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What is the most common treatment for Hirschsprung's disease?
Doctors typically treat Hirschsprung disease with a type of surgery called a pull-through procedure. In some cases, doctors recommend ostomy surgery followed by a pull-through procedure. Children with Hirschsprung disease most often feel better after surgery.
What is the surgical procedure for Hirschsprungs disease?
To fix intestinal obstruction caused by Hirschsprung's disease, surgeons at Boston Children's Hospital perform a type of surgery called a pull-through procedure. The goal of pull-through surgery is to remove the diseased section of your child's intestine and then pull the healthy portion of this organ down to the anus.
Can Hirschsprung disease be treated without surgery?
The only treatment for Hirschsprung's is surgery. All children will have to undergo a procedure at some stage to connect the healthy portion of bowel to their anus in order to allow them to pass bowel motions normally.
Can Hirschsprung disease be cured?
Hirschsprung's disease cannot be cured on its own. It can be fatal for children who do not have surgery. After surgery, most children lead normal lives. They may have minor health problems as a result of the disease.
What is ileostomy surgery called?
Permanent Ileostomy The standard or Brooke ileostomy is the most common ileostomy. Standard ileostomies use external pouches. In the standard or Brooke ileostomy (also known as an end ileostomy), surgeons pull the ileum up and through an incision in the abdomen.
What is the treatment for megacolon?
Patients with idiopathic megacolon or megarectum may require surgery if they have refractory symptoms. Depending on age, pelvic floor, and anal sphincter function, patients who have isolated megacolon can be treated with either subtotal colectomy with ileorectostomy or diverting loop ileostomy.
What is colectomy surgery?
(Hemicolectomy, partial colectomy, or segmental resection) A colectomy is a type of surgery used to treat colon diseases. These include cancer, inflammatory disease, or diverticulitis. The surgery is done by removing a portion of the colon. The colon is part of the large intestine.
What is another name for Hirschsprung disease?
Congenital aganglionic megacolon (Hirschsprung disease).
What is the main cause of Hirschsprung disease?
While a mutation in a single gene sometimes causes the condition, mutations in multiple genes may be required in some cases. The genetic cause of the condition is unknown in approximately half of affected individuals. Mutations in the RET gene are the most common known genetic cause of Hirschsprung disease.
How do you remove impacted stool from a baby?
Your child's healthcare provider may prescribe a stool softener. This will help your child have a bowel movement. In some cases, other methods may be advised to loosen hard stool. These may include a glycerin suppository or laxatives.
How long is Hirschsprung's surgery?
The mean length of the surgery was 181 +/- 40 minutes. Blood transfusions were required in 13 patients with a mean volume of 55 mL given. The complications observed were: an anastomotic leak in two cases, requiring laparotomy and colostomy (both in patients with Down's syndrome), and wound infection in three cases.
What is ostomy surgery?
Definition & Facts. Ostomy surgery of the bowel is an operation that changes the way intestinal contents leave your body when part or all of your bowel is diseased, injured, or missing. During ostomy surgery of the bowel, surgeons connect the large or small intestine to the skin on the outside of your abdomen.
What is colectomy surgery?
(Hemicolectomy, partial colectomy, or segmental resection) A colectomy is a type of surgery used to treat colon diseases. These include cancer, inflammatory disease, or diverticulitis. The surgery is done by removing a portion of the colon. The colon is part of the large intestine.
What is Hirschsprung disease?
Hirschsprung disease is a heterogeneous disease that is caused by a variety of genetic mutations and is characterized by the absence of ganglion cells that are present in the distal intestine. The missing nerve cells result in a lack of peristalsis and functional intestinal ...
What is the surgical treatment for Hirschsprung disease?
Surgical Treatment for Hirschsprung Disease. Once a diagnosis of Hirschsprung disease is made, surgery to remove the aganglionic bowel is performed.
What is Hirschsprung enterocolitis?
For some children, Hirschsprung-associated enterocolitis (HAEC) and functional problems (such as ongoing bowel obstructive symptoms and soiling) continues to occur. It is imperative that family members and primary care physicians stay alert to the symptoms of HAEC.
What happens after Hirschsprung surgery?
Many children thrive after surgery for Hirschsprung disease and experience normal bowel function and an improved quality of life. Initially, loose stools may occur in the early postoperative period, but eventually will improve over time as the child heals.
What are the congenital abnormalities associated with Hirschsprung disease?
These conditions include: Congenital heart disease. Congenital central hypoventilation syndrome. Cleft lip and palate. Dysmorphic features.
What is the RAIR test for Hirschsprung?
Another diagnostic technique to employ with older children is the use of anorectal manometry to look for the presence of the rectoanal inhibitory reflex (RAIR)—which is always absent in children with Hirschsprung disease. Typically the smooth muscle of the internal sphincter relaxes when stool distends the rectum.
How many infants are affected by Hirschsprung disease?
The disease affects approximately one in 5,000 infants. The treatment of Hirschsprung disease is primarily surgical, and involves resection of the aganglionic bowel and reconstruction using one of several techniques: the Swenson, Soave and Duhamel procedures. Although there have been many advances in the diagnostic and surgical approaches ...