensitive of 97% with hemoperitoneum larger than 1 L and can help direct surgical intervention -High-resolution CT scan with IV contrast should be obtained in all patients suspected to have a hepatic injury. -Angiography is generally not helpful and acute setting for diagnosis of liver injury. -Regardless of grade that the liver injury hemodynamic stability of patients dictates whether or not the patient will be managed by surgery or medical management. -CT scan finding associated with medical management include: small hemoperitoneum, unilobar fracture, contained sub capsular or intrahepatic hematoma, or absence of injury to other abdominal organs. -If pt has drop in H&H then repeat CT scan should be ordered to verify if lesion is stable or not. (If a blush of IV contrast is present then bleeding can be addressed by embolization). -CT scans that show active bleeding or major injury require surgery -If bleeding has stopped then suturing wound on liver should not be done -Active bleeding should be managed by clipping or direct suture of the vessels. -The Pringle maneuver can be used to reduce bleeding and permit more accurate ligation of the bleeding vessels. -Absorbent gauze mesh (poly glycolic acid) can sometimes be wrapped around an injured lobe and sutured in a way that maintains pressure and tamponades the bleeding, this is difficult to accomplish without rendering the involved liver ischemic. -In some cases, control of arterial hemorrhage requires ligation of the hepatic artery or one of the accessible major branches in the hilum. -The most difficult problem involves lacerations of the major hepatic veins behind the liver. -Temporary clamping of the inflow vessel has no impact on back bleeding from the inferior vena cava and does not allow adequate inspection and repair of the injured vessels. -For persistent bleeding, the abdominal incision can be extended into a mediansternotomy to improve exposure. -Appropriately placed ligatures around the superior and inferior hepatic vena cava combined with the Pringle maneuver permit total isolation of the liver circulation. -In many cases when bleeding is difficult to control it especially when other injuries must be addressed damage control is the best strategy involves packing the liver to achieve hemostasis. -Packs are left in for 48 to 72 hours during which the patient remains sedated and intubated in the ICU. -The packs are removed in the operating room if persistent bleeding is noted definitive repair of the injury can then be performed in a controlled fashion. -For superficial liver injury is bleeding can be controlled with direct compression topical agents or electrocautery or Eragon beam coagulation. Hepatic Trauma Post op complications: -Pts who rebelled early from liver wound after initial suture ligation should have re-exploration and packing. -Bile leaks can occur with both blunt and penetrating injury/ tx: percutaneous drain -stents can be placed in the =bile ducts and sphincter of Oddi to relieve pressure on biliary system -Abscess can occur after blunt trauma and tx with drain and antibiotics Spontaneous Rupture of the Liver: -Not common -maybe due to hepatic tumors( 5%of hepatocelluar carcinoma tumors can rupture) and during pregnancy, -most are due to hepatic tumors (adenoma >5cm are at risk; 20-40%) -Other conditions that can result in liver future include hepatic hemangioma, typhoid fever, malaria, tuberculosis, syphilis, polyarteritis, and diabetes mellitus. --many cases of normal rupture occur during or after pregnancy r/t preeclampsi/eclampsia or HELLP Syndrome (hemolysis, increased enzymes, decreased PLT) -suspect rupture in all pregnant or postpartum pts (esp. if hypertensive) if complaining of acute discomfort of upper abdomen-Progression: intrahepatic hemorrhage to subcapsular hematoma to capsular rupture to free intra abd hemorrhage -Diagnosis: CT scan; angiography with hepatic artery embolization effective for controlling bleeding; if not successful then emergency laparotomy and intraoperative mgmt. Primary Liver Cancer: -Hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinomia, with HCC being the most common. -Tumor arising from hepatocytes (HCC) or biliary epithelial cells (intrahepatic cholangiocarcinomia) Hepatocellular Carcinoma (HCC): 85-95% of primary hepatic cancers -Causes include Hepatitis B with or without cirrhosis and C with cirrhosis virus and cirrhosis from any cause - fungal metabolites called aflatoxins have produced liver tumors: staple foods(ground nuts and grain) in parts of Africa where HCC is high. - Mass-forming type: characterized by a single predominant mass clearly demarcated from the surrounding liver , occasionally with small satellite nodules - Nodular type: composed of multiple nodules often throughout the liver - Diffuse type: infiltration of tumor throughout the remaining parenchyma - AJCC staging system can be used after pathological exam of the resected liver - 50% of resected tumors are surrounded by a fibrous capsule, encapsulated tumors exhibit low incidence of tumor microsatellities and venous permeation and is a favorable sign for pt - Large portion of pts have either intra-hepatic or extra-hepatic metastases on presentation - Tumors larges than 5cm have vascular invasion which could result in hemorrhage - Metastases is common in hilar and celiac lymph nodes and the lungs, less common is bone and brainIntrahepatic Cholangiocarcinoma: - Less often associated with cirrhosis - Causes include: Infection of liver flukes caused by Clonorchis sinensis (seen in Asia), chronic Hep C, obesity, DM, chronic liver disease, and smoking - Present as a large mass in the liver arising from the extra hepatic biliary tree - Invasive tumors and can grow to be very large- Angiocarcinoma: rare and fatal tumor seen in workers in contact with vinyl chloride for prolonged periods of time in polymerization plants - Hepatoblastomas: common in childhood - Clinical manifestations for Primary Liver Cancers include: asymptomatic in early stages, epigastric pain, RUQ abdominal pain, referred pain to right shoulder, jaundice (rare in small tumors and with pts with god liver function), hepatomegaly, palpable mass, friction rub over liver, arterial bruit over liver, ascites, GI bleeding from varices, - Acute decline of well compensated cirrhosis pt should suspect HCC - Labs in Primary Liver CA: elevated AST and ALT, elevated alkaline phosphatase in absence of underlying liver disease, elevated bilirubin (ominous finding, and reflects liver dysfunction), Low albumin, coagulopathy, thrombocytopenia, may be + for HBsAg or HCV antibody, tumor makers elevated (alpha fetoprotein(AFP)- values above 200 suggest HCC, while levels in the cirrhosis pt of 400 with hyper vascular mass > 2cm is diagnostic) - Imaging: liver US, CT scan and MRI - Triple phase CT scan: (no contrast, arterial phase and portal venous phase), contrast-enhanced helical CT scan of the chest abdomen and pelvis provides the best images of the disease. - Diagnostic angiography: was used to assess liver tumors but is now reserved for treatment (normal liver can clear dye quickly but HCC liver it will remain opacified) - Liver biopsy: percutanoues core or aspiration biopsy - Aspiration biposy is associated with 30% false negative results - Percutaneous biopsy should be done with high suspicion of cancer, risk of bleeding can occur tumor dissemination by seeding may occur - In pts with cirrhosis with hypervascular mass larger than 1cm CT or MRI can predict carcinoma even without biopsy - Monitoring: pts with cirrhosis, Hep B and C should have biannual liver US - High incidence of chronic Hepatitis in Asia - Complications: intra-abdominal hemorrhage from spontaneous bleeding, Budd-Chiari syndrome, liver failure (most common cause of death in these pts).- Budd-Chiari syndrome: results from obstruction of hepatic venous outflow -most cases are caused by spont. thrombosis of hepatic veins associated with myeloproliferative disorders or the use of birth control pills -S/S: begins with right upper quadrant abd pain, post prandial bloating, and anorexia; after weeks or months: gross ascites, hepatomegaly, hepatic failure (increased AST, increased T. Bili, Alk Phos abnormal -Diagnosis: made by venography Treatment for Liver Cancer: Resection, translation, liver direct therapy, ethanol injection, ablation, embolization and systemic therapy - Resection: is the tx of choice in select pts without cirrhosis or with cirrhosis pts with well preserved hepatic function. - Criteria for Resection: disease confined to liver and disease amenable to the complete resection. - Multiple tumors and tumor invasion of major portal or hepatic veins has bad prognosis - Larger tumors may require more extensive resection - Best prognosis for resection pts with solitary small tumors < 3cm, and well preserved liver function - Cirrhosis pts with Child-Pugh A score may have survival benefit form resection - Cirrhosis pts have increased risk for developing new tumors in residual liver after resection and are better tx with transplantation - Overall tumor recurrence is 70% at 5 yrs post op with 5yr survival rate of 40-70% - Transplantation: HCC is the only solid neoplasm for which transplantation play a significant role (can treat the malignancy and the underlying cirrhosis)- Most likely to benefit: single tumor no large then 5cm, or up to 3 tumors no larger then 3cm, no major vascular invasion (Milan criteria). Ethanol Injection: Tx for Liver Cancers: - option for small unresectable HCC - Using US or CT guided 95% ethanol (5-20ml) in injected though a 22 gauge needle directly into