Hirschsprung’s Disease; A Parent’s Guide to Surgical Correction
Hirschsprung’s Disease is a congenital birth defect affecting one out of every 5,000 births. More commonly seen in boys than in girls, the condition is usually identified within the first few months after birth. Characterized by an inability to complete a bowel movement, Hirschsprung’s Disease is life threatening but treatable.
In healthy individuals, the intestinal tract contacts ganglia nerves which are responsible for the muscle contractions required for healthy bowel movement. In children with Hirschsprung’s Disease, the ganglia are missing. The missing ganglia nerves result in the inability of the child to correctly and safely pass feces. The initial concern is a blockage of the intestine exhibited by a distended abdomen. A more sudden indication of the presence of Hirschsprung’s Disease, in newborns, is the failure to pass the first bowel movement, also known as meconium. In fact, in some newborn cases of Hirschsprung’s Disease, the child vomits profusely when the first bowel movement does not occur.
For parents of children with Hirchsprung’s Disease, life can become suddenly frightening. However, with advancements in surgical technology, most children inflicted with Hisrchsprung’s Disease are surgically corrected and lead relatively normal, healthy lives. Of these surgical procedures, the most recent development is the POOP procedure, also known as Perineal One Stage Operation Pull Through Procedure. It is within this procedure the surgeon will remove the area of the intestine or colon which is missing the ganglia nerves and then re-attach health tissue. Hospitalization is generally limited to one to three days and surgical incision sites are minimal as the procedure is generally performed in a laparoscopic procedure.
When POOP is not appropriate in the Hirschsprung’s patient, the surgeon may recommend a two step approach in which the initial surgery involves removal of the damaged colorectal tissue and placement of a stoma; an opening in the intestine leading to the outside of the body and attached to a colostomy bag. Under this two-step procedure, the surgeon will recommend the colostomy bag remain intact for several months in an effort to allow the intestinal tissue to heal from the first surgery. Once healed, the second step of the surgery is completed to remove the stoma and re-attach the healthy intestinal tissue. Under this procedure, hospital admission may require an extended stay.
Following successful completion of either surgery, the Hirschsprung’s infant may continue to suffer from loose bowel movements. As a result, it is highly recommended that a diaper cream be used on a regular basis so as to prevent chronic diaper rash. Additionally, it has been noted that as many as 10-15% of surgically corrected Hirschsprung patients will continue to suffer from enterocolitis, incontinence and ongoing constipation. Your physician may instruct you in home remedies including laxatives and enema administration.
As a parent of a child with Hirschpsrung’s disease, the condition may be frightening. Understanding the cause and origin of the condition, the symptoms of complications and the surgical options will empower the parent and relieve some anxiety associated with a Hirschsprung’s infant. What is important to understand is the Hirschsprung’s Disease patient can lead a normal, happy and healthy life with little to no complications following surgical correction.
