Disease Hirschsprung refers to malformationsleft half of the large intestine. It is characterized by the absence of ganglion cells in the submucosal and intermuscular plexus. Disease Hirschsprung in children occurs in a ratio of 1: 5000 cases. In adolescents, it is found much less often. Boys are sick five times more often than girls. For this pathological condition is characterized by the family nature of inheritance. In some cases, the disease is associated with the emergence of Down syndrome. In adults, Hirschsprung's disease occurs with the condition of localization in the anorectal area. In severe cases, it can spread to the distal part of the small intestine.
In the pathogenesis of the disease, a significant role is played byabsence of influence of the parasympathetic department of the nervous system. The result of this condition is a delay in the relaxation of the internal sphincter of the rectum, which leads to a permanent spastic contraction, oppression of the peristalsis, expansion and hypertrophy. Clinically, the disease manifests itself as a condition called the megacolon.
Hirschsprung disease can occur incompensated, subcompensated and decompensated form. The severity of the pathological condition directly depends on the length of the anglionic site. The decompensated form is manifested by symptoms of severe intestinal obstruction.
Characteristic signs of the disease areintense pains, bloating, rumbling in the abdomen, constipation. The stool delay is chronic (in some cases it reaches a month). Bloating is also a constant sign of the disease, is severe and can lead to difficulty breathing the baby. Bloating remains even after emptying the bowels with enemas. Its consequence can be called the emergence of intense pain. Nausea, vomiting, weakness, deterioration of appetite are added to the main symptoms of the disease. Complications of this pathological condition are anemia, deformation of the rib corners. The presence of inflammatory processes, ulceration, dysbiosis can lead to the emergence of persistent diarrhea, which cause the exhaustion, the collapoid state.
Diagnosis of Hirschsprung's disease begins withexternal inspection. There is an increase in the abdominal area due to constant swelling, peristaltic contractions of the affected bowel, dense stool masses. The change in the contractility of the anal sphincter is determined by finger research. X-ray in the vertical position allows you to get fairly accurate results. There is a change in the intestinal pattern due to the presence of a significant amount of gases that cause obstruction. Other instrumental methods that are used to diagnose this pathological condition are colonoscopy, irrigoscopy. The latter is used to determine the extent and localization of constriction, the diameter of the suprastenotic zone of the colon.
In order to verify the diagnosis is appliedtransanal biopsy. Reduction of the nerve ganglia of the intermuscular plexus is a sign that confirms Hirschsprung's disease. During the operation, this method helps to determine the level of resection.
Treatment of this pathological condition dependsfrom the severity of its current. Compensated forms can be eliminated by conservative methods of therapy, in other cases, urgent surgical intervention is required, which consists in applying a colonostomy, removing the aganglionic zone, decompensated, overly dilated parts of the large intestine. Mortality after treatment is up to four percent.
