Hirschprungs Disease
What is Hirschprungs Disease?
Hirschprungs disease is a condition that affects 1 in 5000 babies. They are born with an abnormality in the nerves of the bowel, rectum and anal sphincters which affects their ability to pass stool and gas.
It affects boys more often than girls (5:1), and can run in families.
It most commonly presents in the newborn period as a baby who does not pass their first stool on day 1 of life (delayed passage of meconium). These babies gradually get a distended abdomen and struggle to feed, or vomit.
Occasionally, Hirschprungs disease can go undetected in the newborn period, and is diagnosed later in childhood in a child with chronic constipation and difficulty with growth and weight gain.
DR ROB’S APPROACH
Dr Rob completed an overseas specialty fellowship in Paediatric Colorectal Surgery, including the care of patients with Hirschprungs disease.
To make a diagnosis of Hirschprungs disease, Dr Rob will perform a biopsy of the rectum. In the newborn period this can be done without anaesthesia using a specially made biopsy instrument. In older children, Dr Rob will arrange for them to have a general anaesthetic.
Once a diagnosis of Hirschprungs has been confirmed, your child will need a procedure called a pullthrough. In this procedure, the part of the large intestine which is affected will be removed, and healthy bowel with normal nerves will be pulled down and attached just above the anal sphincter. This is performed as a combination procedure, with part of the operation completed as keyhole surgery in the abdominal cavity, and part of the operation completed through the inside of the anal canal from below.
Occasionally, the patient may also require a stoma to be formed. This may be done as the first procedure if there is a lot of abnormal bowel, or during the pull through, or afterwards if they are having severe complications.
If your child is given a diagnosis of Hirschprungs, Dr Rob will arrange for their care to be transferred to the Gold Coast University Hospital for their definitive procedure. This is because in addition to operative correction, specialised pathology and nursing is required for the optimal care of children with Hirschprungs disease, which at present is only available at the Gold Coast University Hospital.
Your care will still be carefully coordinated by Dr Rob.
POST OPERATIVE CARE AND RECOVERY
After the pull through procedure, your child should start passing wind and stool from their anus within a day or two. They can usually start feeding the day after the operation. Once they are feeding and stooling well, you will be able to take them home. All their stitches will dissolve, and the glue on their incisions will flake off. You will be able to bath them as normal the day after their operation.
Dr Rob will see you 2 weeks after the operation to check the health of the join in their rectum.
DR ROB McCUSKER
Gold Coast Children’s Surgeon
Dr Rob is dedicated to the highest quality of care and technical excellence for his patients.