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Hirschsprung's disease is a rare, congenital disorder of the innervation of the intestine that is characterized by distension of the intestine to more than 6-7 cm in diameter.
Hirschprung's diseasearises already in the developmental period, when the caudal migration of cells from the nerve crest along thedigestive tractis stopped. The frequency of the disease is estimated at around 1: 5,000 births. The disease is more common in males, the ratio of sick boys to girls is around 4: 1.
Hirschsprung's disease - symptoms
Hirschsprung's disease manifests itself very early, already on the 5-10th day of a child's life. The first symptoms are the inhibition ofmeconium . In addition, newborns have a bloated abdomen and vomit frequently. If a small distal segment is involved, infants alternate between constipation and diarrhea. There is inflammation of the intestines and water and electrolyte disturbances. Occasionally the intestine may perforate.
Hirschsprung's disease - and accompanying defects
The disadvantages of patients with Hirschsprung's disease include :
- heart defects (mainly atrial or interventricular septum defects)
- kidney dysplasia or agenesis
- cleft palate
- defects of the genitourinary system, including hypospadias
- gastrointestinal malformations - e.g. single umbilical artery or inguinal hernia
- facial dysmorphic features, skull defects
Hirschsprung disease - diagnosis
The diagnosis is based on: clinical picture, physical and radiological examination, anal manometry and histopathological examination.
Hirschsprung disease - treatment
In the case of full-blown obstruction, insert a gastric tube and aspirate any remaining content, as well as perform cleansing infusions into the large intestine. In the lightest cases, an enema is performed in a hospital setting. Lack of improvement qualifies the patient for surgery. It involves the removal of the aganglionic segment and the anastomosis of the correctly innervated segment proximal to it with the remaining peripheral segment of the rectum or anus.