2. How to manage pancreatic pseudocysts: a dilemma of choices

3.  Embryology: dorsal and ventral buds from foregut, parenchyma from splanchnic mesoderm, rotation, & fusion  Blood Supply: Celiac & SMA  Innervation: Vagal and Splanchnic  Function:  Exocrine – alkaline ‘juice’ (CCK, secretin, PNS) & proteolytic enzymes (zymogen granules)  Endocrine – insulin (beta) & glucagon (alpha)

7.  History: 2 y/o boy with palpable abdominal mass, 2weeks s/p appendectomy (11/11)  PMH: PDA  PSH: ligation in China  Labs: CBC & BMP nl  Additional information?  Afebrile  Hx of trauma  Early satiety PHYSICAL EXAM

10.  Amylase: 1251 (11/26)  1497 (11/27)  Lipase: 6935 (11/26)  8292 (11/27)  *Low Sn and Sp?  Herman and colleagues looked at 131 pediatric trauma cases with pancreatic injury and noted “Neither intitial nor maximal amylase/lipase has any predictive utility for grade of injury, or length of stay, or outcomes in a child with pancreatic trauma. However, maximal amylase level greater than 1100 U/L was predictive for developing a pseudocyst.”

13.  1992 Atlanta Symposium: Fluid collection >4 weeks old surrounded by a defined wall  “Maturing collection of pancreatic juice surrounded by reactive granulation tissue”  Remember: pseudocyst = nonepithelialized capsule  With “maturation,” pseudocyst capsule becomes a fibrotic rind

14.  Acute or chronic pancreatitis  Ductal obstruction (stricture, stone, etc)  Trauma (blunt, penetrating)  Common Thread?  Ductal disruption  Leakage of pancreatic enzymes, gland autolysis  Inflammation  +/- pancreatic necrosis  Unlike in adults, leading cause of pancreatitis & thus also pancreatic pseudocyts in kids is trauma. (Paul, Teh, Sharma, etc.)  “Pseudocysts can develop in 27%-56% of patients with blunt injury who are managed nonoperatively.” (Fisher) Other sources report 0-69% (Saad).

15.  Medical Management: NPO + TPN + NGT  Indications for drainage I:  Increasing size (>5cm)  Infection  Gastrointestinal obstruction  Bleeding  Rupture  Symptomatic persistence (> 6 weeks) (Lawrence)  Indications for drainage II:  Persistant abdominal pain, nausea, vomiting, FTT (Teh)

16.  1761: Morgagni – first description of a pancreatic pseudocyst  1875: LeDentu – percutaneous drainage of post- traumatic pseudocyst  1882: Bozeman – open removal pseudocyst  1921: 1 st cystgastrostomy  1928: 1 st cystduodenostomy  1931: 1 st cystjejunostomy  Late 70s/early 80s: percutaneous drainage  1989: endoscopic drainage described in adults  1994: laparoscopic cystgastrostomy  1999: endoscopic drainage described in peds

17. SurgeryEndoscopicPercutaneous CystgastrostomyEndoscopic cystgastrostomy Percutaneous drain (IR) Cystenterostomy (direct) Endoscopic cystduodenostomy Cystenterostomy (Roux limb) +/- EUS Distal pancreatectomy+/- needle localization Also: Watchful waiting? Laparoscopy?

18.  Historically, surgery has been the gold standard for treating pancreatic pseudocysts  Reports of associated morbidity and mortality as high as 25% and 5%, respectively  Percutaneous drains also carry high risks, with infection rates up to 50% and increased risk of pancreatic fistula  Endoscopic interventions carry a relatively high risk of bleeding, retroperitoneal perforation, infection, and failure to achieve resolution of the cyst cavity.

19.  Pediatric Literature is limited  Limitations  Retrospective  Not randomized  Inconsistent follow up  Lack of standard reporting  Extremely low power

20.  1988 study looking at 7 children with post- traumatic pancreatic pseudocysts managed with percutaneous drainage  No report of any infections or trouble with fistula formation.  Avg days inpatient ~ 4 weeks  No mention of ultimate follow up

21.  1990 study from The Hospital for Sick Children, Burnweit et al looked at the “Percutanteous Drainage of Traumatic Pseudocysts in Children”  N = 13, 1984-1988  Resolved w/ medical management: 6 children  Operative intervention: 2 children  Percutaneous intervention: 5 children  No adverse events, no infections, no fistulas  Followed 1 mo s/p discharge

22.  1991 study out of North Carolina looking at percutaneous management in adults  Retrospective, spanning 27 years  Group 1: 1965-91 w/ operative intervention, n= 42  Group 2: 1982-91 w/ percutaneous drainage, n= 52  Major complications:  7 pts in Group 1 vs. 4 pts in Group 2  Need for further operations:  4 pts in Group 1 vs. 10 in Group 2

