Annular pancreas is a rare condition in which the second part of the

duodenum The duodenum is the first section of the small intestine in most vertebrates, including mammals, reptiles, and birds. In mammals, it may be the principal site for iron absorption. The duodenum precedes the jejunum and ileum and is the shortest p ...

is surrounded by a ring of

pancreatic The pancreas (plural pancreases, or pancreata) is an organ of the digestive system and endocrine system of vertebrates. In humans, it is located in the abdomen behind the stomach and functions as a gland. The pancreas is a mixed or heterocrine ...

tissue continuous with the

head of the pancreas The pancreas (plural pancreases, or pancreata) is an organ of the digestive system and endocrine system of vertebrates. In humans, it is located in the abdomen behind the stomach and functions as a gland. The pancreas is a mixed or heterocrine ...

. This portion of the pancreas can constrict the duodenum and block or impair the flow of food to the rest of the

intestine The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascular system. T ...

s. It is estimated to occur in 1 out of 12,000 to 15,000 newborns. The ambiguity arises from the fact that not all cases are symptomatic.

Signs and symptoms

Early signs of abnormality include

polyhydramnios Polyhydramnios is a medical condition describing an excess of amniotic fluid in the amniotic sac. It is seen in about 1% of pregnancies. It is typically diagnosed when the amniotic fluid index (AFI) is greater than 24 cm. There are two clini ...

(an excess of

amniotic fluid The amniotic fluid is the protective liquid contained by the amniotic sac of a gravid amniote. This fluid serves as a cushion for the growing fetus, but also serves to facilitate the exchange of nutrients, water, and biochemical products betwee ...

low birth weight Low birth weight (LBW) is defined by the World Health Organization as a birth weight of an infant of or less, regardless of gestational age. Infants born with LBW have added health risks which require close management, often in a neonatal inten ...

, and feeding intolerance immediately after birth, in particular a tendency to develop epigastric distention associated with non-biliary vomiting (the obstruction is generally above the papilla of Vater, therefore superior to the junction with the

bile duct A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates. The bile duct is separated into three main parts: the fundus (superior), the body (middle), and the neck (inferior). Bile is requ ...

s). Different chromosomal diseases (for example

trisomy 21 A trisomy is a type of polysomy in which there are three instances of a particular chromosome, instead of the normal two. A trisomy is a type of aneuploidy (an abnormal number of chromosomes). Description and causes Most organisms that repro ...

and, with a minor frequency,

trisomy 18 Trisomy 18, also known as Edwards syndrome, is a genetic disorder caused by the presence of a third copy of all or part of chromosome 18. Many parts of the body are affected. Babies are often born small and have heart defects. Other features in ...

and

trisomy 13 A trisomy is a type of polysomy in which there are three instances of a particular chromosome, instead of the normal two. A trisomy is a type of aneuploidy (an abnormal number of chromosomes). Description and causes Most organisms that reprod ...

) are present in about 33% of subjects affected by annular pancreas.

Causes

When ventral pancreatic bud splits and encircles the duodenum leads to a construction in the duodenum

Diagnosis

Postnatal diagnostic procedures include abdominal x-ray and ultrasound, CT scan, and upper GI and small bowel series. Abdominal radiography can show the classic sign of the "double bubble": the presence of air in the stomach and duodenum. Unfortunately, this double-bubble sign is not

pathognomonic Pathognomonic (synonym ''pathognomic'') is a term, often used in medicine, that means "characteristic for a particular disease". A pathognomonic sign is a particular sign whose presence means that a particular disease is present beyond any doubt. ...

for annular pancreas, as it can also be observed in other conditions, such as

duodenal atresia Duodenal atresia is the congenital absence or complete closure of a portion of the lumen (anatomy), lumen of the duodenum. It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies. ...

and

intestinal malrotation Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus, ...

. Upper GI series may be suggestive of annular pancreas, especially if they show a duodenal narrowing of the second portion of the duodenum and the concomitant dilatation of the proximal duodenum. In some cases it is possible to have signs of inverse peristalsis of the duodenal tract which is proximal to the narrowing caused by the annular pancreas, and the dilatation of the duodenal portion distal to the anomaly. An abdominal CT scan or an MRI allows to highlight the narrowing of the descending duodenal tract and the ring of pancreatic tissue surrounding the duodenum: this ring can be complete or, in patients with an incomplete annular pancreas, extended in a postero-lateral or anterolateral direction with respect to the second part of the duodenum. ERCP or MRCP with secretin allow precise delineation of the anatomical structure and in particular a good visualization of pancreatic ducts, as well as a careful analysis of pancreatic secretion into the duodenum lumen.

Treatment

In neonates, treatment for relief of obstruction usually is bypassing the obstructed segment of duodenum by duodeno-jejunostomy. In adults, due to the minor duodenal mobility, the approach is laparoscopic gastrojejunostomy or duodenojejunostomy.

References

External links

{{Congenital malformations and deformations of digestive system Congenital disorders of digestive system Anatomical variations