pathology in the pancreas responsible for the dilatation of the biliary tree has mostly led to surgical exploration and diversion in the past. Palpation of a ‘rock hard’ pancreas during laparotomy is an important diagnostic feature of IFP. Histology characteristically demonstrates acinar atrophy with extensive interstitial fibrosis with relative sparing of the pancreatic islets. Treatment modality has previously been divided between aggressive treatment with diversionary procedures and symptomatic, expectant management. In 2002, Sclabas regarded surgical intervention as mandatory. Diversionary procedures have included percutaneous transhepatic biliary drainage, endoscopic biliary stenting, stricture excision and Roux-en-Y with hepaticojejunostomy or longitudinal pancreaticojejunostomy. Interestingly, more recent case reports have shown no difference in outcome between those treated with early diversion and those treated expectedly, supporting the theory that IFP is a self-limiting, transient phenomenon. As there is no evidence that surgical biliary diversion influences the natural history of IFP, it should be avoided until absolutely necessary. However, symptomatic management in patients with cholestatic jaundice can be a challenge, and temporary drainage may allow some relief of troublesome symptoms. For the first time in the literature, UDCA was used to treat symptomatic cholestasis in IFP. Because of the dramatic improvement in cholestasis, we were able to continue our investigations without hastily committing to a surgical solution; no diversionary procedures were undertaken. Thus, we describe UDCA as a valuable drug in the management of IFP, with its dramatic effect thought to be because of its effect on reducing biliary viscosity and subsequent obstruction to flow. Our case is also indicative of the difficulty found in differentiating benign and malignant causes of obstructive jaundice. The diagnosis of IFP is one of exclusion (of congenital and acquired pancreatitis), and if investigations are ultimately inconclusive and unable to allay clinician anxiety, these patients must have tissue sampling to exclude malignancy. While there is evidence that IFP tends to follow a benign, self-limited course, there have been long-term complications reported. Cases have been documented where late onset steatorrhoea has developed and required pancreatic enzyme replacement. Diabetes mellitus has also been reported, although long-term follow-up is limited. Furthermore, inflammatory bowel disease has been reported years after the initial presentation with IFP, and the possible role of parvovirus B19 has also been suggested. Long-term follow-up of these patients may yet reveal more clues as to its aetiopathology.
[1]
M. Ashworth,et al.
Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children
,
2007,
Pediatric Surgery International.
[2]
A. Deshpande,et al.
Idiopathic fibrosing pancreatitis in children--a single-center experience.
,
2006,
Journal of pediatric surgery.
[3]
M. Stringer,et al.
Successful conservative management of idiopathic fibrosing pancreatitis in children
,
2006,
European Journal of Pediatrics.
[4]
H. Naon,et al.
Idiopathic fibrosing pancreatitis in a 3-year-old girl: a case report and review of the literature.
,
2005,
Journal of pediatric surgery.
[5]
A. Burroughs,et al.
Idiopathic fibrosing pancreatitis and Crohn's disease: an interesting association.
,
2000,
European journal of gastroenterology & hepatology.
[6]
B. Shuckett,et al.
Management of fibrosing pancreatitis in children presenting with obstructive jaundice
,
1998,
Gut.
[7]
M. Büchler,et al.
Juvenile idiopathic fibrosing pancreatitis.
,
2002,
Digestive diseases and sciences.
[8]
I. F. Volini,et al.
Chronic relapsing pancreatitis.
,
1949,
Quarterly bulletin. Northwestern University (Evanston, Ill.). Medical School.