Question: What is (are) What I need to know about Hirschsprung Disease ?
Answer: Hirschsprung* disease (HD) is a disease of the large intestine that causes severe constipation or intestinal obstruction. Constipation means stool moves through the intestines slower than usual. Bowel movements occur less often than normal and stools are difficult to pass. Some children with HD cant pass stool at all, which can result in the complete blockage of the intestines, a condition called intestinal obstruction. People with HD are born with it and are usually diagnosed when they are infants. Less severe cases are sometimes diagnosed when a child is older. An HD diagnosis in an adult is rare. *See Pronunciation Guide for tips on how to say the words in bold type.
Source: NIDDK

Question: What is (are) What I need to know about Hirschsprung Disease ?
Answer: The large intestine, which includes the colon and rectum, is the last part of the digestive tract. The large intestines main job is to absorb water and hold stool. The rectum connects the colon to the anus. Stool passes out of the body through the anus. At birth, the large intestine is about 2 feet long. An adults large intestine is about 5 feet long.
Source: NIDDK

Question: What causes What I need to know about Hirschsprung Disease ?
Answer: People with HD have constipation because they lack nerve cells in a part or all of the large intestine. The nerve cells signal muscles in the large intestine to push stool toward the anus. Without a signal to push stool along, stool will remain in the large intestine. How severe HD is depends on how much of the large intestine is affected. Short-segment HD means only the last part of the large intestine lacks nerve cells. Long-segment HD means most or all of the large intestine, and sometimes the last part of the small intestine, lacks nerve cells. In a person with HD, stool moves through the large intestine until it reaches the part lacking nerve cells. At that point, the stool moves slowly or stops, causing an intestinal obstruction.
Source: NIDDK

Question: What causes What I need to know about Hirschsprung Disease ?
Answer: Before birth, a childs nerve cells normally grow along the intestines in the direction of the anus. With HD, the nerve cells stop growing too soon. Why the nerve cells stop growing is unclear. Some HD is inherited, meaning it is passed from parent to child through genes. HD is not caused by anything a mother did while pregnant.
Source: NIDDK

Question: What are the symptoms of What I need to know about Hirschsprung Disease ?
Answer: The main symptoms of HD are constipation or intestinal obstruction, usually appearing shortly after birth. Constipation in infants and children is common and usually comes and goes, but if your child has had ongoing constipation since birth, HD may be the problem. Symptoms in Newborns Newborns with HD almost always fail to have their first bowel movement within 48 hours after birth. Other symptoms include - green or brown vomit - explosive stools after a doctor inserts a finger into the rectum - swelling of the belly, also known as the abdomen - lots of gas - bloody diarrhea Symptoms in Toddlers and Older Children Symptoms of HD in toddlers and older children include - not being able to pass stools without laxatives or enemas. A laxative is medicine that loosens stool and increases bowel movements. An enema is performed by flushing water, or sometimes a mild soap solution, into the anus using a special wash bottle. - swelling of the abdomen. - lots of gas. - bloody diarrhea. - slow growth or development. - lack of energy because of a shortage of red blood cells, called anemia.
Source: NIDDK

Question: What are the treatments for What I need to know about Hirschsprung Disease ?
Answer: Pull-through Procedure HD is treated with surgery called a pull-through procedure. A surgeon removes the segment of the large intestine lacking nerve cells and connects the healthy segment to the anus. The pull-through procedure is usually done soon after diagnosis. Ostomy surgery An ostomy allows stool to leave the body through an opening in the abdomen. Although most children with HD do not need an ostomy, a child who has been very sick from HD may need an ostomy to get better before the pull-through procedure. For ostomy surgery, the surgeon first takes out the diseased segment of the large intestine. The end of the healthy intestine is moved to an opening in the abdomen where a stoma is created. A stoma is created by rolling the intestines end back on itself, like a shirt cuff, and stitching it to the abdominal wall. An ostomy pouch is attached to the stoma and worn outside the body to collect stool. The pouch will need to be emptied several times each day. If the surgeon removes the entire large intestine and connects the small intestine to the stoma, the surgery is called an ileostomy. If the surgeon leaves part of the large intestine and connects it to the stoma, the surgery is called a colostomy. Later, during the pull-through procedure, the surgeon removes the stoma and closes the abdomen with stitches.
Source: NIDDK