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CONGENITAL AND PERINATAL ANOMALIES OF DEVELOPMENT OF THE DIGESTIVE TRACT AND IMPASSABILITY OF INTESTINES INBORN HYPERTROPHIC PYLORIC STENOSIS
Many congenital and perinatal anomalies of development of a digestive tract lead to partial or full obstruction of intestines. Obstructive processes affect generally a rectum and an anus; besides, they are observed in a small bowel. Treat the heaviest defects: pylorostenosis;
atresia or stenosis of a duodenum (separately or in combination with a ring-shaped pancreas); atresia or stenosis of a lean or ileal gut; the wrong turn of an intestinal tube (separately or in combination with torsion of guts);
illness of Girshprunga (aganglionarny megacolon);
doubling and diverticulums of intestines.
The pyloric stenosis, or pylorostenosis, occurs at 1 of 150 newborn boys and at 1 of 750 girls. Some authors consider that this anomaly is more often observed at boys - firstborns. Family character of a disease is noted in 15% of cases, but the mode of inheritance is not established yet.
Etiology. The reason of a pylorostenosis is unknown. In favor of its inborn origin the high frequency of this pathology at enzygotic twins and a relative rarity at biovular testifies; besides, a certain correlation between the frequency of a pylorostenosis and frequency of an atresia of a gullet and hernia of an esophageal opening of a diaphragm is observed. However it is impossible to exclude action of any still unknown acquired factor. At this illness the level of serumal gastrin is increased, but it is still not clear whether it is its reason or a consequence.
Patomorfologiya and pathophysiology. The diffusion hypertrophy and a hyperplasia of smooth muscles of antral department of a stomach turn it into very narrow canal which it becomes easy completely impassable. The antral department is extended, its walls are thickened almost twice and get a cartilaginoid consistence. The muscles thickening is never limited to the isolated bunch of the circular layer called by a peloric sphincter, and occupy all antral department in the proximal direction, and in distal — reaches a duodenum. The delay of evacuation and, therefore, the raised peristaltics conduct to a hypertrophy and dilatation of muscles of all stomach. As a result of a long staz of gastric contents development of gastritis and gastric bleedings is possible. Persistent vomitings cause dehydration and a gipokhloremichesky alkalosis.
Clinical manifestations. The first signs — regurgitation and non-constant not plentiful vomiting — usually appear on 2 — the 3rd week of life, is more rare on the 1st week and are even more rare — on 2 — the 3rd month. In a week after emergence of symptoms vomiting becomes plentiful; it arises usually in time or right after food, but sometimes in several hours after food. In one cases vomiting arises after each meal, in others — has intermittent character. The child is constantly hungry and demands food at once after food. Emetic masses consists only of stomach contents, sometimes in them blood impurity is found; coloring by bile is not observed. The chair is rare, volume a calla small, depends on quantity of the food which passed in intestines.
At inspection of the child reveal dehydration and a stupor of various degree depending on expressiveness of metabolic disturbances. Decrease in body weight is observed. In far come cases the child is extremely exhausted, loss of body weight is so considerable that he weighs less, than at the birth. Elasticity of skin is sharply reduced, hypodermic cellulose is absent. The eyes which sank down, a wrinkled face, as at the old man.
The peristaltic waves seen by an eye going from the left upper quadrant of a stomach in right to the gatekeeper, are especially accurately expressed right after feeding or before vomiting (fig. 12-10). The palpation of a stomach demands patience as it happens successful only at full relaxation of a front abdominal wall and an empty stomach. Long careful suction of gastric contents by means of the probe entered through a nose with simultaneous administration of warm solution of glucose facilitates a palpation of "tumour". It is better to palpate the child on the left side. If there is a stenosis, then in epigastriums, to the right of the average line, under the right direct muscle of a stomach at a liver edge dense education is defined. It without serious consequences, movably, is similar to an acorn or an olive, is better probed right after vomiting. If the hypertrophied gatekeeper is found palpatorno, then is inexpedient to resort to X-ray contrast research.
Fig. 12-10. Peristaltic waves of a stomach at a pyloric stenosis at the child of 3-week age. (With the permission of Dr. Carl Wagner.)
Fig. 12-11. Baric weight in a stomach of the child at which pernicious vomitings are observed. The narrow peloric channel is typical for an inborn hypertrophic pyloric stenosis.
When the stenosis is not found palpatorno, X-ray inspection is shown to establish other reasons of plentiful vomiting. Thus, the diagnosis of a pylorostenosis is made on the basis of a clinical picture and data of a palpation. Any surgeon should not start operation while personally does not propalpirut consolidation and does not identify it as a pylorostenosis.
Radiological signs of a pyloric stenosis are very characteristic. The stomach actively peristaltirut, but evacuation of a baric suspension is sharply reduced or in general is absent. The thin extended peloric channel is contrasted by one (a string symptom) or two narrow strips of barium; the duodenum bulb as an umbrella, hangs over the hypertrophied gatekeeper. Proksimalny the peloric channel it is possible to see an unusual diverticulum (fig. 12-11).
At 2 — 9% of children with a stenosis jaundice is observed; believe that it is caused by deficit of a glyukuroniltransferaza or the increased absorption of bilirubin in intestines. Jaundice usually disappears in 72 h after operation.
