Congenital Abnormalities of the Liverm biliary tree and pancreas

4th, as a diverticulum arising from duodenum

cranial portion

caudal portion, and are canalized by 8wks gest

12th

Idiopathic
Anatomic
Metabolic
Hepatitis
Genetic
Miscellaneous

Biliary Atresia
Choledochal cyst
Spontaneous perforation of the bile duct

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.

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

Ductal plate abnormality
Failure of recanalization
Infantile obstructive cholangiopathy
Ischemic insults
Abnormal bile acid metabolism
Immunologic dysfuntion

Jaundice often not apparent until 3 to 5 weeks of life.
Acholic stools present
Liver firm and enlarged
Elevated alkaline phosphatse

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

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.

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

Hypertrophy of normal pancreatic tissue
Fusion of heterotopic pancreatic rests
Failure of left ventral bud atrophy
Failure of free rotation of right ventral bud

Symptoms may present at any age. Approximately 50% in pediatric age
Vomiting
Peptic ulceration
Abdominal pain
Jaundice
Associated with other congenital defects

Evidence of duodenal obstruction on plain abdominal film(double bubble effect)
Sonographic evidence
Barium studies demonstrating duodenal filling defect
ERCP and laparotomy

Surgical intervention mandatory in cases with obstruction
Recommended approach is a bypass operation, preferably duodenoduodenostomy

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

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

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

Importance of anomaly in clinical disease controversial
Recurrent attacks of pancreatitis or continuous epigastric pain
Diagnosis depends on ERCP

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