Ministry of health of Ukraine

Defects involving intestinal obstruction.

1. Background.

Urgency of the problem lies in the fact that over 2% of children born with birth defects that are incompatible with life and require immediate surgical correction. According to the World Health Organization (WHO), obtained from 24 specialized centers 16 developed countries, this percentage does not tend to decrease.
The structure of infant mortality mortality malformation remains in second place after hypoxia as an etiologic factor. If we consider progressive deterioration of ecological and social situation in Ukraine, maternal age, low birth rate (one or two children in the family), it becomes clear relationship between prenatal and early postnatal diagnosis of defects and their timely surgical treatment. Timely antenatal diagnosis of defects is not only a medical but also a social problem.
2. Specific objectives:
1. Have data on the frequency of prevalence of malformations GIT in Ukraine and abroad.
2. To analyze the risk factors that complicate pregnancy and may be the etiological factor of Pathology intestinal tube.
3. Explain the features of the pathogenesis of defects causing obstructive and strangulative intestinal obstruction at high and low levels.
4. Classified malformations and malformations nosological forms, accompanied by intestinal obstruction.
5. Recognize the major clinical manifestations and determine clinical diagnostic criteria for congenital obstruction.
6. Conduct differential diagnosis of defects that are accompanied by intestinal obstruction, depending on the level of noise.
7. To interpret the general principles of treatment, accompanied by intestinal obstruction.
8. Define the terms and criteria of readiness for surgical treatment, methods of surgical interventions in malformation, causing intestinal obstruction.
9. Have knowledge about the features and principles of postoperative rehabilitation of children with congenital intestinal obstruction.
4.2. Theoretical questions to studies.
1. Embryogenesis intestinal tube.
2. Stages turning "midgut".
3. Pathogenesis of intestinal obstruction.
4. Present a classification of defects, accompanied by intestinal obstruction in children.
3. Present a clinical picture defects involving intestinal obstruction.
4. Master the principles of the use of auxiliary examination methods and interpretation of the data obtained in this pathology.
5. Interpret trends therapy malformations involving intestinal obstruction in children.
6. To analyze the possibility of surgical intervention as a way to influence malformations involving intestinal obstruction.
7. Identify tactics patient with malformations involving intestinal obstruction.
8. Rational infusion and antibiotic therapy of newborns with birth defects, accompanied by intestinal obstruction.

4.3 Practical work carried out in class (level III digestion).

1. Collect medical history, including information about pregnancy and childbirth relatively specific patient.
2. Conduct review of a sick child, palpation, auscultation.
3. Describe the objective status and determine the clinical and radiological symptoms of defects involving intestinal obstruction.
4. To demonstrate the technique of probing the stomach.
5. Interpret the main methods of diagnosis of congenital malformations (radiological, endoscopic, laparoscopic and laboratory).
6. Settle and make a plan of examination and treatment.
7. Demonstration video scenes surgeries in this pathology.
8. Types of intestinal sutures, overlapping ways.
9. Methods of clean surgical wounds.
10. Identify the indications for surgical treatment, especially postoperative care.

