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Inborn megacolon - Diseases of digestive organs at children

Table of contents
Diseases of digestive organs at children
Oral cavity
Diseases of teeth
Malformations of the sky and soft tissues of an oral cavity
Diseases of a mucous membrane of an oral cavity and gums
Diseases of lips and language
Sialadens
Digestive tract
Basic reasons of gastrointestinal frustration
Gullet
Atresia and esophageal and tracheal fistula
Guttural and tracheal and esophageal crevice, inborn stenosis of a gullet
Other diseases of a gullet
Stomach and intestines
Peptic ulcer
Inborn hypertrophic pyloric stenosis
Inborn impassability of intestines
Inborn impassability of a duodenum
Disturbances of turn of intestines
Inborn impassability of a small bowel
Inborn megacolon
Diverticulums and duplikatura
The acquired impassability of intestines
Intestines invagination
Foreign bodys of a stomach and intestines
Motive frustration. stomach and intestines
Anomalies of a structure of anorectal area
Infectious diseases of intestines
Nonspecific ulcer colitis
Illness Krone
Necrotic coloenteritis of newborns
The coloenteritis connected with treatment by antibiotics
Gastrointestinal symptoms of anaphylactoid purpura, gemolitiko-uraemic syndrome
Intolerance of food proteins
Eosinophilic gastroenteritis
Absorption disturbance syndromes
Malabsorption
Immunodeficiency and intestines
Syndrome of "a congestive loop"
Syndrome of a short small bowel
Gee's disease
Sprue after acute enteritis
Tropical to a spr
Whipple's illness, intestines lymphangiectasia, Uolmap's illness, idiopathic diffusion defeat of mucous
Enzymopathies and disturbances of mechanisms of transport of nutrients
Irritable colon
Acute appendicitis
Diseases of an anus, direct and large intestine
Tumors of a digestive tract at children
Hernias of a digestive tract at children
Exocrine part of a pancreas
Pancreatitis
Embryonic development of structure and function of a liver
Diagnosis of diseases of a liver
Cholestatic states at babies
Parenchymatous jaundices at children of chest age
Disturbances of a metabolism of a liver and zhelchevydelitelny system
Anomalies of a structure of bilious ways
Cysts of bilious channels
Cholestasia and diseases of a liver connected with completely parenteral food
Medicinal damage of a liver
Ray's syndrome
Chronic hepatitis
Wilson's illness — Konovalova
Indian juvenile cirrhosis
Damages of a liver at chronic colitis
Cirrhosis and chronic liver failure
Portal hypertensia and varicosity of a gullet
Fatty infiltration of a liver
Cholecystitis
Peritoneum diseases
Peritonitis
Peritoneum hernias
INBORN MEGACOLON (illness of Girshprunga)

