Idiopathic fibrosing pancreatitis in children—a single-center experience

The experience of a single institution on idiopathic fibrosing pancreatitis (IFP) is presented. This is a retrospective review of medical records of affected patients. There were 7 cases with a mean age of 7 years. Upper abdominal pain followed by jaundice was the most common presentation. One child...

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Bibliographic Details
Published in:Journal of pediatric surgery 2006-12, Vol.41 (12), p.1987-1991
Main Authors: Deshpande, Aniruddh V., LaHei, Erik R., Shun, Albert, Martin, Hugh C.O., O'Loughlin, Edward V.
Format: Article
Language:English
Subjects:
Adolescent
Child
Female
Fibrosis
Humans
Jaundice, Obstructive - etiology
Jaundice, Obstructive - surgery
Male
Pancreas - pathology
Pancreatitis, Chronic - complications
Pancreatitis, Chronic - pathology
Portoenterostomy, Hepatic
Retrospective Studies
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Summary:The experience of a single institution on idiopathic fibrosing pancreatitis (IFP) is presented. This is a retrospective review of medical records of affected patients. 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). 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.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2006.08.033