If fetal development does not go wrong, the fetus has anomalies in the structure of the internal organs. One of these mutations is Hirschsprung syndrome (megacolon or aganglion). This disease occurs in one of 5000 children, mostly male.
Hirschsprung's disease - what is it?
The intestinal motility and periodic excretion of feces are regulated by special nerve cells. Congenital megacolon is characterized by their lack or deficiency, because of which the evacuation of feces is significantly hampered. As a result, a large amount of feces accumulates in the large intestine. This leads to an extension and lengthening of the organ.
Hirschsprung's disease in children - symptoms
The clinical picture strongly depends on the localization and the degree of innervation of the intestine. Hirschsprung's disease in infants is manifested in the form of the following symptoms:
- absence of original feces (meconium);
- marked gassing;
- flatulence ;
- vomiting, sometimes bile;
- prolonged constipation ;
- change in the shape of the abdomen;
- inverted or low positioned umbilicus;
- rarely diarrhea.
Disease Hirschsprung in older children is accompanied by additional symptoms:
- impossibility of bowel movement without enema;
- abdominal pain;
- intoxication of the body;
- lag in physical development;
- partial heterochromy;
- presence of hardened feces ("intestinal stones");
- anemia ;
- deformation of the chest;
- hypotrophy;
- enterocolitis;
- expansion of the large intestine;
- propensity to develop bronchitis and pneumonia;
- vomiting and diarrhea after prolonged constipation.
Disease Hirschsprung in children - diagnosis
To confirm suspicions of the described pathology, the doctor first scrupulously examines a small patient. Hirschsprung's disease in newborns can be previously revealed even palpation, feeling the abdomen in the area of the large intestine. An important role is played by the parents' questioning of the child. If the family had cases of the anomaly in question, the likelihood of its occurrence in the baby is significantly increased (hereditary factor).
Rectal examination of a child with Hirschsprung disease
The main diagnostic method for this disease is visual and finger examination of the rectum (sigmoidoscopy). Hirschsprung's disease is accompanied by a high tone of the sphincter along with an empty space in front of the anal opening, even if the emptying did not occur for several days. For more information, hardware research is assigned. Disease Hirschsprung - Diagnosis:
- Irrigoscopy with the introduction of a barium mixture;
- colonoscopy;
- anorectal manometry;
- biopsy with subsequent histological analysis.
Hirschsprung disease - X-ray
An obligatory type of examination in the diagnosis of the described disease is the performance of a survey. Radiography helps to find out exactly where the megacolon (Hirschsprung's disease) is localized, to find out the degree of severity of the pathology. To assess the quality of intestinal motility, it is recommended to introduce contrast medium and additional ultrasound.
Treatment of Hirschsprung disease in children
The only effective way to treat the presented anomaly is surgical intervention. Conservative approach is required only on the eve of the operation. It helps to alleviate and alleviate Hirschsprung's disease - treatment involves eliminating the symptoms of pathology, normalizing the excretion of fecal matter and restoring intestinal motility. After surgical therapy it is important to ensure the child the right care and follow the adherence to a special diet.
Hirschsprung's disease in children - clinical recommendations
When the diagnosis is confirmed and the doctor has appointed the date of the operation, it is necessary to prepare for the procedure. Megacolon in children involves the following treatment measures:
- Elimination of intestinal obstruction. Regularly installed siphon enemas with the introduction of a warm solution of sodium chloride concentration of 0.9%.
- Restoration of intestinal microflora. The kid should take drugs that stimulate the vital activity of beneficial bacteria ( probiotics ).
- Support immunity. The child is prescribed vitamins - E, C, B6, B12.
- Improved faeces withdrawal. To normalize the emptying of the intestines helps massage the stomach and gymnastics.
- Prevention of constipation. Prevent stagnation of feces with a diet enriched with fiber in parallel with the use of clean water.
- Restoration of metabolic processes. With severe eating disorders, intravenous electrolyte solutions, glucose and protein preparations are recommended.
Surgery for Hirschsprung disease
There are several options for surgical intervention to eliminate the disease in question. All of them consist in excision of the damaged sites of a straight line and the sigmoid intestine and imposing between healthy tissues of anastomosis. In infancy, Duhamel surgery is performed primarily in Hirschsprung's disease, but there are other types of effective and safe procedures:
- Rectosigmoidectomy according to Svenson-Hyatt-Isakov;
- a deep colorectal anterior anastomosis according to Rebein;
- Operation Soave.
The listed methods are approximately identical in terms of effectiveness, so the choice of the type of surgical intervention is performed by a specialist. Assigning a certain radical manipulation, the doctor is guided by the age and condition of the small patient, assesses how quickly Hirschsprung's disease progresses. It is important for parents to find an experienced surgeon, all these operations are characterized by a complex technique of implementation.
Disease Hirschsprung - diet
To relieve the symptoms of the described pathology, a special laxative diet is recommended. Nutrition for Hirschsprung disease involves the use of products that soften the stool and stimulate intestinal motility:
- a decoction of bran or dill (for infants);
- dried apricots, prunes, especially compotes on their basis;
- pumpkin;
- buckwheat, oats, wheat porridge;
- one day kefir;
- beet;
- cucumbers;
- yogurt;
- vegetarian soups;
- steam cutlets and soufflé from low-fat diet meat;
- vegetable oils;
- fresh berries, fruits and vegetables.
From the diet, we must exclude everything that strengthens and provokes the processes of fermentation in the intestine:
- pears;
- rice, semolina porridge;
- fresh white and rye bread;
- smoked products;
- bun and pastry;
- cocoa and chocolate;
- seeds;
- cabbage;
- fat meat;
- rich broths and soups;
- potatoes (in limited quantities);
- pasta;
- cherries;
- sweets with oil creams;
- beans;
- whole milk.
Hirschsprung's disease in children - consequences
Without correct and timely therapy, the anomaly considered may end badly, especially if it is diagnosed in a newborn. In 80% of such cases, Harald Hirschsprung's disease leads to the death of the baby. When the surgery was performed on time and qualitatively, the predictions are greatly improved, about 90% of the small patients return to satisfactory rates.
Other common complications of Hirschsprung's disease:
- acute enterocolitis;
- anemia;
- intestinal obstruction ;
- severe intoxication;
- peritonitis;
- fever;
- intestinal bleeding;
- fistula;
- stenosis;
- fecal incontinence.