Hirschsprung’s disease
GastroenterologyHirschsprung's disease is a genetic disorder that affects the intestines. It is mostly diagnosed in children from birth, less commonly in adults. The disease can affect anywhere from a few centimeters to a meter of intestine.
Treatment methods
What is Hirschsprung’s disease
Ganglia – nerve bundles – are responsible for the movement of the intestinal walls. They provide contraction (peristalsis), which is very important for the absorption of nutrients and the passage of fecal matter.
In Hirschsprung’s disease, some area of the intestine does not have these endings. Consequently, peristalsis does not occur there. The pathology appears as early as 5-12 weeks of intrauterine development. For unknown reasons, some parts of the intestine do not have ganglia.
Symptoms
The disease is diagnosed as early as infancy:
- late discharge of primary feces (normally occurs in the first 2 days of life);
- the baby’s not eating well;
- vomiting;
- constipation;
- bloating.
The infant does not gain weight well, becomes lethargic, flatulence is observed. After 1 year of age, the disease acquires classic symptoms: alternation of constipation with diarrhea, incomplete emptying of the intestines, pungent fecal odor.
In adults, the disease manifests similarly. These are probably small areas of intestinal damage, so the body has previously compensated with healthy ones.
Possible complications of the disease:
- Enterocolitis is inflammation of the intestines.
- Fecal obstruction due to narrowing of the intestinal lumen. This leads to fecal intoxication and even death.
Diagnosis of Hirschsprung’s disease
- Gastroenterology consultation
- Blood tests + Urine
- Irrigography is an x-ray with contrast.
- Anorectal manometry is the measurement of pressure in the rectum.
- Biopsy.
Gastroenterologists at the clinic “MDI will prescribe the optimal list of examinations to confirm or deny the diagnosis. At our clinic, diagnosis and treatment are carried out according to the high standards of Israeli medicine.
Treatment
Conservative treatment is designed to support the patient until surgery. Prescribe enemas, probiotics, painkillers and abdominal massage. The radical treatment is surgery to remove a section of the intestine without nerves.
It is conducted in two stages:
- The section of intestine with innervation is dissected out. A temporary colostomy is inserted through the abdomen for fecal output.
- The joining of two sections of intestine.
It is worth noting that a shortened intestine (especially if a significant part of it has been removed) can give complications: constipation and fecal incontinence.
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