Pancreatic anomalies: causes, symptoms, diagnosis, treatment

Pancreatic anomalies are quite common. A large group of anomalies relate to variations in the size, shape, and location of the pancreas and are generally of no clinical significance.

At the same time, hereditary diseases are quite common, much more serious, some of them are incompatible with life and manifest themselves immediately after birth, some anomalies of the pancreas to a greater or lesser extent affect the health of the patient and reduce the quality of life. Speaking about anomalies of the pancreas, it should be remembered that many hereditary diseases are manifested not by one developmental defect, but by several, sometimes even whole complexes of such defects. In addition, an important factor is the increase in the prevalence of hereditary diseases in some countries: for example, in Switzerland, approximately every 13th newborn at birth or during life suffers from a completely or partially genetically determined disease.

However, this chapter will mainly consider those anomalies of the pancreas with which patients can live for a long time and whose symptoms (or complications) lead them to seek medical attention; and doctors are often able to help such patients.

In mild cases of congenital anomalies, the pancreas may be split along its longitudinal axis, or the head of the pancreas may be located separately from its body.

Duplication of the pancreas is an extremely rare malformation; splitting of its tail is somewhat more common.

In the pancreas, heterotopia of spleen tissue is sometimes observed; in these cases, the tail of the gland is usually fused with the spleen; on section, pancreatic islets of dark red tissue several millimeters in size are determined.

Duplication of the pancreas and heterotopia of spleen tissue into the pancreas in themselves do not seem to pose any real threat to the child's development, unless they are just one of the manifestations of much more complex developmental defects, such as Patau and de Lange syndromes. Dystopias and anomalies of the shape of the pancreas occur in cases of situs viscerum inversus (the gland is located on the right "mirror" to its usual location), and also (to a lesser extent) when its position is displaced due to an increase in adjacent organs (a sharply enlarged spleen, large left-sided diaphragmatic hernias, etc.).

Annular gland. The so-called annular pancreas is relatively common, arising in embryogenesis due to uneven growth of the dorsal and ventral rudiments of this organ. The tissue of the pancreas in this developmental defect is located in the form of a ring, which completely or almost completely covers the descending part of the duodenum. In some cases, which is relatively common, the annular pancreas is a relatively harmless type of abnormal development of the gland, which either does not cause much concern to the patient, or, with sufficiently pronounced stenosis, is relatively easily corrected by surgery. At the same time, it should be borne in mind that the annular pancreas can be combined with other anomalies in the development of the digestive system, and in some cases - other organs. In addition, this anomaly can be included only as one of the components in very complex combined developmental defects, sometimes incompatible with life. If we consider more common cases, when annular pancreas is not combined with other developmental defects, then its clinical manifestations are largely determined by the degree of compression of the duodenum and the obstruction of its patency.

If the compression is sufficiently pronounced during the period of fetal development, then immediately after birth it manifests itself as regurgitation, vomiting and other symptoms, resembling congenital pyloric stenosis or pylorospasm.

In older age and in adults, even intact tissue of this "pancreatic ring" can compress the lumen of the duodenum, and in the case of chronic pancreatitis or cancer of the head of the pancreas, such compression usually occurs up to the development of high complete intestinal obstruction, chronic or even acute. In some cases, with annular pancreas, mechanical jaundice (subhepatic) can occur due to compression and involvement of the common bile duct in the process. In contrast radiographic examination of the stomach and duodenum, a limited (over 2-3 cm) circular narrowing is usually detected in the descending part of the latter, while the mucous membrane of the duodenum in the narrowing zone appears unchanged, the folds of the mucous membrane are preserved. Gastroduodenoscopy reveals a narrowing of the descending part of the duodenum (in different cases - to varying degrees), the mucous membrane of the duodenum is unchanged. This sign allows us to exclude inflammatory-cicatricial lesions of the duodenum, for example, due to scarring of an extrabulbar ulcer, narrowing due to tumor adhesion of the intestinal wall in cancer of the duodenum or the head of the pancreas.

Treatment for severe compression and obstruction of the duodenum is surgical.

