Management of fibrosing pancreatitis in children presenting with obstructive jaundice

Abstract

Background-Children with fibrosing pancreatitis are conventionally treated surgically to relieve common bile duct (CBD) obstruction caused by pancreatic compression. Residual pancreatic function has not been formally tested in these patients. Aims-To evaluate the usefulness of nonsurgical temporary drainage in children with fibrosing pancreatitis and to assess pancreatic function after resolution of their CBD obstruction. Patients-Four children (1.5-13 years; three girls).Methods and results-Abdominal sonography and computed tomography revealed diVuse enlargement of the pancreas, predominantly the head. The CBD was dilated due to compression by the head of the pancreas. Pancreatic biopsy specimens obtained in three patients showed notable acinar cell atrophy and extensive fibrosis. Cystic fibrosis was excluded. No other cause of pancreatitis was identified. Pancreatic tissue from one patient contained viral DNA sequences for parvovirus B19 detected by polymerase chain reaction; serum IgM to parvovirus was positive. Three patients had temporary drainage of the CBD and one patient underwent a choledochojejunostomy. Serial imaging studies revealed resolution of the CBD obstruction with reduction in pancreatic size. Exocrine pancreatic function deteriorated. Three patients developed pancreatic insuYciency within two to four months of presentation. The fourth patient has notably diminished pancreatic function, but remains pancreatic suYcient. None has diabetes mellitus. Conclusions-Temporary drainage of the CBD obstruction is recommended in fibrosing pancreatitis in children along with close monitoring of the clinical course, before considering surgery. (Gut 1998;43:715-720)

Idiopathic fibrosing pancreatitis and spontaneous resolution of pancreatic masses in children

“…In the former report [18] three of four patients (aged 1.5-13 years) underwent temporary stenting, with resolution of jaundice allowing stent removal at 2-4 months without further complications, at 1.5 to 3.5 years follow up [18], a situation parallelling our 3rd case. Another report [7] performed endoscopic drainage in one patient, again, with subsequent removal without complications.…”

“…In a smaller number of patients stenting (with or without biopsy) has been carried out either surgically [18], percutaneously [18] or endoscopically [7,8,18,19]. In the former report [18] three of four patients (aged 1.5-13 years) underwent temporary stenting, with resolution of jaundice allowing stent removal at 2-4 months without further complications, at 1.5 to 3.5 years follow up [18], a situation parallelling our 3rd case.…”

“…Modern imaging allows detailed assessment and the possibility of percutaneous/transduodenal sampling [7] may even avoid laparotomy and biopsy where anxiety about possible malignancy is high. The management of the jaundice may be expectant although temporary drainage either percutaneous or endoscopic, may allow relief of troublesome symptoms [7,18,19].…”

Unusual causes of extrahepatic biliary obstruction in children: a case series with review of literature

“…Cases 4 and 5 in the current series belonged to the non-calcific category and the reported etiologies for this form in the literature are congenital or acquired lesions of pancreatic duct, trauma, sphincter of Oddi dysfunction, and idiopathic fibrosing chronic pancreatitis. While series on adult patients with chronic pancreatitis report a 20% incidence of associated biliary obstruction, the same is infrequently reported in the pediatric age group [6].…”

“…Only 50 cases have been described in literature so far [7]. The following are the features of this entity as described in the literature: (1) non-calcific pancreatitis, (2) age at presentation ranging from 4 months to 20 years (mean 10 years), (3) focal head involvement (4) obstructive jaundice, often accompanied with painful abdomen is the leading symptom (70%) although painless jaundice is also seen in a minority (17%) [8], (5) clinical evidence of exocrine or endocrine dysfunction at initial presentation is rare although many patients on long-term follow-up develop steatorrhea [6], (6) histology reveals acinar atrophy with extensive interstitial fibrosis with relative sparing of islets of Langerhans, and (7) specific etiologies of chronic pancreatitis have been excluded. A diagnosis of ''idiopathic fibrosing pancreatitis'' was entertained for cases 4 and 5 in this study on the basis of the same criteria.…”