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22 Cards in this Set

  • Front
  • Back
Liver appears during what week of gestation
4th, as a diverticulum arising from duodenum
Parenchymal tissue and bile ducts form from
cranial portion
gallbladder and cystic duct are formed from
caudal portion, and are canalized by 8wks gest
Intrahepatic duts formed from cell plates derived from hepatic duct and is complete by what week of gestation
12th
Disorders associated with cholestasis in newborns- intrhepatic
Idiopathic
Anatomic
Metabolic
Hepatitis
Genetic
Miscellaneous
Disorders associated with cholestasis in newborns-extrahepatic
Biliary Atresia
Choledochal cyst
Spontaneous perforation of the bile duct
Billiary Atresia
Progressive (mostly developmental) 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 (more congenital) occur
Embryonic or fetal type-Approximately 34% of cases. More commonly associated with other abnormalities
Perinatal type-Approximately 66% of cases. Later onset of jaundice.
Etiology of Biliary Atresia
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
Biliary Atresia Thoeries
Ductal plate abnormality
Failure of recanalization
Infantile obstructive cholangiopathy
Ischemic insults
Abnormal bile acid metabolism
Immunologic dysfuntion
Biliary Atresia- Clinical
Jaundice often not apparent until 3 to 5 weeks of life.
Acholic stools present
Liver firm and enlarged
Elevated alkaline phosphatse
staged evaluation of infant with suspected cholestasis
Clinical evaluation ( hx, PE), labs, stool color, PT/PTT, viral and bact cultures, viral serology, alpha 1 antitrypsin phenotype, thyroxin and TSH, sweat cloride, metabolic screen, ultrasound, hepato bili scan, percuataneous liver bx
Choledochal cysts
Congenital. cystic dilatation of biliary tree,
incidence is 1 in 13-2000000, 30% diagnosed before age 1, more common in females, incr risk of carcinoma.
Pancreatic 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
Annular Pancreas- Pathologies
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 Dx
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
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 dyplasia
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
Pancreatic ductal anomalies, 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
Pancreatic Ductal Anomalies Pancreas Divisum-Clinical
Importance of anomaly in clinical disease controversial
Recurrent attacks of pancreatitis or continuous epigastric pain
Diagnosis depends on ERCP
Pancreatic Ductal Anomalies Common Channel Syndrome
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