Content themes

Malformations and diseases of the digestive system
Congenital intestinal obstruction
Congenital intestinal obstruction in children is 10-15% of acute intestinal obstruction.
Newborn etiological factors of congenital intestinal obstruction is intestinal tube defects in embryogenesis (atresia, stenosis, doubling), defects that are associated with the violation of innervation and blood supply to the intestinal tube (congenital pyloric stenosis, mehaduodenum, neurogenic ileus and Hirschsprung's disease), rotational anomalies and those that develop as a result of incomplete rotation of the intestinal tube - syndrome Ladd (midgut volvulus with compression of the duodenum strands of the peritoneum, which fix perched cecum).
One of the causes of congenital intestinal obstruction may be abnormalities of other organs. This is the wrong development of the pancreas, which covers in the form of ring duodenum, which narrows its lumen, aberrant vessels, diaphragm defects, cysts and tumors, abnormalities vitelline duct.
Malformations of the intestinal tube are formed during organogenesis first 3-4 weeks of fetal development, when disrupted one of the processes of formation of the intestinal wall, the lumen of the intestine. In the development of intestinal tube stage is proliferation of the epithelium, resulting in the intestine completely closed. Later vacuolization process normally should lead to the restoration of its lumen, but under certain conditions, this phase is disturbed and intestinal lumen tube remains closed. In case of violation of recanalization in a small area it is closed by a thin membrane - membranous atresia. If the process is incomplete recanalization in peretyntsi formed holes of different sizes - membranous stenosis. If the lumen intestinal tube closed at a great distance, formed atresia in a fibrous strand. Sometimes it is caused by underdevelopment of the relevant branch of mesenteric vessels.
Atresia may be single or multiple. Often these defects occur in areas of complex embryonic processes - large duodenal papilla in a place of transition duodenum into the small, in the distal ileum.
The transition from primary fetal position - phase physiological embryonic hernia - occurs by rotation of the intestinal tube. Violation of this process (the delay) at different stages causes various defects that may cause intestinal obstruction. In case of delay in the first stage rotation child is born with incomplete intestinal rotation. It midgut (from duodenum to mid transverse colon) remains fixed at one point in the projection discharge of the superior mesenteric artery. Loops of small intestine are located in the right half of the abdominal cavity, blind - epigastric under the liver or in the left upper quadrant and colon - left. With this fix, there are conditions for the development of twisting around the root of the mesentery and the development of acute intestinal obstruction strangulative.
If disturbed second phase rotation, cecum, which is located in the epigastric region, fixed embryonic strands in front of the duodenum, compressing it. Possible combination of compression of the duodenum and twisting around the superior mesenteric artery - a syndrome Ladd full, without twisting - incomplete. Atypical location of the cecum with the appendix difficult to diagnose acute appendicitis in older children and even adults.
If disturbed the third phase rotation, changing fixation intestines that leads to the formation of defects in the mesentery, or the formation of various depressions and pits, which may zaschemlyuvatysya intestinal loops. Accordingly, these anomalies are considered false or true internal hernias.
Especially by their nature are abnormalities of other organs, leading to intestinal obstruction. Mekoniyevyy ileus caused by congenital kistofibrozu pancreas. Lack of enzyme activity and the absence of pancreatin enhance the viscosity of the meconium, which clog the lumen of the ileum to the iliac slipokyshkovym valve (bauhiniyevoyu valve).
According to the classification SJ Doletskoho during diagnosis and determination of treatment strategy should be considered types of congenital intestinal obstruction, namely, full and partial, which in turn is divided into high, low and intestinal.
To include such high Defects duodenal atresia, initial of the small intestine, the duodenum stenosis of the internal, external stenosis - Ladd syndrome or its individual components; annular pancreas, aberrant vessel.
By enteric: ileum atresia, stenosis of the internal ileum, duplication (enterokistoma) pinched in birth defects ripples; genuine internal hernia, isolated midgut volvulus, internal hernia false; mekoniyevyy ileus.
To include low anorectal malformation.
According to the classification of GA Bayirova a blemish divided into acute, chronic and recurrent. Each of them is divided into high and low intestinal obstruction.
Prenatal diagnosis of congenital intestinal obstruction is to determine the -fetoprotein, ultrasound examination of amniotic fluid forlevel of karyotype. All this allows in some cases to identify a risk group among pregnant women and raise the question of abortion.
Polyhydramnios occurs in 50% of all cases of high intestinal atresia, its presence is an indication for fetal ultrasound.
Increasing the concentration of bile acids in the amniotic fluid in polyhydramnios is a diagnostic feature atresia intestines.
Another method is to fence amniotic fluid and entitled "amniocentesis". For getting into the amniotic cavity, needle pierce the abdominal wall and uterus of the mother and take about 20-50 ml of fluid. This procedure is usually not performed until the 14th week of pregnancy due to the lack of fluid so far. -fetoprotein.The liquid is analyzed for This substance is a protein of the fetus, which is present in high concentrations in the amniotic fluid in the abdominal cavity defects, Gastroschisis, omfalotsele. This protein is also present in the serum of the mother, where it can be determined, though with less certainty, rather than in the amniotic fluid.
