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Chronic Pancreatitis: The Puestow ProcedureA case presentation
Gina Kroeplin, MSN, CRNP Children’s Hospital of Philadelphia
Division of General, Thoracic, & Fetal Surgery
Disclosure Information
No Disclosures
Objectives
• The learner will demonstrate understanding of the etiologies of chronic pancreatitis and the long term implications.
• The learner will have an understanding why surgical intervention is necessary and be familiar with labs and diagnostics done prior to surgery.
• The learner will have an understanding of the Puestow Procedure and post-operative care.
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Function of pancreas:Dual Purpose
Endocrine gland – produces several hormones including insulin, glucagon, somatostatin, and pancreatic polypeptide which circulate in the blood.
Digestive organ - secretes pancreatic juice containing digestive enzymes that assist digestion and absorption of nutrients in the small intestine. The enzymes further breakdown carbohydrates, proteins and lipids in the chyme.
Pancreas Landmarks
Van Dyke Carter, H. (1918). [Anatomy of the Human Body]. Retrieved from URL
https://commons.wikimedia.org/wiki/Category:Pancreatic_duct#/media/File:Gray_1100_Pancreatic_duct.png
Pancreas Landmarks
Blausen.com staff. (2014). [Medical gallery of Blausen Medical 2014]. Retrieved from URL WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436. .
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Chronic Pancreatitis
Etiological Factors
Obstructive Systemic Toxic
Ductal Obstructive Pancreatitis
Pancreas divisum with obstructive papillae
Choledochal Cyst
Annular Pancreas
Trauma related obstructions
Cradel, A. (2015). 1) normal pancreas 2) pancreas divisum. Retrieved from https://commons.wikimedia.org/w/index.php?search=pancreas+divisum&title=Special:Search&profile=default&fulltext=1&searchToken=cfvmo6frb80rwlan51856xrdt#/media/File:%CE%94%CE%B9%CF%83%CF%87%CE%B9%CE%B4%CE%AD%CF%82_%CF%80%CE%AC%CE%B3%CE%BA%CF%81%CE%B5%CE%B1%CF%82.gif
Systemic
Pancreatitis part of multi-organ disease
HypertriglyceridemiaSLECystic fibrosisIgG4- related pancreatitis
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Toxic
Caused by certain drugs
Most frequently ethanol
More common in adult population
Elham, A. (2014). [Pancreapedia: Exocrine Pancreas Knowledge Base]. Retrieved from DOI:10.15347/wjm/2014.010. ISSN 2002-4436.
Hereditary Pancreatitis
Genetic disease of autosomal dominant inheritance
Intrinsic process of pancreatic auto-digestion based on mutations in variety of genes that codify different components of pancreatic fluid
Pancreatitis Genetics
PRSS1 – cationic trypsinogen geneSPINK1 – serine protease inhibitor, Kazal type1CTRC – chymotrypsin CCFTR – cystic fibrosis transmembrane conductance regulator
*Mutations in any of these genes may result in pancreatitis
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Chronic pancreatitis
No consensus of exact definitionIn general, diagnosed when patient with any type of sustained
pancreatic damage has anatomical changes in pancreas onimaging
*calcifications, atrophy, ductal dilation
Chronic pancreatitis:Clinical features
Abdominal pain
Pancreatic Insufficiency:
ExocrineEndocrine
Etiology of pain:Pancreatic Duct Dilation
Elevated pressure
Pseudocystformation
Stasis of pancreatic
fluid
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Pancreatic Duct Dilation
Primary obstruction
Acquired obstruction – chronic inflammation, scarring, and fibrosis
Pancreatic duct
Right hepatic duct
The pancreatic duct should be SMALLERthan the right hepatic duct.
The pancreatic duct (arrow) should be invisible on a CT scan.
The pancreatic duct gets dilated because there is an obstruction to the flow. In some cases the pressure is so high that the duct ruptures, like in this case. The leak of pancreatic fluid forms a pseudocyst(star).
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Chronic Pancreatitis:Implications
Chronic pain issuesNarcotic dependenceMalnutritionPancreatic Enzyme DeficiencyInsulin Dependent DiabetesPotential for malignancy later in life
Chronic pancreatitis:Work up
Labs-CBC, Amylase, Lipase, LFT’s, CMP
Imaging - Ultrasound, MRCP, CT
Genetic work upSweat test Upper endoscopyERCP
Chronic Pancreatitis:Medical management
Analgesia – strive for non narcoticEnzyme therapy –(Viokase) Not much data
- An effort to control diarrhea/steatorrhea- Helps to achieve adequate weight gain- Used in combination with acid suppression to help inhibit pancreatic secretion andpossibly decrease pancreatic intraductalpressure
Nutritional support
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Chronic Pancreatitis:Nutritional Support
Malnutrition is result of inappropriate digestion and malabsorption of fats, carbohydrates, and protein
Important to correct malabsorption High protein, high calorie dietsNG/NJ enteral feeds TPN
Chronic pancreatitis:Surgical Management
Goal: alleviate pain and preserve exocrine and endocrine functions of the pancreas
Decompression of pancreatic duct by surgical technique has been performed since beginning of 20th century.
