"If it ain't broke, don't fix it."

But its more important to know when its broken

Jaundice

Definition

• Yellow discoloration of sclera, skin, mucous membranes due to deposition of bile pigment

Classification

• Pre Hepatic, Hepatic, Post hepatic

• Hemolytic, Hepatocellular, Obstructive

• Conjugated or Non Conjugated Hyperbilirubinemia

CASE 1: PEDIATRICS

A five year old female child Muslim by religion

•Jaundice•Itching all over the body•Pain in abdomen occasionally

Presented with

History• Jaundice since the age 20 MONTHS OLD

• Full term normal delivery with birth weight of 2800

• Pregnancy uneventful

• Postnatal period passed without problems

• No delayed meconium

• No history of, constipation, feeding difficulties, vomiting, diarrhea, fever, convulsion or bleeding from any site

• Sleeping disturbance and had history of frequent inconsolable crying

• Family: parents are first degree cousins

BASIC EVALUATION :Examination

• Deeply jaundice• Child very irritable• Multiple scratch marks all over his body• Weight 16.8kg• Height 99 cm• Head circumference was 47cm • Below 25th centile• Spleen enlarge to 1.5cm BCM• Liver enlarge to 3 cm BCM• NO Ascites• No signs of chronic liver disease• No sign of rickets

INTRAHEPATIC EXTRAHEPATICIdiopathic Extrahepatic Billiary atresia

Toxic (TPN, DRUG) Choledochal cyst

Genetic (TRISOMY 18,21) Bile duct stenosis

Infectious SPBD

Metabolic Cholelithiasis

Miscellaneous Inspissated bile/mucus plug

External Bile Duct compression

BASIC EVALUATION: Blood IgCBC and reticulocyte count NORMAL

Electrolytes, BUN, creatinine,

calcium, phosphate

NORMAL

AST

ALT

GGT

alkaline phosphatase

509

231

72

552

Total BILIRUBIN

direct bilirubin

20.2

8.7

Prothrombin time

Partial Thromboplastin time

Total protein, albumin, cholesterol,

14/14 (1.0 INR)

30/32

Normal

Basic Evaluation : Infection

Indicated cultures of blood,

Urine, CSF

Negative

TORCH titers, RPR/VDRL Negative

Urine for CMV Negative

Hepatitis B and C serology Negative

Basic Evaluation : Radiological

– Ultrasonography• Slight increase echogenicity of enlarged Liver

Basic Evaluation: MetabolicProtein electrophoresis, alpha-1-antitrypsin level and phenotype

Normal

Thyroid function tests Normal

Serum Zinc Normal

Serum amino acids

Chromotography

Normal

Urine reducing substances Not Detected

Urine bile acids Not Detected

Blood Pyruvate Normal

Blood Lactate Normal

Advance Evaluation: Invasive

– Duodenal intubation

– Percutaneous liver biopsy

– Percutaneous transhepatic cholangiography

– Endoscopic retrograde cholangiopancreatography (ERCP)

– Exploratory laparotomy with intraoperative cholangiogram

23%56%1%

7%

13%

COMMON ETIOLOGIES

• Premature infants– Sepsis/Acidosis

– TPN-associated

– Drug-induced

• Idiopathic neonatal hepatitis

• Extrahepatic biliary atresia

• Alpha-1-antitrypsin deficiency

• Intrahepatic cholestasis syndromes (Byler’s syndrome)

What is diagnosis?

PFIC : Progressive Familial Intrahepatic Cholestasis

Type of PFIC : 3

PELD score: 14

What would be the right Option?

• Liver transplantation

• Partial External biliary diversion or PIBD

• Cholecystocolostomy using jejunum or colon

59%

12%

29%

NIH recommended option of Partial Internal Biliary Driannage as Gold standard option in Neonatal Cholestasis for Lower PELD score patient in place of Liver Transplantation

Partial Biliary Diversion

• External : Partial External Biliary Diversion

– A vascular jejunum segment connecting Gall bladder is exteriorize

• Internal : Partial Internal Biliary Diversion

– Using Jejunum as Interposition

– Cholecystocolostomy

Other Neonatal cases

• Extrahepatic biliary atresia

• Choledochal cyst

• Bile duct stenosis

• SPBD

• Cholelithiasis

• Inspissated bile/mucus plug

• External Bile Duct compression

EXTRAHEPATIC BILIARY ATRESIAKASAI PROCEDURE

• Performed for biliary atresia that is not surgically correctable with excision of a distal atretic segment.

• Roux-en-Y portoenterostomy

• Bile flow re-established in 80-90% if performed prior to 8 weeks-old.