Clinical features of congenital intestinal obstruction depends largely on the level and type of intestinal obstruction. At high intestinal obstruction in infants from the first hours of life there is vomiting, sometimes relentless, mixed with bile, if an obstacle is localized below the large duodenal papilla (Fig. 1 and 2). Very fast-growing phenomenon exsicosis and aspiration pneumonia. In children with high congenital intestinal obstruction is usually observed discharge of meconium. If the location is above the obstacles of large duodenal papilla, the number and color of meconium is not changed and there is a discharge of his 3-4th day. For low number of meconium ileus much less consistency it more viscous, grayish color. These features are caused by the inability of the passage of bile and amniotic fluid in the distal colon. When atresia and subtotal stenosis is a single allocation changed meconium or selection in small portions during the first 2 days.
The behavior of a child with high intestinal obstruction in the 1-st day as usual. With the deterioration of the general condition of the child refuses to feeding.
In the dynamics is the progressive reduction of body weight to 250 grams per day. On the 2nd day the first signs of dehydration.
Determined reconfiguration abdomen. In the upper (epihastralya area) it is swollen at the bottom - hollow. The abdomen is usually mild and painless. If volvulus child restless, tense and painful abdomen.
Radiological studies begin with a sightseeing radiographs of the abdomen in a straight line and lateral projections in the upright position. At high intestinal obstruction in the direct projection shows two gas bubbles with horizontal liquid level, according to the stretched stomach and duodenum, a similar pattern is determined by the images in the lateral projection. Diagnostic value of research increases when the solution of barium sulfate: 1 teaspoon of barium sulfate dissolved in 30-50 ml of breast expressed milk. Other parts of the abdominal cavity not visualized due to lack of gas in the intestines.
Having specified radiological picture and clinical data suggests a diagnosis of high intestinal obstruction undeniable, but his final confirmation GA Bayirov proposes to hold iryhohrafiyu (40-60 ml of water-soluble contrast material) to specify the location of the colon. If the colon, which is partially filled with contrast medium is left, it must be assumed that the newborn midgut volvulus. Clarifying the cause of obstruction allows substantiate the correctness of the calculation time for preoperative preparation.
The differential diagnosis must be made with other congenital diseases that have similar clinical signs.
Pilorospazm evident in the first days of vomiting, which is intermittent and less frequent than in the case of congenital intestinal obstruction. In vomit at pilorospazmi no admixture of bile as obstruction of the duodenum above the large duodenal papilla. Survey radiographs in two projections can clarify the diagnosis by typical congenital intestinal obstruction symptoms and no two bowls (or small number) of gas in the intestines.
Congenital pyloric stenosis, which is characterized by partial obstruction of the intestines, has similar symptoms: epigastric abdominal area with the presence of visible peristalsis of the stomach, constant vomiting, scant feces, hypotrophy. However, permanent color stagnant bile vomit completely exclude the presence of a child's congenital pyloric stenosis. To confirm the diagnosis conducting X-ray examination of the abdomen, during which exhibit a large gas bubble in the extended projection of the stomach, bowel loops is determined by a uniform distribution of gas. To clarify the diagnosis carried out x-ray with contrast material.
In congenital diaphragm hernia may experience vomiting, but the fore signs of dysfunction of the respiratory and cardiovascular systems. X-ray study indicate gut loops moving in the chest cavity.
Birth trauma brain, whether manifested vomiting bile, meconium discharge is not violated. When a brain injury symptoms determined injury of the central nervous system. Diagnosis is confirmed by x-rays and ultrasound.
At low congenital intestinal obstruction are all symptoms of the disease develop slowly: first appears bloating, no discharge of meconium. After entering the vapor tube or enema formulation in infants allocated glomeruli colorless mucus. Vomiting starts later with a 2-3-day old. Vomit stained with bile, then take mekoniyevoho character and odor - "fecal vomiting." On the 2nd - 3rd day after birth appears motor excitation, the child tapping foot, refuses to eat or suck very frail. Condition gradually worsened, growing phenomenon of intoxication, the skin becomes gray and earthy colors, sickly child, adynamic, enlarged abdomen, intestinal peristalsis increases, becomes visible through the anterior abdominal wall. Percussion is timpanichesky shade percussion sound over the entire abdominal wall, auscultatory - liquid, deaf intestinal noises.
Violations of water-electrolyte metabolism and toxicity effects develop slowly, if no necrosis and perforation pereroztyahnenoho blind end of intestine development mekonialnoho peritonitis. Condition of the child deteriorates: continuous vomiting, fever, anterior abdominal wall pasty, pronounced venous pattern, soft tissue perineal and inguinal areas swollen and congested. Anterior abdominal wall is tense. Pa plain film of the abdomen shows dilated bowel loops with multiple levels, which is typical for obstruction of ileum and large intestine. When obstruction empty and ileal intestines, ileus mekoniyevomu define several large gas bubbles and ample fluid levels. Perforation of the intestines extended above the place of obstruction radiographically characterized by the presence of free gas in the subdiaphragmatic space abdomen.
For suspected low intestinal obstruction expedient introduction of water-soluble contrast medium into the rectum, with radiographically defined sharply narrowed colon.
The differential diagnosis of low congenital intestinal obstruction spend with paralytic intestinal obstruction and Hirschsprung's disease.
For paretic ileus typical slow start against a background of severe general illness (peritonitis, pneumonia, sepsis, enterocolitis, etc.).. Meconium in the child normally departs after setting enemas or vapor tube. The introduction of contrast medium into the rectum during X-ray helps in the differential diagnosis, while in paretic intestinal obstruction exhibit well established direct and sigmoid colon with normal clearance.
Hirschsprung's disease (excluding acute decompensation), unlike congenital intestinal obstruction, you can get a discharge of gas and feces conservative method (abdominal massage, the introduction of vapor tube enema formulation). Final diagnosis is confirmed by X-ray contrast study in two projections - Hirschsprung's disease defined expansion of the lumen of the colon with the presence of constricted zone ahanhliozu.
