Hirschsprung's disease is a congenital disorder in which there are no nerves in the affected region of the bowel. The most prominent symptom is severe constipation. Other symptoms may include vomiting, abdominal pain, diarrhea and slow growth. Most babies with HSCR experience symptoms after they turn 2 months old. Complications may include enterocolitis, colic, obstruction and perforation of the intestinal tract.
The major types of Hirschsprung disease include:
Hirschsprung disease is more likely to affect
Children of any race or ethnicity may get Hirschsprung disease.
Hirschsprung disease affects about 30% of newborns. Some of the other common conditions that plague people affected by the Hirschsprung disease include:
Some newborns with Hirschsprung disease have early signs and symptoms of intestinal blockage, which include
Hirschsprung disease in newborns may potentially show signs or symptoms of problems.
Hirschsprung disease may go undiagnosed and untreated in infants who do not exhibit early signs and symptoms of intestinal blockage. Typical signs in older infant and children include
Hirschsprung disease symptoms, like constipation, may resemble those of other illnesses in older infants and kids. As a result, medical professionals might not immediately suspect and diagnose Hirschsprung illness.
Hirschsprung may not always be identified and treated until adulthood. Hirschsprung disease is often diagnosed in adults who have a history of chronic constipation and abdominal swelling that doesn't improve with oral laxative use.
Why the ganglion cells don't migrate all the way to the end of the rectum is a mystery to scientists. However, genetic factors might be at play, particularly if there are longer intestines involved or if there is a family member who also has the illness.
For instance, if one parent has Hirschsprung's disease, there is a higher likelihood that the child will also have the disease. (If the mother has Hirschsprung's illness, the chances are higher.) There is a 3–12% risk that another child from the same parents will also have Hirschsprung's disease if the family has one child who does.
Boys experience Hirschsprung's disease five times more often than girls do. Children who have Down syndrome are also at an increased risk.
If your child exhibits these symptoms, you should call your doctor right away. They can use a few particular tests to validate Hirschsprung's:
The disorder known as Hirschsprung's disease is exceedingly dangerous. However, it can enable your child lead a somewhat normal life if it is discovered quickly.
Typically, doctors will do one of two kinds of surgery:
Pull-through procedure: The big intestinal section that lacks nerve cells is simply removed during this procedure. The remainder of the intestine is then directly attached to the anus.
Ostomy surgery: The intestine is guided to an opening created in the body by this procedure. In order to retain the intestine waste, the doctor next fastens an ostomy bag to the exterior of the orifice. In order to wait until the youngster is prepared for the pull-through treatment, ostomy surgery is typically a temporary solution.
Some kids may experience constipation, diarrhoea, or incontinence after surgery (lack of control over bowel movements or urination).
Enterocolitis may affect a few people. Rectal haemorrhage, fever, vomiting, and an enlarged abdomen are symptoms. Take your child right away to the hospital if that occurs.
However, with the right care, particularly a healthy diet and enough of water, these issues should improve, and most kids can resume having regular bowel movements within a year of treatment. Some kids will experience gastrointestinal problems intermittently throughout childhood and into adulthood. Hirschsprung's disease should be followed throughout one's life because it is congenital.
Hirschsprung's disease risk factors include the following:
In most kids, symptoms become better or disappear following surgery. A tiny percentage of kids could struggle to control their faeces or have constipation (fecal incontinence). Children with less disease (which enables the surgeon to leave more healthy bowel in place) and those who receive treatment early typically have better outcomes.
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