Annular pancreas

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ORPHA:675OMIM:167750Q45.1
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18Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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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:

Annular pancreasHP:0001734High intestinal obstructionHP:0005250Duodenal stenosisHP:0100867
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Annular pancreas.

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No actively recruiting trials found for Annular pancreas at this time.

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Specialists

18 foundView all specialists →
MK
Monika Konarska-Włosińska
Specialist
1 Annular pancreas publication
PO
Patryk Ostrowski
Specialist
1 Annular pancreas publication
MB
Michał Bonczar
Specialist
1 Annular pancreas publication
WM
Weronika Michalik
Specialist
1 Annular pancreas publication
VC
Vikram Chaudhari
Specialist
2 Annular pancreas publications
SS
Shailesh V Shrikhande
Specialist
2 Annular pancreas publications
MB
Manish S Bhandare
Specialist
2 Annular pancreas publications
CY
Chaima Yakoubi
Specialist
1 Annular pancreas publication
SA
Souhaib Atri
Specialist
1 Annular pancreas publication
HM
Houcine Maghrebi
Specialist
1 Annular pancreas publication
MK
Montasser Jameleddine Kacem
Specialist
1 Annular pancreas publication
HA
Haithem Hussein Ali Almoamin
Specialist
1 Annular pancreas publication
SK
Sadik Hassan Kadhem
Specialist
1 Annular pancreas publication
AS
Ansam Mahmood Saleh
Specialist
1 Annular pancreas publication
DP
Dawid Plutecki
Specialist
1 Annular pancreas publication
HK
Hedy Kindler
CHICAGO, IL
Specialist
PI on 7 active trials6 Annular pancreas publications
LM
Lauren Henke, M.D.
SAINT LOUIS, MO
Specialist
PI on 1 active trial
MM
Manabu Kawai, MD
Specialist
PI on 2 active trials

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Annular pancreas.

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Community

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Latest news about Annular pancreas

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Caregiver Resources

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

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.