Accessory (aberrant) pancreas. A relatively common developmental defect consisting of heterotopia of pancreatic tissue into the wall of the stomach, intestine, gall bladder, liver, and very rarely into other organs.

It is believed that the first description of an accessory pancreas belongs to Schulz (1727), who discovered it in a diverticulum of the ileum (Meckel's diverticulum). S. A. Reinberg proposed the term "choristoma" for the aberrant pancreas (from the Greek choristos, meaning "separable, separate").

By 1927, i.e., over 200 years since the first description, according to I. I. Genkin, only 60 cases of aberrant pancreas had been described, and in the following 20 years, another 415. By 1960, 724 cases of "choristoma" had been described in domestic and foreign literature; according to pathologists, aberrant foci of pancreatic tissue occur in 0.3-0.5% of all autopsy cases, which is due to more accurate morphological diagnostics.

When localized in the stomach and intestine, the accessory gland is usually located in the submucosa or muscular coat. The most common localization is in the gastroduodenal zone (from 63 to 70%) with predominant localization in the pyloric section of the stomach. Aberrant pancreas is more common in men. Sufficiently large islets of pancreatic tissue usually look like a polyp (or polyps) on a broad base during X-ray and endoscopic examination. In the center of such sufficiently large polyps, a small accumulation of contrast mass is sometimes determined during X-ray examination - this is the mouth of the accessory pancreas duct. In most cases, the aberrant pancreas is asymptomatic.

Complications of an accessory pancreas include inflammatory lesions, necrosis and perforation of the gastric or intestinal wall, malignancy, and possible bleeding from the accessory pancreas.

If the aberrant pancreas is localized in the gastroduodenal zone, pain in the epigastric region, nausea, and vomiting may be observed. The diagnosis is clarified by X-ray, endoscopic, and especially morphological (histological) examination of biopsy specimens. Given the high risk of malignancy, most authors believe that surgical treatment is indicated for patients with an aberrant pancreas.

Agenesis (aplasia). It is considered an extremely rare developmental defect, and newborns with this anomaly usually die very quickly. The literature describes only isolated cases where patients with this developmental defect lived for several years, casuistically such patients rarely live more than 10 years - and in addition to the absence or underdevelopment of the pancreas, they usually have a complex of primary (i.e. congenital) and secondary (acquired) changes in the body.

Shwachman syndrome. A unique symptom complex characterized by hypoplasia or aplasia of the exocrine pancreas with its lipomatosis, bone marrow hypoplasia, delayed maturation of granulocytes, fatty hepatosis and liver fibrosis, endocardial fibroelastosis, metaphyseal chondrodysplasia, was first described in Russian literature by T. E. Ivanovskaya and E. K. Zhukova in 1958. However, it received its name (Shwachman syndrome) from one of the authors who described it in 1963.

This syndrome is quite rare. It is characterized by hereditary insufficiency of the exocrine function of the pancreas (autosomal recessive inheritance), dysfunction of the bone marrow. It usually manifests itself in infancy. Chronic recurrent diarrhea, growth retardation and general development occur while maintaining mental development, the delay of which is rare. During examination, signs of intestinal malabsorption syndrome are found: body weight deficiency, edema, positive symptom of muscular ridge, manifestations of hypo- and avitaminosis, anemia, etc. During laboratory and instrumental examination, fasting hypoglycemia, decreased tolerance to galactose, metabolic acidosis, metaphyseal dysostosis, hematological disorders (neutropenia, anemia, thrombocytopenia, pancytopenia), and steatorrhea are detected.

Patients have been described in whom Shwachman syndrome was combined with other congenital malformations, biochemical and hematological anomalies, although the pancreatic islets are usually not affected, isolated cases have been described that occur with dysfunction of the endocrine apparatus of the pancreas and the development of diabetes mellitus. Patients most often die in the first 10 years from bacterial infections, usually pneumonia, or from severe metabolic disorders. At the same time, patients have been described who lived to 30 years or more.

Morphologically, hypoplasia of the pancreas with lipomatosis is detected, in which the glandular tissue and ducts are replaced by fatty tissue; sometimes the endocrine parenchyma of the gland is also affected.

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