Chronic intestinal obstruction is mainly due to stenosis of the duodenum, squeezing her annular pancreas or embryonic strands. Much less can be stenosis of the distal parts of the digestive system, often as a result of tumor.
Clinical manifestations of partial intestinal obstruction: vomiting occurs, the child is restless, reduced appetite, weight is not increasing. The diagnosis is confirmed by radiopaque study of the digestive system of barium sulfate (oral). Characteristic is suprastenotychne expansion bowel barium sulfate delayed it.
Recurrent intestinal obstruction often associated with midgut volvulus or compression of the intestines of the internal hernia, a fluctuating course with periods of exacerbation of symptoms of acute intestinal obstruction. The child is excited, complains of pain in the abdomen, is a forced situation, in severe cases, the pain develops shock.
Palpable defined sharp pain in the navel, the phenomena of peritonitis, increased peristalsis. After setting all the signs enema temporarily disappear.
Treatment of congenital intestinal obstruction remains a problem in pediatric surgery. Results of treatment depends on early diagnosis, adequate surgical correction of defects, individual preoperative preparation and management of postoperative care.
If the diagnosis is made promptly, in the 1st day after birth, the preoperative preparation lasted 3-6 hours. Perform puncture the subclavian vein (by Seldinherom) baby warm, cordial injected means conduct oxygen therapy, administered through the nose stomach tube to evacuate the contents and gastric lavage to prevent aspiration pneumonia.
If hospitalization of the child later on 2nd - 3rd day after birth, preoperative preparation lasts 12-24 hours in the absence of congenital twisting midgut. In this case, due to the risk of intestinal necrosis, duration of preoperative preparation reduces to 3-4 hours. In the preoperative period children are in incubators at 28-32 ° C, constantly receiving oxygen, actively pursued infusion therapy (10% glucose, protein drugs) and anti-inflammatory therapy.
At low intestinal obstruction preoperative preparation lasts 2-3 hours, due to the severe complications that develop early (bowel perforation, peritonitis), the aim of preoperative preparation is the warming of the child, the introduction of cardiac drugs, vitamins, antibiotics, gastric lavage, in severe cases (for presence of intoxication and hyperthermia) - active infusion therapy.
Chronic and recurrent intestinal obstruction should seek to operate in a planned manner. The extent and duration of preoperative preparation is largely dependent on the patient's general condition and degree of endogenous disorders.
Surgical intervention for congenital intestinal obstruction in newborns: hold right sided paramedian incision length is 9-10 cm nature of the intervention depends on the type of obstruction. When atresia and stenosis of the internal iliac and empty intestines perform bowel resection atrezovanoyi with extended its division (15-20 cm), for the period of deficient in functional terms. Sometimes diameter Drive intestine reduces by intestynoplikatsiyi that keeps absorptive mucosal surface. For the small intestine is physiological anastomosis end to end. For a better comparison of all gut loops using the method Rehbein protybryzhovoho wedge cut edge of the distal end of the intestine. Impose inline ukruchuvanyy atraumatic suture needles.
When atresia and stenosis of the internal duodenum perform longitudinal duodenotomiyu 1 cm above the clutter. Membrane circular cuts to move the mucosa to the intestinal wall. At mucosa after determining the localization of large duodenal papilla impose circular seams. The wound was stitched bifariamous duodenal suture (Figure 3).
In recent years, preferring duodeno-duodenoanastomozu "end-to-end" with wedge resection dilyatovanoyi wall. In the case of wide rings pancreas, pancreatic-biliary bifurcation duct when papilla fateri opens in the upper segment of the lower 12 duodenal ulcer using duodeno-yeyunoanastomoz the short loop "side-to-side" inline inverted seams threads PDS, Maxon 6/0 (T.I.Danshyn, 2003). Remember about technical errors: injured papilla fateri, requiring plastic faterova papilla and not pidshyte window ripples colon above the anastomosis sutures, which can zazemlyuvatysya as driving and discharge of bowel loops.
The syndrome Ladd operation consists of removing twisting midgut by turning around the intestines swell the movement clockwise and fetal autopsy tended to compress the lumen of the duodenum. Do not try to restore the normal position of the cecum and its fixation in the right iliac region, because such manipulation complicate surgery and lead to a recurrence of intestinal obstruction (Fig. 4). T.I.Danshyn and others (2003) form the duodeno-yeyunalnyy curved place by pidvertannya duodenum by the superior mesenteric artery and overlapping the lower horizontal branches 12 duodenal ulcer parietal peritoneum.
During operations in the annular pancreas (Fig. 5) and aberrant vessel shown imposition pozaduobodovoho bypass anastomosis between the duodenum and jejunum.
When mekoniyevomu ileus to eliminate intestinal obstruction resection spend most extended and completed meconium plot using double ileostomiyi by Mikulic (Fig. 6) and U-shaped anastomosis followed by washing the intestinal lumen enzymes of the pancreas and retroperitoneal closing fistulas.
In the postoperative period for 3-4 days spend parenteral nutrition. Stomach constantly draining thin probe through the nasal passage. After feeding the mouth begin only after the restoration of gut motility and appearance of stool.
After surgery for intestinal obstruction Children under medical supervision: determining the dynamics of weight gain, adjust feeding, treats dysbiosis, enzyme deficiency and anemia.
In severe forms of intestinal obstruction after resection of large terminal part of the small intestine and ileocecal valve syndrome occurs short bowel. Enteral feeding leads to diarrhea, steatorrhea, dehydration and exhaustion. Prolonged parenteral nutrition promotes healing children. Metabolic disorders occur much less frequently. But the family of the child should be provided with psycho-social support.
Mortality in the surgical treatment of infants with intestinal malformations in recent years gradually reduced to 10.6% (T.I.Danshyn, 2003). Its main causes are: prematurity, pulmonary pathology, combined severe abnormalities of other organs, heart disease, septic complications, which worsen the underlying disease.

