The disease of Girshprunga is the disease resulting from congenital violation of development of the nervous textures providing an innervation of a large intestine. Pathology is followed by locks and violation of absorption in a thick gut. Easily proceeding disease of Girshprunga can be shown only at adult age, the heavy current is characterized by a meconium neotkhozhdeniye (first-born a calla) at the newborn. The disease is diagnosed by means of a X-ray contrast and endoscopic research of intestines. Treatment surgical – is made removal of the struck part of a gut.
Disease of Girshprunga
Disease of Girshprunga (, congenital megacolon) – the congenital malformation of a digestive tract which is characterized by violation of an innervation of a part of a large intestine or its total defeat. Depending on localization and the extent of an aganglionarny zone in a proktologiya allocate rectal, rektosigmoidalny, segmentary, subtotal and total forms of a disease of Girshprunga. Men have this pathology four times more often than women. In most cases (90%) the diagnosis is exposed to newborn babies. At 20% of newborns at the same time reveal other congenital pathologies of nervous system, and also a disease of cardiovascular system, digestive organs and urinogenital frustration. With a Down syndrome the disease of Girshprunga occurs at children by 10 times more often than at persons without chromosomal violations.
Reasons and risk factors
The disease of Girshprunga develops vnutriutrobno. During incubation to a heel on the twelfth week there is a formation of the nervous congestions further providing an innervation of a gastrointestinal tract. Formation begins from nervous textures in a mouth and goes down on a gullet to a stomach, intestines and an anus. In certain cases for the obscure reasons this process is interrupted that leads to lack of nervous textures on the site of a thick gut. Extent of not innervated site can be various and make as several centimeters, and to include all large intestine. Extent of the thick gut deprived of nervous textures also causes weight of a course of a disease.
It is supposed what violations of development of nervous system of a large intestine is caused by mutations in gene structure of DNA. As the disease of Girshprunga has the congenital and hereditary nature, probability of development of this pathology in a family where one child already suffers from it, much higher. Besides, it is necessary to pay attention that boys by 4-5 times more likely can have this disease.
Symptoms of a disease of Girshprunga
Expressiveness of clinical symptomatology is various, depending on weight (extent of damage of intestines). Most often symptoms are found right after the birth, but they can sometimes come to light at teenage or youthful age. Symptoms of a disease of Girshprunga at newborns: in the first days after the birth meconium does not depart (first-born kcal), there is vomiting (sometimes with bile), locks or diarrhea, the increased gas generation.
At children of more advanced age tendency to persistent locks, the stomach increased in volume, the lag in growth and development connected with the broken absorption and development of a syndrome of malabsorption can be noted. Anemia owing to continuous losses of blood with a stake, besides, perhaps accession of an infection and development of an enterokolit is quite often noted that is shown by temperature increase of a body, diarrhea and vomiting. At the expressed intoxication expansion of a thick gut can be noted.
Diagnostics and inspection
The diagnosis is exposed after survey of the proctologist and carrying out the corresponding researches. As diagnostic measures of detection of a disease of Girshprunga apply tool procedures and functional tests. At roentgenoscopy of an abdominal cavity sites of expansion of a large intestine come to light, and absence in it a calla above an anus is found. If to make an irrigoskopiya after an enema with contrast substance, it is possible to visualize well walls of intestines and to find the narrowed and expanded sites.
The disease of Girshprunga is characterized by lack of a reflex of weakening of an internal sphincter at rectum ampoule stretching. Lack of this reflex can be revealed by means of an anorektalny manometriya (the research allowing to measure pressure in thick intestines and an internal sphincter). For confirmation of lack of nervous cages in a wall of a thick gut carry out a kolonoskopiya during which take a biopsy, and then make a histologic research.
Treatment of a disease of Girshprunga
The only effective way of elimination of pathology is surgical treatment. Operation consists in excision of an affected area of a thick gut and connection of healthy department with the terminal site of a rectum. In case of one-stage surgery all stages are made at once, but in certain cases choose two-moment operation. The first stage of two-moment operational tactics includes removal of an affected area of intestines (resection) and removal of an extremity of a healthy gut through an operational section on a stomach (kolostomiya). Removal a calla happens in the special capacity which carries with itself sick.
After adaptation of the patient to new conditions of digestion in the shortened intestines make the second stage of expeditious treatment - reconstructive operation during which the free end of healthy intestines is connected to a rectum, and take in an opening on a stomach (closing of a kolostoma). After surgical treatment patients usually have a gradual normalization of a chair though at some diarrhea originally can be noted. Sometimes patients note tendency to locks which are, as a rule, resolved after reception of depletive.
At diarrhea or locks for regulation of activity of intestines by the patient after operation for a disease of Girshprunga the diet rich with vegetable cellulose can be recommended. After operation the risk of infection of intestines and development of an enterokolit is high. In case of emergence in the postoperative period of the following symptomatology: fever, diarrhea, vomiting, an abdominal distension, symptoms of intestinal bleeding – it is necessary to see a doctor at once.
At easily proceeding disease of Girshprunga in case of damage of intestines on the site of small extent it is possible to refuse surgical treatment and it will be limited to siphon enemas. Enemas do throughout all life. It is necessary to make them with a large amount of water (to two liters), for simplification of passing of kalovy masses inside accept vaseline oil. Besides, the special remedial gymnastics is recommended to patients with this pathology.
Forecast and prevention
At a timely initiation of treatment forecast usually satisfactory. About 80% of the babies who are not receiving treatment perish in the first months of life. Data on restoration of functions of intestines after operations differ. About 1% of patients is required imposing of a constant kolostoma because of an incontience of kalovy masses. The disease of Girshprunga has the congenital, genetically caused character and cannot be warned. Early detection of a disease and timely rendering medical assistance promotes improvement of quality of life of patients.