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Updated: Aug 2, 2026
Collection Protocol for Human Pancreas
Published on: May 23, 2012
Pancreas divisum in a family with hereditary pancreatitis
A R Muzaffar1, M S Moyer, J Dobbins
1Department of Pediatrics, Pediatric Gastroenterology and Hepatology, Yale University School of Medicine, New Haven, Connecticut, USA.
Insights
Hereditary pancreatitis may be linked to pancreas divisum, a rare congenital condition where pancreatic ducts don't fuse. This family study suggests a potential genetic link, impacting clinical significance and treatment discussions.
Area of Science:
- Gastroenterology
- Genetics
- Pediatric Medicine
Background:
- Hereditary pancreatitis presents with early-onset symptoms and autosomal-dominant inheritance.
- Pancreas divisum is a congenital anomaly where pancreatic ducts fail to fuse, potentially causing pancreatitis.
Observation:
- A family presented with recurrent pancreatitis across three generations.
- The mother and son showed documented pancreas divisum via ERCP.
- The daughter had a distal pancreatic duct stricture.
Findings:
- This is the first reported case of familial pancreas divisum.
- The findings suggest a potential genetic association between pancreas divisum and hereditary pancreatitis.
Implications:
- Highlights the controversial clinical significance of pancreas divisum.
- Raises questions about the appropriateness of surgical intervention for pancreas divisum.
- Emphasizes the need for further research into the genetic basis of pancreatitis.
Abstract:
Hereditary pancreatitis is characterized by an autosomal-dominant mode of inheritance with incomplete penetrance, onset of symptoms in childhood or early adolescence (mean age of onset approximately 13 years), and an approximately equal sex incidence. Pancreas divisum is a congenital variant of pancreatic ductal anatomy in which the ventral and dorsal pancreatic ductal systems fail to fuse, so that two functional papillae drain the exocrine secretions of the pancreas. In recent years, several reports of pancreatitis associated with pancreas divisum in children have appeared. We now report a family in which the mother, son, and daughter all had presented with recurrent pancreatitis from an early age. Both the mother and son have endoscopic retrograde cholangiopancreatography-documented pancreas divisum, whereas the daughter has a stricture in her distal pancreatic duct. To our knowledge, this is the first such report of "familial" pancreas divisum. The implications of these findings in the setting of hereditary pancreatitis highlight the controversial issues of the clinical significance of pancreas divisum and the appropriateness of surgical therapy.
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