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12.74. TUMOURS OF THE DIGESTIVE TRACT AT CHILDREN
Juvenile polyps of intestines. It is the most frequent type of tumors of intestines at children. They treat gamartoma and do not malignizirutsya. Usually polyps arise at children 12 months are more senior and only in rare instances persistirut at teenagers 15 years are more senior. About 80% of polyps are localized in a distal segment of a large intestine within reach of a sigmoskop, and only 10% of polyps find proksimalny a splenic bend. Occasionally multiple juvenile polyps occur at members of one family; but structure they do not differ from single polyps and too do not malignizirutsya.
Typical clinical sign of polyps — painless rectal bleedings. Blood can be on a surface of fecal masses or is mixed with them, quantity its moderate. Microscopic losses of blood are possible, and the iron deficiency anemia becomes the first symptom of an illness in such cases. Invagination, polyp or it. a prolapse through an anus are followed by painful attacks. The majority of polyps nekrotizirutsya spontaneously and disappears.
At the differential diagnosis it must be kept in mind other forms a polypose, in particular family polyposes, Mekkel's diverticulum, a crack. an anus and inflammatory processes, including infectious diseases, and also an illness Krone and other forms of colitis.
The diagnosis is made on the basis of rectal research. About 1/3 polyps are available, to detection by a finger though it is quite difficult to feel them at the same time. At a sigmoskopiya polyps look as the smooth, having a leg educations containing gray-white cysts. Irrigoskopiya with a double contrast study allows to reveal the polyps which are above reach of a sigmoskop. Now prefer to use colonoscopes on a fiber optics by means of which it is possible to examine the descending colon and even a cross colon. Detection of a polyp at endoscopic research forms the basis for a biopsy with the purpose to confirm its gamartomny type.
Treatment is conservative unless there are life-threatening bleedings. Low located polyps can be removed with special nippers by means of a rectal speculum. Experience on removal of polyps by means of a colonoscope is saved up, but so far this method widely is not applied. The laparotomy is recommended only at the located polyps multiple proksimalno if they do not manage to be removed by means of a colonoscope.
Family polyposes. This rare group of hereditary syndromes which general sign is intestines polyposes treats precancerous states that causes a cancerophobia in the patient and members of his family.
Family adenomatous polyposes of a large intestine. This precancerous disease which is transmitted on dominant type with the reduced penetrance is characterized by existence of a large number of adenomatous polyps in distal department of a large intestine. In literature cases of emergence of polyps are described
at early age, but usually they arise by the end of the first decade and at teenage age. At an early stage of development polyps of a bessimptomna and in many cases those also remain. At some patients ponosa, bleedings and pristupoobrazny pains are noted. The malignancy can occur at children 10 years are more senior.
It is possible to suspect this form a polypose on the basis of the corresponding family anamnesis, however the methods allowing to predict precisely development a polypose at the child do not exist. The diagnosis is made on the basis of results of X-ray inspection (at an irrigoskopiya with a double contrast study multiple defects of accumulation are visible), and also by sigmoskopiya and kolonoskopiya at which polyps of the different sizes are visible. Polyps are multiple, adenomatous, without cysts and the signs of an inflammation inherent to juvenile polyps.
Treatment includes careful supervision over family members, genetic consultation, the patient make a pan-colectomy. In the past imposed ileosty, but new methods of creation of an anastomosis provide integrity of intestines therefore on operation keep a serous and muscular layer of a rectum.
Peytts's syndrome — Egersa. This rare syndrome is transferred on dominant type and characterized by pigmentation of a mucous membrane of lips and gums, and also gamartoma of a stomach and a small bowel. Such polyps do not malignizirutsya. Accurately delimited nevus pigmentosus is usually visible already at the birth or appears at chest age on a mucous membrane of lips, cheeks and even around a mouth. Signs of damage of intestines — bleeding, painful attacks, obstruction of intestines and even invagination — arise later.
Treatment consists in supervision over family members and genetic consultation. At inspection of relatives it is possible to reveal patients with partial or full manifestations of this syndrome. If polyps of intestines cause considerable disturbances, then they should be removed, however so most part of a gut is usually struck that all polyps cannot be removed.
Gardner's syndrome. This rare, is prepotent the inherited frustration is characterized by numerous polyps of intestines, and also tumors of soft tissues and bones, in particular a mandible. Tumors of soft tissues and an osteoma arise already in the childhood, and polyps in intestines are found only in adults. They can be localized in any departments of intestines and show a tendency to malignant regeneration. Therefore at this syndrome radical surgical removal of polyps is shown.
