Biliary Atresia Diagnosis, Management protocols and Recent advances Dr Ravi Kanojia PGIMER, Chandigarh India
Objectives Spectrum of surgical jaundice How do we diagnose EHBA Pre op management by the surgical team Post op management Prognostic factors Follow up PGI experience Message and lessons learnt Recent advances in EHBA we will not be talking on Surgical details and options Liver transplant
Spectrum of surgical jaundice
How do we diagnose EHBA
Pre op management by the surgical team
Post op management
Prognostic factors
Follow up
PGI experience
Message and lessons learnt
Recent advances in EHBA
we will not be talking on
Surgical details and options
Liver transplant
Common case scenario Term 60 days old female infant Jaundice since birth Passing pale white stools with mustard color urine Palpable firm hepatomegaly Referred by a Pediatrician after a exhaustive non conclusive work up spending precious prognostic days
Term 60 days old female infant
Jaundice since birth
Passing pale white stools with mustard color urine
Palpable firm hepatomegaly
Referred by a Pediatrician after a exhaustive non conclusive work up spending precious prognostic days
Surgical causes of jaundice Biliary atresia Choledochal cyst Bile duct perforation Inspissated bile syndrome
Biliary atresia
Choledochal cyst
Bile duct perforation
Inspissated bile syndrome
Alpha1-anti-trypsin deficiency Idiopathic neonatal hepatitis Algillie syndrome Inborn errors of bile acid synthesis Nonsyndromic intrahepatic bile duct hypoplasia Total parenteral nutrition–associated (TPN) cholestasis Viral infections (eg, toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex [TORCH]) Other Causes
Alpha1-anti-trypsin deficiency
Idiopathic neonatal hepatitis
Algillie syndrome
Inborn errors of bile acid synthesis
Nonsyndromic intrahepatic bile duct hypoplasia
Total parenteral nutrition–associated (TPN) cholestasis
Viral infections (eg, toxoplasmosis, other infections, rubella, cytomegalovirus infection, and herpes simplex [TORCH])
Diagnosis Clinical Suspicion by Prolonged jaundice Conjugated hyperbilirubinemia Acholic stools dark urine Firm to hard palpable hepatomegaly Antenatally by abnormal maternal usg Cyst in the porta Spleenic malformations
Clinical Suspicion by
Prolonged jaundice
Conjugated hyperbilirubinemia
Acholic stools dark urine
Firm to hard palpable hepatomegaly
Antenatally by abnormal maternal usg
Cyst in the porta
Spleenic malformations
Investigations Non invasive & Invasive Non invasive Biliary USG HIDA/Mebrofennin Scan MRCP Invasive Liver biopsy Per op cholangiogram Serial Duodenal intubations ERCP Laparoscopy Hematology Routine LFT Gamma GGT Sr Lipoprotien X TORCH Minimum recommended (PGI Practice) USG + HIDA (with priming) + hematology (>85% sensitive for pre op diagnosis) Per op cholangiogram
Investigations
Non invasive & Invasive
Non invasive
Biliary USG
HIDA/Mebrofennin Scan
MRCP
Invasive
Liver biopsy
Per op cholangiogram
Serial Duodenal intubations
ERCP
Laparoscopy
Hematology
Routine LFT
Gamma GGT
Sr Lipoprotien X
TORCH
Minimum recommended (PGI Practice)
USG + HIDA (with priming) + hematology (>85% sensitive for pre op diagnosis)
Per op cholangiogram
Biliary USG Triangular Cord Sign GB motility GB length Measure GB length remains same before and after feeding All the 3 factors combined gives positive predictive value of > 90% JPS (2007) 42, 2093–2096
Triangular Cord Sign
GB motility
GB length
HIDA Hepatobiliary excretion scans Pretreatment with phenobarb 5mg/kg/d for 3-5 days Simultaneous administration of UDCA may be done DISIDA – not reliable when sr bil levels are very high (>10) 99mTc-mebrofenin IDA Recent reports of technetium-99m-mebrofenin iminodiacetate scans combined with estimation of gama GGT increases the negative predictive value to > 80%
Hepatobiliary excretion scans
Pretreatment with phenobarb 5mg/kg/d for 3-5 days
Simultaneous administration of UDCA may be done
DISIDA – not reliable when sr bil levels are very high (>10)
99mTc-mebrofenin IDA
Recent reports of technetium-99m-mebrofenin iminodiacetate scans combined with estimation of gama GGT increases the negative predictive value to > 80%
MRCP Is done when usg demonstrates cysts at porta Reliably differentiates between choledochal cyst and cystic variants of EHBA
Is done when usg demonstrates cysts at porta
Reliably differentiates between choledochal cyst and cystic variants of EHBA
Liver Biopsy 90% sensitivity and specificity for biliary atresia. Biopsies are not usually diagnostic in those younger than 2 weeks Portal bile ductular proliferation, bile plugging, portal-portal fibrosis, and an acute inflammatory reaction Not our preferred practice because most of the patients coming late and have a deranged coagulogram and practical problems
90% sensitivity and specificity for biliary atresia.
