Idiopathic fibrosing pancreatitis in children--a single-center experience.
OBJECTIVE
The experience of a single institution on idiopathic fibrosing pancreatitis (IFP) is presented.


METHODS
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).


CONCLUSIONS
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.
Deshpande
Aniruddh V
AV
Department of General Surgery, The Children's Hospital at Westmead, Westmead, Sydney N.S.W. 2145, Australia. sushrudh@hotmail.com
LaHei
Erik R
ER
Shun
Albert
A
Martin
Hugh C O
HC
O'Loughlin
Edward V
EV
D000293:Adolescent; D002648:Child; D005260:Female; D005355:Fibrosis; D006801:Humans; D041781:Jaundice, Obstructive / Q000209:etiology / Q000601:surgery*; D008297:Male; D010179:Pancreas / Q000473:pathology*; D050500:Pancreatitis, Chronic / Q000150:complications* / Q000473:pathology; D011171:Portoenterostomy, Hepatic; D012189:Retrospective Studies