What are the long term effects of Hirschsprung disease?

What are the long term effects of Hirschsprung disease?

Analysis of long-term function in children after surgical management is difficult. The most commonly encountered problems include constipation, incontinence, enterocolitis and the overall impact of the disease on lifestyle (quality of life). Other complications are less frequent.

What are three 3 clinical manifestations of Hirschsprung’s disease in an infant?

The main signs and symptoms of Hirschsprung disease are constipation or intestinal obstruction, usually appearing shortly after birth. Most often, an infant or a child with Hirschsprung disease will have other symptoms, including growth failure, swelling of the abdomen, unexplained fever, or vomiting.

Is Hirschsprung a disability?

Hirschsprung disease is a congenital disability or birth defect. It’s present at birth. Approximately 5,000 babies are born with the disease every year. The condition affects three to four times as many males as females.

What is the complication of Hirschsprung disease?

Enterocolitis, chronic obstruction, incontinence, constipation, and late mortality may occur late after surgery. Rectovesical fistulas have also been reported in the literature. Enterocolitis accounts for significant morbidity and mortality in patients with Hirschsprung disease and can progress into toxic megacolon.

What to expect after surgery for Hirschsprung’s?

Immediately following successful surgery, many children with Hirschsprung’s disease have diarrhea for weeks. Diarrhea may break down sensitive skin under the diaper, and the child recognizes intense pain after each bowel movement.

What is the prognosis for surgically treated patients with Hirschsprung disease?

Background. Hirschsprung disease (HD) is a congenital disorder of the bowel resulting in pathological dysmotility of the colon. While many patients who undergo surgical correction attain satisfactory bowel function, a significant percentage still report poor outcomes (i.e., constipation, diarrhea, impaired continence).

Is Hirschsprung serious?

Children who have Hirschsprung’s disease are prone to a serious intestinal infection called enterocolitis. Enterocolitis can be life-threatening and requires immediate treatment.

What diagnostic procedure that confirm Hirschsprung’s disease?

Hirschsprung’s disease should be confirmed using rectal suction biopsy. Serial rectal irrigation should be performed before surgery to help prevent enterocolitis. Surgery is the recommended treatment for patients with Hirschsprung’s disease.

Can you grow out of Hirschsprung disease?

Fortunately, most children who have surgery are fully cured and able to pass bowel movements (BMs) normally. Hirschsprung disease can cause constipation, diarrhea, and vomiting. Sometimes it leads to serious colon complications, like enterocolitis and toxic megacolon, which can be life-threatening.

Is Hirschsprung disease autosomal dominant?

If HSCR occurs as part of a genetic syndrome, then it is inherited in a specific pattern, according to the specific syndrome . For example, the inheritance may be autosomal recessive , autosomal dominant , or X-linked recessive , depending on the exact cause of the syndrome.

What is the main pathophysiological problem in Hirschsprung’s disease?

Hirschsprung’s disease occurs when nerve cells in the colon don’t form completely. Nerves in the colon control the muscle contractions that move food through the bowels. Without the contractions, stool stays in the large intestine.

What is the possible complication of Hirschsprung disease after surgery?

Most people who undergo surgery for Hirschsprung’s disease recover without problems. Any time a person has a general anesthesia and surgery, however, there is a possibility of complications. These include bleeding, infection or a blockage of the bowel after surgery due to scarring or other factors.

How does Hirschsprung disease affect an older child?

Older kids with Hirschsprung disease might have a growth delay because the condition can affect the body’s ability to absorb nutrients. What Causes Hirschsprung Disease? Hirschsprung disease prevents bowel movements (stool) from passing through the intestines due to missing nerve cells in the lower part of the colon.

Where are the nerve cells missing in Hirschsprung disease?

Rarely, nerve cells are missing in the entire large and small intestine. In a child with Hirschsprung disease, stool moves through the bowel until it reaches the part lacking nerve cells. At that point, the stool moves slowly or stops. The large intestine, which includes the colon and rectum, is the last part of the gastrointestinal (GI) tract.

What is the treatment for Hirschsprung disease ( HSCR )?

HSCR may occur as an isolated finding or as part of a another disorder. Isolated HSCR is associated with mutations in several genes. Treatment is with surgery, removing the aganglionic intestinal segment. [2]

What foods can I Feed my Child with Hirschsprung’s disease?

Serve high-fiber foods. If your child eats solid foods, include high-fiber foods. Offer whole grains, fruits and vegetables and limit white bread and other low-fiber foods. Because a sudden increase in high-fiber foods can worsen constipation at first, add high-fiber foods to your child’s diet slowly.