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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……