title: Modified Puestow procedure for the management of chronic pancreatitis in children.
abstract: OBJECTIVE
  To present our experience with the modified Puestow procedure in the management of children with chronic pancreatitis.
  
  
  METHODS
  Retrospective chart review of patients treated between 2003 and 2012.
  
  
  RESULTS
  Six patients underwent a modified Puestow procedure (lateral pancreaticojejunostomy) for the management of chronic pancreatitis, three females and three males. Four patients had hereditary pancreatitis (three with confirmed N34S mutation in the SPINK1 gene), one patient had chronic pancreatitis of unknown etiology, and one patient with annular pancreas developed obstructive chronic pancreatitis. The pancreatic duct was dilated in all cases, with a maximum diameter of 5 to 10mm. Median time between onset of pain and surgery was 4 years (range: 1-9). Median age at surgery was 7.5 years (range: 5-15). Median hospital stay was 12 days (range: 9-28). Median follow up was 4.5 years (range: 5 months to 9 years). All patients had temporary postoperative improvement of their abdominal pain. In two patients the pain recurred at 6 months and 2 years postoperatively and eventually required total pancreatectomy to treat intractable pain, 3 and 8 years after surgery. Two patients were pain free for two years and subsequently developed occasional episodes of pain. The two most recent patients are pain free at 1 year (obstructive chronic pancreatitis) and 5 months (hereditary pancreatitis) follow-up. Two patients developed type I diabetes mellitus 10 and 12 months postoperatively (one with hereditary and one with idiopathic chronic pancreatitis).
  
  
  CONCLUSIONS
  We conclude that the modified Puestow procedure in children is feasible and safe. It seems to provide definitive pain control and prevent further damage to the pancreas in patients with obstructive chronic pancreatitis. However, in patients with hereditary pancreatitis, pain control outcomes are variable and the operation may not abrogate the progression of disease to pancreatic insufficiency.
authors.affiliation: Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Pablo
authors.initials: P
authors.lastname: Laje
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000293:Adolescent; D002352:Carrier Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003922:Diabetes Mellitus, Type 1 / Q000209:etiology; D018450:Disease Progression; D004380:Duodenal Obstruction / Q000601:surgery; D010188:Exocrine Pancreatic Insufficiency / Q000209:etiology; D005240:Feasibility Studies; D005260:Female; D006801:Humans; D007409:Intestinal Atresia; D007902:Length of Stay / Q000706:statistics & numerical data; D008297:Male; D059408:Pain Management; D010179:Pancreas / Q000002:abnormalities; D010180:Pancreatectomy; D010183:Pancreatic Ducts / Q000473:pathology / Q000601:surgery; D010193:Pancreaticojejunostomy / Q000379:methods*; D050500:Pancreatitis, Chronic / Q000150:complications / Q000235:genetics / Q000601:surgery*; D011183:Postoperative Complications / Q000209:etiology / Q000601:surgery; D012074:Remission Induction; D012189:Retrospective Studies; D016896:Treatment Outcome; D014359:Trypsin Inhibitor, Kazal Pancreatic; D014361:Trypsin Inhibitors / Q000235:genetics
chemical_list: D002352:Carrier Proteins; C486642:SPINK1 protein, human; D014361:Trypsin Inhibitors; D014359:Trypsin Inhibitor, Kazal Pancreatic
keywords: Chronic pancreatitis; Modified Puestow procedure