Chronic Pancreatitis: The Puestow Procedure A 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. 1 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. 2 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=cfvmo6frb80rwlan5 1856xrdt#/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%C E%B5%CE%B1%CF%82.gif Systemic Pancreatitis part of multi-organ disease Hypertriglyceridemia SLE Cystic fibrosis IgG4- related pancreatitis 3 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 gene SPINK1 – serine protease inhibitor, Kazal type1 CTRC – chymotrypsin C CFTR – cystic fibrosis transmembrane conductance regulator *Mutations in any of these genes may result in pancreatitis 4 Chronic pancreatitis No consensus of exact definition In general, diagnosed when patient with any type of sustained pancreatic damage has anatomical changes in pancreas on imaging *calcifications, atrophy, ductal dilation Chronic pancreatitis: Clinical features Abdominal pain Pancreatic Insufficiency: Exocrine Endocrine Etiology of pain: Pancreatic Duct Dilation Elevated pressure Pseudocyst formation Stasis of pancreatic fluid 5 Pancreatic Duct Dilation Primary obstruction Acquired obstruction – chronic inflammation, scarring, and fibrosis Right hepatic duct Pancreatic duct The pancreatic duct should be SMALLER than 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). 6 Chronic Pancreatitis: Implications Chronic pain issues Narcotic dependence Malnutrition Pancreatic Enzyme Deficiency Insulin Dependent Diabetes Potential for malignancy later in life Chronic pancreatitis: Work up Labs -CBC, Amylase, Lipase, LFT’s, CMP Imaging - Ultrasound, MRCP, CT Genetic work up Sweat test Upper endoscopy ERCP Chronic Pancreatitis: Medical management Analgesia – strive for non narcotic Enzyme 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 and possibly decrease pancreatic intraductal pressure Nutritional support 7 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 diets NG/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 identified Pancreatic duct is filleted open its entire dilated length Any stones that are present are removed 35 cm Roux-en-Y jejunal limb is created End to side jejunostomy is performed Pancreaticojejunostomy is then performed 8 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 9 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 endoscopy Negative sweat test Negative outpatient ultrasounds Case Presentation Episodes severe, progressed to lethargy with bilious emesis Admitted to OSH Labs notable for lipase 294, amylase 177 LFT’s normal WBC 19.1 U/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 pain CHOP ED for deterioration in clinical picture At this time patient was also now having diarrhea 10 Case Presentation In the ED she was voluntarily guarding with diffuse abdominal tenderness, obvious dehydration Labs: Amylase 440, Lipase 3114 U/S: Heterogeneous mass in pancreas, suggestive of atypical early pseudocyst Fluid resuscitation, IV Nubain Admission to GI floor Case presentation MRCP 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 sent NG 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 11 Review of repeat MRCP Pancreatitis, small 1 cm intraluminal soft tissue defect next to duodenal ampulla No associated dilation of CBD Some dilation of pancreatic duct in mid portion of pancreas, maximal diameter 2.2mm Question 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 admission 3rd MRCP shows changes now progressing to prominence in CBD and narrowing as it courses through pancreatic head Pancreatic duct tortuous and dilated with small stones Pseudocyst 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 12 What Next? 4 hospital admissions, +PRSS 1 inability to drain pancreas via ERCP mutation Surgery Persistence of Elevation of labs & inflammation on evidence of pancreatic imaging with ductal insufficiency changes Pre-operative work up Up to date history and PE Anesthesia evaluation Factor V Leiden labs CBC w/ diff, T&C, 1 unit of blood Arrangements made for Intra-op ultrasound - RUQ 13 Day of Surgery Child life Versed in OR holding area Intra-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 minutes PICU post op Post op management NPO NG to low wall suction Epidural catheter with ropivicaine infusion Morphine and nubain PRN Toradol started on POD #1 Foley Catheter Zosyn post op prophylaxis TPN started on POD # 1 140 ml PRBC’s Post op management Return of bowel function POD # 3 NG removed Clear liquid diet started NGT tube feeds started 1/3 goal volume JP drain left in place while feeds were advanced 14 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 bound POD# 14 Current clinical status Free of abdominal pain Only moving bowels 2-3 times per day No nausea or vomiting No longer requiring NG feeds Loves burgers, pasta, chicken, peanut butter Socially she is not as fearful, more interactive Now attending preschool Amylase – 47, Lipase – 17 15 Growth Chart Life is good…… 16.