the tumor - Can achieve complete narcosis of 90-100% on tumor in small tumors <2 cm - Necrosis declines in efficacy as the tumor gets larger in size - Follow treatments may be needed for residual tumor or new primary tumors Radio-frequency Ablation (RFA): TX for Liver Cancers - Percutaneous ablative approach and useful in small tumors - Using US or CT guided a needle is red to access the lesion and the needle is attached to a radio-frequency generator that generates thermal energy to bring the tumor destruction- Can be used percutaneous, laparoscopically or laparotomy - Goal is the same as the ethanol injection-tumor necrosis - Effective in tumors < 2cm Arterial Embolization: TX for Liver Cancers - Pt must have good liver function - Child-pugh C cirrhosis of thrombosis of the portal vein are not candidates - Gelfoam can be used and dissolves in a few weeks - Some centers use invert particles without chemotherapy (bland embolization), but most employ chemotherapy medications (transarterial chemoembolization or TACE) - Chemo medications used in TACE: doxorubicin, mitomycin, and cisplatin. - Lipiodol lodges in the tumor and can be used as a carrier of chemo medications - May take multiple treatments Metastatic Neoplasms of the Liver: - Metastatic cancer in the liver is more common than primary liver tumors - Cancers that metastases to the liver: GI tract cancers (colon, pancreas, esophagus, stomach, and neruoendrocrine), breast, lung, GU tract (kidney and adrenal), ovary and uterus cancers - Spread can be through the systemic or portal venous circulation - Most common is colorectal cancer then lung cancer - General s/s: weight loss, fatigue, pain, anorexia, ascites, jaundice, fever, abdominal pain, friction rub one liver, - Labs: anemia, low albumin, increased alkaline phosphatase, tumor markers - Imaging: CT scan, MRI, PET scan- Treatment: most patients with metastatic liver disease systemic chemotherapy is the only choice - Not curative just palliative management except for colorectal cancer which with resection and other tx can be effective Hepatic hemangiomas : -most common site is liver and more common with women -are cavernous type rather than capillary type -most are small solitary subcapsular growths found during laparotomy or imaging study; pain is uncommon in tumors< 8-10cm diameter -Complications: hemorrhagic shock from spont. rupture and Kasaback-Merritt syndrome(children); very rare -Large Hemangiomas of Liver: may be associated with others in skin; may cause large volume AV shunting leading to cardiac hypertrophy leading to CHF -diagnosis made with CT with contrast or MRI -Hallmark features: nodular peripheral enhancement on arterial phase with progressive central enhancement on the more delayed images; MRI appear very bright on T2 weighted images -only reasons to resect are for symptoms: pain or diagnostic uncertainty -Symptomatic hemangiomas: can be excised by lobectomy or enucleation -vast majority incidentally found remain stable and asymptomatic -progressive growth of asymptomatic in a short time is considered indication for resection Hepatic adenomas: -predominantly seen in women of childbearing age and appears to be related to oral contraceptives (Mestranol containing compounds) -tumors are soft, yellow tan, well circumscribed masses of moderate size (2-15cm); symptomatic are 8-15cm -50% of pts are asymptomatic -Symptoms: right upper quadrant pain -Complications: spont. hemorrhage into tumor with subsequent rupture intraperitoneal bleeding ; strong association with acute bleeding episodes from adenoma with pregnancy -general recommendation is that they should be resected because of risks of malignant changes and spont. hemorrhage -lesions <5cm should be watched with serial images-small adenomas may regress if oral contraceptive is discontinued -symptomatic and large asymptomatic should be resected -if hemorrhaging, emergent resection or hepatic artery embolization Cirrhosis: -Clinical manifestations: ascites, RUQ pain, hepatorenal syndrome, hyponatremia, renal insufficiency, coagulopathy, encephalopathy, varices, bleeding - Treatment: TransplantationPortal Hypertension (PH): In all but a few instances, the basic cause is increased resistance to portal flow which are subclassified as prehepatic, hepatic, and posthepatic. hepatic causes -subclassified as presinusoidal, sinusoidal, and postsinusoidal. Most common causes of PH- Cirrhosis from alcohol use (85%), Postnecrotic cirrhosis, biliary cirrhosis. hepatic schistosomiasis largest single group in some part of the world. Idiopathic PH- mostly in southern Asia. After cirrhosis, extrahepatic portal venous thrombosis or occlusion is the most common cause of portal hypertension in the United States. Patients with this condition are generally younger than cirrhotics, and many are children. Posthepatic obstruction due to Budd–Chiari syndrome (BCS) or constrictive pericarditis is rare. Pathophysiology: PH- is defined as a hepatic venous pressure gradient (HVPG) >5 mm Hg, but usually become clinically significant when this gradient reaches 10 mm Hg. Portal venous pressure normally ranges from 7 to 10 mm Hg. In portal hypertension, portal pressure exceeds 10 mm Hg, averaging around 20 mm Hg and occasionally rising as high as 50-60 mm Hg. Pressure = Flow × Resistance, PH could result either from increased volume of portal blood flow or increased resistance to flow. Pathophysiology -In alcoholic liver disease, the abnormal resistance is predominantly hepatic and postsinusoidal, as indicated by the results of wedged hepatic vein pressure studies.* The causes of increased resistance in this disease are thought to be: (1) distortion of the hepatic veins by regenerative nodules and (2) fibrosis of perivascular tissue around the hepatic veins and the sinusoids. Even in the absence of cirrhosis, acute alcoholic hepatitis can raise portal pressure by producing centrilobular swelling and fibrosis. Sinusoidal resistance to flow is also increased by engorgement of adjacent hepatocytes with fat and resultant distortion and narrowing of vascular channels. Documented cases of normalization or reduction in portal pressure have occurred with resolution of the pathologic changes. Schistosomiasis can produce a unique formof hepatic presinusoidal obstruction to blood flow from deposition of parasite ova in small portal venules. The subsequent chronic inflammatory reaction leads to fibrosis and cirrhosis. Many patients with schistosomiasis are also at risk for chronic hepatitis, which can exacerbate the liver damage. BCS (hepatic vein thrombosis) results from obstruction of flow through the hepatic veins. The resulting sinusoidal hypertension produces prominent ascites and hepatomegaly. Conditions (veno-occlusive disease, inferior vena cava obstruction by tumor or congenital webs, right-sided heart failure) that reduce flow through the hepatic veins result in a similar clinical picture. Banti syndrome was defined as liver disease secondary to primary splenic disease and was incorrectly considered as the cause of portal hypertension now known to result from cirrhosis and other hepatic disorders rather than a consequence of such conditions. Portal hypertension from splenomegaly and increased splenic vein flow has been described in patients with hematologic diseases or tropical splenomegaly and apparently normal liver function. This is extremely uncommon, however, and given the great reserve of the liver to handle increases in portal flow, many such patients probably have some component of liver disease. In cirrhosis, the increased splenic blood flow accompanying “congestive” splenomegaly may occasionally be great enough to warrant splenic artery ligation or splenectomy to decrease portal pressure and improve symptoms, but this situation is rare. Development of Portosystemic Collaterals and Varices The obstacle to flow through the liver promotes expansion of collateral channels between the portal and systemic venous systems. As the pathologic process develops, portal pressure increases until a level of about 40 cm H2O (30 mm Hg) is reached. At this point, increasing hepatic resistance, even to the point of occlusion of the portal vein, diverts a greater fraction of portal flow through collaterals without significant increments in portal pressure. In extrahepatic portal vein thrombosis (without liver disease), collaterals in the diaphragm and in the hepatocolic, hepatoduodenal, and gastrohepatic ligaments transport blood into the liver around the occluded vein (hepatopetal). In cirrhosis, collateral vessels circumvent the liver and deliver portal blood directly into the systemic circulation (hepatofugal); these collaterals give rise to esophageal and gastric varices. Of the many large collaterals that form as a result of portal hypertension, spontaneous bleeding is relatively uncommon except from those at the gastroesophageal junction; spontaneous bleeding from gastric varices can sometimes occur and carries a higher rate of death than gastroesophageal varices. Compared with adjacent areas of the esophagus and stomach, the gastroesophageal junction is especially rich in submucosal veins, which expand disproportionately in patients with portal hypertension. The cause of variceal bleeding is most probably rupture due to sudden increases in hydrostatic pressure. Esophagitis is usually mild or absent. Acutely Bleeding Varices -Varices develop in 5-15% of cirrhotic patients. Most patient who have cirrhosis develop varices, but only ⅓ experience variceal hemorrhage. Each bleeding episode is associated with mortality rate up to 25% and 70% untreated