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home - Pancreas - Other - Pancreatic Minutiae Written by Dr Sebastian Zeki

Pancreatic Minutiae

Heterotrophic Pancreas-anywhere in GI tract (75 % in stomach or oesophagus) -very occasionally can get CA pancreas -On endoscopy: submucosal nodule central depression Agenesis/ Hypoplasia of the Pancreas -Agenesis: -Causes total endocrine and exocrine insufficiency -May be caused by intra-uterine infection -Hypoplasia: - Is lipomatous pseudo hypertrophy of the pancreas Pancreatic Minutiae Shwachman-Diamond syndrome Annular pancreasRare congenital abnormality characterized by a ring of pancreatic tissue surrounding descending portion of duodenum.Due to incomplete rotation of pancreatic ventral bud. Symptomatic from any age; 2/3rds asymptomatic for lifeIf symptomatic >2/3 neonates with features of gastric outlet obstruction.Infant associationMaternal polyhydramnios Congenital abnormalities (Down's syndrome, esophageal/duodenal atresia, imperforate anus, Meckel's diverticulum).Adults (usually between 20-50yrs): Gastric outlet obstructionUpper GI bleeding (from peptic ulceration)Acute or chronic pancreatitis (due to fibrotic partial obstruction of pancreatic duct esp in head of pancreas)Biliary obstruction (rare) Pancreas divisum Aetiology: Non-fusion of Santorini duct and Wirsung duct so most drainage is via accessory duct Epdidemiology: 7% of population (most common pancreatic congenital anomaly) Clin Pres: 95% are asymptomatic Small proportion get recurrent pancreatitis due to remediable papillary narrowing. Diagnosis: ERCP/MRCP However, a finding of a dilated dorsal duct or isolated changes of chronic pancreat - tis in the dorsal system (ie, with a normal ventral duct) suggests pathologic narrowing of the minor papilla. Treatment: Usually conservative as unclear whether pancreas divisum causing symptoms The greatest likelihood of a response occurs in patients with recurrent episodes of apparently idiopathic acute pancreatitis. We generally evaluate the dorsal pancreas and perform minor papilla therapy in patients having two or more bouts of pancreatitis or one bout of severe pancreati - tis- sphincterotomy +/- stent Treatment of minor papilla narrowing in pancreas divisum has traditionally been approached surgically.However, similar results are being obtained in experienced centers with endoscopic approaches. AR Shwachman-Bodian-Diamond syndrome gene - Skeletal abnormalities - Short stature - Dental dysplasia- Learning and behavioral difficulties- Hepatomegaly and inc ALT, early in life - Exocrine pancreatic insufficiency - Bone marrow failure with neutropenia is the most frequent abnormality, although anemia and thrombo-cytopenia also may occur. -A subset of heterozygously-affected patients can develop aplastic anemia -Cytopenia can be intermittent.- Increased risk for developing myelodysplastic syndrome and/or acute myelogenous leukemia DiagnosisThe diagnosis of Shwachman-Diamond Syndrome is based upon clinical criteria that demonstrate exocrine pancreatic insufficiency and bone marrow failure. Clinical Presentation: Any medium that shows gastric outlet obstruction-ERCP/ EUS/ MRCP may have a role to for HPB obstruction Written by Dr Sebastian Zeki Epidemiology- Clin Pres: Classification:Type I (the most common form) in which the annular duct flows directly into the main pancreatic ductType II (the second most common variant) in which the duct of Wirsung encircles the duodenum but still drains at the major papilla.The other four subtypes are much less common. Treatment:-Bypass annulus (duodenoduodenostomy (for neonates), gastrojejunostomy, or a duodenojejunostomy (latter 2 for adults))-Resection of the annulus leads to complications (pancreatitis, pancreatic fistula formation, and incomplete relief of obstruction) so avoidIf obstructive jaundice, biliary bypass with choledochoenterostomy/ internal stent recommended.If pancreatolithiasis or periampullary lesions esp.suspected malignancy, pancreaticoduodenectomy. Management- Hematopoietic stem cells transplantation (HSCT) is the only potential current curative treatment for bone marrow failure in patients with Shwachman-Diamond Syndrome, but data are limited regarding patient outcome of this intervention.In patients with Shwachman-Diamond Syndrome, HSCT can be considered for the treatment of acute myelogenous leukemia. 1:10,000 Diagnosis