Biopsies are not usually diagnostic in those younger than 2 weeks
Portal bile ductular proliferation, bile plugging, portal-portal fibrosis, and an acute inflammatory reaction
Not our preferred practice because most of the patients coming late and have a deranged coagulogram and practical problems
Per op cholangiogram Decision algorithm ? EHBA Laparotomy Bile in GB Aspirate GB if patent with lumen White bile Obtain Cholangiogram Proceed for Kasai Small non patent fibrotic GB EHBA confirmed
Decision algorithm
Duodenal Intubation Aspiration of duodenal contents via an endoscope Preferred Japanese practice Reliably rules out BA if bile is seen aspirate Sensitivity can be increased by combining with HIDA – if aspirated contents shows some radioactivity then BA is ruled out
Aspiration of duodenal contents via an endoscope
Preferred Japanese practice
Reliably rules out BA if bile is seen aspirate
Sensitivity can be increased by combining with HIDA – if aspirated contents shows some radioactivity then BA is ruled out
ERCP Pediatric scope looking for ampula of vater and trying for retrograde dye study Technical limitations are there with the scope as well as the skill No added advantages over other investigations
Pediatric scope looking for ampula of vater and trying for retrograde dye study
Technical limitations are there with the scope as well as the skill
No added advantages over other investigations
Laparoscopy Has a role in obtaining a POC with minimally invasive technique KPE procedure can be accomplished through it Major advantages offered are Magnification while dissecting at the porta Minimal adhesions at the time of liver transplant
Has a role in obtaining a POC with minimally invasive technique
KPE procedure can be accomplished through it
Major advantages offered are
Magnification while dissecting at the porta
Minimal adhesions at the time of liver transplant
Diagnosis pathway followed Clinical Suspicion (conjugated jaundice), acholic stools Hepatobiliary USG Hepatic nuclear excretion scans If no bilioenteric drainage POC and proceed
Pre op management protocol Physicians role Identify Conjugated jaundice + pale stools go for USG followed by HIDA EARLY REFERAL
Physicians role
Identify
Conjugated jaundice + pale stools go for USG followed by HIDA
EARLY REFERAL
Pre op management Phenobarbitone Vit k Antibiotics Nutrition
Phenobarbitone
Vit k
Antibiotics
Nutrition
Post operative management Supportive Pharmacological options improving outcomes Phenobarbitone UDCA Steroids Vitamin supplements Antibiotics
Supportive
Pharmacological options improving outcomes
Phenobarbitone
UDCA
Steroids
Vitamin supplements
Antibiotics
Role of steroids Basis Reduces inflamation Immune suppression Cholerectic action- increases canalicular electrolyte transport and stimulate bile flow independent of the bile salt concentration. With these basis it Decreases protal fibrosis Increased bile flow Decreasing cholangitis Our practice Not routinely prescribed In the event of cholangitis or persistent white stools 2mg/kg/d prednisolone
Basis
Reduces inflamation
Immune suppression
Cholerectic action- increases canalicular electrolyte transport and stimulate bile flow independent of the bile salt concentration.
With these basis it
Decreases protal fibrosis
Increased bile flow
Decreasing cholangitis
Our practice
Not routinely prescribed
In the event of cholangitis or persistent white stools 2mg/kg/d prednisolone
Prognostic factors Age at presentation Bile duct size at porta after excision of the fibrotic cord Liver histology at the time of KPE Presence of portal hypertension Associated malformations MAP Score JPS, Vol31, No 10,1996: pp 1387-1390
Age at presentation
Bile duct size at porta after excision of the fibrotic cord
Liver histology at the time of KPE
Presence of portal hypertension
Associated malformations
Follow up protocol Repeat LFT at 1 mth Repeat HIDA at 3 mth 3 monthly review subsequently Registration for LTx registry
Repeat LFT at 1 mth
Repeat HIDA at 3 mth
3 monthly review subsequently
Registration for LTx registry
Recent Advances Ductal plate malformation – aberrant morphological distribution of intrahepatic bile ducts seen using immunohistochemical staining for cytokeratin 19 (CK-19) in liver biopsy
Ductal plate malformation – aberrant morphological distribution of intrahepatic bile ducts seen using immunohistochemical staining for cytokeratin 19 (CK-19) in liver biopsy
Recent advances in molecular pathology Increased expression of ICAM AND VCAM molecules in the sinusoidal epithelium Now serum measurements of the molecules is possible Few candidate genes jagged 1 mutation CFC HNF-6 INV
Recent advances in molecular pathology
Increased expression of ICAM AND VCAM molecules in the sinusoidal epithelium
Now serum measurements of the molecules is possible
Few candidate genes
jagged 1 mutation
CFC
HNF-6
INV
PGI Experience 15 pt in single unit in 14 mth Age range 1-6 mth Mean age 87 days Mean bil levels 10.4 (range 6-17) Predominant usg findings Small contracted GB or gb not visualised Cysts at the porta HIDA scan Good hepatocyte uptake with absent billio enteric drainage
15 pt in single unit in 14 mth
Age range 1-6 mth
Mean age 87 days
Mean bil levels
10.4 (range 6-17)
Predominant usg findings
Small contracted GB or gb not visualised
Cysts at the porta
HIDA scan
Good hepatocyte uptake with absent billio enteric drainage
Atretic GB with complete atresia of the EHBT Ty IIA Extrahepatic cyst with proximal atresia Per op findings 12 1 2
Outcome (short term) All passed yellow stools after the procedure Persistent jaundice (levels les than pre op) Appetite increased Gaining weight Mortality none
Outcome (short term)
All passed yellow stools after the procedure
Persistent jaundice (levels les than pre op)
Appetite increased
Gaining weight
Mortality none
Lessons learnt Poor prognosis even after good bile flow Surgery improves quality of life in terms of feeding and thriving Prognosis does not has a marked difference with early v/s late operated patients Early sx reduces the incidence of complications, post op cholangitis, better response to drug treatment and delayed appearance of secondary PHT
Poor prognosis even after good bile flow
Surgery improves quality of life in terms of feeding and thriving
Prognosis does not has a marked difference with early v/s late operated patients
Early sx reduces the incidence of complications, post op cholangitis, better response to drug treatment and delayed appearance of secondary PHT
Kasai Portoenterostomy Video part 1
Video part 1
Kasai Portoenterostomy Video part 2
Video part 2
Kasai Portoenterostomy Video part 3
Video part 3
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