Metabolic disturbances. Plentiful and long vomitings at a pyloric stenosis (as well as at other forms of high impassability of intestines) can lead to sharply expressed insufficiency of potassium, sodium and chlorides as which reflection serves their low content in blood serum. Decrease in level of chlorides, increase pH and a delay in a carbon dioxide organism, i.e. typical biochemical signs of a gipokhloremichesky alkalosis are especially dangerous. It is possible to Korrigirovat these chemical disturbances by administration of potassium and sodium. Intravenous administration of solution of chloride ammonium is contraindicated. Infusion of 5% of solution of glucose in isotonic solution of sodium chloride in which potassium chloride is added (to concentration of 30 — 50 mmol/l) allows to compensate gradually and sufficiently the calculated deficit of potassium, sodium and chloride ions. This method allows to prevent such serious complication as a hyponatremia which can arise if for compensation of insufficiency of electrolytes at persistent vomiting enter hypotonic salt solutions. Level chloride ions in serum (which varies from normal to 70 mmol/l) can be regarded as an approximate indicator of content of potassium in an organism; if the level of chlorides in serum normal, then deficit of potassium can be minimum. In such cases it is necessary to watch to oversaturating an organism this element.
After dehydration is eliminated, it is necessary to enter into an organism enough liquid constantly.
Differential diagnosis. In typical cases the diagnosis is made on the basis of characteristic clinical signs and a palpation of dense weight in the field of the gatekeeper. Frequent vomitings in the first weeks of life can arise if the child excessively actively reacts to external stimuluses if he is looked after by the inexperienced or angry persons or if adequate connection necessary for the child with mother is not established so. Such children sometimes remind patients with a pyloric stenosis, they can have constant plentiful vomitings. Peristaltic waves can be observed at the exhausted child even in the absence of a stenosis. On the first week of life of vomiting appear at a halaziya of a gullet and at hernia of an esophageal opening of a diaphragm which are differentiated from a stenosis by means of X-ray inspection. External signs, similar to peloric stenosis, are observed at insufficiency of adrenal glands, but lack of the palpated weight, the metabolic acidosis, the increased content of potassium in serum and sodium in urine allow to make the correct diagnosis. Vomiting in combination with diarrhea forces to think of a gastroenteritis, though at a pilorosteioza ponosa are sometimes possible. Occasionally the pylorostenosis should be differentiated with the gastroesophagal reflux which arose independently or in combination with phrenic hernia. At last, very seldom severe vomitings, a visible peristaltics and yes -
palpatorno the defined tumor can take place at a duplikatura of the peloric channel.
Treatment. Method of the choice is surgical elimination of impassability of a pylorus right after the diagnosis is established and disturbances of a metabolism are liquidated. Children without dehydration and deficit of electrolytes it is necessary to operate immediately, at heavy dehydration it is possible to delay operation on 24 — 36 h for carrying out replacement therapy by means of intravenous injections. Before operation contents of a stomach are deleted by means of a probe. On operation a serous muscular layer of antral and peloric departments of a stomach make cuts, then muscles stratify blunt instrument (a pyloromyotomy according to Freda — to Ramshtedt). Already in 4 — 6 h after operation of the child it is possible to feed in the natural way, since small portions and gradually increasing them. Now the following mode is accepted: in the first 4 feedings each hour give on 4 ml 5% of glucose in normal saline solution. If vomiting does not arise, then in the following of 4 h give 8 ml; in each subsequent 4 h volume is increased, gradually replacing solution with milk mix. By the end of second day of the child it is possible to feed normally. If the child takes a breast, then it is recommended already at the first feeding after operation to put it to each breast for 1 min., increasing suction duration at each subsequent feeding.
Other option of postoperative maintaining consists in that within the first days to enter necessary liquids parenterally and only in 24 h to begin to feed in the natural way. If after the first meal there is vomiting, then feeding is postponed for 4 h and if necessary all cycle is repeated at first. Persistent vomitings in the postoperative period indicate that the sphincter is dissect insufficiently or that there are associated diseases (phrenic hernia, a halaziya). In general single vomitings are frequent during this period, apparently, owing to the accompanying gastritis. Additional intravenous administration of liquids in the volume corresponding to a condition of a water salt metabolism helps to get rid of them. As it was specified, the vomitings proceeding within 3 — 5 days after operation testify to an incomplete section of a hypertrophied peloric sphincter. In such situation the repeated laparotomy can be required. As a rule, after operation of vomiting stop. Sometimes X-ray inspection reveals narrowing of the peloric channel within many months, despite the lack of clinical symptoms. Usually the child can be written out from a hospital in 48 h after operation.
Nonsurgical methods of treatment. The recovery process duration (2 — 8 months) at conservative treatment, its high cost, a high rate of a lethality, negative influence of long hospitalization on an emotional condition of the child — all this led to almost full refusal of nonsurgical methods of treatment of a pylorostenosis. If nevertheless there is a need to treat the child in therapeutic way, then apply frequent feeding in the small portions, include thick porridges in a diet, keep the child in vertical position within an hour after each food, appoint sedatives and anticholinergic drugs, according to indications enter intravenously nutrients. If to food the stomach is stretched, then evacuation of its contents through a probe reduces probability of vomiting.
Forecast. At early diagnosis and an adequate preparation for surgery the postoperative lethality makes less than 1%. Nonsurgical treatment is followed by higher lethality. The long sharply expressed insufficiency of food adversely affects the subsequent development of the child.