• Bile flow re-established in less than 20% if performed after 12 weeks-old

INTERVENTIONS

• Surgical

– Kasai procedure for biliary atresia

– Limited bile duct resection and re-anastomosis

– Choledochal cyst excision

– Cholecystectomy

– Liver transplantation

LIVER TRANSPLANTATION

• Survival rates approach 80% at 1 year and 70% at 5 years.

• Biliary atresia is the most common indication for transplant and may be the initial treatment when detected late or may be used as a salvage procedure for a failed Kasai.

• Used early in cases of tyrosinemia.

• Increasing swelling of his abdomen

• Swelling on feet over the last 2 months.

• Increasing tired over this time

• Nauseous and is off his food.

• Eyes have turned yellow over last few days.

• Works in a warehouse

• Smokes 10 cigarettes a day.

• Drinking 4 – 8 (150ml) of alcohol a Day.

• A half bottle of whisky a day.

CASE 2: Adult

54 year old gentleman presents

Examination

• Jaundice

• He has no hepatic flap

• He is orientated in time, place and person.

• His abdomen is distended but soft and non-tender.

• There is no palpable organomegaly

• There is shifting dullness.

GVAN-T

• Viral

• Autoimmune - Autoimmune hepatitis, PBC, PSC

• Genetic - Wilson’s, Haemochromatosis, Alpha 1 antitrypsin deficiency

• Toxic / Drugs – alcoholic, paracetamol

• Non-alcoholic fatty liver (DM / metabolic syndrome, pregnancy, idiopathic)

Chronic Liver Disease - Etiology

Re – Cap of the Sign of LCF

NEED OF INTERVENTION• ACUTE BLEEDING

– ( ESOPHAGO-GASTROSCOPY)

• ASCITES– Tapping

• DIAGNOSIS DELIMMA (LIVER BIOPSY)– Auto immune hepatitis– PBC, PSC– WILSON DISEASE– ALPHA 1 ANTI TRYPSIN DEFICIENCY– HEREDITARY HEMOCHROMATOSIS

MELD SCORE

Survival probability of a patient with end-stage liver disease is estimated based on the following variables.– Serum bilirubin– Serum Creatinine– International Normalized ratio– (etiology)

UTILITY: 1. To predict dealth within three months of TIPS 2. For prioritizing allocation of liver transplants

instead of the older Child-Pugh Score

MODEL FOR END-STAGE LIVER DISEASE

A stenting between Hepatic system and Portal System thro Liver Parenchyma

Risk

• Damage to blood vessels

• Fever

• Hepatic Enchephalopathy

• Infection, bruising, or Bleeding

• Reactions to medicines or the contrast dye

• Stiffness, bruising, or soreness in the neck

Transjugular Intrahepatic Portosystemic Shunt (TIPS)

DDW guidelines for SHUNT / Devascularization SURGERIES

In cirrhotic,

• Elective operations should be preferred– using portal flow preserving techniques

• such as a selective distal splenorenal shunt (Warren)

• a partial portocaval small diameter interposition shunt (Sarfeh)

– Rarely, end-to-side portocaval shunt may serve as a salvage procedure

Until definitive results from randomized trials are available patients with good prognosis (Child-Pugh A and B) should be regarded as candidates for surgical shunts

Portosystemic shunt surgery represents the only effective therapy

which leads to freedom of recurrent bleeding and repeated endoscopies for many years,

and improves hypersplenism without deteriorating liver function or encephalopathy.

Gastro esophageal Devascularization and other direct variceal ablative procedures should be restricted to treat endoscopic therapy failures without shuntable portal tributaries.

DDW guidelines for SHUNT / Devascularization SURGERIES

Noncirrhotic portal hypertension or

Extrahepatic portal vein thrombosis.

Indications for Transplant•Irreversible cirrhosis with at least two signs of liver insufficiency

•Fulminant hepatic failure: Coma Grade 2

•Unresectable hepatic malignancy confined to the liver that is less than 5 cm. in diameter

•Metabolic liver disease that would benefit from liver replacement

•MELD score of 15 or higher

Other specific indications for liver transplantation include:

•Budd-Chiari Syndrome,

•Benign hepatic tumors,

•Autoimmune liver diseases.

