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Disease of Girshprunga


Disease of Girshprunga (N. of Hirschsprung, 1887) - the malformation of the left half of a large intestine caused by total absence of ganglionic cells in intermuscular (auerbakhovy) and submucosal (meyssnerovy) textures. Aganglionarny megacolon as a matter of fact is a neurogenic form of intestinal impassability. The disease is inborn pathology (1:5000 been born children are marked out in the ratio). It is more often shown at children and teenagers, is more rare at adults. Boys are ill 4 - 5 times more often than girls. The disease of Girshprunga has the family character caused by an abnormal locus in a structure of the 10th chromosome. About 5% of children with a disease of Girshprunga have a Down syndrome. If the aganglionarny site of a gut small is also localized in anorectal department, then symptoms of a disease can appear at mature age. This type of a disease call "a disease of Girshprunga of adults".

Bolezni Girshprunga symptoms:

At a short aganglionarny segment symptoms of a disease are easily stopped by purpose of cleansing enemas and laxatives, at long - the disease can proceed in the form of obturatsionny impassability.

On a clinical current distinguish compensated (easy), subcompensated (medium-weight) and dekompensirovanny (heavy) forms. Weight of a disease depends on the extent of the aganglionarny site of a large intestine. At the small extent of the aganglionarny site at children the easy form of a disease develops. Good care of the child and regular bowel emptying by means of enemas or colonic tubes promote long compensation. At a dekompensirovanny form from the first days of life the disease is shown by symptoms of low colic impassability.

Disturbance of a passage of intestinal contents and accumulation a calla in the form of "stopper" from first weeks of life define clinical displays of a disease of Girshprunga which main symptoms are locks, swelling, pains, abdominal murmur. Locks happen persistent and are observed from the moment of the birth of the child or at early children's age. The delay of a chair can be long - from several days to several months. A constant symptom of a disease is also the abdominal distention (meteorism) reaching sometimes such degree that complicates breath of the patient. It is characteristic that swelling remains even after bowel emptying by means of an enema. Owing to a lock and abdominal distention there are pains on all stomach disappearing later bowel emptyings. The long delay of a chair can be followed by weakness, nausea, vomiting, deterioration in appetite, at children anemia, a hypotrophy, expansion of a costal corner of a thorax develop. Sometimes the lock is replaced by the diarrhea (a paradoxical diarrhea) lasting several days and leading to an exhaustion, and sometimes and to a kollaptoidny condition of the patient. The diarrhea is caused by inflammatory changes in expanded loops of a gut, sometimes an ulceration of a mucous membrane, dysbacteriosis.

At survey reveal increase in a stomach (a constant meteorism), sometimes it is possible to observe peristaltic reductions of a gut, to probe dense fecal masses. At a manual research of a rectum define increase in a tone of a sphincter of an anus, an empty ampoule spastic of the reduced rectum. At X-ray inspection in vertical position the intestines loops characteristic of intestinal impassability inflated by gas come to light. During a kolonoskopiya because of a rectum spasm in the beginning ректоскоп moves ahead hardly, and then it as if fails in its expanded part which is always filled with the fecal masses and fecal stones. Irrigoskopiya allows to reveal a narrowing zone, its extent and localization, extent of an expanded suprastenotichesky zone of a large intestine which diameter usually makes 10-15 cm. When studying a passage of a baric suspension on a digestive tract reveal long (within several days) a suspension delay in expanded departments of a large intestine.

The transproctal biopsy of a rectum allows to verify the diagnosis. At a histologic research of a bioptat reveal аганглиоз or sharp reduction ганглиев in intermuscular neuroplex. The biopsy during operation is especially valuable to determination of level of a resection of the struck department of a gut.

At slow development of process patients long enough can compensate it, trying to obtain bowel emptying by means of cleansing enemas, and only at a decompensation (already at mature age) there are indications to surgical treatment.

Complications. The disease of Girshprunga can be complicated by intestinal impassability, an inflammation and an ulceration of a mucous membrane in expanded departments of a large intestine, perforation of intestines, krovotechekniy.

Etiologies of Girshprunga:

The Aganglionarny zone most often begins from the anorectal line and in 80-90% of observations extends to rectosigmoid department, in 10% it extends to a splenic bend or to all large intestine and distal department of a small bowel. Lack of a parasympathetic innervation leads to a delay of a relaxation of an internal sphincter of a rectum, also the lack of synthesis of nitrogen oxide (NO) causing a sphincter relaxation is noted. The site of a gut deprived intramural nervous ганглиев constantly spastic is reduced, does not peristaltirut, the departments of a gut having a normal innervation as a result above-located extend and hypertrophy, there is a megacolon.

Bolezni Girshprunga treatment:

Conservative treatment is only a preparatory stage to operation. It consists in the measures directed to bowel emptying (a dietary regimen, purgatives, cleansing enemas, about kinetics - координакс, etc.). Intestinal impassability which does not manage to be resolved conservative actions serves as the indication to imposing of a kolostoma on proximal departments of a large intestine. Many specialists consider colostomy as the first stage of operation (training of the patient for radical surgical intervention). Surgical treatment consists an aganglionarny zone and dekompensirovanny expanded departments of a large intestine with the subsequent recovery of passability of intestines at a distance. At children Duhamel's operation, Soava, Svensson is most often applied. The postoperative lethality, as a rule, does not exceed in recent years 2-4%.

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