The Role of Ductal Anomalies in the Progression of Hereditary Pancreatitis in Children 563

Abstract

Three pediatric age siblings with hereditary pancreatitis required therapeutic intervention for intractable pain resulting in malnutrition. One had recurrent pseudocysts. The first patient, a 6-year-old girl was status post biliary sphincterotomy and pancreaticojejunostomy. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated narrowing proximally and distally within the remaining pancreatic duct. A stent was placed across the pancreatic duct. This relieved her abdominal pain. Serial stents were placed at variable intervals and then removed. The patient experienced prolonged symptom-free intervals, but required additional periods of serial stent placement for 2 recurrences of abdominal pain. Her brother developed pancreatic pseudocysts at 2 years of age. ERCP showed pancreas divisum and multiple pseudocysts. He was treated with external drainage and sphincterotomy. The pseudocysts resolved and he did well. At 5 years of age, he had recurrence of pseudocysts. ERCP with cannulation of the minor papilla demonstrated a large pseudocyst communicating with an irregular pancreatic duct. A stent was placed across the minor papilla and past the origin of the pseudocyst. The pseudocyst resolved within 1 week. The stent was replaced twice and then removed. The patient has been free of abdominal pain with no recurrence of pseudocysts for more than one year. The last patient had ERCP performed at 6 years of age for reoccurence of abdominal pain that demonstrated a dilated pancreatic duct, which bifurcated and ended abruptly in the body of the pancreas. At 9 years of age, she developed intractable abdominal pain. Repeat ERCP showed total occlusion of the dilated pancreatic duct in the head of the pancreas. Biliary sphincterotomy did not relieve her symptoms. She subsequently underwent a 90% subtotal pancreatectomy. The body and tail of the pancreas were shrunken, nodular, and calcified. The patient has remained well in 6 months of follow-up. Conclusions: Ductal anomalies may play a role in symptomatology and possibly progression of disease in hereditary pancreatitis in children. Endoscopically-placed stents are effective therapy for hereditary pancreatitis. Persistent relief of symptoms may be due to dilating strictures within the pancreatic duct. Pancreas divisum may contribute to disease when present in patients with hereditary pancreatitis.