General clinical and biochemical indications for liver transplantation

• Serum bilirubin > 8-10 mg/Dl• Intractable pruritus• Intractable bone disease• Malnutrition or recurrent bacterial cholangitis• Severe or intractable encephalopathy

•Serum albumin < 3.5 g/dL•Prothrombin time > 3 seconds above control or INR > 1.3•Encephalopathy•Ascites•Bilirubin > 2 mg/dL

In patients with chronic hepatocellular diseases

Patient Selection Criteria for Liver Transplantation

• Severe fatigue

• Unacceptable quality of life

• Recurrent variceal bleeding

• Intractable ascites

• Recurrent or severe hepatic encephalopathy

• Spontaneous bacterial peritonitis

• Hepatorenal syndrome

• Development of small hepatocellular carcinoma on hepatic imaging

Contraindications to Transplant

Absolute

• AIDS or HIV positivity

• Irreversible brain damage

• Multi-system failure

• Malignancy outside the liver (not skin cancer)

• Infection outside the hepatobiliary system

• Active alcohol or substance abuse

• Advanced cardiopulmonary or other systemic disease

Contraindication for Liver Transplant

• Advanced age

• Advanced chronic renal failure

• Cholangiocarcinoma /Hepatocellular Carcinoma

• Chronic hepatitis B virus infection

• Hypoxemia from intrapulmonary shunts

• Massive ascites

• Portal vein thrombosis

• Prior portosystemic shunt surgery / Biliary Surgery

• Severe malnutrition

• Severe abdominal atherosclerosis

Relative Contraindication

Timely early Referral

• For preoperative evaluation for liver transplantation is critical

• Allows evaluation before the development of multiple or advanced complications

• Allows close follow-up of patients with end-stage liver disease with the referring physician(MELD)

• In addition, the family members and patient have ongoing education regarding the liver transplant process.

CASE 3: ADULT

A 56 year old woman was admitted with jaundice and severe pain in abdomen

Brief History

• Recent past history of recurrent pain

• Antecedents of cholelithiasis

• Cholecystectomy done before 2 years

• Recurrent history of pancreatitis

• Overweight diabetic using Metformin

INVESTIGATION

• USG was normal other than an incidence of bulky pancreas

• With no stone in CBD• Ct scan showed normal Hepatobiliary region with

CBD. The fat plan of pancreas was obscure over the body and head(indicating recent pancreatitis)

• MRCP was done, showed a small peanut growth in Ampullary region

• ERCP : Transpapillary biopsy : Ampullary AdenoCa• ERCP stenting was done to relieve jaundice

Whipples Procedure

Care in pancreatic surgery

•Parenchyma•Duct size•With stomach or jejunum•Pylorus Preserving•Duodenum Preserving•Pancreatic fistula•Duodenal fistula•Spleen Salvaging Distal Pancreatectomy

Obstructive of bile Ducts

• Compression obstruction from tumors

• Pancreatic Mass ( Inflammatory/ Malignant)

• Intraluminal gallstones

• Stenosis-postoperative or inflammary

CAUSES OF OBSTRUCTIVE JAUNDICE

Rampant increase in Biliary Stricture

• After 1990, especially after 2001 in India

• Increase use of Laparoscopic Gall Bladder Surgery

• 2 Dimensional Surgical Skills and training

• Most Injury go un-noticed during Surgery

• Faulty Technique in Gall Bladder Retraction and Calot’s Dissection

Intervention in Hemolytic Jaundice

• Patient with HyperSplenism and Pancytopenia

• Refractory to Medical Management

• Partial / Total Splenectomy

• Splenosis

• Gaucher Disease the only indication for Partial Splenectomy

Magnetic Resonance Cholangio-

Pancreatography (MRCP)

Two stones in the common bile duct

Primary sclerosing cholangitis (PSC)

with stricture due to

cholangiocarcinoma.

Retrograde Cholangiogram - ERCP

Retrograde Cholangiogram - ERCP

Bile leak from the cystic duct after

cholecystectomy

Irregular dilation of intrahepatic and

extrahepatic ducts.

Retrograde Cholangiogram - ERCP

Primary Sclerosing Cholangitis

Normal

Extra

hepatic BD

Narrowed abnormal

intra-hepatic bile ducts.

CT Abdomen

A large mass with a

hepatoma.

GOALS OF TIMELY EVALUATION

• Diagnose and treat known medical and/or life-threatening conditions.

• Identify disorders amenable to surgical therapy within an appropriate time-frame.

• Surgical intervention in rightly choose diseases.

• Early Evaluation and intervention reduces morbidity and mortality

When to refer to Liver Surgeon

•Unexplained Jaundice

•Suspected biliary obstruction

•Acute hepatitis - severe or fulminant

•Unexplained abnormal LFTs persisting (for 6 months or greater)

•Unexplained cholestatic liver disease ( adult or Pediatric)

•Cirrhosis (in non-alcoholic) for consideration of liver transplant

•Suspected hereditary hemochromatosis /Wilson's/autoimmune

•Non cirrhotic Portal Hypertension

•Suspected Obstructive Jaundice

Thanks to IMA BHARUCH

Dr. Tapan ShahMCh Surgical GastroenterologyBoard Certified G I Surgeon, ( U.S.A)PhD ( Pediatric Liver Transplantation)www.drtapan.com9925033348