Editing Cirrhosis


''Cirrhosis is a histological diagnosis characterised by the development of fibrous septa surrounding hepatocellular nodules, and results in synthetic deficiencies and portal hypertension.''

== Aetiology ==

* Chronic viral hepatitis
* Alcoholic liver disease
* Haemochromatosis
* Nonalcohol fatty liver disease
* Also:
** Autoimmune hepatitis
** Primary and secondary biliary cirrhosis
** PSC
** Medications
** Wilson disease

== Pathophysiology ==

* The end result of a healing process initiated by chronic liver injury
* Fibrous septa develop around regenerating hepatocellular nodules
** Distortion of hepatic architecture
* May be reversible in early stages if cause is treated

== Presentation ==

* Compensated
** Anorexia
** Weight loss
** Weakness
** Fatigues
* Decompensated
** Jaundice
** Pruritis
** Upper GI bleeding
** Ascites
** Confusion
* Look for jaundice, spider angiomata, gynaecomastia, ascites, splenomegaly, palmar erythema, digital clubbing, asterixis

== Diagnosis ==

* Cirrhosis is a histological diagnosis established on liver biopsy
* However presence can be suspected based on clinical signs of chronic liver disease and portal hypertension along with imaging signs 
* Fibroscan
** Assesses the stiffness of liver via transient elastography - a 'vibration wave'/'shear wave' is generated on the skin, and velocity of this wave is assessed by determining the time taken to travel to a particular depth inside the liver
** Because fibrous tissue is harder/stiffer than normal liver, the higher the stiffness, the higher the likelihood of cirrhosis
** Cutoff is >14kPa

== Management ==

* No effective treatment
* Treat cause
* Palliative: treat portal hypertension and treat complications
* Definitive: liver transplant

== Complications ('''decompensated - primarily CAVE - coagulopathy, ascites, variceal bleed, encephalopathy''') ==

* ''Risk factors/triggering events for decompensation:''
** ''Bleeding''
** ''Infection''
** ''Alcohol intake''
** ''Medications''
** ''Dehydration''
** ''Constipation''

* Variceal bleed
** Large volume, melaena, shock
* Portal hypertensive gastropathy bleed
** Often slower, IDA
* Ascites
** Requires portal HTN
** Occurs in 50% of previously compensated cirrhotic patients over a 10 year period, and marks a 2-year survival of 50%. Associated with a deterioration in QoL.
** Consider concomitant viral hepatitis/vascular disease, along with cardiac/renal/malignant disease.
** Exam
*** Shifting dullness = 2L of ascites
** Generally treated with diuretics and sodium restriction, but pay require therapeutic paracentesis or TIPS
* SBP
** Infected ascites collection
* Hepatic encephalopathy
* HCC
* Hepatorenal syndrome
** Renal failure in a patient with advanced liver disease
* Hepatopulmonary syndrome
* Umbilical herniae often occur in this setting, which presents a management dilemma
** Ruptured/incarcerated herniae are repaired immediately
** Those with symptomatic herniae/skin changes should be referred for elective repair
** Asymptomatic herniae are managed conservatively - involves aggressive management of ascites
** Repair asymptomatic herniae at time of liver transplantation
* Coagulopathy
** Rebalanced haemostasis with both procoagulant and anticoagulant effects
*** Impaired haemostasis - coagulation factor deficiencies, most notably I, II, V, VII, IX, X and CI; thrombocytopaenia/platelet dysfunction; altered fibrinolytic system
*** Prothrombotic changes: reduced protein S, C and antithrombin; increased VWF

== Indications for albumin in cirrhosis ==

* Prevention of paracentesis-induced circulatory dysfunction
* Diagnosis and treatment of hepatorenal syndrome AKI
* Treatment of SBP

== Prognosis ==

* Child-Pugh classification was originally developed to assess risk of non-shunt operations in patients with cirrhosis
** 1-year mortality of 0%, 20% and 55% for A, B and C respectively
** Mortality rate after abdominal surgery
*** A 10%
*** B 30%
*** C 70-80%
** Scoring
*** Score of 5-15
*** 5-6 = CPA
*** 7-9 = CPB
*** 10-15 = CPC
** Breakdown
*** 1, 2 or 3 points for each category
*** Ascites: Absent, slight, moderate
*** Bili: <35, 36-51, >51
*** Albumin: >35, 28-35, <28
*** INR: <1.7, 1.7-2.3, >2.3
*** Encephalopathy: None, Grade 1 or 2, grade 3 or 4
* MELD (Model for End-stage Liver Disease)
** Determines prognosis and prioritises liver transplant allocation
** Versions
*** Original MELD
*** MELD-Na (2016)
*** MELD 3.0 (2022) - swap out ascites and encephalopathy for sodium and creatinine levels
**** Creatinine level (receive max value if on dialysis)
**** Bilirubin level
**** INR
**** Sodium level
**** Albumin level

* Elective surgery well tolerated in CP-A or MELD <10
* Might be able to optimise a CP-B/MELD 10-15 to safety
* All surgery contraindicated in CP-C/MELD >15
** Only if there is no other option, and should be clearly informed about their high mortality risk



== '''Operating on patients with cirrhosis''' ==

* Risks
** Surgical factors
*** Recanalised umbi veins
*** Stiff, nodular liver
** Physiological
*** Coagulopathy - bleeding and clots
*** Decompensated liver
*** Renal failure
*** Infection
*** Leaking ascites and hernia recurrence
*** Mortality - see above
* '''Pre-op'''
** Ensure seeing a gastroenterologist - optimise liver function, treat underlying cause
** Consider timing and location
*** Should it be done by HPB surgeon?
*** Is a transplant happening soon, and it should be done at the same time?
*** Should be done in a centre with gastroenterologist, experienced anaesthetist and ICU
** Minimise ascites
** Correct malnutrition, including vitamin K
** Optimise coagulopathy
*** Correcting the coagulopathy with FFP is difficult and frequently does not help; vitamin K is obligatory but frequently also doesn't help. There is an expert opinion in Schein's that an INR of 2.5 in a cirrhotic patient is not necessarily as prone to bleeding as an INR of 2.5 in a patient taking warfarin, and that he wouldn't give FFP to any liver patients pre-op. This is supported by modern studies - INR and PLT don't seem to predict bleeding risk, whereas abnormal TEG does.
*** Use ROTEM/TEG to guide clotting ability
*** Generally aim fibrinogen >1 and platelets >50
*** Avoid prothrombinex in severe chronic liver disease - very prothrombotic - risk of dead toes etc.
** Identify abdominal wall varices - CT
** Check for portal hypertension and avoid operating near portal system if present
** Ensure group and hold done
* '''Intra-op'''
** Obsessive haemostasis, haemostatic agents and energy devices available
** Meticulous respect for peritoneal integrity
** Avoid abdominal wall varices
** Drains are controversial - risk of infection - may be better off performing periodic ascitic tap
** Conservative intra-op fluids, and generally avoid saline due to higher risk of hyperchloraemic metabolic acidosis - give Hartmann's instead, or albumin
* '''Post-op'''
** High-risk for infections, haematomas, ascites and renal failure. Restrictive fluid management, possibly with low-dose diuretics. Oliguria may mean volume overload.
** Protein losses of prolonged post-op drainage are prohibitive.
** Consider AWS/thiamine
** Resume diet and lactulose early to reduce the risk of decompensation

[[Category:Liver]]