Pediatric Surgery Specialist Prof. Dr. Feryal Gün Soysal explained what you need to know about Hirschsprung disease in children and its treatment.
It is a disease that concerns the nervous system of the intestine. This should be taken into consideration, especially in babies and children who have not pooped since infancy without stimulation such as suppositories or cream, and who have a delay in passing the first poop, which we call meconium, within the first 24 hours after birth. Symptoms of intestinal obstruction, characterized by bilious vomiting, abdominal bloating and gas, and inability to defecate, may occur at any time in life.
The course of the disease; In newborn babies, it includes bilious vomiting, abdominal distension, inability to pass meconium, and intestinal obstruction confirmed by radiology. It may begin with sudden abdominal distension, bilious vomiting, foul-smelling and sometimes bloody diarrhea, and high fever, and may lead to death in a short time with hypovolemic shock, septicemia, and intestinal perforation. It is usually a disease of a healthy term baby. However, Down syndrome can be seen at a rate of 5%, genitourinary system anomalies at the same rate, and more rarely, cardiac anomalies, digestive system atresias, diseases of neuroectodermal origin, and CNS anomalies.
Foul-smelling stools are the most typical symptom. Examination is performed using radiological techniques. Definitive diagnosis is made by rectum biopsy.
How is it treated?
The diagnosis is made by rectum biopsy.
After the diagnosis is confirmed, the aganglionic part is removed with a one-stage transanal endorectal pull-through operation and the ganglion-containing intestine is connected to the anus. In complicated and delayed cases, it may be necessary to temporarily apply an emergency colostomy as the first stage of the 3-stage surgical treatment. Closed or open surgical techniques can be applied.
Each patient, even if they have gone through all the surgery stages, is monitored for a maximum of 3 years, and diet and toilet training are specially planned and organized.
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