Cme at bharuch 27.6.13
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Transcript of Cme at bharuch 27.6.13
"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