25.  1998 study out of Emory  Retrospective review of medical records, n = 96  Meeting Atlanta Criteria, dx by CT  Operative (n=32) vs. Percutaneous management (n = 27), group assignment determined by cyst size and location  Results:  Operative group  2/32 with failure of resolution, need for further debridement  2/32 with abscess formation  Percutaneous drainage group  17/27 showed resolution  7/27 had significant clinical deterioration or 2 nd infection, leading to urgent debridement or cystgastrostomy  1 death

26.  2006 study out of Mayo  Retrospective review looking at different management strategies of PP & their affects on outcomes in children  N=24, Jan 1976-Dec 2003  All managed conservatively initially (avg 13.1wk)  7 w/ symptom resolution (29%)  17 w/ need for intervention ▪ 13 w/ surgical intervention (cystogastrostomy (8), cystojejunostomy (2), lateral longitudinal pancreaticojejunostomy (2), Frey’s procedure (1)) ▪ 4 w/ endoscopic/radiologic intervention  Only indicative factor for surgical intervention was etiology of PP  5 of 11 patients with trauma-induced PP underwent surgical intervention  12 of 13 patients with non-trauma-induced PP underwent surgical intervention  Diseased vs. Non-diseased parenchyma?

27.  Of the operative intervention…  all patients experienced resolution of their symptoms  no mortality & no recurrence (73.3 mo follow up)  morbidity in 2 patients (11.1%): 1 wound infection & 1 pancreatic leak  Of the endoscopic interventions…  1 had choledocolithiasis, 1 developed an infection  Factors significant in indicating need for intervention: non-trauma (vs. trauma)

28.  2008 study from India looking at long term outcomes (up to 10y) in pediatric patients who underwent endoscopic management of pseudocyst  N = 9, 8 with cystogastrostomy + stenting and 1 with cystoduodenostomy + stenting  100% resolution of symptoms  Did not report significant morbidity and mortality  ERCP in 2 patients only, before drainage  Observational study? No randomization, no control, no comparison.

30. SurgeryEndscopicPercutaneous Avoid risk for superinfection Avoid major operation Avoid risk for fistula Specimen available for histology Less risk of exocrine insufficiency, pancreaticogenic diabetes, or splenectomy (with distal pancreatectomy) Operator dependentRisk of fistula Risk of infection

31.  2005 study looking at laparoscopic cystgastrostomy in children  2 case reports, reporting success with laparoscopic approach to pseudocyst drainage.  Will this be our middle ground?

32.  Fisher J, Kuenzler K, Bodenstein L, and Chabot J. Central pancreatectomy with pancreaticogastrostomy. Journal of Pediatric Surgery (2007) 42, 740-746.  Herman et al. Utility of amylase and lipase as predictors of grade of injury or outcomes in pediatric patients with pancreatic trauma. Journal of Pediatric Surgery (2011) 46, 923-926.  Iqbal et al. Management of chronic pancreatitis in the pediatric patient: endoscopic retrograde cholangiopancreatography vs. operative therapy. Journal of Pediatric Surgery (2009) 44, 139-143.  Lautz T, Chin A, and Radhakrishman J. Acute Pancreatitis in children: spectrum of disease and predictors of severity. Journal of Pediatric Surgery (2011) 46, 1144- 1149.  Paul M and Mooney D. The management of pancreatic injuries in children: operate or observe. Journal of Pediatric Surgery (2011) 46, 1140-1143.  Sharma S and Maharshi S. Endoscopic management of pancreatic pseudocyst in children—a long term follow up. Journal of Pediatric Surgery (2008) 43, 1636- 1639.  The et al. Pancreatic pseudocyst in children: the impact of management strategies on outcome. Journal of Pediatric Surgery (2006) 41, 1889-1893.

33.  Cannon et al. Diagnosis and Management of Pancreatic Pseudocysts, Collective Review. Vol 209; 3, September 2009.  Bergman S and Melvin S. Operative and Non-operative Managent of pancreatic pseudocysts. Surgical Clinics of North America (2007), 87, 1447-1460.  Burnweit et al. Percutaneous Drainage of Traumatic Pancreatic Pseudocysts in Children. The Journal of Trauma Vol 30; 10, October 1990.  Jaffe et al. Percutaneous Drainage of Traumatic Pancreatic Pseudocysts in Children. AJR: 152, March 1989  Medical Embryology (Langman).  Handbook of Pediatric Surgery (Sinha, Davenport).  Pediatric Surgery Secrets (Glick).  Essentials of General Surgery (Lawrence).  Access Surgery.  UpToDate.

34. Thank you.

37.  4 th week of gestation  Dorsal and ventral buds from endodermal lining of foregut (dorsal from duo, ventral from liver)  Rotaion & fusion of ducts (2/2 differentiation and gut rotation), by 7 th week  Surrounding splanchnic mesoderm forms parenchyma  Ventral pancreas  caudial portion of head + uncinate process  Ventral duct (& distal dorsal duct)  duct of Wirsung  Dorsal pancreas  cranial portion of head + body + tail  Dorsal duct  proximal dorsal duct