Hirschsprung's Disease

There are many different conditions that affect the proper functioning of the colon, according to Colon-cancer-digestion-health-zone, a website on disorders of the digestive system and colon. The website points out that accuracy in diagnosis is essential because many colon conditions present similar symptoms, such as diarrhea or constipation, but may require different treatments. One colon disorder where accurate diagnosis is vital is Hirschsprung's disease, a condition named after Dr. Harald Hirschsprung, a Danish physician who first correctly diagnosed the disease in 1886.
  1. Definition

    • Hirschsprung's disease is a congenital condition usually diagnosed in newborns, according to the Mayo Clinic. It occurs because the baby is born without all the nerve cells or ganglia that normally line the entire colon. Sometimes only a small section of ganglia is missing, but at other times a much longer section is absent.
      The missing ganglia affect the functioning of the large intestine—or colon—chiefly in the processing of stool, according to the Cincinnati Children's Hospital. This digestive process is known as "peristalsis." The Mayo Clinic indicates that sometimes the infant must be treated right away; sometimes the condition requires treatment as the child gets older.

    Symptoms

    • In addition to difficulty passing stool, symptoms of Hirschsprung's disease in infancy include difficulty absorbing nutrients. Severe cases of the condition are marked by the inability of the baby to have a bowel movement within the first or second day after being born, according to the Mayo Clinic. Other symptoms include vomiting, diarrhea, bloating and constipation. Symptoms in older children include failure to thrive, chronic constipation, anemia, malnutrition, bloating and enterocolitis.

    Enterocolitis

    • Enterocolitis, a severe infection of the colon, can occur in conjunction with Hirschsprung's disease. This is a serious infection that can affect both infants and young children suffering from Hirschsprung's disease, according to the Cincinnati Children's Hospital. Symptoms of enterocolitis include fever, vomiting and diarrhea.

    Testing and Diagnosis

    • Tests for Hirschsprung's disease that your child's pediatrician may wish to perform include an abdominal X-ray to determine bulging or narrowing in the colon or a barium enema to map the colon and rectum, according to the Cincinnati Children's Hospital. Tests on older children include a biopsy or surgical removal of a small section of colon tissue, to search for areas where ganglia is missing.

    Surgical Treatment

    • The Mayo Clinic cites a procedure known as "pull-through surgery" as the most effective and preferred treatment for Hirschsprung's disease. In pull-through surgery, a portion of the diseased colon (without nerve ganglia) is removed and then the two healthy sections are "pulled" together and reconnected.

    Surgery Risks

    • The Mayo Clinic indicates that children undergoing pull-through surgery remain at risk for enterocolitis until the surgery is fully healed. Patients should be monitored following surgery for fever, abdominal swelling, diarrhea, vomiting and rectal bleeding. Other after-effects of pull-through surgery include diarrhea and difficulty passing stool due to lack of colon muscle coordination.

    Research

    • Research to identify the cause of Hirschsprung's disease continues. It is possible that the disease is inherited. The Cincinnati Children's Hospital asserts that Hirschsprung's disease is about 10 times more likely in a child with Down syndrome.

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