Overview
Annular pancreas is a rare congenital anomaly in which a ring or collar of pancreatic tissue surrounds the second part of the duodenum (the first section of the small intestine just beyond the stomach). This abnormal band of pancreatic tissue can partially or completely obstruct the duodenum, interfering with the normal passage of food and digestive contents. The condition results from a failure of normal embryological rotation and fusion of the ventral pancreatic bud during fetal development. Annular pancreas primarily affects the gastrointestinal system. In neonates, it typically presents with signs of duodenal obstruction, including vomiting (often bilious), feeding intolerance, abdominal distension, and failure to pass meconium. A characteristic "double bubble" sign may be seen on prenatal ultrasound or postnatal abdominal X-ray, indicating duodenal obstruction. In some cases, the condition may remain asymptomatic until adulthood, when patients may develop symptoms such as postprandial abdominal pain, nausea, vomiting, peptic ulcer disease, or pancreatitis. Annular pancreas is frequently associated with other congenital anomalies, including Down syndrome (trisomy 21), intestinal malrotation, esophageal atresia, congenital heart defects, and other forms of duodenal atresia or stenosis. Treatment is surgical and is indicated when the annular pancreas causes symptomatic obstruction. The standard surgical approach involves a bypass procedure, most commonly a duodenoduodenostomy or duodenojejunostomy, which creates an alternative pathway for intestinal contents to flow around the obstruction. Division of the pancreatic ring itself is avoided due to the high risk of pancreatic fistula and injury to the pancreatic duct. Prognosis after surgical correction is generally excellent, particularly in the absence of other significant congenital anomalies.
Clinical phenotype terms:
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Annular pancreas.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Annular pancreas.
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Common questions about Annular pancreas
What is Annular pancreas?
Annular pancreas is a rare congenital anomaly in which a ring or collar of pancreatic tissue surrounds the second part of the duodenum (the first section of the small intestine just beyond the stomach). This abnormal band of pancreatic tissue can partially or completely obstruct the duodenum, interfering with the normal passage of food and digestive contents. The condition results from a failure of normal embryological rotation and fusion of the ventral pancreatic bud during fetal development. Annular pancreas primarily affects the gastrointestinal system. In neonates, it typically presents w
How is Annular pancreas inherited?
Annular pancreas follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Which specialists treat Annular pancreas?
18 specialists and care centers treating Annular pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.