Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children

Abstract

A symptomatic pancreatic mass poses both a diagnostic and therapeutic conundrum. First, benign conditions may mimic malignancy and second, the management of benign lesions has not been well defined. We reviewed four such cases and discuss the management of juvenile idiopathic fibrosing pancreatitis in the light of these. In four children with symptomatic pancreatic masses/enlargement (three with jaundice) all resolved spontaneously without definitive intervention. Histopathology, obtained in two cases, revealed a fibrotic chronic inflammatory reaction. Pancreatic masses in children may not be malignant. Resolution of symptoms including obstructive jaundice and a mass secondary to juvenile idiopathic fibrosing pancreatitis may occur spontaneously, avoiding the need for resection or definitive drainage procedures.

Autoimmune pancreatitis: an adolescent case and review of literature

“…Another entity, idiopathic fibrosing pancreatitis, unlike AIP, usually presents with jaundice because of obstruction and abdominal pain; however, the pathologic hallmark is fibrosis rather than lymphoplasmocytic infiltration as is the case in AIP. There are no reports associating idiopathic fibrosing pancreatitis to autoimmune diseases, although some AIP cases are also without such association [18].…”

Ursodeoxycholic acid's role in avoiding diversionary surgery in idiopathic fibrosing pancreatitis

“…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 4,5,7 . As there is no evidence that surgical biliary diversion influences the natural history of IFP, it should be avoided until absolutely necessary.…”

“…It typically presents with obstructive jaundice, often accompanied with abdominal pain, although painless jaundice is seen in a minority (17%) 3 . There are now only 58 cases of IFP reported in the literature, and there is no specific test to diagnose this rare condition 1–8 …”

A refugee with vomiting and weight loss

“…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. 4,5,7 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.…”

“…However, symptomatic management in patients with cholestatic jaundice can be a challenge, and temporary drainage may allow some relief of troublesome symptoms. 7 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.…”