Congenital anomalies
Dept of Surgery
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Anatomy
Lies transversely in the retroperitoneal space, between
the duodenum on the right and the spleen on the left
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Related anteriorly to the omental bursa above, the
greater sac below, and the transverse mesocolon
Fixed organ
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level of the L2 vertebra
From the duodenal C loop
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to the hilum of the spleenDivided into four parts
Cross-sectional relation of the pancreas to other
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abdominal structures
Embryology
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Pancreatic Ducts
Main pancreatic duct (duct of Wirsung), joins
the CBD to empty into the duodenum at the
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ampulla of VaterDiameter -- 2 to 4 mm
Pressure --15 to 30 mm Hg
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Lesser duct (duct of
Santorini)
Drains superior portion
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of head of the PancreasEmpties separately into the
2nd part of duodenum
through the minor papilla
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Arterial SupplyCeliac
Superior mesenteric artery
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Splenic artery
Venous Drainage
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Suprapancreatic portal vein
Retropancreatic portal vein
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Splenic veinsInfrapancreatic SMV
Lymphatic Drainage
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Drain into five main nodal groups? Superior nodes drain the upper half of the head of the pancreas
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? Anterior lymphatic drain to the prepyloric and infrapyloric nodes? Inferior group of nodes drain to the superior mesenteric and periaortic nodes
? Posterior pancreaticoduodenal lymph nodes drain into right periaortic nodes
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? Splenic group of nodes drain into the interceliomesenteric lymph nodes
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The absence of a peritoneal barrier on the posterior
surface of the pancreas results in direct communication
of the intrapancreatic lymphatics with retroperitoneal
tissues, and this contributes to the high incidence of
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recurrence after presumably curative resections ofpancreatic cancer
Histology
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Exocrine Structure
Acinar cells secrete the enzymes responsible for
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digestionDuctular network carry the exocrine secretions into
the duodenum
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Constitute 80% to 90% of the pancreatic mass
Endocrine Structure
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Islets of Langerhans responsible for the secretion ofhormones that control glucose homeostasis
Contains
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alpha (A)-- glucagon
beta (B)-- insulin
delta (D)-- somatostatin
pancreatic polypeptide (PP) or F cel s? PP
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Accounts for 2% of the pancreatic mass
Pancreatic Exocrine Enzymes
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Pancreatic Endocrine Enzymes
Investigations
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? Serum MarkersAmylase, lipase, trypsinogen, and elastase
CA 19.9 (Tumor Marker)
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? Lundh meal test? Ultrasound
? EUS
? CECT Scan
? MRCP
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? ERCPUltrasound image of normal pancreas (left) and acute pancreatitis (right).
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Evidence of parenchymal edema and peripancreatic fluid accumulation is seenNon necrotizing acute pancreatitis
Congenital Anomalies
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The complex process by which the dorsal and
ventral pancreatic primordia fuse during
pancreatic development gives rise to
congenital variations
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Agenesis
Very rarely, the pancreas may be totally absent
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The homeodomain transcription factor IPF1(PDX1) is critical for the development of the
pancreas, and germ line (inherited)
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homozygous mutations in the IPF1 gene on
chromosome 13q12.1 have been reported in a
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patient with pancreatic agenesisPancreas divisum
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Most common clinically significantIncidence of 3% to 10%
Caused by a failure of the fetal duct systems of
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the dorsal and ventral pancreatic primordia tofuse
Cystic fibrosis
vAutosomal recessive
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vMore in Caucasians
vIncidence 1 in 2500 live births in U.K.
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vDefect is mutation in CFTR gene on Ch 7vHigh levels of Na2+,Cl2- ions in sweat
Annular pancreas
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Develops when one portion of the ventral pancreatic primordium
becomes fixed, while the other portion of this primordium is drawn
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around the 2nd or 3rd duodenumCan be associated with congenital duodenal atresia/ stenosis
Common in children with Down's syndrome
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Present with signs and symptoms of duodenal obstruction such as
gastric distention and vomiting
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Tx- DuodenoduodenostomyEctopic pancreas
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Found in about 2% autopsy specimenSites for ectopia are stomach and duodenum, followed
by the jejunum, Meckel diverticula, and ileum
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May cause pain from localized inflammation, or, rarely,
may incite mucosal bleeding
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Approximately 2% of islet cell tumors arise in ectopicpancreatic tissue.