patients die within a year of the first hemorrhage.Co-existing factors: malnutrition, pulmonary aspiration, infections, coronary artery disease. Complicating factors: lack of cooperation with treatment and acute alcohol withdrawal, delirium tremens adds greatly to the high mortality rate. Clinical findings: previous documentation of cirrhosis, varices, hematemesis, significant hemorrhage with alteration of mental status, hypotension, and tachycardia, often in hypovolemic shock. Bleeding from varices cannot be accurately diagnosed on clinical grounds alone. Laboratory findings: Bilirubin ↑, serum albumin ↓ 3g/dl. Leukocyte count maybe ↑. Anemia may be a reflection of chronic liver disease or hyperslenism as well as acute hemorrhage. CT Scan and marked elevation of serum �-fetoprotein make the diagnosis. Thrombocytopenia and coagulopathy are common. Treatment of Acute bleeding: GOAL of TREATMENT is CONTROL THE BLEEDING AS QUICKLY AND RELIABLY AS POSSIBLE WITH THE FEWEST POSSIBLE SIDE EFFECT. ● Medical-vasoconstrictor (somatostatin analogs, Vasopressin, terlipressin) ● Medical-Antibioprophylaxis (norfloxacin, ciprofloxaxin, ceftriaxone (if doubt quinolone resistance)● Interventional, non-surgical ( Endoscopic variceal ligation, transjugular intrahepatic portosystemic shunt TIPS) ● Mechanical (Balloon tamponade (Sengstaken-Blakemore) ● Surgical (Emergency portosystemic Shunts, Esophageal transection and reanastomosis, Esophagogastric devascularization, suture ligation of varices) Critical initial Steps: 1. Airway protection (especially in those who have AMS or hemodynnamic instability) 2. Resuscitation with fluids and blood products. 3. In cirrhotic patient correction of coagulopathy and thrombocytopenia be started early. 4. Antibiotic therapy (Ceftriaxone is preferred) 5. Vasoconstrictors: octreotide and vasopressin/terlipressin 6. Endoscopic sclerotherapy or ligation by Esophagogastroscopy 7. TIPS procedure with balloon tamponade if necessary; if not possible 8. Surgical Shunt NON-Bleeding Varices Gastroesophageal Varices are present in half od patient with cirrhosis at the time of diagnosis. Development and growth of esophageal varices each occur at a rate of 7% per year. Patient with varices who have never bled have a30% of chance of bleeding at some point. Of those who have bled 50% die. Those diagnosed in the first year and do not bleed the risk decreases by half and continues to decrease. Those who have bled once have a 60-70% chance of bleeding again and about ⅔ of repeat bleeding is fatal.Evaluation: Measurement of pressure and flow in the splanchnic vasculature is used for diagnosis and a guide to therapy and prognosis in portal hypertension. Wedged hepatic venous pressure (WHVP)accurately reflects free portal pressure when portal hypertension is caused by postsinusoidal resistance. Hepatic Venous pressure gradient (HVPG) predict decompensation and death. HCPG is valuable only in those who have primarily in alcoholic liver disease, it is an invasive study. Duplex ultrasonography is an accurate non-invasive means of assessing the amount and direction of flow in the portal vein. Pre-operatively duple US is useful to determine patency of the portal vein and direction of flow. Spontaneous thrombosis about 10% of patients of cirrhosis have a portal vein unsuitable for a portacaval shunt. Treatment: Consists of expectant management, endoscopic sclerotherapy, nonselective beta blockers (propranolol, nadolol), portosystemic shunts, devascularization of the esophagogastric junction, and miscellaneous rarely used operations.. Treatment of those with varices whom has never bled prophylactic therapy is used (prophylaxis endoscopic variceal ligation (EVL) or prophylactic propranolol) Prophylactic therapy is valuable CHILD-PUGH classification is used to determine risk of hemorrhage. CHILD-PUGH uses size of varices, presence of wale markings (whip marks). This classification can identify high-risk patient (up to 65% risk of bleeding within a year) who is most likely to benefit from prophylactic treatment.Treatment for whose who have bled: 1. Non-selective beta blocker therapy- reduces risk of recurrent bleeding episodes, work by decreasing cardiac output, splanchnic blood flow and consequently portal blood pressure. Chronic therapy of propranolol 20-160mg twice daily, decreases about 40% the frequency of re-bleeding. Benefits are greater in Child-Pugh A and B than Child-Pugh C cirrhotics. 2. Endoscopic band ligation- effective means of preventing recurrent bleeding episodes and is superior to sclerotherapy. 3. Endoscopic sclerotherapy- was used routinely but band ligation has replaced this method. 4. Transjugular intrahepatic portosystemic shunt (TIPS) this advantage is offset by its higher morbidity and mortality rate and development of hepatic encephalopathy and liver failure. TIPS is usually only used for salvaging patients who faily endoscopic and/ or pharmacologic treatment. Extrahepatic Portal Venous Occlusion (Doherty, 573) Is one of the causes of non-cirrhotic portal hypertension, the other common cause is noncirrhotic portal fibrosis. The clinical manifestation of portal hypertension in the absence of significant hepatic parenchymal dysfunction. Causes in adults are hepatic tumors, cirrhosis, trauma, pancreatitis, pancreatic pseudocysts, myelofibrosis, thrombotic states (protein C deficiency) and sepsis; in particular cirrhosis and/or hepatocellular carcinoma needs to be considered.Splenic Vein Thrombosis (Doherty, 574) Isolated thrombosis of the splenic vein is a rare cause of variceal bleeding that is cured by splenectomy. The splenic venous blood is blocked from its normal routem flows through the short gastric vessels to the gastric fundus and then into the left gastric vein. This phenomenon is call left sided portal hypertension. The principle cause is pancreatitis, pancreatic pseudocyst, neoplasm, and trauma. The mean incidence of splenic vein thrombosis associated with acute and chronic pancreatitis is estimated at 22.6% and 12.4% and overall bleeding rate of 12.3% Splenomegaly is present in ⅔ of patients diagnosis is made by splenic arteriography that opacifies the venous phase, but more common now is CT scan with portal phase imaging. Splenectomy is curative. Budd-Chiari Syndrome (Doherty, 575) Results from obstruction of hepatic venous outflow. Most cases are caused by spontaneous thrombosis of hepatic veins associated with myeloproliferative disorders (polycythemia vera, essential thrombosis) or the use of birth control pills. Other common associated conditions include Factor V Leiden and factor II gene mutations. Other predisposing factors include protein C and S deficiencies, antiphospholipid syndrome, antithrombin III deficiency, paroxysmal nocturnal hemoglobinuria, Behcet syndrome and trauma.S/S begins with vague right upper quadrant abd pain, post prandial bloating, and anorexia; after weeks or months: gross ascites, hepatomegaly, hepatic failure (↑AST, ↑Serum Bilirubin, Alk Phos abnormal) Diagnosis: Venography. Treatment: Anticoagulation is recommended in presence of long-standing thrombosis to allow recanalization or to avoid propagation of venous thrombosis. Hepatic Encephalopathy (Doherty, 577) Hepatic encephalopathy clinical indications range from lethargy to coma, some may be as minor as personality changes to psychosis-asterixis to paraplegia. Hypothermia and hyperventilation may precede coma. Changes may be very subtle detectable with neuropsychological or neurophysiological testing. It is a reversible metabolic neuropathy that results from the action of chemicals absorbed from the gut of the brain. Aggravating factors of hepatic encephalopathy are: GI Bleed, constipation, azotemia, hypokalemic alkalosis, infection, excessive dietary protein, and sedatives. Laboratory findings: Ammonia ↑. Glutamine in CSF ↑(best to distinguish hepatic encephalopathy from other causes) Treatment: Acute encephalopathy treatment control precipitating factors: stop all dietary protein intake, cleansing the bowel with purgatives and enemas, and administer antibiotics(neomycin or ampicillin) or lactulose. Neomycin PO or GT (2-4x daily) or rectally as an enema (1%1-2x daily) blood volume must be maintained to avoid prerenal azotemia. After patient responds to initial therapy, dietary protein can be started at 20g/d and increased in increments of 10-20g every 2-5 days as tolerated. Chronic encephalopathy is treated by dietary restriction of protein, avoidance of constipation and elimination of sedatives, diuretics, and tranquilizers. Avoid protein depletion, protein intake must not be chronically reduced below 50g/d. Vegetable protein is better tolerated then animal protein. Lactulose is a disaccharide unaffected by intestinal enzymes, drug of choice for long term control. Hepatic Abscess (Doherty, 579) Hepatic Abscess may be bacterial, parasitic or fungal. In US Pyogenic abscess are most common followed by amebic abscess. 90% fo right lower lobe abscesses are solitary, while only 10% of left lobe is solitary. Many abscesses are spread from biliary infections such as empyema of the gallbladder or protracted cholangitis. 