Congenital pyloric stenosis

According to various authors, the incidence of congenital pyloric stenosis ranges from 0.8 to 4 cases per 1000 newborns (YF Isakov, EA Stepanov, T. Krasovska, 1988). The disease is genetically heterogeneous, family and ancestral origin found in nearly 7%. Value for boys and girls 4-1.
It is based on impaired patency vorotarnoyi part (pars pylorica) stomach, which is caused by a bug of its structure: hyperplasia and hypertrophy of smooth muscle fibers of circular (circular) layer, a lack of clear delineation of circular and longitudinal layers, a large number of fibrous connective tissue structures. In pyloric stenosis show degenerative changes of nerve cells.
Clinic and diagnostics. If classic paintings congenital pyloric stenosis diagnosis is not difficult. The main clinical symptoms are:
• vomiting, usually without any mixture of bile in the form of a fountain;
• hiperperystaltyka stomach epigastric by type hourglass;
• weight loss;
• constipation;
• reduce urination.
Depending on the time of the first symptoms and the nature of the disease NS Mankin distinguishes its acute and protracted stage. Analysis of observational data for the children of our clinic indicates that the typical pattern of disease is in children aged 3-4 weeks, which is a severe stenosis of the pylorus.
In newborns the first two weeks of life, preterm infants with pyloric stenosis and mild classical picture of the disease is absent, that, perhaps, is one reason for late diagnosis of the disease.
First there is vomiting a fountain between feedings. Vomit are stagnant, the type of clotted milk with a sour smell, their volume exceeds the volume of each feeding.
Child thinner, there are signs of dehydration, accompanied rarer urination and scanty feces. In the acute form of the disease in the clinic predominate symptoms of dehydration II-III degrees and decompensated alkalosis.
In case of subacute disease symptoms develop slowly at first vomiting, then single or double vomit which frequent and leads to malnutrition. This form is not accompanied by severe disturbances of water and electrolyte metabolism.
On examination, the child pay attention to the severity of malnutrition and exsicosis. The degree of malnutrition is directly dependent on the duration of clinical manifestations of pyloric stenosis. According to our data normotrofiya occurs in 6% of I degree malnutrition in 30.2% of the second degree in 35,6%, III degree of 28.2%. To detect gastric motility child lay on your back and watching it for 10-15 minutes. In doubtful cases, the appearance of peristaltic waves give your child to drink from a bottle teat with 10 ml of 5% glucose solution or milk and easily stroking hand epigastric (upper quadrant of the abdomen). Visible peristalsis of stomach shaped like hourglass: between extended upper and lower wave is clearly narrowing. Periodically, these waves can be repeated. During abdominal palpation can often determine hypertrophied goalkeeper.
While laboratory studies show signs of thickening blood: increased hemoglobin and hematocrit, signs of metabolic alkalosis, hypokalemia, hipohloremiyu. The level of gastrin and somatostatin in children with congenital pyloric stenosis exceeding benchmarks, especially in children with acute course (Solovyov AE, Spahy OV and others 2007).
During the X-ray (5% aqueous suspension of barium sulfate diluted breast milk in volume 50-60 ml) after 30 minutes determined sehmentopodibna peristalsis of the stomach and delay initial evacuation of gastric contents into the duodenum.
In normal newborns and infants complete evacuation of gastric contents occurs within 2.5-3 hours and it starts from the first minutes taking the contrast medium.
In congenital pyloric stenosis observed delay evacuation of gastric contents. Depending on the degree of constriction and spasm of pyloric stomach full release occurs within 6 - 10 hours, and sometimes up to 36 hours. In recent years, radiologists have great diagnostic value of symptoms of constriction and elongation vorotarnoho channel. Vorotarna Cave (antrum pyloricum) is in the form of the beak or thin ribbon. At its edges showing indentation and lengthened, not filled with barium sulfate Between vorotarnoyu cave and duodenal bulb.
The most promising and reliable for the diagnosis of pyloric stenosis is the use fibroezofagogastroduodenoscopy (fibrogastroduodenoscopy).
Fibroezofagogastroduodenoscopy makes it possible to evaluate not only the degree of obstruction vorotarnoyi part of the stomach, but also examine the esophagus, to determine the severity of reflux esophagitis.
Also used sonographic (ultrasound) diagnosis of congenital pyloric stenosis, which, according to Kelleh N. (1987), in 91-100% of cases helps in establishing the diagnosis. With ultrasound pyloric - the presence of gastric pyloric length more than 16 mm increase in diameter pylorus than 14 mm, the thickness of the muscular layer of more than 4 mm.
The differential diagnosis spend with pilorospazmom, psevdopilorostenozom (adrenogenital syndrome Debreu - Fibihera), malformations of the duodenum, gastro-oesophageal reflux.
When pilorospazmi vascular dystonia due for a nice type of the first signs of disease occur shortly after birth - frequent vomiting small amounts, the amount is less than the volume of each feeding, the baby is gaining weight, rarely observed motility of the stomach. Very informative to exclude pilorospazmu is spasmolytic therapy appointment or 0.1% solution of atropine sulfate and 2 drops per 1 kg of body weight per day, the daily dose divided into 3-4 receptions for 15 minutes before feeding. In case of hypertrophic pyloric ste ¬ nozu course of drug therapy is ineffective.