The illness of Girshprunga makes a third of all cases of impassability of intestines at newborns (though it is seldom observed at premature children) and is a basic reason of impassability of a large intestine. Sometimes this illness has family character. The atresia of a large intestine meets extremely seldom.
Etiology. Believe that as an etiology serves inability of cells of an embryonal nervous rudiment to migrate in a wall of a large intestine or inability of intramural and submucous plexuses grow together with an intestinal wall in the caudal and cranial direction.
Pathogeny. Inborn megacolon is characterized by lack of ganglionic cells in a wall of a large intestine on a certain site which usually begins at an anus. At 80% of patients the denervated segment is limited to rectosigmoid area, at 15% process extends to the right bend of a colon and at 3% all large intestine is deprived of ganglionic cells.
The defective parasympathetic innervation of an aganglionarny segment leads to disturbance of a vermicular movement, locks and functional impassability of intestines. Proksimalny a transitional zone between normally innervated gut and department of a gut with an insufficient innervation the muscular hypertrophy which leads to a thickening of an intestinal wall develops. The fecal masses and gases accumulating in intestines cause its sharp expansion.
Clinical manifestations. The first symptoms of megacolon are very various: from full acute impassability at newborns to chronic locks at children of advanced age. Often physical development of the child is late; ponosa are sometimes noted.
At newborns symptoms can arise right after the birth or throughout the first pedela of life in the form of vomiting, increase in a stomach and absence a calla. The temporary relief can come after manual research of a rectum when there is a sudden rough otkhozhdeniye a calla and gases. Emetic masses is painted by bile and has a muddy appearance; the child grows thin, the expressed dehydration develops. In the neonatal period diarrhea which is combined with symptoms of impassability of intestines can become the leading sign. The enteropathy connected with protein loss is followed by a hypoproteinemia and hypostases.
The periods of locks and ponos alternate with the periods of normal functioning of intestines. Diarrhea can pass into the fulminant coloenteritis causing the most severe dehydration owing to loss of liquid and electrolytes through an intestinal wall. At a fulminant coloenteritis it is not possible to allocate any specific microorganisms. If not to take vigorous measures, then the disease quickly progresses and can end letalno already by the end of the first days. The condition of the child worsens also owing to intestines stretching a stake and gases.
The illness of Girshprunga causes locks and increase in a stomach in children of advanced age. When collecting the anamnesis it becomes clear that from the first days of life the child experiences difficulties at bowel emptying. In the left lower quadrant of a stomach dense fecal masses is palpated, but manual research shows that the rectum is not expanded and free from a calla. Kal consists of small lumps, sometimes taenioid or has a liquid consistence. Patients to functional locks have no plentiful chair and fecal "stones". In mild cases the condition of food of the child significantly does not change; at a heavy current hypodermic cellulose disappears and there is a growth inhibition. Thin extremities and a big evaginated stomach are characteristic of such children, however in some cases this picture can be taken for an absorption disturbance syndrome (especially at ponosa). Mine { et to develop hypochromia anemia. The impassability attacks caused by obstruction of a gut fecal masses are followed by pains and temperature increase. This disease needs to be distinguished from more often found acquired megacolon (fig. 12-14), a chronic idiopathic lock and other states (tab. 12-7).
толстая кишка - запоры
Fig. 12-14. The roentgenogram (after a barium enema) a large intestine of 14 flyings of the boy suffering from heavy locks. Excessive expansion of a direct and sigmoid gut is characteristic or the acquired megacolon.
Rare option of inborn megacolon is extremely short aganglionarny segment limited to a zone of an internal proctal sphincter, the proctal channel and the closest piece of a rectum. Such patients can suffer from an incontience a calla. Biopsy material needs to be taken from the most distal site of a gut, otherwise it is possible to find ganglionic cells in a bioptata and to make the wrong diagnosis.
Diagnosis. Authentically it is possible to prove availability of inborn megacolon only by means of a puncture or aspiration biopsy. In a bnoptata lack of ganglionic cells in submucous and intermuscular neuroplexes in combination with the normal or increased amount of nerve fibrils comes to light. As in distal department of a rectum and the proctal channel the quantity of ganglionic cells is reduced even at healthy faces, bioptat it is necessary to take at distance not less than 2 cm from the crested line.
The radiological survey of children of chest age with the impassability of intestines caused by an illness of Girshprunga reveals expanded loops of intestines (the direct roentgenogram at vertical position of the patient). On lateral roentgenograms gas in a rectum, usually visible in presakralny area is not observed. At an irrigoskopiya the following radiological signs are defined: 1) sharp narrowing of the gut segment deprived of ganglionic cells

Table 12-7. The comparative characteristic of the inborn and acquired megacolon


Symptoms

Inborn
megacolon

Acquired
megacolon

Anamnesis

 

 

Since the birth Coloenteritis
Rectal bleeding the Habit to empty intestines the Incontience a calla the Volume of fecal masses

Always
It is possible
No
No
Never
Small

Never Is absent Perhaps Usually is available Always
Very big

Inspection

 

 

Reduced food Increase in a stomach the Hypersthenic thorax the Palpation of fecal masses through an abdominal wall Proctal treshchiia the Tone of a proctal sphincter Fecal masses in a rectum ampoule

Perhaps
It is characteristic
Usually
Never
It is raised
No

It is not observed
No
Often
Are possible
It is reduced
The ampoule is hammered-i scrap

Barium enema

 

 

Empty ampoule of a rectum of Fekalom in an ampoule an evacuation Delay

It is usual
No
It is usual

It is not observed Always
It is not observed

Biopsy

 

 