40% have an underlying malignancy, other cases develop after general sepsis from bacterial endocarditis, renal infection or pneumonitis. Rare causes include secondary bacterial infection of an amebic abscess, hydatid cyst or congenital hepatic cyst. Organism is of enteric origin: Escherichia coli, Klebsiella pneumoniae, baceteriodes, enterococci, anaerobic streptococci, and microaerophilic streptococci are most common.Signs and Symptoms: abscesses from another intra abdominal infections such as diverticulitis there are increased toxicity, higher fever, jaundice, and generally a deteriorating clinical picture. Right upper quadrant pain and chills may appear. Other times less obvious, malaise and fatigue followed by several weeks of fever, epigastric of right upper quadrant pain is present in half the cases, pain maybe aggravated by motion or may be referred to the right shoulder. Laboratory findings: Leukocytosis is present and usually over 15,000/ul. Small group may fail to develop leukocytosis. Anemia is present in most and average HCT is 33% Serum bili is normal except in those who have multiple abscesses or biliary obstruction.Alk Phos is ↑ even with the presence of normal bili. Imaging studies: US and CT scans are the most useful diagnostic test. Treatment: Antibiotics to be started promptly, initial coverage before culture results are available. Coverage should be adequate for E. Coli, K. Pneumoniae, bacteroides, enterococci, and anaerobic streptococci and consequently would usually include aminoglycoside, clindamycin or metronidazole, and ampicillin. Drainage catheter inserted percutaneously under US or CT guided is the most appropriate therapy catheters are removed 1-2 weeks after output become nonpurulent and scant. 40% of patient the drains do not drain well and need to be repositioned when there are difficulties are encountered with percutaneous drainage, a laparotomy should be performed promptly. Jaundice (icterus) o Categorized as prehepatic, hepatic, or post-hepatic. o Prehepatic cause are hemolysis which involves increased production of bilirubin (most common) and Gilbert disease and Grigler-Najjar syndrome. o Hepatic jaundice can be put in to 2 subcategories hepatocellular and cholestatic, hepatocellular includes acute viral hepatitis and alcoholic cirrhosis. Some cases of intrahepatic cholestasis may be indistinguishable clinically and biochemically from cholestasis d/t to bile duct obstruction. Primary biliary cirrhosis, toxic drug jaundice cholestatic jaundice of pregnancy and postoperative cholestatic jaundice are the most common forms. o post-hepatic (also called extra hepatic jaundice or obstructive jaundice)- is often from biliary obstruction by a malignant tumor, choledocholithiasis or biliary stricture, pancreatic pseudocyst, chronic pancreatitis, sclerosing cholangitis, metastatic cancer. o Physical exam  hepatomegaly is common in both hepatic and post hepatic jaundice  palpation of liver may suggest cirrhosis or metastatic CA, but is unreliable  secondary stigmata of cirrhosis such as liver palms, spider angiomas, ascites collateral veins on the Abd. Wall and splenomegaly suggest cirrhosis. o Labs  Normal values  Direct Bilirubin (conjugated)- 0.1-0.4mg/dl  Indirect Bilirubin (unconjugated)- 0.3-1.1mg/dl  Bilirubin that is bound to a protein (albumin) in the blood is called unconjugated, or indirect bilirubin whereas Conjugated, or direct bilirubin travels from the liver into the small intestine. You need to understand the difference in this as it will help you with your differential diagnosis’.  Prehepatic (hemolytic) the increased bilirubin is principally in the unconjugated indirect fraction this is insoluble to water, and the jaundice in hemolysis is acholuric. With hemolysis the total bili rarely exceeds 4-5mg. dl.  Hepatic disease is characterized by elevations of both conjugated and unconjugated bili. An increase in the conjugated always signifies disease within hepatobiliary system.  hepatic cholestasis and extrahepatic obstruction will raise the direct bilirubin, the indirect will only rise slightly, since direct bili is watersoluble bilirubinuria develops  with complete extrahepatic obstruction bili plateaus around 25- 30mg/dl at which point loss in the urine equals the daily production. If you see higher values think concomitant hemolysis or decreased renal function. Obstruction of a single duct does not usually cause jaundice.  Obstruction jaundice d/t common duct stones often produce transient bili increases in the range of 2-4 and the level rarely exceeds 15. ASTrises are common but levels as high as 1000u/L are seen in pts. with common duct stones and cholangitis, AST level > 1000u/L suggests viral hepatitis. o Diagnosis  The principal diagnostic objective is to distinguish surgical (obstructive) from nonsurgical jaundice.  H&P and labs allow an accurate diagnosis to be made in most cases without invasive tests.  Use step wise approach with each test selected according to the information available.  Only severe or worsening cholangitis requires urgent intervention.  Most jaundice is mild and if recent passes with 24-48h if persistent jaundice the first test is usually an u/s, this will show dilated intrahepatic bile ducts which indicates obstruction or gallbladder stones.  And ERCP is preferred when a lower end duct is believed to be obstructed, (suspected carcinoma of the pancreas or other periampullary tumors).  THC is preferred for proximal lesions (biliary stricture neoplasm of the bifurcation of the hepatic ducts) because it gives better opacification of the duct proximal to the obstruction and therefore provides better imaging in planning surgery.  If a neoplasm is suspected a CT scan is can be done as this gives a better image of mass.  Gallstones (Cholelithiasis) o Two types cholesterol (75%) and pigment (25%) o Cholesterol stones  Cholesterol gallstones (CG) result from secretion by the liver of bile supersaturated with cholesterol, and when the phospholipid vesicle that transports the bile’s holding capacity is exceeded cholesterol crystals are formed.  The incidence of CG disease is highest in American Indians, lower in Caucasians, and lowest in blacks, with a twofold gradient from one group to the next.  75% of American Indian women >40 affected.  Women are affected more than men until after menopause than it lessens.  Hormonal effects are also reflected in the increased incidence of gallstones with multiparity and increased cholesterol saturation of bile with oral contraceptives.  Obesity is another major risk factor o Pigmented Stones  Pigmented stones are black to brown 2-5mm in diameter, and amorphous.  They are composed of a mixture of calcium bilirubinate, complex bilirubin polymers, bile acids and other unidentified substances. About 50% are radiopaque (opaque) and in the U.S. they constitute 2/3 of all radiopaque gallstones.  Pigment stones are rare in American Indians but make up about 60% of all stones in Japan.  Predisposing factors are cirrhosis, bile stasis (stricture or markedly dilated common duct) and chronic hemolysis.  Bacteria play role in formation of pigmented stones, which explains why pts. With pigment gallstone disease have sepsis more often than do those with cholesterol gallstones. o Asymptomatic Gallstones  Only about 30% of people with gallstones end up going to surgery  s/s of gallstones disease generally do not change in severity, only about 2% of pts. with asymptomatic gallstones develop symptoms.  When should a pt with asymptomatic disease have a cholecystectomy, 1. Large stones >2cm because they produce acute cholecystitis, 2. A calcified gallbladder because it is associated with carcinoma. o Gallstones & Chronic cholecystitis (biliary colic)  The term Cholecystitis is used when gallstones are involved  Biliary colic, also known as a gallbladder attack or gallstone attack, is when a colic (sudden pain) occurs due to a gallstone temporarily blocking the cystic duct.  Repeated minor episodes of obstruction of the cystic duct causes biliary colic which cause inflammation and formation of scars. o s/s  Biliary colic- begins abruptly and subsides gradually lasting for a few minutes to several hours. The pain of biliary colic is usually steadynot intermittent, for some the attach occurs postprandially, in others, there is no relationship to meals.  Nausea and vomiting may accompany the pain.  Epigastric and precordial pain is common  Pain may radiate to back or scapula area  In severe attacks pts. may curl up in bed changing positions often to get more comfortable  RUQ tenderness  Indigestion, heartburn, eructation (burping or belching) may also be present. o Differential diagnosis’  Essentials of diagnosis’- episodic abd. pain, dyspepsia, gallstones evident on imaging  Biliary colic may also simulate the pain of duodenal ulcer, hiatal hernia, pancreatitis, and MI. o Test  U/S  ECG and chest x-ray (to r/o MI) T6-T10 dermatomes (osteoarthritis, spurs, vertebral lesions or tumors), pain may be confused with biliary colic so you may want to do a x-ray of the spine. o Treatment  Medical  avoid offending foods  dissolution of cholesterol gallstones ursodiol, this is marginal effective, the stones need to be <5mm and devoid of calcium, it takes two years to dissolve them and they usually reoccur.  Acute Cholecystitis o 80% of acute cholecystitis is from obstruction of cystic duct by a gallstone impacted in Hartmann’s pouch o Gallbladder becomes inflamed and distended. o Pathologic changes evolve in a typical pattern- subserosal edema and hemorrhage and patchy mucosal necrosis are the first changes. Later PMN (polymorphonuclear neutrophils) appear. The final stage is development of fibrosis, gangrene and perforation may occur. o About 20% of acute cholecystitis is in the absence of calculous this is known as acalculous cholecystitis this is usually d/t tumor, cystic artery occlusion or primary bacterial infection from bacteria like E coli, clostridia, or occasionally salmonella typhi. Most patients who suffer from this are already hospitalized for another reason this condition is particularly common in trauma pts. and in pts. getting TPN. o s/s  the same as chronic cholecystitis biliary colic n/v, but with acute cholecystitis the pain symptoms do not go away and is associated with abd tenderness, and vomiting is rarely severe.  