Pancreatic anomalies: causes, symptoms, diagnosis, treatment

Pancreatic abnormalities are quite common. A large group of anomalies refers to variants of the size, shape and location of the pancreas and are largely of no clinical significance.

At the same time, hereditary diseases are much more frequent, much more serious, some of them are incompatible with life and appear immediately after birth, some abnormalities of the pancreas more or less affect the patient's health and reduce the quality of life. Speaking of pancreatic anomalies, it should be remembered that many hereditary diseases are manifested not only by some kind of developmental malformation, but by several, sometimes even whole complexes of such vices. 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 a lifetime suffers a completely or partially genetically determined disease.

However, in this chapter, we will mainly consider those pancreatic anomalies with which patients can live for a long time and about the symptoms (or complications of which) they turn to doctors; and such sick physicians often manage to help.

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

Doubling the pancreas is an extremely rare developmental disorder; the splitting of its tail is more common.

In the pancreas, there is sometimes a heterotopy of the spleen tissue; in these cases, the tail of the gland is usually fused with the spleen; pancreatic islets of dark-red tissue several millimeters in size are defined on a cut.

The doubling of the pancreas and the heterotopy of the spleen tissue in the pancreas by themselves do not seem to represent any real threat to the development of the child unless they are only one of the manifestations of much more complex developmental defects, for example the Patau and de Lange syndromes. Dystopia and anomalies in the form 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 shifted due to the increase in adjacent organs (sharply enlarged spleen, large left diaphragmatic hernia, etc.).

Ring-shaped gland. Relatively often occurs the so-called annular pancreas arising in embryogenesis due to uneven growth of the dorsal and ventral bookmark of this organ. The tissue of the pancreas at this developmental malformation is located in the form of a ring that 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 form of abnormal development of the gland, which either does not cause any special anxiety to the patient, or, with sufficiently pronounced stenosis, is relatively easy to correct by surgery. At the same time, what should be borne in mind, 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 only enter as one of the components in very complex combined developmental defects, sometimes incompatible with life. If we consider more frequent cases when the 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 violation of its patency.

If the compression is sufficiently pronounced during the development of the fetus, then immediately after birth, it manifests as regurgitation, vomiting and other symptoms, reminiscent of congenital pyloric stenosis or pilorospasm.

In the older age and in adults, even intact tissue of this "pancreatic ring" can squeeze the lumen of the duodenum, and in the case of chronic pancreatitis or pancreatic head cancer such compression occurs, usually up to the occurrence of high total intestinal obstruction, chronic or even acute. In some cases, jaundice of mechanical type (subhepatic) may arise with the annular pancreas due to compression and involvement of the common bile duct in the process. In contrast radiographic examination of the stomach and duodenum, a limited (for 2-3 cm) circular narrowing is usually detected in the descending part of the latter, while the mucosa of the duodenum appears unchanged in the constriction zone, the folds of the mucous membrane are preserved. With gastroduodenoscopy, the narrowing of the descending part of the duodenum (in different cases - of different degrees) is revealed, the mucosa of the duodenum is not changed. This sign allows to exclude the inflammatory-cicatricial lesion of the duodenum, for example, due to scarring of extra-ulcerous ulcers, narrowing due to tumor growth of the intestinal wall in case of cancer of the BSD or head of the pancreas.

Treatment with pronounced compression and violation of patency of the duodenum operative.

Additional (aberrant) pancreas. Relatively common developmental defect, consisting of heterotopy of pancreatic tissue in the wall of the stomach, intestines, gall bladder, the liver and very rarely - in other organs.

It is believed that the first description of the additional pancreas belongs to Schulz (1727), who discovered it in the diverticulum of the ileum (Meckel's diverticulum). SA Reinberg proposed for the aberrant pancreas the term "choristoma" (from the Greek choristos, which means "separable, separate").

By 1927, that is, 200 years after the first description, according to II Genkin, only 60 cases of the aberrant pancreas were described, in the next 20 years - 415. By 1960, in domestic and foreign the literature describes 724 cases as "chorister"; according to pathologists, aberrant foci of pancreatic tissue are found in 0.3-0.5% of all cases of autopsy, which is due to more accurate morphological diagnosis.

When localized in the stomach and intestines, the accessory gland is usually located in the submucosa or muscle envelope. The most frequent localization is in the gastroduodenal zone (63 to 70%) with predominant localization in the pyloric stomach. Aberrant pancreas is more common in men. Large enough islets of the pancreatic tissue with X-ray and endoscopy studies usually have the appearance of a polyp (or polyps) on a broad base. In the center of such large enough polyps with X-ray examination is sometimes determined by a small accumulation of contrast mass - this is the mouth of the duct of the extra pancreas. In most cases, the aberrant pancreas exists asymptomatically.

Complications of the additional pancreas are its inflammatory lesions, necrosis and perforation of the gastric or intestinal wall, malignancy, bleeding from the extra pancreas is possible.

When localizing the aberrant pancreas in the gastroduodenal zone, pain in the epigastric region, nausea, vomiting can be observed. The diagnosis is specified for radiographic, endoscopic and, especially, morphological (histological) studies of biopsy specimens. Given the high risk of malignancy, most authors believe that patients with aberrant pancreas are indicated for surgical treatment.

Aging (aplasia). It is considered an extremely rare developmental disorder, and newborns with this anomaly usually die very quickly. In the literature, only a few cases have been described, when patients with this developmental defect have lived for several years, casually rarely such patients live for more than 10 years - besides, in addition to the absence or underdevelopment of the pancreas, a complex of primary (i.e., congenital) and secondary (acquired) changes in the body.

Syndrome of Shvakhman. A peculiar symptom complex characterized by hypoplasia or aplasia of the exocrine part of the pancreas with its lipomatosis, bone marrow hypoplasia, delayed maturation of granulocytes, fatty hepatosis and liver fibrosis, endocardial fibroelastosis, metaphyseal chondrodysplasia was first described in the domestic literature by TE Ivanovskaya and E. K Zhukova in 1958. However, his name (Syndrome Shvakhman), he received the name of one of the authors who described him in 1963.

This syndrome is rare. It is characterized by hereditary insufficiency of the exocrine function of the pancreas (autosomal recessive inheritance), bone marrow dysfunction. Usually occurs in infancy. There are chronic relapsing diarrhea, delayed growth and general development while maintaining mental development, the delay of which is rare. The examination reveals signs of a syndrome of impaired intestinal absorption: lack of body weight, swelling, a positive symptom of the muscular roller, manifestations of hypo- and avitaminosis, anemia, etc. In laboratory and instrumental studies, fasting hypoglycaemia, reduced tolerance to galactose, metabolic acidosis, metaphyseal dysostosis, hematological disorders (neutropenia, anemia, thrombocytopenia, pancytopenia), as well as steatori.

Patients with Shvakhman syndrome combined with other congenital malformations, biochemical and hematological abnormalities are described, although pancreatic islets are usually not affected, single cases occur that violate the endocrine function of the pancreas and the development of diabetes mellitus. Patients are more likely to die in the first 10 years from bacterial infections, usually pneumonia, or from severe metabolic disorders. At the same time, patients who have lived up to 30 years or more are described.

Morphologically they detect hypoplasia of the pancreas with lipomatosis, in which the glandular tissue and ducts are replaced by fat tissue; sometimes affects the endocrine gland parenchyma.

[1], [2], [3], [4], [5]