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.