Idiopathic Fibrosing Pancreatitis Presenting with Obstructive Jaundice in a Child

Abstract

Idiopathic fibrosing pancreatitis (IFP) is a rare cause of obstructive jaundice in children. The obstruction is caused by circumferential compression of the intrapancreatic portion of the common bile duct (CBD). Various forms of biliary decompression, including surgical sphincteroplasty, choledochoduodenostomy, and Roux-en-Y choledochojejunostomy are employed as a treatment. We report a 14-year-old boy with IFP who was successfully treated by a temporary drainage of compressed CBD with the help of a stent placed by endoscopic retrograde cholangiopancreatography (ERCP). The diagnosis of IFP was confirmed histologically after open surgery. The patient remains well after 3.5 years of follow-up with no evidence of pancreatic insufficiency. This report offers an additional case of IFP causing obstructive jaundice treated by temporary internal drainage of CBD, which is safer and less invasive than biliodigestive surgical decompression.

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.…”

“…Most of the cases reported (36/46, 78%) presented with abdominal pain, discomfort, or cramping, with or without jaundice. Painless jaundice was a presenting symptom in a minority of the cases (8/46, 17%) reported [2][3][4][5]14,16,17]. Other reported symptoms include weight loss, nausea, vomiting, dark urine, pale stools, and fatigue/lethargy .…”

“…In one case reported by Amerson and Ricketts [12], a stent was placed but the patient had to be taken to the operating room for exploration and surgical diversion. Sylvester et al [14] reported successful decompression of the CBD in 2 of 4 patients with stent placement (one placed percutaneously and the other endoscopically), and Keil et al [16] were also able to decompress the CBD with an endoscopically placed stent. Their patient still had to undergo surgery to biopsy the pancreas and make a definitive diagnosis.…”

“…Endoscopic retrograde choledocho-pancreatography was performed in most cases for diagnosis and was followed by surgery for biopsy and biliary-enteric diversion. In 5 cases [12,[14][15][16], a stent was placed endoscopically to relieve the obstruction. In one case reported by Amerson and Ricketts [12], a stent was placed but the patient had to be taken to the operating room for exploration and surgical diversion.…”

Successful conservative management of idiopathic fibrosing pancreatitis in children

“…Since the eventual outcome was not significantly different, they recommended that CBD stenting, as opposed to major surgical intervention, was sufficient to treat this condition. Another recent report reached a similar conclusion [7].…”