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22 Cards in this Set

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Liver embryology
Liver appears during 4th week of gestation as diverticulum arising from duodenum
Cranial portion forms parenchymal tissue and bile ducts
Caudal portion forms gallbladder and cystic duct
Bile canaliculi embryology
Bile canaliculi well developed by 6 weeks of gestation
Intrahepatic ducts formed from cell plates derived from hepatic duct and is complete by 12 weeks of gestation
Gallbladder and cystic duct canalize by 8 weeks of gestation
Pathologic jaundice
Conjugated fraction comprises more than approximately 20% of total
Cholestasis defined physiologically as a reduction in canalicular bile flow
There are multiple causes of cholestatis in early life
-Idiopathic (20-30%)
-Biliary Atresia (25-30%)
-Choledochal cyst
-Spontaneous perforation of the bile duct
-Alpha-1 AT deficiency
Biliary atresia: overview
Progressive disease characterized by dynamic fibroobliteration of the bile ducts
Occurs in a range of 1 in 8000 to 25000 live births
In some cases associated malformations occur
Biliary atresia: phenotypes
Embryonic or fetal type-Approximately 34% of cases. More commonly associated with other abnormalities
Perinatal type-Approximately 66% of cases. Later onset of jaundice.
Biliary atresia: etiology
May represent either a congenital or acquired abnormality
The congenital form may represent failure of recanalization of bile duct
Acquired form seems to result from a progressive destruction of the bile ducts by an inflammatory process

Theories:
Ductal plate abnormality
Failure of recanalization
Infantile obstructive cholangiopathy
Ischemic insults
Abnormal bile acid metabolism
Immunologic dysfuntion
Biliary atresia: clinical presentation
Jaundice often not apparent until 3 to 5 weeks of life.
Acholic stools present
Liver firm and enlarged
Elevated alkaline phosphatase
Biliary atresia: pathology
In approximately 90% there is inflammatory obliteration of all or portions of extrahepatic biliary tract
Hepatic architecture generally intact with features of extrahepatic obstruction. Bile stasis and bile duct proliferation
Staged evaluation of infant with suspected cholestasis
Clinical evalulation (history, PE)

Initial lab studies
-Fractionated bilirubin
-SGOT/SGPT
-GGT

Stool color

Measure of hepatic synthetic function
-PT/PTT

Viral and bacterial cultures

Viral serology
-HBSAg
-TORCH
-VDRL

Alpha 1 AT phenotypes

Thyroxin and TSH

Sweat chloride

Metabolic screen (urine or serum amino acids, urine reducing substances)

Ultrasonography

Hepatobiliary scan (DISIDA)

Percutaneous liver biopsy
Extrahepatic cholestasis vs intrahepatic cholestasis: clinical presentation
Extrahepatic born at normal birth weight
-Earlier onset of symptoms

Intrahepatic low birthweight
-Present later
Biliary atresia and liver transplant
#1 reason for babies with jaundice to have liver transplant
Choledochal cyst: overview and epidemiology
Cystic dilation of biliary tree

Incidence 1 in 13,000-2,000,000

30% diagnosed before age 1 year

More common in females

Increased risk of carcinoma
Pancreas embryology
Originates as dorsal bud and two ventral buds
The left ventral bud atrophies
Rotation of stomach and duodenum with elongation carries right ventral bud posteriorly
Fusion of right ventral with dorsal bud completes head and uncinate process
Right ventral bud forms major duct
Dorsal bud forms minor duct
Annular pancreas: pathogenesis
Numerous possibilities:
Hypertrophy of normal pancreatic tissue

Fusion of heterotopic pancreatic rests

Failure of left ventral bud atrophy

Failure of free rotation of right ventral bud
Annular pancreas: clinical presentation
Symptoms may present at any age. Approximately 50% in pediatric age
Vomiting
Peptic ulceration
Abdominal pain
Jaundice
Associated with other congenital defects
Annular pancreas: diagnosis
Evidence of duodenal obstruction on plain abdominal film(double bubble effect)
Sonographic evidence
Barium studies demonstrating duodenal filling defect
ERCP and laparotomy
Annular pancreas: management
Surgical intervention mandatory in cases with obstruction
Recommended approach is a bypass operation, preferably duodenoduodenostomy
Ectopic pancreas: overview
Presence of pancreatic tissue lacking anatomic and vascular continuity with the main body of pancreas
70-90% occurs in the upper GI tract mostly in gastric antrum
Usually incidental finding
Pancreatic agenesis, hypoplasia, and dysplasia
Complete agenesis rare and usually incompatible with life
Clinical manifestations attributable to both exocrine and endocrine pancreatic dysfunction
Diagnosed at autopsy, surgery, or with radiographic studies
Pancreas divisum: pathogenesis
Refers to abnormality resulting from incomplete fusion of dorsal and ventral pancreatic ductal systems
Dorsal duct functions as main drainage system but opens into smaller accessory papilla
Development of pancreatitis
Pancreas divisum: clinical
Importance of anomaly in clinical disease controversial
Recurrent attacks of pancreatitis or continuous epigastric pain
Diagnosis depends on ERCP
Pancreas common channel syndrome: overview
Anomalous junctions of common bile duct and main pancreatic duct
The presence of a long common channel with pancreaticobiliary junction located outside of duodenal wall associated with pancreatitis and choledochal cyst
Reflux of pancreatic juice