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Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology
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Pancreas Congenital Anomalies (agenesis, pancreas divisum, annular pancreas, ectopic pancreas, congenital cysts) - reference mostly from Textbook of Robbins Pathology

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CONGENITAL ANOMALIES OF PANCREAS. (including clinicopathological references - morphology, lab diagnosis, complication, prognosis, etc)

CONGENITAL ANOMALIES OF PANCREAS. (including clinicopathological references - morphology, lab diagnosis, complication, prognosis, etc)

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  • 1. • To explain about anatomy of pancreas: – Gross : blood supply, lymphatic drainage, innervations, ducts system, embryology – Histology : exocrine, endocrine • To explain about congenital anomalies of pancreas: 1. Agenesis 2. Pancreas Divisum 3. Annular Pancreas 4. Ectopic Pancreas 5. Congenital Cysts
  • 2. Anatomy of Pancreas Gross Histology
  • 3. • A gland with both exocrine and endocrine functions. • 15-25 cm long • 60-100 g • Location: retro-peritoneum, 2nd lumbar vertebral level • Extends in an oblique, transverse position • Parts of pancreas: head, neck, body and tail
  • 4. Arterial Supply: – splenic artery – pancreaticoduodenal arteries Venous Drainage:  Suprapancreatic PV  Retropancreatic PV  Splenic vein  Infrapancreatic SMV • Ultimately, drains into portal vein Innervations: • Sympathetic fibers (from the splanchnic nerves) • Parasympathetic fibers (from the vagus)
  • 5. • The pancreatic duct begins in the tail of pancreas. • Joins CBD at the Ampulla of Vater. • 2 – 4 mm in diameter, 20 secondary branches • Lesser duct (Santorini) drains superior portion of head and empties separately into 2nd portion of duodenum
  • 6. • Has rich periacinar networks that drain into 5 nodal groups: – Superior nodes – Anterior nodes – Inferior nodes – Posterior PD nodes – Splenic nodes
  • 7. • Pancreatic acini + duct system. • Produces digestive enzymes (amylase, trypsin, lipase) and alkaline fluid. Exocrine Parts Endocrine Parts • Islets of Langerhans (pancreatic islets) • Regulator of glucose, lipid, protein homeostasis.
  • 8. • cells at central – Produce insulin • cells peripheral – Produce glucagon • cells peripheral – Produce somatostatin
  • 9. • The openings of two ducts carried into line with each other. • Two parts of the gland fuse to form one pancreas. • Dorsal pancreatic duct becomes the accessory pancreatic duct. • Duct of ventral bud forms the main pancreatic duct. • Terminal parts of branching ducts form pancreatic acini.
  • 10. • Highest mortality rateAgenesis • Most commonPancreas Divisum Annular Pancreas Ectopic Pancreas Congenital Cysts
  • 11. • Characterized by: – absence of a critical mass of pancreatic tissue or, in rare cases, totally absent. • Cause: – Genetic mutation of PDX1 gene, located on chromosomal locus 13q12.1 • Normal pancreatic development requires pancreatic duodenal homeobox 1, a homeodomain transcription factor. • Severity depends on amount of functional pancreatic tissue present. PANCREAS WITH ABSENT BODY AND TAIL
  • 12. Associated disease: • Pancreaticobiliary duct anomalies – can lead to acute/chronic pancreatitis Symptoms: • Abdominal pain • Diabetes (dorsal pancreas agenesis) • Lipid-rich stool (caused by defect of pancreatic enzymes) Exam and Tests: • Abdominal ultrasound • Abdominal CT scan Management: • treatment of the diabetes and exocrine deficiency, when present. • Infusion of subcutaneous insulin, administration of pancreatic enzyme. Prognosis: • Variable, depending on the quality of treatment received.
  • 13. • The duct systems of the fetal pancreatic primordia fail to fuse. • most common birth defect. • The cause of the defect is unknown. • Many cases go undetected. • Pathogenesis: – The ventral bud failed to fuse with dorsal bud during fetal development.
  • 14. Clinical features: • Abdominal pain and swelling, nausea or vomiting. • Note: symptoms absent without pancreatitis Exams and Tests: • Abdominal ultrasound • Abdominal CT scan • Amylase and lipase blood test Treatment: • Surgery. Complication: • Pancreatitis Prognosis: • The outcome is usually good.
  • 15. • A ring of pancreatic tissue that completely encircles the duodenum. • Pathogenesis: – May occur when the ventral bud splits (become bifid), the two segments may encircle the duodenum .
  • 16. • Symptoms:  Newborns  spit up more than normal, do not drink enough milk  Adult  fullness after eating, nausea or vomiting • Symptoms occur when the ring of pancreas squeezes and narrows the small intestine. • Conditions that may be associated with annular pancreas include: – Down syndrome – Excess amniotic fluid during pregnancy (polyhydramnios) – Other congenital gastrointestinal problems – Peritonitis
  • 17. Exams and Tests: • Abdominal ultrasound • Abdominal x-ray Treatment: • Surgical bypass of the blocked part of the duodenum. Prognosis: • Good outcome with surgery. Complications: • Obstructive jaundice • Pancreatic cancer • Peptic ulcer • Perforation (tearing a hole) of the intestine due to obstruction
  • 18. • also known as heterotopic pancreatic tissue. • condition where pancreatic tissues lie outside and separate to pancreatic gland. • Most are completely asymptomatic. • Common sites: – Duodenum – Jejunum – Ileum – Meckel’s Diverticulum (remnant of yolk sac)
  • 19. Symptoms: • Usually non-specific • Abnominal pain & dyspepsia Diagnosis: • GI endoscopy Complication: • Intestinal obstruction • Carcinoma of the ectopic pancreatic tissue Treatment: • Surgery if symptomatic
  • 20. • Results from anomalous development of the pancreatic ducts. • Morphology: – unilocular cysts, range from 5cm in diameter. – Lined by either uniform cuboidal or flattened epithelium and are enclosed in a thin, fibrous capsule. – Contains clear serous fluid – important point from pancreatic cystic neoplasms (which is mucinous)
  • 21. Symptoms: • Abdominal swelling • Palpable mass • Vomit Exams and Tests: • Ultrasonography Management: • Surgery
  • 22. • There are five congenital diseases, agenesis, pancreas divisum, annular pancreas, ectopic pancreas, and congenital cysts. • The most common is pancreas divisum. • Agenesis has the highest mortality rate. • All treatments are via surgery except in agenesis, insulin infusion (and other pancreatic secretion components) is needed. • Polyhydramnios can occur in annular pancreas cases. • Cancer mostly occurs in the head of pancreas.
  • 23. • Textbook of Robbins Basic Pathology • Textbook of Gray’s Anatomy • www.healthline.com • www.medscape.com
  • 24. 1 2 3 4 5 1. The following are congenital diseases of pancreas A. Congenital cysts B. Pancreatic divisum C. Heterotopic pancreatic tissue D. Agenesis E. Annular pancreas TTTTT C. Other name for Ectopic Pancreas
  • 25. 2. Pancreas divisum is the most common congenital disease. T/F 1 2 3 4 5 T
  • 26. 3. Annular pancreas is the incomplete fusion of the ducts of pancreas during fetal development. T/F 1 2 3 4 5 F Pancreas divisum incomplete fusion Annular pancreas encircling duodenum
  • 27. 4. Congenital cysts can be managed by cystectomy. T/F 1 2 3 4 5 T
  • 28. 5. Common sites for ectopic pancreas are A. Jejunum B. Meckel’s diverticulum C. Caecum D. Rectum E. Ileum 1 2 3 4 5 TTFFT
  • 29. THANK YOU

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