Intestines hemangioma. This rare benign tumor can be complicated massive, sometimes by fatal bleeding. The leading symptom are the painless bleedings beginning already in the early childhood. In one cases the loss of blood is hardly noticeable and has chronic character, in others it is sudden and plentiful. There are no intestinal symptoms, but in case of invagination there are signs of impassability of intestines. Approximately 50% of patients have skin hemangiomas; in some cases the disease occurs at members of one family. Approximately at a half of patients of a hemangioma are localized in a large intestine and are visible at a kolonoskopiya, in the period of bleeding the selection mezenterialny arteriography can help to identify character and localization of defeat.
Leiomyoma. This rare high-quality new growth arises mainly in a stomach and a jejunum. A long time it remains asymptomatic, but if the leiomyoma sizes considerably increase, then it can become the reason of invagination and abdominal pains.
Carcinoma. Epithelial tumors of a digestive tract at children are extremely rare therefore too active approach to diagnosis of these new growths in this age group is not justified. Gardner's syndrome, nonspecific ulcer colitis, and also, to a lesser extent, an illness the Krone and some syndromes connected with ruptures of chromosomes contribute to development of an adenocarcinoma family polyposes. At such syndromes of a tumor usually develop only at adults.
Most often the carcinoma is localized in a large intestine and shown by feeling sick of the patient, abdominal pains, bleedings, the tumor palpatorno defined in an abdominal cavity. These tumors often happen rather undifferentiated and high-malignant.
Lymphosarcoma. As it was stated above, malignant damages of a digestive tract are very rare; the majority of them belongs to lymphosarcomas. The tumor is usually localized in the lower part of a small bowel. Its clinical symptoms — a bad general state, abdominal pains and anemia. Rather often the lymphosarcoma arises at adults, long time having a gluten illness; it is proved that even continuous observance of a diet is not prevented by developments of a new growth.
Carcinoid tumors. The carcinoid tumor proceeds from enterokhromaffinny cells of intestines, is localized mainly in a worm-shaped shoot; the probability of its malignancy is very small. Symptoms are same, as at appendicitis; after a resection they do not arise any more even if the tumor extended to lymph nodes.
The carcinoid tumors which are out of an appendix usually metastasize, and metastasises give rise to a carcinoid syndrome. This syndrome develops as a result of effect of the hormonal active agents produced by a tumor. It includes periodic hyper motility of intestines (diarrhea), vasculomotor frustration and bronchospasms. The most active substance is serotonin. The diagnosis is based on detection in serotonin metabolite urine — 5-oksiipdoluksusny acid. Such hormonal and active tumors very seldom occur at children.
The hormonal and active tumors causing diarrhea.
The increased hormonal activity of some tumors serves as the reason of heavy chronic diarrhea. The most widespread of these. tumors come, from nervous tissue. The neuroblastoma and a ganglioneuroma most often arise in adrenal glands, but to mogut.razvitsya in any part of a sympathetic trunk (section 18.28). The diarrheal syndrome, apparently, is caused by effect of vasoactive intestinal polypeptide (LOOK) which is produced by a tumor. Diarrhea is noted approximately at 10% of patients with a tumor, it can be various intensity, but usually has profuse character and is followed by disturbances of a water salt metabolism. The diagnosis is based on detection of a tumor and its secrets, including catecholamines and their metabolites. At a pheochromocytoma of diarrhea does not happen. Occasionally heavy diarrhea accompanies Zollinger's syndrome — E l of l and with about N and (section 12.22); at this frustration clinical symptoms are caused by gastrinnrodutsiruyushchy. tumor of the insular device of a pancreas. Seldom found tumors of podzheludochyy gland which are not producing gastrin can be also followed by diarrhea, in these cases they, apparently, cosecrete the VIP.
Nodular hypertrophy of an adenoid tissue.
Lymphoid follicles in own plate of a wall of a digestive tract are concentrated in peyerovy plaques. They look as submucosal nodes, are distinguishable on roentgenograms, and they can be taken for pathological educations mistakenly. The quantity of peyerovy plaques in distal department of a small bowel are much higher, than in proximal; some patients have lymphoid follicles, giperplazirutsya. This process usually takes a large intestine, but can extend also to thin. The hyperplasia of follicles in a small bowel takes place at immunodeficiency., combined (or not combined) with a lambliasis. Symptoms of a lymphoid hyperplasia are expressed unsharply; rectal bleedings, diarrhea and abdominal pains which appear on the 3rd year of life. It is important to emphasize that this picture is similar to displays of other more serious diseases. The lymphoid hyperplasia is allowed spontaneously and does not demand specific treatment.