Ganglionic cells

No

Are found

Note. The illness of Girshprunga with very short struck segment has signs of the acquired megacolon.
(fig. 12-15); 2) the uneven "jagged" contours of the struck segment; 3) parallel cross folds in expanded proximal department of a gut; 4) reinforced, knotty, edematous mucous membrane of proximal department of a large intestine characteristic of a belkovodefitsitny epteropatiya; 5) delay of evacuation of baric weight. Inspecting newborns, it is necessary to enter only very small amount of a contrast agent through a thin catheter which end have at once behind a sphincter. Procedure is carried out under roentgenoscopic control in a slanting position. Characteristic sharp change of caliber of a gut can be not noticed if a large number of baric weight is entered.
The barium enema at newborns with the impassability of intestines caused by an illness of Girshprunga not always reveals typical signs as distinctions in sizes of the struck and not struck part of a gut did not manage to develop yet. The X-ray pattern is even less characteristic when all large intestine is deprived of ganglionic cells. It should be noted, however, that at such patients a contrast agent is late in a large intestine of more than a day.
болезнь  Гиршпрунга
Fig. 12-15. The roentgenogram (after a barium enema) a large intestine of the 3-year-old girl with an illness of Girshprunga. The Aganglionarny distal segment is narrowed, over it the stretched normally innervated site of a large intestine is visible.
The anorectal manometriya by method of inflating of the cylinder placed in a rectum ampoule showed that at increase of intra rectal pressure the tone of an internal proctal sphincter at the healthy child sharply falls, and at an illness of Girshprunga increases. Accuracy of this diagnostic reception exceeds 90%, however at newborns it yields less reliable results.
Megacolon can be diagnosed for children of advanced age on the basis of locks which the child suffers since the birth, and detection of an empty ampoule of a rectum at manual research. As confirmation serve results of rentgenokoptrastny research (see fig. 12-15) and a proctal manometriya. It must be kept in mind that X-ray inspection can give the wrong idea of character and vyrazhepnostn pathological process. At children with suspicion on megacolon it is reasonable to carry out an irrigoskopiya without preliminary cleaning of intestines more accurately to reveal a difference between the struck and intact segment of a gut.
Treatment. If at the newborn the diagnosis of inborn megacolon is precisely established, then surgical intervention is shown. After a laparotomy take a large number of bioptat and impose kolosty in the most distal part of normally innervated gut. Some surgeons impose kolosty in the right half of a cross colon without preliminary biopsy. They believe that such kolostoma is adequate to the most common form of a disease at which the aganglionarny segment extends to a rectosigmoid zone. If the upper bound of an affected area lies at the level of the left bend or proksimalny, then, apparently, instead of a kolostoma it is necessary to impose a transverzoanalny anastomosis to avoid removal of an intermediate piece of a healthy gut. For simplification of care of children from kolostomy several fine models of kalopriyemnik are created.
Treatment by means of enteroclyses until the child does not grow up, was not repaid as it is accompanied by danger of development of a life-threatening coloenteritis. At early imposing of a kolostoma mortality from a coloenteritis makes 4%, and during creation of a kolostoma after development of a coloenteritis — 33%. At the age of 6 — 12 months carry out radical having risen novitelpy operation on Svepsott, Duhamel or the modified method to Soava. At the same time delete the segment deprived gangliyev, and reduce the remained healthy gut to an anus where create an anastomosis with the proctal channel of a pas distance about 2,5 cm from the crested line.
At children of advanced age surgical treatment is also begun with colostomy, but after operation on Svensson and Duhamel an ostomy leave open for some time.
Ultrashort aganglionarny segment at an illness of Girshprunga. If the struck segment is so short that radiological and clinical signs of an illness are indistinguishable from those at the acquired megacolon, then extensive surgical intervention is inexpedient. When conservative treatment is inefficient, it is enough to carry out excision of a small cavity of an internal proctal sphincter (an internal proctal mioektomiya).
Total aganglioz large intestine. Tactics of treatment of this defeat which is often extending to terminal part of an ileal gut consists in imposing of an ileoanalny anastomosis. However at the same time the incontience of fecal masses from which excoriation of skin of a perianal zone, extremely resistant to treatment, and buttocks results is possible.
Forecast. Results of treatment of an illness of Girshprupga generally satisfactory; at most of patients ability to hold kcal remains. As in most cases the illness is distinguished and treat in the neonatal period, it is impossible to estimate a deduction mechanism condition right after operation. Development of a normal rhythm of bowel emptying usually drags on; within several years the periodic incontience from ponosa is observed, however over time at most of children the chair is normalized.



 
"Diseases of bodies of an urinary system at children   Diseases of the lacrimal bodies"