Psevdopilorostenoz (adrenogenital syndrome Debre-Fibihera) - a disease accompanied by vomiting fountain, sometimes mixed with bile, weight loss, weakness, exsicosis, tendency to diarrhea. Signs found in the first 3-4 weeks of life the newborn. The pathogenesis of this disease is complex and consisted in the fact that in the adrenal cortex is disrupted enzymatic synthesis of glucocorticoids, the end product of which is hydrocortisone, which enters the blood. As a result of reduction of its content pituitary hyperfunction occurs that leads to the synthesis of a large number of adrenocorticotropic hormone that stimulates the function of adrenal cortex. Develops their hyperfunction and hyperplasia. Hyperfunction cause the formation of large quantities of androgens and aldosterone, which promotes the excretion of sodium ions. As a result of the loss of sodium and chloride excretion increased potassium in the blood. Therefore, the differential diagnosis of pyloric stenosis is primarily to determine the level of potassium in the blood. In pyloric stenosis contents of the electrolyte in the normal range, sometimes - hypokalemia. Fibrogastroduodenoscopy and x-ray reveals unchanged permeability pylorus (goalkeeper).
If pylorus atresia or complete obstruction by closing its lumen membrane of the stomach or duplications in vorotarniy of vomiting without impurities bile comes from the first hours of life.
Plain radiographs stomach stretched, gas loops colon missing. Not excluded regurgitation of contrast medium in cardiac esophagus.
At high congenital intestinal obstruction at the duodenum vomiting occurs in the 1st day after birth. During X-ray of the abdomen showing two levels of fluid in the stomach and duodenum, while the latter expanded.
For gastroesophageal reflux typical onset practically from birth, manifested vomiting and regurgitation of gastric contents into the baby lying down.
The presence of fibro-ulcerative esophagitis, cardiac ziyannya esophagus and gastro-esophageal reflux confirmed by endoscopic and radiological examination.
Treatment. Congenital pyloric stenosis requires surgical treatment. Preoperative preparation aimed at correcting hypoproteinemia, hypokalemia and alkalosis.
Performed by Fred piloromiotomiyu-Ramshtedtom (Fig. 7). Divide serous layer and stupid by separating the muscular layer to the mucosa prolapse pylorus.
For intraoperative assessment of goalkeeper macroscopically distinguish its two varieties: "Heavy Oliva" (hard, hryaschopodibna consistency) and "soft Oliva" (plotnoelastychna consistency). Acute clinical course option corresponds to "stiff Oliva", subacute and chronic "soft Oliva" (O.V.Spahi and others, 2004).
Recently, the proposed dual piloromiotomiya and use of laparoscopic treatment of congenital hypertrophic pyloric stenosis (A.Ye.Solovyov and others, 2005).
During piloromiotomiyi may have two complications: dissection of the mucous membrane of the duodenum and bleeding from a wound pylorus. Because control is mandatory tsilostnosti mucosal pyloric part hemostasis.
In the absence of complications in 3 - 6 hours after the operation the child begins to drink 5-10 ml isotonic sodium chloride solution, and then expressed breast milk by 5 - 10 ml every 2 hours. Between feedings give 10 ml of 5% glucose solution. On the 2nd day of each feeding volume of 20 ml, 3 rd - 30 ml, 4 th - 40 ml, 5 th - 50 ml. At the 6 th day child gets to 60 - 70 ml of milk every 3 hours, and then be transferred to normal breastfeeding with mandatory daily weighing.
In the postoperative period, especially in the first 2 days, spend parenteral nutrition and infusion therapy aimed at correcting the deficiency of fluid, protein, electrolytes. Also, designate microclysters with Ringer-Locke and 5% glucose devel ¬ way of 25-30 ml 4-5 times a day.
In the long run it may be recurrent pyloric stenosis, due to insufficient muscle layer section relating to technical errors (0.67%). Also after surgery have been violated gastroduodenal junction, characterized by varying degrees of "dysplastic stenosis reflex", which increases the risk of gastro-duodenitis and peptic ulcer 12 duodenal ulcer (O.V.Spahi and others, 2004).
Weather favorable. After discharge from the hospital, the child is under the supervision of a pediatrician and child surgery clinics, where the correction of vitamin deficiency and malnutrition.
Doubling the digestive system
Doubling the digestive system - a malformation of the digestive tube - formed during organogenesis (4-8-week embryonic period). According Vremer the cause doubling is a deviation in the development stage vacuolization primary cancer, which leads to disruption of the digestive tube recanalization. Group vacuoles vidshnurovuyetsya from channel wall, forming isolated or blend with the main lumen tube cavity. The wall is lined with epithelium formation, characteristic of the department of the digestive system. Another theory identifies doubling as a violation of the development process neurenteric canal or clumping neuroectodermal leaf with endo (F. Grob).
Doubling the digestive system is characterized by the following features:
1) the presence of the cavity, which contains a secret and is lined with epithelium;