Mild icterus in about 10% of cases  High fever and chills are uncommon and should suggest the possibility of complications or a wrong diagnosis.  RUQ pain with a palpable gallbladder (sometimes)  Positive Murphy’s sign o Labs  WBC usually between 12-15, but normal cts. are not uncommon, but if ct. goes above 15 you should start to consider complications.  Mild elevation of serum bili (2-4) related to secondary inflammation of the common bile duct. If the bili is >than this, you are probably looking at a secondary stone in the common bile duct.  Occasionally amylase will be elevated as high as 1000u/dl but this is transient. o Imaging  Ultrasound  U/S will show gallstones, sludge, and thickening of the gallbladder wall.  You will have a ultrasonic positive murphy’s sign, but this is absent if the gallbladder is gangrenous, usually this is the only test needed. HIDA scan  Needed when U/S is not conclusive  This test cannot demonstrate gallstones, but can r/o with imaging of ducts (it will show patency with the radiograph dye), pts should not get narcotics for 4-6 hours o Differential diagnosis’  Peptic ulcer with or without perforation  Acute pancreatitis  Acute appendicitis in patients with a high cecum  RUQ pain may develop with high fever in acute gonococcal perihepatitis (Fitz-Hugh- Curtis syndrome), clues to this may be found in tenderness in the adnexa, vaginal discharge that shows gonococci and a disparity between the pts. high fever and lack of general toxicity. o Complications of acute cholecystitis are empyema, gangrene, and perforation and perforation with pericholecystic abscess. o Treatment  Medical and surgical  NPO  IV fluids  NG tube (I have never done this, but this is what the book recommends)  Mild cases give Cefazolin 2-4g daily with severe cases give Pcn 20million units daily, clinda, and gent. Or single-drug therapy like imipenem.  Cholecystectomy- if possible, stabilize pt first, then do choli. It will reduce complications  Reasons not to wait high fever >39c, WBC’s >15 or chills that suggest suppurative progression, Acalculous acute choli. should automatically be placed in this category.  Emphysematous cholecystitis o This is a rare condition in which bubbles of gas from anaerobic infection appear in the lumen of the gallbladder, its wall, the pericholecystic space, and on occasion the bile ducts. o Most common bacteria responsible is clostridia species but E coli, or anaerobic streptococci may be found. o Effects men>women o Diabetes Mellitus is present in 20% of pts. o Sudden onset and rapidly progressive pain o Fever and WBC reach high levels quickly, pts. look more toxic than with regular acute choli. o Needs cholecystectomy  Cholangitis (bacterial cholangitis) o This is a bacterial infection of the biliary ducts which signifies biliary obstruction. The obstruction can be partial or less common complete. The principal causes are choledocholithiasis, biliary stricture, and neoplasm.Uncommon causes are chronic pancreatitis, ampullary stenosis, pan. pseudocyst, duodenal diverticulum, congenital cyst, and parasitic invasion. o s/s  Charcot’s triad – which consists of biliary colic, jaundice, and chills and fever, this is present in 70% of pts.  Leukocytosis  Elevated bili  Elevated Alkaline phosphatase  The predominant organisms in bile (in decreasing frequency) are E coli., Klebsiella, Pseudomonas, enterococci, and proteus Bacteroides fragilis  Bacteria is usually present o Treatment  You need to treat the infection first, then you can deal with the hepatobiliary problem  Antibiotic choice is Cefazolin for mild cases, if disease is severe or worsening add gent., plus clinda, or Flagyl.  If it does not improve the bile duct needs to be decompressed, the most common cause of cholangitis is choledocholithiasis for which the best treatment consists of emergency endoscopic sphincterotomy  If the underlying cause is a neoplastic obstruction than insertion of a transhepatic drain into the bile duct is choice.  Choledocholithiasis o Choledocholithiasis refers to the presence of one or more gallstones in the common bile duct. There are two possible origins for common duct stones, 1. Stones (both cholesterol and pigmented) coming from the gallbladder these are known as secondary stones and 2. De novo which develop within the common duct, these are called primary common duct stones. It is about 60 to 40 ratios with cholesterol having 60 but a more severe clinical manifestation is seen with pigmented. o s/s  principal clinical findings are caused by obstruction to the flow of bile or pancreatic juice these include  Biliary colic  Cholangitis  Jaundice  Pancreatitis  Up to 50% of pts. can be asymptomatic  Common duct can dilate to 2-3cm proximal to an obstructing lesion, and truly huge ducts form with biliary tumors.  In choledocholithiasis or biliary stricture the inflammatory reaction restricts dilation, so the dilation is less marked.  Icteric and toxic, with high fever and chills  RUQ tenderness may be present but not as marked as with acute cholecystitis If it is developing into cholangitis than leukocytosis with valve of 15 is common it is not uncommon to see valves as high as 20 or above.  Elevated serum bili of 10 or under (usually) appears within 24h after the onset of symptoms, it is usually 2-4 with the direct fraction exceeding the indirect, Bili does not reach high levels as seen with malignant tumors because the obstruction is usually incomplete, and transient, in fact this is a reliable differentiate between benign and malignant obstruction.  Alkaline phosphatase rises,  AST and ALT mildly with extrahepatic obstruction of the duct. o Imaging  Radiopaque gallstones may be seen on plain abd. film or CT  if the pt. has had a previous cholecystectomy, you go right to an ERCP, if not U/S to assess for gallstones.  If a cholecystectomy has not been done a cholangiography should be part of operative management. o Differential diagnosis  The same differentials as with acute cholecystitis:  Acute pancreatitis  Acute cholecystitis  Alcoholic cirrhosis  Alcoholic hepatitis  Cholangitis  Intrahepatic cholestasis from drugs, pregnancy, o Complications  Infection can produce intrahepatic abscess.  Hepatic failure  Acute pancreatitis o Treatment  If they pt. has cholangitis along with Choledocholithiasis then systemic IV antibiotics is needs. If you do not see a big improvement within 2-4 days, they need to go to surgery.  The surgical treatment of choice is a cholecystectomy if they still have their gallbladder.  If they do not have their gallbladder then endoscopic sphincterotomy is done unless it has been determined that the stones are >2cm then they will need a Laparotomy.  Pancreatitis (Doherty page 610): A common non-bacterial inflammatory disease caused by activation, interstitial liberation, and autodigestion of the pancreas by its own enzymes. This process may or may not be accompanied by permanent morphologic and functional changes in the gland.  Acute pancreatitis, characterized by sudden upper abdominal pain, nausea and vomiting, and elevated serum amylase.  Chronic pancreatitis characterized by chronic pain pancreatic calcification on x-ray and exocrine (steatorrhea) or endocrine (DM) insufficiency. Acute attacks can happen to patients with chronic pancreatitis. Acute relapsing pancreatitis is defined as multiple attacks of pancreatitis without pancreatic scaring most often associated with biliary pancreatitis.  Most cases of pancreatitis are caused by gallstone disease or alcoholism; a few results from hypercalcemia, trauma, hyperlipidemia and genetic predisposition and the remainder are idiopathic.  Cause: Alcohol, Drug Induced, Idiopathic, Blockage  Labs: • Serum amylase • Serum lipase • Aspartate transaminase (AST), alanine transaminase (ALT), bilirubin • Serum electrolytes: *Hyperkalemia and Hyperlipidemia • CBC • Abdominal x-ray • Chest x-ray • Abdominal US (if biliary’  Acute:  Elevated amylase levels may occur with gangrenous cholecystitis, small bowel obstruction, mesenteric infarction, and perforated ulcer (rarely > 500 IU/dL) • Leukocytosis > 12,000/ L is unusual in the absence of abscess  Hematocrit may be elevated due to dehydration as a result of abdominal blood loss in hemorrhagic pancreatitis.  Usually a moderated leukocytosis  LFT’s usually normal, but mild elevation in serum bilirubin  Serum amylase concentration rises to more than 2 ½ times normal w/in 6 hours after the onset of and acute episode and generally remains elevated for several days. Values in excess of 1000 IU/dL occur early in the attack of in 95% of pts with biliary pancreatitis and 85% of patients with acute alcoholic pancreatitis. Severe disease levels below 1000.  Elevated serum lipase  Test: Plain Abdominal X-ray, CT, ERCP  TRT: Gastric Suction, Fluids, Antibiotics, Oxygen, Calcium and Magnesium replacement, Nutrition  Pancreatic pseudocyst: encapsulated collection of fluid with high enzyme concentrations that arise from the pancreas.  DX with: Epigastric mass and pain  Mild fever and leukocytosis  Persistent serum amylase elevation  Pancreatic cyst demonstrated by ultrasound or CT scan  The walls of the pseudocyst are formed by inflammatory fibrosis of the peritoneal mesenteric, and serosal membranes which limits spread of the denotes absence of an epithelial lining whereas true cyst are lined by epithelium.  