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The modified Puestow procedure for complicated hereditary pancreatitis in children
1Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City 52242, USA.
Insights
The modified Puestow procedure significantly improves quality of life for children with hereditary pancreatitis by enhancing pancreatic function and reducing hospitalizations. Direct duct localization offers lower morbidity compared to distal pancreatectomy.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Hereditary Pancreatitis Research
Background:
- Hereditary pancreatitis (HP) is a severe genetic disorder often presenting in childhood with significant morbidity.
- Complicated HP requires effective surgical interventions to manage symptoms and improve long-term outcomes.
- The modified Puestow procedure is a surgical option for managing complex cases of HP.
Purpose of the Study:
- To evaluate the efficacy of the modified Puestow procedure in treating complicated hereditary pancreatitis in pediatric patients.
- To assess the impact of the procedure on surgical outcomes, pancreatic function, and nutritional status.
- To compare the morbidity associated with different duct localization techniques during the procedure.
Main Methods:
- Retrospective review of 12 pediatric patients (<18 years) undergoing the modified Puestow procedure for complicated HP (1973-1998).
- Analysis of surgical morbidity, mortality, pre- and postoperative pancreatic function (steatorrhea), hospitalization rates, and ideal body weight (IBW) percentile.
- Comparison of outcomes between direct transpancreatic duct localization and distal pancreatectomy for duct localization.
Main Results:
- No surgical mortality was observed. Postoperative steatorrhea resolved in 4 of 5 patients.
- Hospitalizations for pancreatitis decreased significantly post-surgery (0.4 vs. 3.5 per 5 years; P=.01).
- Ideal body weight percentile showed a significant increase by the third postoperative year (P=.01). Direct duct localization had lower blood loss and fewer complications than distal pancreatectomy.
Conclusions:
- The modified Puestow procedure effectively improves quality of life in children with complicated HP.
- Key benefits include improved pancreatic function, reduced hospitalizations, and increased ideal body weight.
- Direct pancreatic duct localization is associated with lower morbidity, and early surgical intervention may preserve pancreatic function.
Purpose:
The aim of this study was to evaluate the role of longitudinal pancreaticojejunostomy (modified Puestow procedure) in the treatment of complicated hereditary pancreatitis (HP) in children.
Methods:
The authors reviewed their experience with the modified Puestow procedure for complicated HP in patients less than 18 years of age at a single tertiary care facility between 1973 and 1998. Main study outcomes included surgical morbidity and mortality, pre- and postoperative pancreatic function, number of hospitalizations, and percentile ideal body weight (IBW).
Results:
Twelve patients (6 boys and 6 girls) with a mean age of 9.3 years were identified. Presenting diagnoses were abdominal pain (n = 10), failure to thrive (n = 4), pancreatic pleural effusion (n = 2), and pancreatic ascites (n = 1). Blood loss was greater in patients who underwent distal pancreatectomy to localize the duct (n = 6) than in those who underwent direct transpancreatic duct localization (n = 6; 29.1+/-6.8 v. 8.3+/-3.7 mL/kg; P = .03). Other complications in patients who underwent distal pancreatectomy included splenic devascularization requiring splenectomy (n = 1) and postoperative intraabdominal bleeding with subsequent left subphrenic abscess (n = 1). There was no surgical mortality. Five patients had steatorrhea preoperatively that resolved in 4 patients postoperatively and was well controlled in the fifth. Mean number of hospitalizations for pancreatitis in the 5 years after surgery were markedly less than in the 5 years preceding surgery (0.4+/-0.2 v. 3.5+/-0.5; P = .01, n = 9). Percentile ideal body weight tended to increase within the first postoperative year (24.6+/-6.8 v. 45.0+/-8.3; P = .07, n = 9), and by the third year this trend was clearly significant (27.0+/-7.2 v. 60.9+/-9.5; P = .01, n = 8).
Conclusions:
In children with complicated HP, the modified Puestow procedure improves the quality of life by improving pancreatic function, decreasing hospitalizations, and increasing the percentile ideal body weight. Direct pancreatic duct localization during the procedure had a lower morbidity rate than localization via distal pancreatectomy. It is our impression that surgery performed in the early stage of complicated disease may preserve pancreatic function.