2) formation of more localized along the lateral edge of any of the digestive system, which can be shared with him the muscle and serosa, and communication with him;
3) the total of the main parts of the digestive system and blood supply innervation.
Formation can be spherical or longitudinal form. There incomplete (spherical cystic doubling dyvertykulopodibni, tubular) and complete doubling. Tubular doubling usually combined with adjacent lumen of the digestive system.
Clinical manifestations depend on the presence of a combination of twice the organ lumen of the digestive system. Isolated forms may compress adjacent parts of the digestive system, causing symptoms of partial or complete obstruction, twisting. The combined form of doubling can run long asymptomatic or manifest acute or chronic gastrointestinal bleeding due to the presence of heterotopic islets in the gastric mucosa at the junction of doubling. Sometimes twice authority may be in the chest cavity. Clinic doubling stomach occurs with signs of obstruction, gastrointestinal bleeding or perforation, and doubling of peritonitis. Sometimes, the doubling of the stomach, determine tumor formation in the upper abdomen with a smooth surface, sharp edges, moving is usually painless. This clinical picture is typical for isolated forms of doubling.
For doubling antrum typical clinic pyloric stenosis: regurgitation, vomiting, discomfort in the epigastric pain. Clinical symptoms in dynamic progress. When there is bleeding bloody vomiting, dihtepodibni emptying in growing phenomenon dynamics anemia.
When massive bleeding develop haemorrhagic shock.
The differential diagnosis spend with tumors of the stomach and retroperitoneum, hydronephrosis. Urology study, ultrasound can eliminate these diseases.
Treatment operative. For it is impossible to remove isolated doubling rezektuyut stomach. For large doubling two options transactions: gastrostomy and marsupializatsiya.
Doubling the small intestine. More than 58% of all cases observed doubling jejunum, ileum mainly.
Symptoms caused by compression of the lumen of the intestine and the violation of its blood supply.
The main clinical symptoms are abdominal pain, nausea, vomiting, defecation disorders, the presence of tumor formation in the abdominal cavity. If inflammation disease is acute course, feigning hospital acute appendicitis. The cause of bleeding is squeezing blood vessels and poor circulation in the nearby department or intestine ulcers conceal ektopirovanoyi mucosa in the oral doubling. This bleeding may be subacute and chronic.
Doubling can cause intestinal intussusception, which generally takes recurrent nature.
Diagnosis of the disease is very difficult.
Anamnestic data indicate the frequency of bleeding from the rectum, recurrent abdominal pain. During abdominal palpation (using muscle relaxants) can identify tumor formation and suspected doubling. Fluoroexam informative in the development of intestinal obstruction due to compression of the colon when expanding drive her department and found the level of fluid in the loops of the small intestine.
Additional information for the final diagnosis is possible when using laparoscopy.
Treatment operative. In isolated and combined forms of doubling, which is located along the mesenteric edge, economical expedient bowel resection with area doubling and blending anastomosis end to end.
When conjugated forms with a narrow base sometimes resort to wedge resection of the bowel. Trying enucleation twice the department may cause damage to the blood vessels that krovopostachayut major intestine, and necrosis of the bowel wall appeared ¬ tion with peritonitis.
Doubling the colon. Doubling the colon relatively rare. The combined forms occurring doubling the type of fistula that department (rektovahinalni, rektovezykalni, rektouretralni) with the development of chronic infection of the urinary and reproductive systems. Sometimes a combination of the mentioned shortcomings noted with a doubling of the urinary and reproductive systems.
Doubling the rectum may open itself out one or two vidhidnykom that septate. When combined forms connected with major parts of the digestive system of clinical symptoms may be absent, and they were mostly found during other surgery or autopsy.
In isolated forms clinically noted intestinal obstruction, bleeding.
Sometimes during palpation and rectal examination determined roundish, slightly elongated, smooth and movable entity that is determined in a side channel or pelvis.
Diagnosis doubling difficult. Perform x-ray (iryhohrafiyu, proctoscopy, fibrokolonoskopiyu). When descending colon doubling and rectum when there is communication with the urinary and genital systems, transmitting color samples or double staining using X-ray.
Treatment. For all types of doubling the colon indicated surgery. In cystic and tubular forms shown resection doubling the plot healthy colon and overlapping end anastomosis in the end. Ryhl (Czech Republic, 2007) showed that the ileocecal resection leads to the risk of ulcers of anastomoses in the remote period, as in the cases of iron deficiency anemia in these children, and a positive test for "occult blood" should be performed colonoscopy.
When tubular forms doubling when there is communication with the vagina, bladder or urethra, conduct complex multi-stage operation. Voles vagina recommended to close in the second stage of the operation at an older age.
Operative intervention on tubular doublings rectum conducted according to the principles which apply when fistulas anomalies rectum.

