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Therapeutic step-up strategy for management of hereditary pancreatitis in children
S Kargl1, M Kienbauer2, H C Duba3
1Department of Pediatric Surgery, Women's and Children's Hospital Linz.
Insights
A step-up treatment plan using early endoscopic retrograde cholangiopancreatography (ERCP) and surgery effectively manages hereditary pancreatitis in children, preventing recurrence and ensuring a normal quality of life.
Area of Science:
- Pediatric Gastroenterology
- Hereditary Pancreatitis Treatment
- Minimally Invasive Surgery
Background:
- Hereditary pancreatitis in childhood presents diverse treatment challenges.
- Current therapeutic options require a structured approach.
Purpose of the Study:
- To propose and evaluate a therapeutic pathway for childhood hereditary pancreatitis.
- Emphasis on integrating endoscopic and surgical interventions.
Main Methods:
- Prospective inclusion of 12 pediatric patients with hereditary pancreatitis (2006-2013).
- Implementation of a therapeutic step-up schema involving diagnostic work-up and interventions.
- Evaluation of treatment outcomes correlated with etiological factors and pathoanatomic findings.
Main Results:
- Early endoscopic retrograde cholangiopancreatography (ERCP) was successful in 10 of 12 patients.
- Eight patients with obstructive pancreatitis required sphincterotomy, dilation, and stenting.
- Open surgical drainage was performed for two patients with unsuccessful ERCP.
- After a mean 32-month follow-up, all patients were free of pancreatitis recurrence.
Conclusions:
- A step-plan approach with early ERCP is effective for hereditary pancreatitis in children.
- Open surgical drainage is a viable option for cases with impossible or insufficient endoscopic treatment.
- This strategy prevents recurrent pancreatitis and ensures a normal quality of life with minimal complications.
Background/Purpose:
Various different regimes exist for the treatment of hereditary pancreatitis in childhood. Here, we propose a therapeutic pathway with emphasis on endoscopic and surgical procedures.
Methods:
From 2006 to 2013, 12 patients with a diagnosis of hereditary pancreatitis were prospectively included in a therapeutic step-up schema. The treatment outcome was evaluated and correlated to aetiological factors and pathoanatomic findings.
Results:
After diagnostic work-up (laboratory data, ultrasound examination, magnetic resonance cholangiopancreatography and genetic testing), all 12 patients underwent early endoscopic retrograde cholangiopancreatography (ERCP), which was successfully performed in ten children. Obstructive pancreatitis was found in eight children, and required sphincterotomy, dilation and stenting for 12 months. In two children with unsuccessful ERCP, open surgical drainage procedures were performed. After a mean follow-up of 32 months all children are free of recurrence of pancreatitis without any impairment of everyday activities.
Conclusions:
For children with hereditary pancreatitis, a therapeutic step plan with early ERCP and open surgical drainage procedures in case of impossible or insufficient endoscopic treatment prevents recurring pancreatitis and offers a normal quality of life without any major complications.
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