2 processes involved many occur as complications of severe pseudocyst where extravasation of pancreatic juice and glandular necrosis from a sterile pocket of fluid that is not reabsorbed as inflammation subsides.  S/SX: pseudocyst should be suspected when a patient with acute pancreatitis fails to recover after a week of treatment. Pain is the most common finding  Fever, weight loss, tenderness, and palpable mass are present in about half of patient. A few have jaundice a manifestation of obstruction of the intrapancreatic segment of the bile duct. Early stages of a pseudocyst formation are often demonstrates radiographically before specific clinical findings appear. 1st clinical manifestation is usually a palpable tender mass in the epigastrium consisting of a swollen pancreas and contiguous viscera. With time the mass may subside, but if it persists it is likely a pseudocyst.  An elevated serum amylase and leukocytosis are present in about half of patients. Elevated bilirubin levels reflect biliary obstruction. Dilated common bile duct suggest biliary obstruction either from the cyst or from underlying chronic pancreatitis. Serum amylase remains elevated about 3 weeks in those patients with pancreatic and about half will have a pseudocyst.  CT scan is the diagnostic study of choice. The size and shape of the cyst and its relationship to other viscera can be seen. Acute pseudocyst are often irregular in shape chronic pseudocyst are most often circular or nearly so. An enlarged pancreatic duct may be present in patients with chronic pancreatitis.  Diff/DX: pancreatic pseudocyst must be distinguished form pancreatic abscess and acute pancreatic phlegmon.Patient with an abscess will exhibit signs of infection. Rarely patient with pseudocyst present with weight loss, jaundice and nontender palpable gallbladder and are first thought to have pancreatic carcinoma.  Treatment: principle indication for treating pancreatic pseudocyst are to improve symptoms and to prevent complication. A few untreated cyst resolve spontaneously even after being stable for months. Expectant management is especially important in the first 6-12 months of existence of the cyst that have arisen during an attack of acute pancreatitis.  Excision: most definitive treatment bit is usually confined to chronic pseudocyst in the tail of the gland. This is recommended especially for the cyst follow trauma. most cyst should be drained either externally or internally  External drainage: best for critically ill patients when cyst wall has not matured sufficiently for anastomosis. A large tube is sewn into the cyst lumen and its end is brought out through the abdominal wall. Complicated in a third of patients by a pancreatic fistula that sometimes requires surgical drainage, but on average closed spontaneously in several months.  Internal drainage: preferred method of treatment is internal drainage where the cyst is anastomosed to a Roux- en-y limb of the jejunum, to the posterior wall of the stomach or the duodenum. The interior of the cyst should be inspected for evidence of a tumor.  The procedure consists of making a lateral duodenotomy opening into the cyst through the medial wall of the duodenum and then closing the later duodenotomy. Following the drainage, the cyst becomes obliterated with a few weeks.  Nonsurgical drainage: can be established by a percutaneous catheter placed into the cyst under radiographic or ultrasound control. This is the preferred method for infected pseudocyst.  Pancreatic insufficiency: condition which occurs when the pancreas does not make enough of a specific enzyme the body uses to digest food in the small intestine. This may follow pancreatectomy or pancreatic disease. Patients with many varying degrees of pancreatic insufficiency have no symptoms and require no treatment.  Patients benefits from rational medical regimen. Malabsorption and steatorrhea do not appear until more than 90% of pancreatic exocrine function is loss. With 2-10% of normal function steatorrhea is mild to moderate with less than 2% of normal function steatorrhea is severe. Pancreatic insufficiency affects fat adsorption more than that of protein or carbohydrate because protein digestion is aided by gastric pepsin and carbohydrate digestion by salivary and intestinal amylase. On a diet of 100g/d of fat per day normal subjects excrete 5-7 g/d and the efficiency of assimilation is similar over a wide range of fat intake. Malabsorption of vitamins is rarely a significant problem. Vitamin B12 malabsorption has been detected in some patient with pancreatic insufficiency but it is rare.  DX Test: Secretin or Cholecystokinin- pancreatic juice is obtained by peroral duodenal intubation, and the response to an intravenous injection of secretin or CCK measured.  Pancreatic fluid should normally have a bicarbonate concentration greater than 80mEq/L and bicarbonate above 15mEq/30min.   Pancreolauryl test: Fluorescein dilaurate is given orally with breakfast and urinary fluorescein excretion is measured.  PABA excretion (Bentiromide) test: patient ingests 1g of the synthetic peptide Bentiromide and urinary excretion of aromatic amines is measured. Patients with chronic pancreatitis excrete about 50% of the normal amount of PABA.  Fecal Fat Balance Test: patient ingests a diet containing 75-100g fat each day for 5 days. The amounts of dietary fat should be measured and should be the and the same each day. Clinically significant steatorrhea is present when fat malabsorption exceeds about 25%. Total pancreatectomy results in about 70% fat malabsorption.   Treatment: diet should aim for 3000-6000 kcal/d, emphasizing carbohydrate (400g or more) and protein (100-150g). Patients with diarrhea may be restricted to 50g of fat the amount increased until diarrhea appears.  Pancrelipase replacement may be accomplished with pancreatic extracts containing 30,000- 50,000 units of lipase distributed throughout each of four daily meals. If enzymes alone do not improve the malabsorption enough the problem is probably due to destruction of lipase by gastric acid.  Adenocarcinoma of the Pancreas: After tumors of the lung, prostate and colon, pancreatic carcinoma is the fourth leading cause death in men, and trails lung, breast and colon in women. Factors assoc. with an increased risk of pancreas cancer are cigarette smoking, dietary consumption of meat (especially fried meat), fat, previous gastrectomy (>20 years earlier) and race.  Peak incidence in 50 and 60’s years of age. 2/3 of cases the tumor is located in the head of the gland the remainder occurs in the tail of the gland.  Ductal adenocarcinoma mainly of a poorly differentiated cell pattern account for 80% of the cancers. The remainder is islet call tumors and cystadenocarcinomas  S/Sx: carcinoma of the head of the pancreas presents with wt loss, obstructive jaundice, and deep-seated ABD pain in 75% of patients. Back pain occurs in 25% of patient and is associated with a worse prognosis.  Smaller tumors of the pancreas have less pain  Wt loss averages 20lbs (44kg)  Hepatosplenomegaly present in half of patients but does not necessarily indicate spread to the liver.  Palpable mass found in 20% of patients nearly always signifies surgical incurability  Jaundice is unrelenting in most patients, fluctuates in about 10%. Jaundice is often accompanied by pruritus, especially of the hand and feet.  Palpable nontender gallbladder in a jaundiced patient suggests neoplastic obstruction of the common duct (COURVOISIER sign) most often d/t pancreatic cancer this is present in about half patient  Diff/DX: the other periampullary neoplasm carcinoma of the ampulla of Vater distal common bile duct or duodenum may also present with pain wt loss, obstructive jaundice and palpable gallbladder. Preoperative cholangiography and gastrointestinal xrays may suggest the correct diagnosis, but laparotomy may be required.  Lab findings and test for:  Elevated alk phos and bilirubin levels reflect either common duct obstruction or hepatic mets  Bilirubin level with neoplastic obstruction averages 18mg/dL much higher then generally seen with benign disease of the bile duct.  Serum aminotransferase levels are rarely markedly elevated  Repeated exam of stool specimen for occult blood gives positive reaction in many cases.  CA 19-9 serum markers are elevated in most patients with pancreatic cancer, but the sensitivity in resectable <4cm lesions is too low 50% for tis to serve as a screening tool. (the greatest usefulness of CA 19-9 measurements may be in following the results of treatments after the resection of the tumor levels drop to normal but rise again with recurrence.  CT scan show a pancreatic mass in 95% of cases  ERCP is indicated in the absence of a mass (it is the most sensitive test 95% for detecting pancreatic cancer though specificity in differentiating between cancer and pancreatitis is low. Consequently, a pancreatogram should be obtained early in cases where the existence of a pancreatic lesion is suspected. Finding consist of stenosis or obstruction of the pancreatic duct Upper GI series is not sensitive in detecting pancreatic cancer but provides information about the patency of the duodenum that may be useful in deciding whether a gastrojejunostomy will have to be performed.  Angiography has not proved reliable and ultrasound is poor second to CT scan  Aspiration Biopsy of the pancreatic mass lesions is positive 85% of malignant tumors. Procedure is relatively safe but there is risk of spreading a localized resectable tumor, so it is contraindicated in patients who are candidates for surgery.  