Congenital Anomalies of the Pancreas

Congenital Anomalies of the Pancreas

The pancreas arises from the foregut just distal to the stomach by ventral and dorsal anlagen. The for­mer is frequently derived from the out­pouching for the liver and biliary ducts (see page 2). This origin explains the many locations of the most frequent pancreatic anomaly, aberrant or ectopic pancreatic tissue. Nests of aberrant ■pan­creatic tissue are most frequently found in the duodenum, stomach and small boxvel but have also been reported in the biliary ducts, gallbladder, liver, spleen, peritoneum, omentum, colon and organs within the mediastinum, such as the esophagus. The favorite site of these aberrant pancreatic nodules is the second portion of the duodenum, followed by the diverticula in the small intestine, such as Meckel’s diverticulum. It has even been suggested that these pancre­atic cells might be causative agents in diverticulum formation. In more than one half of the cases, the nodules of pan­creatic tissue are embedded in the sub – mucosa or, in other instances, in the muscularis and, least commonly, they are subserosal or involve all layers of the wall. On palpation they may seem polyp­oid, and characteristically they have a central dimple. It is rare that more than a single nodule can be detected. The structure is usually similar to that of the normal pancreas but may become cystic, and instances where the mass was pri­marily or entirely made up of islet cells have been reported. The ectopic masses are usually small, seldom being over 5 cm., and usually 1 to 2 cm., in diam­eter. Acute or chronic pancreatitis or tumor, including malignant degenera­tion, may occur in these ectopic tissues.

Aberrant pancreatic tissues many times give rise to rather bizarre symptoms. They may be the cause of pyloric obstruction or of ulceration when developing in the stomach, or of intussusception when lodged in the intestine, especially in a diverticulum. With a predominance of islet tissue, signs and symptoms of an islet cell tumor may try the ingenuity of the clinician and surgeon, who must search all the common areas of aberrant tissue for the islet tumor if it is not found in the pancreas proper.

In the rare anomaly known as annular pancreas, the head of the gland surrounds the second portion of the duodenum. It is more common in males than in females, and all grades from an over­lapping of the posterior duodenal wall to a complete ring may be found. If the ring is incomplete, the defect is always on the anterior surface of the duode­num. The pancreatic duct in the annular tissue arises anteriorly and courses laterally to the right and around the duodenum to its posterior wall, terminat­ing in the main pancreatic duct, the common bile duct or the papilla of Vater. The origin of this anom­aly rests with a failure of the ventral anlage of the pancreas to migrate normally to the left behind the duodenum.

Congenital Anomalies

Congenital Anomalies of the Pancreas

Annular pancreas may be associated with a com­plete or partial atresia of the duodenum. The annular portion of the pancreas may fall heir to any of the diseases of the pancreas, such as acute or chronic pan­creatitis or tumor. Complete or partial obstruction of the duodenum, with dilatation of the proximal portion and the stomach, is to be expected. Secondary ulceration of the duodenum or stomach, not rare with this anomaly, may lead to the discovery of the lesion. Surgery is, of course, the only treatment of this condi­tion. In view of the inherent complications and the possibility of the existence of a duodenal atresia, some authors recommend gastro-enterostomy or duodeno – duodenal anastomosis around the obstruction in all cases, rather than section of the annular pancreas.

Abnormalities in fusion of the anlage of the gland may result in all degrees of division of the gland, including the presence of two separate glands with individual ducts to the duodenum, known as pancreas divisum. Other anomalies of the gland are extremely rare.

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