Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice in childhood

Abstract

Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

Idiopathic fibrosing pancreatitis in a 3-year-old girl: a case report and review of the literature

“…The presentation of our patient with abdominal pain and obstructive jaundice was similar to the presentation of 25 (54%) of the reported cases of this disorder [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]. Most of the cases reported (36/46, 78%) presented with abdominal pain, discomfort, or cramping, with or without jaundice.…”

“…Advanced fibrosis, ductal dilatation, relative sparing of islets Alvear and Petro [23] Dilated CBD, narrowing at ampulla of Vater Not reported Alwmark et al [24] Enlarged pancreas, partially soft and hard Fibrinoid degeneration, edema, numerous inflammatory cells Holcomb et al [2] Firm, nodular pancreas Not reported Meneely et al [4] Hard, nodular, fibrotic, shrunken pancreas Not reported Ghishan et al [5] 2 patients: hard, thickened pancreas Not reported 4 patients: fibrotic, hard head of pancreas Isaacs et al [17] Chronic pancreatitis Not reported Atkinson et al [6] 1 patient: brock hardQ pancreas Dense fibrosis surrounding intact acini 1 patient: btumor massQ in pancreatic head Wheatly and Coran [7] 1 patient: fibrotic hard pancreas Inflammation and fibrosis consistent with chronic pancreatitis in both 1 patient: not reported Elitsur et al [8] Enlarged, firm, smooth, pancreas Fibrous replacement of pancreatic tissue, islets preserved Choi et al [9] Diffusely enlarged pancreas, rubbery, hard Fibrosis, intact exocrine acini and islets Buchta and Bell [10] bRock hardQ pancreas. Dilated CBD and gallbladder Chronic inflammation, severe fibrosis Little et al [25] Not reported Not reported Stephen et al [11] Enlarged, hard pancreas; liver green and smooth Diffuse fibrosis and inflammation including acini Barkin et al [3] Nodular hard pancreas in both cases 1 diffuse fibrosis with relative sparing of acini 1 not reported Amerson and Ricketts [12] 2 patients: hard mass at head of pancreas 4 chronic fibrosing pancreatitis 2 patients: diffuse fibrosis of pancreas Lewin-Smith et al [13] Enlarged, indurated, fibrotic pancreas.…”

Idiopathic fibrosing pancreatitis in children—a single-center experience

“…Hence, it was difficult to differentiate choledochal cysts from IFP and also to rule out a mass at the lower end of the bile duct. This diagnostic problem has been previously documented [8]. The specific diagnostic accuracy of these tests is poor (Table 2).…”

Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children

“…Radiological imaging typically demonstrates a pancreatic mass or enlargement with dilatation of the intra and extra hepatic biliary tree where jaundice is present (72% of previously reported cases [6][7][8]). Histology shows fibrosis of the pancreas with relative preservation of acinar structure and no evidence of auto digestion [8][9][10]. In two of our cases histology was obtained and both cases showed interstitial fibrosis, parenchymal loss, intralobular fibrosis and a chronic inflammatory cell infiltrate (Fig.…”

“…In many patients this has been accompanied by a definitive drainage procedure. The latter includes: cholecystduodenostomy [8][9][10], choledochoduodenostomy [6,[8][9][10][11], choledochojejunostomy [8,9,12] and cholecystjejunostomy [8,13]. More minor procedures undertaken at the time of exploratory surgery include sphinterotomy [14,15] and transduodenal sphincteroplasty [14,16].…”