Treatment: Pancreatic resection for pancreatic cancer is appropriate only if all gross tumor can be removed with a standard resection.  The lesion is considered resectable if the following areas are free of tumor:   Hepatic artery near the origin of the gastroduodenal artery   Superior mesenteric artery where is courses under the body of the pancreas and is so close to the portal vein and the superior mesenteric vessels (these structures may be removed early)   The liver and regional lymph nodes  About 20% of the head of the pancreas can be resected, but with local and distant spread this is rarely possible for lesions of the body and tail.  Histologic diagnosis can be made with aspiration biopsy (may be difficult to obtain for histologic diagnosis, b/c much of the palpable mass may consist of inflamed pancreatic tissue.  Curable lesions of the head pancreaticoduodenectomy (WHIPPLE procedure) is required this involves resection of the common bile duct, the gallbladder, the duodenum, and the pancreas to the mid body. There is increasing tendency to preserve the antrum and pylorus. Involvement of a short <1.5cm segment of the portal vein is not a contraindication to a curative resection.  Postoperative deaths are d/t complications such as pancreatic and biliary fistulas hemorrhage and infections  Hypersplenism (Doherty page 635): Overactive spleen, exaggeration of normal splenic function primarily associated with red pulp. In the past the term hypersplenism or increased splenic function have been used to denote the syndrome characterized by splenic enlargement, deficiency of one or more of the cell lines, normal or hyperplastic cellularity or deficient cell lines in the marrow, and increased turnover of the effected cells.  Hypersplenism is not synonymous with hypersplenism.  Differential diagnosis:   Leukemia and lymphoma are diagnosed by marrow aspiration, lymph node biopsy, and examination of the peripheral blood (white count and differential).   Spherocytosis there are spherocytes, osmotic fragility is increased, and platelets and white cells are normal.   Thalassemia major become apparent in early childhood, and the blood smear morphology is characteristic.  Myelofibrosis the bone marrow shows proliferation of fibroblasts and replacement of normal elements   Idiopathic thrombocytopenia purpura (ITP) the spleen is normal or only slightly enlarged and the marrow is fatty.  Diagnostic Studies:  Before it become palpable an enlarged spleen may cause dullness to percussion above the left costal margin. Splenomegaly is manifested on supine x-ray of the abdomen by medical displacement of the stomach and downward displacement of the transverse colon and splenic flexure. CT scan is useful for differentiating the spleen from other abdominal masses and for demonstrating splenic enlargement or intrasplenic lesions.  Treatment options (please see table 27 -2 on page 636 for surgery options):  The course, response to treatment and prognosis of hyper-splenic syndromes differ widely depending one the underlying disease and its response to treatment and will be decided for or against splenectomy.  Splenectomy may decrease transfusion requirements decrease the incidence and number of infections and prevent hemorrhage and reduce pain.  The course of congestive splenomegaly due to portal hypertension depends upon the degree of venous obstruction and liver damage.  Hypersplenism is rarely a major problem and is almost always overshadowed by variceal bleeding or liver dysfunction.  Chronic Lymphocytic Leukemia (pg. 637) o CLL is a low-grade neoplasm of B cell lineage characterized by accumulations of populations of lymphocytes that are mature morphologically but functionally incompetent. o Makes up 25-30% of all leukemias, mean age dx of 72 o Initially causes little to no pain, eventually causes massive splenomegaly. o Most symptoms related to the spleen are from thrombocytopenia and anemia due to hypersplenism. o Decreased cellular production from the bone marrow is another cause of cytopenia. o Splenectomy corrects thrombocytopenia in 70-85% of cases, neutropenia is 60-70% of cases, and anemia in 50-60% of cases.  Myelodysplastic Syndrome (pg. 637)o Heterogenous group of clinical hematopoietic stem cell disorders manifested by pancytopenias, and dysplasia of the bone marrow. o Pathologic changes include extensive bone marrow fibrosis, extramedullary hematopoiesis in the spleen and liver, and a leukoerythroblastic blood reaction that may evolve into acute myeloid leukemia over time. o Bone marrow is usually completely replaced by fibrous tissue. o Symptoms are attributable to anemia (weakness, fatigue, dyspnea) and to splenomegaly (abd fullness, pain). o Pain over spleen from splenic infarcts is common. o Spontaneous bleeding, fatigue, secondary infection, bone pain, and hypermetabolic state are frequent. o Portal hypertension develops in some cases as a result of fibrosis of the liver, increased splenic blood flow or both. o Hepatomegaly is present in 75% of cases and splenomegaly with a firm and irregular spleen in all cases. o Splenectomy often provides relief. o Treatment  Primarily supportive using transfusions, androgenic steroids, antimetabolites and hematopoietic growth factors.  New therapies include immunomodulatory drugs like thalidomide or antibodies to VEGF and TNF.  Splenectomy indicated in the following: major hemolysis unresponsive to medical treatment, severe symptoms of massive splenomegaly withmass effect of the spleen, life threatening thrombocytopenia, portal hypertension with variceal hemorrhage  Hemolytic Anemia (pg. 641) o Essentials of diagnosis  Fatigue, pallor, jaundice  Splenomegaly  Persistent anemia and reticulocytosis. o Autoimmune hemolytic anemias have been classified according to the optimal temperature at which autoantibodies react. This is important because patient with cold antibodies will not benefit from a splenectomy, but those with warm antibodies may. o Hemolysis with a negative Coombs test may occur in uremia, cirrhosis of the liver, cancer, and certain infections (usually Coombs test positive). The antibody in IgG autoimmune hemolytic anemia is specifically directed against the Rh locus on the erythrocyte. Initiation of this disease is either idiopathic or secondary to drug exposure, connective tissue disorders, or lymphoproliferative disorders. o Hemolytic anemia due to cold antibodies is less common and always a secondary immune response. Cold agglutinin hemolytic anemia is due typically to an IgM directed against the I red cell antigen. Penicillin, quinidine, hydralazine, and methyldopa have been most commonly implicated in this syndrome. o Signs and Symptoms Can occur at any age, most common after age 50.  Occurs twice as often in females  Usually acute, consisting of anemia, mild jaundice, and sometimes fever.  Spleen palpably enlarged in 50% of cases  Rarely a sudden severe onset produces hemoglobinuria, renal tubular necrosis, and 50% death rate. o Laboratory Findings  Hemolytic anemia is diagnosed by demonstrating a normocytic normochromic anemia, reticulocytosis, erythroid hyperplasia of the marrow, and elevation of serum indirect bilirubin.  Serum haptoglobin is usually low or absent.  Direct Coombs test is positive because the red cells are coared with immunoglobulins or complement. o Treatment  Treat associated diseases.  Stop offending agent, if drug induced.  Corticosteroids produce a remission in about 75% of patients, but only 25% of remissions are permanent.  Avoid transfusion if possible  cross-matching very difficult. May need washed red cells and saline-active antisera. Rituximab is an effective second line therapy now producing durable responses 40% of steroid resistant cases.  Splenectomy is indicated for patients with warm-antibody hemolysis who fail to respond to 4-6 weeks of high-dose corticosteroid therapy, for patients who relapse after an initial response, and for patients in whom steroid therapy is contraindicated (TB).  Splenectomy is effective because it removes the principal site of red cell destruction.  Splenectomy may identifies the presence of an underlying disorder, i.e. lymphoma.  50% of patients who fail to respond to splenectomy will respond to azathioprine or cyclophosphamide.  Immune Thrombocytopenia Purpura (ITP) (pg. 642) o Essentials of diagnosis  Petechiae, ecchymoses, epistaxis, and easy bruising  No splenomegaly  Decreased platelet count, prolonged bleeding time, poor clot retraction, normal coagulation time. o General considerations  ITP is a hemorrhagic syndrome with diverse causes that can occur in an acute or chronic form and is characterized by marked reduction in the number of circulating platelets, abundant megakaryocyte in the bone marrow, and a shortened platelet life span. It may be idiopathic or secondary to a lymphoproliferative disorder, drugs or toxins, bacterial or viral infection (especially in children), SLE, or other conditions.  Splenectomy should be reserved for those with signs of blood loss, since surgical complications are high and survival may be short.  The spleen is the primary site of platelet destruction and may also be a significant source of autoantibody production. o Symptoms and signs  onset may be acute, with ecchymoses or showers of petechiae, and may be accompanied by bleeding gums, vaginal bleeding, GI bleeding, and hematuria.  Acute form most common before age 8 and begins 1-3 weeks after a viral URI.  Chronic form can start at any age, more common in women.  Cyclic remissions and exacerbations may continue for several years. o Laboratory findings  Platelet count is moderately to severely decreases, always <100,000 and platelets may be absent from the peripheral blood smear.  