Materials for self-

Situational tasks
Task 1. Newborn to 1 day, hospitalized with complaints of vomiting, restlessness. On examination: abdomen swollen epigastric, rectal meconium gray. Plain radiographs of the abdomen in the upright position there are two liquid level and two gas bubble.
1. What is the most likely diagnosis?
2. Tactics physician in detecting the disease.
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Task 2. Newborn, went to hospital with a clinical picture of intestinal obstruction. Determined restlessness, vomiting greens, swollen abdomen, lack of bowel movements, slight admixture of blood from the rectum. Made plain film abdomen in upright iryhohrafiya, which defines high position of the cecum in the left upper quadrant.

1. What is the most likely diagnosis?
2. Tactics physician in detecting the disease.
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 3. Infant 1 month. For two weeks after each feeding baby blyuye clotted milk. Losing weight is 15 percent, the state hard, second-degree malnutrition. Abdomen palpation accessible, visible peristalsis of the stomach in the form of "hourglass".

1. What is the most likely diagnosis?
2. Tactics physician in detecting the disease.
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 4. Baby 3 days old, moved from a house with a sexual clinic congenital intestinal obstruction. During surgery revealed mekoniyevyy ileus.

1. What caused this pathology?
2. Tactics physician in detecting the disease.
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 5. Newborn to 5 days, hospitalized with complaints of vomiting, restlessness. On examination: abdomen swollen epigastric, asymmetrical. From the rectum meconium gray. Plain radiographs of the abdomen in the upright position there are two liquid level and two gas bubble.

1. What is the most likely diagnosis?
2. What examination is necessary to determine the cause for this disease?
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 6. Baby 3 nights that hospitalized in the surgical ward of the clinic congenital intestinal obstruction: with mixed with bile vomiting, anxiety child, the presence of tumor formation in the abdominal cavity, made viewing and contrast X-ray examination of the abdomen in the upright position. Determine the level two liquid and two gas bubble on iryhohrami - high position of the cecum in the left upper quadrant.

1. What is the most likely diagnosis?
2. Tactics physician in detecting the disease.
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 7. Newborn to 1 day, hospitalized with complaints of vomiting, restlessness. On examination: abdomen swollen epigastric, rectal meconium gray. Plain radiographs of the abdomen in the upright position there are two liquid level and two gas bubble.

1. What is the most likely diagnosis?
2. What diagnostic methods necessary to the patient to determine the factor of disease?
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Task 8. Newborn, went to hospital with a clinical picture of intestinal obstruction. Determined restlessness, vomiting greens, swollen abdomen, lack of bowel movements, slight admixture of blood from the rectum. Made iryhohrama with barium mixture which defined high position of the cecum in the left upper quadrant.

1. What is the most likely diagnosis?
2. What diseases should conduct differential diagnosis?
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 9. Infant 3 weeks. Even a week after each feeding baby blyuye clotted milk. Losing weight is 10 percent, the state hard, second-degree malnutrition. Abdomen available palpation, gastric peristalsis defined as "hourglass".

1. What is the most likely diagnosis?
2. What diseases should conduct a differential diagnosis?
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Problem 10. Newborn to 5 days, hospitalized with complaints of vomiting, restlessness. On examination: abdomen swollen epigastric, asymmetrical. From the rectum meconium gray. Plain radiographs of the abdomen in the upright position there are two liquid level and two gas bubble.

1. What is the most likely diagnosis?
2. What diseases carried differential diagnosis?
3. Etiology and pathogenesis of disease.
4. Name the main areas of treatment.
5. What features of clinical supervision for the child after recovery?

Tests.
1. Baby 3 nights that hospitalized in the surgical ward of the clinic congenital low intestinal obstruction survey performed RO - gram of the abdomen in the upright position. What radiographic signs we expect to get?

A. Lack of gas in the intestine "dumb stomach"
B. The presence of two levels of liquid and gas bubbles
C. The presence of many large liquid levels and low gas bubbles
D. Increased intestinal gas filling evenly
E. Sharp advanced gas colon

2. Newborn to 1 day, hospitalized with complaints of vomiting, restlessness. On examination: abdomen swollen epigastric, rectal meconium gray. Plain radiographs of the abdomen in the upright position there are two liquid level and two gas bubble. What kind of pathology should think a child?

A. Congenital pyloric stenosis
B. Congenital low intestinal obstruction
S. paralytic intestinal obstruction
D. Congenital duodenal obstruction high
E. Pilorospazm

3. A newborn who came to the hospital with a clinical picture of intestinal obstruction, defined anxiety, vomiting greens, swollen belly, no stool, slight admixture of blood from the rectum. Made plain film abdomen in upright iryhohrafiya, which defines high position of the cecum in the left upper quadrant. What possible kind of obstruction?

A. Ladd Syndrome
B. Acute Hirschsprung disease
S. Atresia ileum
D. Duodenal atresia
E. annular pancreas

4. All defects that manifest congenital obstruction grouped into four main groups, depending on the violation utero. What bug development, which refers to a group of defects, disorders of embryogenesis of the intestinal wall.

A. Ladd Syndrome
V. Mekoniyeva obstruction
S. annular pancreas
D. The acute form of Hirschsprung disease
E. form membranous atresia

5. All defects that manifest congenital intestinal obstruction grouped into four main groups according to the type of violation utero. For one of them include defects that are caused by violation of the fetal circulation intestines. What type of obstruction may be in violation of the second phase circulation guts?

A. Mekoniyeva obstruction
V. annular pancreas
S. membranous atresia Form 12 - duodenum
D. Syndrome Ladd
E. Shnuropodibna form ileum atresia

6. All defects that manifest congenital intestinal obstruction grouped into four main groups, depending on the type of violations of fetal development. What type of intestinal obstruction refers to a group of malformations that are caused by violation of the abdominal cavity?

A. Ladd Syndrome
V. Membranous atresia form
S. Isolated torsion of the intestine
D. Annular pancreas
E. Shnuropodibna form atresia

7. All defects intestine, manifested symptoms of congenital intestinal obstruction grouped into four main groups according to the type of violation utero. What type of obstruction include a group of birth defects that are caused by violation of embryogenesis intestinal tube?

A. Ladd Syndrome
V. annular pancreas
S. membranous atresia form