Disease of Girshprunga

Disease of Girshprunga – one of the most widespread anomalies of development of a large intestine which is shown by lack of an innervation of a certain department or all gut. The disease of Girshprunga has a genetic etiology. The disease is characterized by lack of ganglionic cells in distal department of intestines. The disease has hereditary character and is caused by a mutation in the 10th chromosome. At untimely diagnosis can provoke development of acute inflammatory processes in intestines, and also lead to a lethal outcome.

Pathogeny of a disease of Girshprunga

Ganglionic (or ganglionic) cells – the general name of some types of large neurons which in intestines are responsible for communication between nervous structures. During ontogenesis ganglionic cells migrate from upper parts of intestines to its distal department. Disturbance of migration leads to formation of the aganglionarny site of a gut (a zone of functional narrowing) with the lowered or completely absent innervation (ability to make forward сокращательные the movements). In certain cases the aganglionarny site can abnormally extend, creating megacolon that is one of serious complications of a disease of Girshprunga.

The place of localization of the aganglionarny site, as a rule, is the distal department of a large intestine. In rare instances forms of a disease of Girshprunga at which the aganglionarny segment forms on the site from a splenic column, or develops аганглиоз all large intestine meet. At such form of a disease at patients total paralysis of a vermicular movement is observed.

Disease of Girshprunga at children

Owing to the etiology the disease of Girshprunga is diagnosed for newborns or in the first year of their life. However there are special forms of a disease which fully develop only in an adult organism. Both the hereditary factor, and disturbance of pre-natal development is the reasons of a course of a disease of Girshprunga at children in ontogenesis. Frequency of diagnosing of a disease of Girshprunga at children makes about 1:5000. The disease is more often diagnosed for boys, than for girls (5:1). At timely diagnosis and treatment the forecast favorable. At most of patients the peristaltics of intestines completely renews and the natural excrement is normalized. At 1% of patients need of a kolostoma (brought to a front abdominal wall and the opened end of a colon fixed there) for removal of fecal masses and gases remains.

Disease of Girshprunga: symptoms, diagnostic methods

The main symptoms of a disease of Girshprunga are:

• Locks;
• Nausea, vomiting;
Abdominal
• cavity pains;
• Abdominal distention, meteorism.

The aganglionic segment of a large intestine plays a role of a functional stenosis above which abnormal expansion of a gut where there is accumulation of fecal masses forms. Expressiveness of symptoms of a disease of Girshprunga will be directly proportional to the extent of a segment of the struck gut.

In many cases at a palpation accumulation of fecal masses is found. The main symptom of a disease of Girshprunga at children is the meconium otkhozhdeniye delay in the first 24-48 hours. At some children development of symptoms can begin only during the period an otnyatiya from a breast and upon transition to firm food.

In rare instances at a disease of Girshprunga which symptoms are very similar to symptoms at intestinal impassability at patients the coloenteritis (an inflammation of a large and small bowel) provoking diarrhea develops.

The main diagnostic methods of a disease of Girshprunga are a radiographic research of intestines using contrast agents, and also a biopsy of a large intestine. The data obtained by these methods are quite enough for diagnosis and definition of localization and a stage of development of a disease. Also ultrasonography and an anorectal manometriya are in rare instances applied.

Treatment of a disease of Girshprunga: main methods

Treat the main methods of treatment of a disease of Girshprunga:

• Dietotherapy;
• Conservative treatment;
• Radical treatment.

Dietotherapy and conservative treatment of a disease of Girshprunga – temporary measures when training the patient for radical (operational) treatment. The dietotherapy is directed to reduction of manifestation of symptoms at the patient while conservative treatment is applied to elimination of symptoms or their simplification.

Radical treatment of a disease of Girshprunga assumes an operative measure at which the aganglionic site of intestines with imposing of an anastomosis is excised. Until recently, operations at a disease of Girshprunga were performed with opening of an abdominal cavity and imposing of a kolostoma before resuming of a normal vermicular movement of intestines. A repeated operative measure was carried out later certain time for closing of the removed kolostoma and sewing together of healthy sites of intestines.

Modern low-invasive laparoscopic techniques allow to carry out at a disease of Girshprunga operation in one step assuming excision of the site deprived innervations and formation of an anastomosis through rectal access. Now at a disease of Girshprunga operations with open access are performed only in the presence of serious complications, such as a coloenteritis or megacolon which are resistive to conservative methods of treatment.