ICD-10
Specialty
Onset
Reviewed
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OverviewOverview
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Etiology & Risk FactorsEtiology & Risk Factors
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PathophysiologyPathophysiology
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Clinical PresentationClinical Presentation
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History TakingHistory Taking
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Physical ExaminationPhysical Examination
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InvestigationsInvestigations
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DiagnosisDiagnosis
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ManagementManagement
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ComplicationsComplications
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PrognosisPrognosis
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Key Points / Clinical PearlsKey Points / Clinical Pearls
Overview
- May be complete or incomplete depending on how much of the duodenum is encircled.
- Many individuals are asymptomatic, while others present in infancy or adulthood.
- Symptoms are mainly due to duodenal obstruction and may include feeding difficulties and vomiting in neonates, or abdominal pain, nausea, vomiting, postprandial fullness, and pancreatitis in adults.
- Asymptomatic patients usually require no treatment, whereas symptomatic cases are typically managed with surgical bypass procedures (e.g., duodenoduodenostomy).
Etiology & Risk Factors
Etiology
- Congenital condition caused by defective migration and malrotation of the ventral pancreatic bud during embryonic development.
- This abnormal development forms a complete or partial ring of pancreatic tissue around the second part of the duodenum.
Risk Factors / Associated Conditions
- Annular pancreas is commonly associated with:
- Down syndrome
- Congenital heart defects
- Intestinal malrotation
- Duodenal atresia
- Meckel diverticulum
- There are no known acquired risk factors because annular pancreas is a congenital embryologic anomaly.
Pathophysiology
Defective migration and malrotation of the ventral pancreatic bud (embryonic development) → Ring of pancreatic tissue forms around the second part of the duodenum → Compression/narrowing of the duodenum → Partial or complete duodenal obstruction → Impaired passage of gastric contents → Vomiting, feeding difficulty, abdominal distention (or abdominal pain/postprandial fullness in adults) → May also lead to pancreatitis and biliary obstruction
Clinical Presentation
Many patients are asymptomatic. Symptoms depend on the age at presentation.
Prenatal
- Polyhydramnios
Neonates
- Feeding difficulties
- Abdominal distention
- Nonbilious or bilious vomiting
Adults
- Abdominal pain
- Nausea and/or vomiting
- Postprandial fullness
- Symptoms of pancreatitis
- Peptic ulcer disease
- Jaundice (uncommon)
History Taking
Ask about:
- When did the symptoms start?
- Is there vomiting? Is it bilious or nonbilious?
- Does vomiting occur after feeding or meals?
- Is there feeding difficulty or poor weight gain? (infants)
- Is there abdominal pain? Is it related to meals?
- Do you feel full quickly after eating (postprandial fullness)?
- Have you had recurrent episodes of pancreatitis?
- Have you noticed yellowing of the eyes/skin (jaundice)?
- Is there a history of Down syndrome or other congenital anomalies?
Physical Examination
- General appearance: May appear well if asymptomatic.
- Signs of dehydration: Dry mucous membranes, sunken eyes, tachycardia (from persistent vomiting).
- Abdominal distention: Especially in neonates with duodenal obstruction.
- Epigastric tenderness: If associated with acute pancreatitis.
- Visible vomiting (bilious or nonbilious), especially after feeds in infants.
- Poor weight gain/failure to thrive: In infants with chronic obstruction.
- Jaundice: May be present if there is biliary obstruction (uncommon).
- Signs of malnutrition: In prolonged or severe cases.
Investigations
Laboratory tests
- Usually not diagnostic
- Serum amylase/lipase if pancreatitis is suspected
- Liver function tests (bilirubin, ALP) if jaundice or biliary obstruction is suspected
Imaging (mainstay of diagnosis)
- Prenatal ultrasound: Polyhydramnios and features of duodenal obstruction
- Abdominal X-ray: Double bubble sign (duodenal obstruction)
- CT scan or MRI (best diagnostic imaging):
- Pancreatic tissue completely or partially encircling the second part of the duodenum
- Duodenal narrowing/thickening
- Proximal duodenal dilatation
diagnostic finding
- Ring (complete or partial) of pancreatic tissue surrounding the second part of the duodenum on cross-sectional imaging.
Diagnosis
-Diagnosis is based on :
- Diagnosis is based on clinical suspicion plus imaging findings.
Diagnostic criteria
- Symptoms of duodenal obstruction (or incidental finding in asymptomatic patients)
Cross-sectional imaging (CT or MRI) showing:
- Complete or partial ring of pancreatic tissue surrounding the second part of the duodenum (diagnostic finding)
- Duodenal narrowing/thickening
- Proximal duodenal dilatation
- Prenatal ultrasound or abdominal X-ray may support the diagnosis by showing a double bubble sign (duodenal obstruction).
Related Topics
Management
1. Initial/Supportive Management
- Keep NPO (nil by mouth) if there is significant obstruction.
- IV fluids to correct dehydration and electrolyte imbalance.
- Nasogastric (NG) tube for gastric decompression if persistent vomiting or obstruction.
- Treat associated complications (e.g., acute pancreatitis or biliary obstruction) as indicated.
2. Definitive Management
- Asymptomatic patients: No treatment required; observation and follow-up.
- Symptomatic patients: Surgical intervention is recommended.
3. Surgery (Treatment of Choice)
- Duodenoduodenostomy (preferred bypass procedure)
- Other bypass procedures may be used if needed.
- Do not resect the annular pancreatic tissue, as this increases the risk of pancreatic injury and complications.
Complications
- Duodenal obstruction (most common)
- Acute pancreatitis
- Chronic pancreatitis
- Biliary obstruction (may cause jaundice)
- Peptic ulcer disease
- The main complications result from compression of the duodenum and nearby biliary/pancreatic ducts.
Prognosis
- Excellent prognosis in asymptomatic patients; no treatment is usually needed.
- Good prognosis after surgical bypass (e.g., duodenoduodenostomy), with relief of duodenal obstruction in most patients.
- Prognosis depends on the severity of obstruction and the presence of complications (e.g., pancreatitis or biliary obstruction).
- Early diagnosis and treatment generally lead to favorable outcomes.
Key Points / Clinical Pearls
- Rare congenital anomaly with pancreatic tissue surrounding the second part of the duodenum.
- Caused by abnormal migration of the ventral pancreatic bud.
- Presents with duodenal obstruction or may be asymptomatic.
- CT or MRI confirms the diagnosis.
- Asymptomatic: Observation only.
- Symptomatic: Duodenoduodenostomy (bypass surgery) is the treatment of choice.
- Main complications: Duodenal obstruction, pancreatitis, and biliary obstruction.
- Aleem A, Desai H, Shah H. National Center for Biotechnology Information (NIH). Annular Pancreas, StatPearls.
- Vlad C, Todor C, Iancu G, et al. Clinical Analysis of Congenital Duodenal Obstruction and the Role of Annular Pancreas. Diagnostics (Basel). PMC11857210.
- Annular Pancreas Presenting with Intermittent Duodenal Obstruction in Early Childhood: A Diagnostic Masquerade. Reports. PMC12821695.
- MedlinePlus, National Library of Medicine (NIH). Annular Pancreas: Medical Encyclopedia.
- National Center for Biotechnology Information (NIH). Duodenal Atresia and Stenosis, StatPearls.
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