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Anatomy
Anatomy
Embryology
Embryology
• Liver and Biliary tree develops from liver
primordium which emerges from the
endoderm around 3rd week.
• It consists of a cranial portion and a caudal
portion
• Cranial portion forms liver and intrahepatic
biliary tract.
• Extra hepatic bile ducts and the gallbladder
arise from the caudal portion.
Congenital Anomalies
Congenital Anomalies
• Floating gall bladder
• Double gall bladder
• Absence of the cystic duct
• Low insertion of the cystic duct
• An accessory cholecystohepatic duct
• Biliary Atresia
• Choledochal cyst
Biliary Atresia
Etiology
Etiology
• Idiopathic
• Congenital
• Traumatic
• Infections /Infestation
• Autoimmune
• Neoplastic (Benign/Malignant)
• Degenerative
Etiology
• Not known.
• Arrest of development at solid stage.
• Autoimmunity triggered by viral infection
Clinical Features
Clinical Features
• Demography
• Symptoms
• Signs
• Prognosis
• Complications
Demography
Demography
• 1 in 5000 – 1in 20,000
• More in Asians.
Symptoms & Signs
Symptoms & Signs
• Low birth weight
• Conjugated Jaundice
• Pale Stools
• Yellow urine
• Failure to thrive
• Bleeding tendency
• Portal hypertension
• Splenomegaly
• Oesophageal varices
Associated Malformations
Associated Malformations
• Polysplenism
Pathophysiology
Pathophysiology
• Occlusive pan ductular cholangiopathy.
• Not simply mechanical
• Inflammation +nt
• Intrahepatic bile ducts are not dilated
Investigations
Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histology
Diagnostic Studies
Diagnostic Studies
Imaging Studies
• X-Ray
• USG
• CT
• Angiography
• MRI
• Endoscopy
• Nuclear scan
Investigations
Investigations
• Technitium-99m iminodiacetate(DISIDA)
scan.
• Normal alpha1 antitrypsin level.
• USG to exclude choledochal cyst.
• Liver biopsy.
• Barbiturate therapy.
• ERCP
• Surgical exploration and cholangiogram
Differential Diagnosis
Differential Diagnosis
• Prolonged Physiological jaundice
• Obstructive jaundice
– Neonatal hepatitis
– Inspissated bile syndrome
– Choledochal cyst
• Hematological disorders
– ABO/Rh incompatibility
– Spherocytosis
• Metabolic disorders
• Congenital infections
Differential Diagnosis
• Physiological jaundice
• Obstructive jaundice
• Hematological disorders
• Metabolic disorders
– Alpha1 antitrypsin deficiency
– Galactosemia
– Pyruvate kinase deficiency
• Congenital infections
– Syphilis
– rubella
Operative Therapy
Operative Therapy
• Kasai operation- Portoenterostomy
• Liver Transplantation
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