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Idiopathic fibrosing pancreatitis in children--a single-center experience
Aniruddh V Deshpande1, Erik R LaHei, Albert Shun
1Department of General Surgery, The Children's Hospital at Westmead, Westmead, Sydney N.S.W. 2145, Australia. sushrudh@hotmail.com
Insights
Idiopathic fibrosing pancreatitis (IFP) in children often presents with biliary obstruction. Surgical intervention provides effective long-term relief, though precise preoperative diagnosis remains challenging.
Area of Science:
- Pediatric Gastroenterology
- Hepatobiliary Surgery
- Pancreatology
Background:
- Idiopathic fibrosing pancreatitis (IFP) is a rare condition.
- It primarily affects children and presents with biliary obstruction.
Purpose of the Study:
- To present the experience of a single institution with idiopathic fibrosing pancreatitis.
- To evaluate diagnostic methods and treatment outcomes.
Main Methods:
- Retrospective review of medical records for 7 pediatric patients diagnosed with IFP.
- Analysis of clinical presentation, diagnostic imaging, surgical, and endoscopic treatments.
Main Results:
- The most common presentation was upper abdominal pain followed by jaundice.
- Preoperative diagnosis was difficult, with limited sensitivity of noninvasive imaging (ultrasonography, CT, MRCP).
- Surgical biliary enteric bypass was performed in 6 patients, with one successful endoscopic biliary stenting; no postoperative complications occurred.
Conclusions:
- IFP in children typically manifests as biliary obstruction, with challenging preoperative diagnosis.
- Surgery offers satisfactory long-term relief of biliary obstruction.
- Endoscopic biliary drainage shows promise; long-term follow-up is crucial for pancreatic insufficiency, recurrent biliary issues, and potential inflammatory bowel disease.
Purpose:
The experience of a single institution on idiopathic fibrosing pancreatitis (IFP) is presented.
Methodology:
This is a retrospective review of medical records of affected patients.
Results:
There were 7 cases with a mean age of 7 years. Upper abdominal pain followed by jaundice was the most common presentation. One child had varicella and 1 developed Crohn's disease 3 years later. In 5 cases, diagnosis was established intraoperatively, whereas 2 cases were diagnosed preoperatively. Ultrasonography suggested the diagnosis in 2 of the 7 cases, contrast computed tomography scan in 1 of the 3 cases, and magnetic resonance cholangiopancreatography in 1 of the 4 cases. Six patients were treated by biliary enteric bypass surgery. Treatment by endoscopic biliary stenting was successful in one. There were no postoperative complications. Pancreatic biopsies showed fibrosis of exocrine elements with preservation of islets. Three patients have pancreatic atrophy, and none has diabetes at follow-up (mean, 62 months).
Discussion:
Idiopathic fibrosing pancreatitis presents as biliary obstruction in children. Precise preoperative diagnosis of IFP is difficult. Noninvasive imaging has limited sensitivity. Surgery offers satisfactory long-term relief of biliary obstruction. Treatment using temporary endoscopic biliary drainage appears promising in treatment of IFP. Patients should be followed up for pancreatic insufficiency, long-term biliary obstruction, and inflammatory bowel disease.
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