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Annular Pancreas: Causes, Symptoms and Treatment

Annular pancreas is a rare congenital anomaly in which a ring of pancreatic tissue surrounds the duodenum, potentially causing a partial or complete intestinal obstruction.

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Things worth knowing about "Annular Pancreas"

Annular pancreas is a rare congenital anomaly in which a ring of pancreatic tissue surrounds the duodenum, potentially causing a partial or complete intestinal obstruction.

What is Annular Pancreas?

Annular pancreas is a rare congenital malformation of the pancreas in which a band or ring of pancreatic tissue encircles the second part of the duodenum (the first section of the small intestine). This ring can compress the duodenum, causing a partial or complete blockage. The condition arises during embryonic development and is most often detected in newborns, though it may remain undiagnosed until adulthood in milder cases.

Causes and Development

During normal fetal development, the pancreas forms from two embryonic buds: the ventral and dorsal pancreatic buds. In annular pancreas, the ventral bud fails to rotate properly and instead wraps around the duodenum before fusing with the dorsal bud. The exact mechanism behind this developmental error is not fully understood. Annular pancreas is frequently associated with other congenital conditions, including:

Down syndrome (Trisomy 21)
Esophageal atresia
Congenital heart defects
Intestinal malrotation
Duodenal atresia

Symptoms

In Newborns and Infants

When the duodenal narrowing is severe, symptoms appear shortly after birth:

Bilious (green-tinged) vomiting
Abdominal distension in the upper abdomen
Failure to pass stool (meconium)
Poor feeding and weight loss

In Children and Adults

In cases of incomplete obstruction, symptoms may not appear until later in life and can include:

Recurrent nausea and vomiting after meals
Upper abdominal pain
Delayed gastric emptying
Chronic pancreatitis
Peptic ulcer disease

Diagnosis

Several imaging techniques are used to diagnose annular pancreas:

Ultrasound: Often the first investigation; prenatal diagnosis is possible in some cases
X-ray with contrast: Reveals the classic double-bubble sign in newborns, indicating duodenal obstruction
Computed tomography (CT) or magnetic resonance imaging (MRI): Provides detailed visualization of the pancreatic ring and surrounding structures
Endoscopic retrograde cholangiopancreatography (ERCP): Used in adults to evaluate the pancreatic and bile ducts

Treatment

Treatment of annular pancreas is surgical. The encircling pancreatic ring is never cut or divided, as this risks causing serious complications such as pancreatic fistula or acute pancreatitis. Instead, a surgical bypass is created to restore bowel continuity:

Duodenoduodenostomy: Connecting the duodenum above and below the obstruction -- the preferred technique in newborns
Duodenojejunostomy: Connecting the duodenum to the jejunum (a further section of the small intestine)
Gastrojejunostomy: Connecting the stomach directly to the jejunum, used less frequently

Surgery is highly effective in symptomatic patients and generally results in the permanent relief of the obstruction. Incidentally discovered, asymptomatic cases may be managed conservatively with regular monitoring.

Prognosis

The long-term prognosis following surgical repair is generally excellent. Rare complications such as recurrent obstruction or chronic pancreatitis may occur and require further management.

References

Skandalakis, J.E. et al. - Surgical Anatomy: The Embryologic and Anatomic Basis of Modern Surgery, 2nd Edition, Paschalidis Medical Publications (2004)
Zyromski, N.J. et al. - Annular Pancreas: Degree of Duodenal Obstruction Influences Clinical Presentation and Management. World Journal of Surgery, 32(4), 560-565 (2008). PubMed PMID: 18219533
Esposito, C. et al. - Annular Pancreas in Newborns: Surgical Management and Outcome. Pediatric Surgery International, 36(9), 1087-1091 (2020). PubMed PMID: 32556553

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