White and red cell counts usually normal, may have Fe deficiency anemia  Bleeding time is prolonged  Capillary fragility (Rumpel-Leede test) greatly increased, and clot retraction poor Pt, PTT, INR are normal  Can measure rate of disappearance of radioactivity from the blood. A spleen/liver ration greaten than 2:1 indicates significant splenic pooling and suggests that splenectomy would be beneficial. o Differential diagnosis  Other causes of nonimmunologic thrombocytopenia must be ruled out  Leukemia  aplastic anemia  macroglobulinemia  Thrombocytopenia and purpura may be caused by ineffective thrombocytopoiesis (pernicious anemia, preleukemic states) or by nonimmune platelet destruction (septicemia, disseminated IV coagulation, or other causes of hypersplenism) o Treatment  Treatment for ITP depends on the age of the patient, the severity of the disease, the duration of the thrombocytopenia, and the clinical variant.  Secondary ITP are best managed by treating the underlying primary disorders  Patients with mild or no symptoms need no specific therapy but should avoid contact sports, elective surgery, and all unessential medications  Corticosteroids are indicated in patients with moderate to severe purpura of short duration. 60mg prednisone daily until the platelet count returns to normal and then is gradually tapered after 4-6 weeks.  Corticosteroids produce a response in 70-80% of patients, but sustained remissions in only 20% of adults.  Second line therapy with Rituximab improves platelet counts in 30-40% of patients and sustained complete response in 10-20%  Splenectomy is the most effective form of therapy and is indicated for patients who do not respond to corticosteroids, for those who relapse after an initial remission on steroids, and for steroid-dependent patients  If indicated, platelet transfusions are given intraoperatively only after ligation of the splenic artery or removal of the spleen, since platelets from earlier transfusion would be rapidly sequestered in the spleen.  For temporary treatment of the thrombocytopenia, IVIG is effective  Splenectomy produces a sustained remission in about 68% of patients.  Splenectomy and corticosteroids success rates are better with acute disease.  Platelet count may double 24 hours after a splenectomy  If the platelet count remains elevated 2 months after treatment, the patient is considered cured.  When corticosteroids and splenectomy have failed, immunosuppressive drugs (azathioprine, vincristine) achieve remission in 25% of cases. o Prognosis In patients under age 16, 80% have complete and permanent spontaneous remission. This occurs rarely in adults Intestines, Hernias, and Abdominal Wall Lesions  Evaluation, differential diagnosis, diagnostic studies, and treatment options  Small Bowel Obstruction: o Causes: Prior abdominal surgeries, adhesions, hernias, or cancer o Symptoms:  nausea, vomiting bilious dark green (bowel contents back up into stomach), abdominal distention (Obstruction becomes large, dilated, filled with fluid and air), cramping and discomfort from bowel trying to squeeze things forward o Diagnosed by XRAY or CT  many SBO can be resolved after resting the bowel, insertion of an NGT can suction contents from stomach, keep NPO, give IV hydration  If SBO doesn’t resolve a Lysis of Adhesions may need to be performed to free up trapped bowel  Enteritis: o Caused by eating/drinking contaminated bacteria or viruses; germs settle in small intestine and cause inflammation and swelling o Other causes include Crohns DX, NSAIDS, cocaine, damage from radiation therapy, celiac disease, tropical sprue, whipple dx o Inflammation can involve the stomach (GASTRITIS) or large intestine (COLITIS) o Risk Factors: Recent stomach flu, recent travel, exposure to unclean water o Types include bacterial, campylobacter, e-coli, food poisoning, radiation, salmonella, shigella, staph aureus o symptoms abdominal cramping, diarrhea, n/v, loss of appetite, bleeding, fever o Treatment includes proper hydration, electrolyte supplements, in severe cases may need IV Fluids  Crohns Disease: o Inflammatory bowel disease that causes inflammation in digestive tract that leads to abdominal pain, diarrhea, fatigue, weight loss, malnutrition. o Causes include diet, stress, malfunctioning immune system, heredity o Risk factors: Age, ethnicity, fam hx, smoking, NSAID use o Complications include bowel obstruction, ulcers, anal fissure, malnutrition, colon cancer o Categories of drug treatments are:  Antibiotics: Metronidazole, ciproflaxin  Amino salicylates: Decrease inflammation in intestinal linings MILD SYMPTOMS Corticosteroids: Reduce inflammation by suppressing immune system MODERATE-SEVERE SYMPTOMS  Immune Modifiers: Suppress immune system  Biologic Therapies: Suppress immune system and are given to people who have not responded to conventional therapy  Non-Pharmacological: Antidiarrheals, pain relievers, and nutritional supplements  Mesenteric Ischemia: o Occurs when narrowed or blocked arteries restrict blood flow to intestine o Symptoms include: Tachycardia+, peritonitis, marked Abd guarding o ACUTE: Abrupt abdominal pain, urgent need to have a BM, fever, n/v o CHRONIC: Abdominal pain that starts about 30 minutes after eating, pain that worsens over an hour, pain that goes away within 1-3 hours o Causes: decreased blood flow, acute infarct usually by a blood clot in main mesenteric artery o If not promptly treated can lead to sepsis, irreversible bowel damage, death o Diagnosed by a CT o Treatment: Embolectomy, revascularization w/ or w/out a bowel resection, vasodilators, thrombolysis, long-term anticoagulation or antiplatelet therapy o Once intestines infarct, mortality is 70-90% Short bowel syndrome- Typically presents as a complication of extensive bowel resection. o The intestine adapts over time to be more efficient at nutrient absorption; the extent of adaptation determines the extent of recovery. o Treatment and prognosis are dependent on the length, health, and location of remaining bowel. 1st Tests To Order: CBC, serum electrolytes, BUN and creatinine, serum albumin, serum calcium, zinc, selenium, folate, vitamins A, B1, B2, B6, B12, C, D, and E, methylmalonic acid (MMA), and INR o Differentials- active Crohn disease, celiac disease, small bowel malignancy, and bacterial overgrowth o Management includes electrolyte and fluid replacement, provision of adequate macronutrients, and prevention and treatment of micronutrient deficiencies during intestinal adaptation. o Primary anastomosis of residual small bowel to colon, when possible, is the most important surgical procedure to reduce the need for parenteral nutrition (PN). o There should be an awareness of the possibility for medication malabsorption. o Physicians and patients need to watch closely for signs of dehydration, micronutrient deficiency, liver disease, and PN-related complications such as catheter infections and occlusions.Constipation- A detailed history and physical examination, including a digital rectal examination, is the first step in the evaluation of constipation. o Diagnostic evaluation may include a colonoscopy if indicated, colonic transit study, anorectal manometry, and a balloon expulsion study. o 1st Tests To Order: complete blood count (CBC), thyroid function tests, serum electrolytes, calcium, and magnesium, blood glucose, abdominal x-ray, barium enema o Differentials – anal fissure, medication induced constipation, hypercalcemia, hypothyroidism, DM o Initial treatment consists of lifestyle modification and laxatives. Acute symptoms <3 months  treatment of any identified underlying cause  diet + lifestyle advice + laxatives or prunes and/or stool softeners  with fecal impaction  evacuation measures  opioid-induced  methylnaltrexone o Medical treatment should be tailored to the underlying etiology. Patients who do not respond to medical treatment may need specialized evaluation for pelvic floor dysfunction and biofeedback therapy. Surgery is only rarely indicated. Large bowel obstruction - A surgical emergency which must be quickly differentiated from pseudo-obstruction to ensure that timely and correct treatment is provided. o Key Factors • colicky abdominal pain • abdominal distention • tympanic abdomen • change in bowel habits • hard feces • empty rectum • soft stools • recent weight loss •  renal function  serum amylase/lipase  coagulation studies  erect chest x-ray  plain abdominal x-ray o Consider malignancy in all patients who present with large bowel obstruction. o Suspect bowel perforation where there is persistent tachycardia, fever, and/or abdominal pain and tenderness. o Differentials- acute colonic pseudo-obstruction, chronic/idiopathic megacolon, toxic megacolon, endometriosis o Treatment Options: Presumptive- acutely ill: supportive measures, suspected or impending perforation, emergency surgery Acutesigmoid volvulus  no peritonitis or mucosal gangrene  flexible or rigid sigmoidoscopy  surgery  peritonitis or mucosal gangrene  surgery cecal volvulus  surgery colorectal malignancy  surgery  endoscopic stenting diverticular disease  surgery  endoscopic stenting foreign body ingestion  transluminal removal or laparotomy benign strictures  treat underlying cause endometriosis  resection pelvic abscesspercutaneous or transrectal drainage  resection Cancer - Third leading cause of cancer deaths in the US in men and women. Rare below 40 years of age. Key Factors  increasing age  positive FHx  rectal bleeding  change in bowel habit riteria)  radical resection