Hirschsprung’s disease (congenital aganglionosis) is a condition in which the nerves of the lower part of the intestine that usually allow the bowel to relax are absent, resulting in the inability of the infant to have a bowel movement. It occurs in 1 in 1000 to 1500 live births with males affected more than females by 4 to 1.

Diagnosis
Failure to pass meconium (baby’s first bowel movement) in the first 24-48 hours will prompt your doctor to evaluate your baby for Hirschsprung’s disease. The severity of an infants symptoms can vary from being completely obstructed (unable to pass gas or stool) with abdominal distension, to a delay of constipation for several weeks. In some cases, the child will have mild constipation for several years before a diagnosis is made.

A history and physical exam will be performed by your surgeon. He will be looking for stool in the rectum and will evaluate your baby’s abdomen for distension. A contrast X-ray study will be performed called a barium enema. It will look for an area of the colon that is narrow closer to the anus, with a transition to dilated colon upstream.

A suction biopsy of the rectum (lowest part of the intestine) is the next step. Your surgeon will do this at the bedside without anesthesia. The pathologist will look for ganglion cells (nerve cells) in the biopsy.

Treatment

If you child is otherwise healthy, your surgeon may choose to perform a one-stage pullthough procedure. This may be done in a variety of ways, including open (involving an abdominal incision), laparoscopically (using 3 small incisions), or perhaps transanal (with the entire procedure done through the anal opening).  All 3 single stage procedures avoid a temporary colostomy and may be performed in the newborn period. You and your surgeon will decide which procedure is best for your child. Regardless of the procedure chosen, all methods have more than a 90% success rate overall.

Depending on the severity of the infant’s condition, a decision will be made to either perform the procedure in two-stages. If the two-staged procedure is chosen, the infant will first go to the operating room, and under general anesthesia, a colostomy will be performed. A colostomy is a procedure in which the colon upstream to the transition point is brought up to the level of the skin and sutured there. The child will wear a bag to capture the stool which will exit through colostomy. Once the child has reached an adequate size (20 pounds or more), a formal procedure is done. There are a variety of procedures which all involve removing the segment of bowel that does not relax, and sewing the functional bowel a centimeter inside the anus.

Postoperative Care
Your child will be kept in the hospital after surgery. He will be closely monitored and in the first days, will receive fluids by vein. Once your child is stable from a surgical standpoint, his pain is controlled with oral pain medications, and he is tolerating a regular diet, he will be able to go home. This usually occurs in 3-5 days.

Medications
Your surgeon will give your child a prescription for a mild narcotic pain medication which can be given every four to six hours as needed. These medications can be irritating to your child’s stomach so it is best to take them with food. It is important to give your child plenty of fluids in order to keep him well-hydrated and to avoid constipation.

Any other medications your child required before the operation should be continued on the regular schedule afterward.

Diet
Your child should continue the regular diet that was started prior to their discharge. It is important to give your child plenty of fluids in order to keep him well-hydrated and to avoid constipation.

Wound care
Always wash your hands before touching or cleaning the incision area, if there is one on the abdomen. Some blood staining of the paper tapes on the incision is common. If the blood is dry and not spreading, the staining is not a problem. If the blood seems fresh, the amount is increasing, or if the paper tape is blood soaked and partially floating above the skin, apply gentle pressure with a clean washcloth for five to six minutes. If the bleeding does not stop after five minutes, call your surgeon at (602) 254-5561.

You should be vigilant about changing your baby’s diaper to keep the area clean and dry. This is especially important during the first few weeks. Call the office or go to the emergency room if your child develops redness or yellowish or bloody drainage around the anus.