Lateral pancreaticojejunosotomy – described by Puestow
Puestow Procedure
Abdomen entered via transverse supraumbilical or Chevron incision
Dilated main pancreatic duct identifiedPancreatic duct is filleted open its entire dilated
lengthAny stones that are present are removed35 cm Roux-en-Y jejunal limb is createdEnd to side jejunostomy is performedPancreaticojejunostomy is then performed
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The bowel is on the pancreas, and the pancreatic duct is open.
Roux en Y
Puestow Procedure:Long-term outcomes
Depend largely on etiology of chronic pancreatitis and whether causative factors persist after surgery
- Obstructive: likely to have definitive relief as long as pancreatic duct remains decompressed
- Toxic (seen with adults): likely to have definitive relief unless patient begins to consume alcohol again
- Hereditary pancreatitis: intrinsic cause of disease does not appear after operation, most patients improve pain score, however long-term results are variable
Case Presentation
26 month old female presents with a 1 year history of episodic abdominal pain.
Episodes last 3-5 days, severe at times, associated with anorexia
Nothing would make the pain better
Episodes occurring more frequently
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Case presentation
Pain has been followed by PCP and felt to be functional abdominal pain
Began seeing outside GI practice in July 2013
Normal upper endoscopyNegative sweat testNegative outpatient ultrasounds
Case Presentation
Episodes severe, progressed to lethargy with bilious emesisAdmitted to OSH Labs notable for lipase 294, amylase 177LFT’s normalWBC 19.1U/S – normal biliary architecture, but could not visualize pancreas
well
Case Presentation
After fluid bolus, and advancement of diet patient was discharged home with instructions to encourage eating/drinking
While at home she continued to vomit and have abdominal painCHOP ED for deterioration in clinical pictureAt this time patient was also now having diarrhea
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Case Presentation
In the ED she was voluntarily guarding with diffuse abdominal tenderness, obvious dehydration
Labs: Amylase 440, Lipase 3114U/S: Heterogeneous mass in pancreas, suggestive of atypical early
pseudocystFluid resuscitation, IV NubainAdmission to GI floor
Case presentationMRCP
No pancreatic mass is identified. Diffuse enlargement of the pancreas with enhancement and signal
characteristics of pancreatitis. Surrounding peripancreatic inflammatory change and ascites also
present. No pseudocyst is noted
Hereditary pancreatitis labs sentNG enteral feeds initiated for nutritional support (Vivonex)
Case presentation
L.D. readmitted 2 weeks after discharge with recurrence of symptoms
Hereditary pancreatitis +PRSS1 Gene
NJ tube placement
Persistently high amylase/lipase
Worsening diarrhea; pancreatic enzymes started in addition to MCT oil
General Surgery consulted
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Review of repeat MRCP
Pancreatitis, small 1 cm intraluminal soft tissue defect next to duodenal ampulla
No associated dilation of CBDSome dilation of pancreatic duct in mid portion of pancreas, maximal
diameter 2.2mmQuestion raised: could defect be small choledochocele? Unlikely in absence of biliary ductal dilation, most likely secondary to
edema
Case Presentation
Another bout of pancreatitis and subsequent hospital admission3rd MRCP shows changes now progressing to prominence in CBD
and narrowing as it courses through pancreatic headPancreatic duct tortuous and dilated with small stonesPseudocyst present
Case Presentation
Diagnostic and therapeutic attempts via ERCP were not successful in cannulating the pancreatic duct
Congenital loop ductal malformation of pancreatic head vs ductal changes due to inflammatory related phenomenon
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What Next?
4 hospital admissions, inability to drain
pancreas via ERCP+PRSS 1 mutation
Persistence of inflammation on
imaging with ductal changes
Elevation of labs & evidence of pancreatic
insufficiency
Surgery
Pre-operative work up
Up to date history and PE Anesthesia evaluationFactor V Leiden labsCBC w/ diff, T&C, 1 unit of blood Arrangements made for Intra-op ultrasound - RUQ
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Day of Surgery
Child lifeVersed in OR holding areaIntra-op U/S performed for
localization of normal anatomic structures
Left subclavian double lumen catheter placement
Jackson Pratt placed via RLQ abdominal wall
Case length: 6 hours 15 minutesPICU post op
Post op management
NPONG to low wall suction Epidural catheter with ropivicaine infusionMorphine and nubain PRNToradol started on POD #1 Foley CatheterZosyn post op prophylaxisTPN started on POD # 1140 ml PRBC’s
Post op management
Return of bowel function POD # 3NG removedClear liquid diet startedNGT tube feeds started 1/3 goal
volumeJP drain left in place while feeds
were advanced
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Post op management
POD # 11 L.D. developed fevers
Cefepime started 48hr prophylaxis as per protocol
UA, CXR, Blood Cx sent, all normal
U/S done- no fluid collections appreciated
Fevers resolved after 48 hours
Central line and JP removed on POD # 13
Homeward boundPOD# 14
Current clinical status
Free of abdominal painOnly moving bowels 2-3 times per dayNo nausea or vomitingNo longer requiring NG feedsLoves burgers, pasta, chicken, peanut butterSocially she is not as fearful, more interactiveNow attending preschoolAmylase – 47, Lipase – 17
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Growth Chart
Life is good……