ongoing help, when you and your baby are discharged

home.
Dilatations
Two weeks after the pull-through surgery, you may need to
begin a program of rectal dilatations in order to stretch the
area, where the newly joined intestine is, to a normal size.
You begin with a dilator that fits snugly, and perform the
dilatations twice a day. Every 1-2 weeks the size of the
dilator will be increased until the correct size is reached.
At this point if your baby has a colostomy it can be closed.
The dilatations continue, but the frequency is gradually
decreased until they are no longer required.
Colostomy Closure and Nappy Rash
When the colostomy is closed baby will start passing stool
through the anus again, it will be frequent and loose. During
this time, they are susceptible to severe nappy rash. Good
bottom care is required with the use of a skin barrier cream.
There are a variety of barrier creams available and it may
take some trial and error to find one that works well for your
baby. Please ask your nurse for some advice.
It is advisable to start preparing babys bottom a week/
fortnight prior to this operation. This can be done by placing
some stool from babys colostomy onto the nappy and
having baby wear the nappy for 5-10 mins, and/or applying
skin prep wipes to babys bottom. This should be done
several times a day.
Adjusting after pull-through
Some children may have diarrhoea for a while, and good
bottom care is required.
Eventually the stool will become more solid and the child will
need to go to the bathroom less often.
Toilet training may be delayed, as the child learns how to use
the bottom muscles only after the pull-through surgery.
Older children might stain their underwear for a while after
the surgery. It is not their fault. They can not control this
problem, but it improves with time supported by diet and
regular toileting.
Infection
Infections can be very dangerous for a child with
Hirschsprung's disease. Infection of the large and small
intestines is called enterocolitis. It can happen before or after
surgery to treat HD. Here are some of the signs to look for:
fever
diarrhoea loose watery motions
vomiting (especially bile (green) stained vomit)
Tummy distended (swollen)
Crying for no reason. Drawing knees up to tummy.
Pale and lethargic (no energy), not moving arms & legs
vigorously.
If the problem is enterocolitis, your child should be admitted
to hospital.
Enterocolitis can be life threatening, so watch for the
signs and do not delay in seeking medical help.
SSH/WI-3033/008 updated 01/06 SSH/WI-3033/008 updated 01/06 SSH/WI-3033/008 updated 01/06
Adapted from the National Institute of Diabetes and
Digestive and Kidney Diseases (USA), and Pull-thru Network.
www.digestive.niddk.nih.gov
www.pullthrough.org
Disclaimer: Disclaimer: Disclaimer: Disclaimer: Due to the dynamic state of the internet we
cannot recommend or endorse these website.
Parents Support Group:
Bowel Group for Kids Inc. (Australasian)
www.bgk.org.au
P.O. Box 40
Oakdale NSW 2570
Ph: 02 4659 6186
Fax: 02 4659 6381
Compiled by J Rowe Surgical Nurse Specialist
Department of Paediatric Surgery
Starship Hospital
Level 4
Phone: 64 9 3074949 ext 6381
Fax: 64 9 3078952
A Parents
Guide
Note: This is general information only and
the situation can vary from case to case
Hirschsprungs Hirschsprungs Hirschsprungs
Disease Disease Disease
Department of Paediatric Surgery
Starship Hospital
Auckland
Your baby has been born with Hirschsprungs disease (HD).
This brochure is intended to provide you with general
information about HD and the operations that are done to
deal with it.
What is Hirschsprung's Disease (HD)?
Hirschsprungs disease is a form of intestinal obstruction
caused by the absence of ganglion cells (special nerve
cells) in the wall of the intestine.
Introduction
These ganglion cells produce the wave-like motion of the
intestine, which pushes stool towards the anus where stool
then leaves the body. This movement of the intestine is
called peristalsis.
In HD normal peristaltic movement of the large intestine is
not possible.
Background/Incidence
HD was first described in 1886. HD develops before a baby
is born. Normally the ganglion cells grow from the top of the
gastrointestinal tract all the way to the anus. With HD, the
ganglion cells stop growing before they reach the anus. No
one knows why the ganglion cells stop growing.
The incidence of HD is 1 in 5000 newborns and it occurs
more frequently in boys than girls. The ratio being 4 males to
1 girl.
What happens in HD?
In a person with HD, the healthy muscles of the intestine
push the stool until it reaches the part without nerve cells.
At this point, the stool stops moving. New stool then begins
to stack up behind it causing a blockage of the intestine.
The length of affected intestine varies. If the diseased
section includes only part of the large intestine, it may be
standard length or short segment HD.
Sometimes the ganglion cells are missing from the whole
large intestine and even parts of the small intestine. This is
called long-segment HD or total colonic HD.
Management/Treatment
When the pull-through operation is done will depend on how
baby is doing.
Babies who have been very sick may first need surgery to
form a colostomy. This helps the child become healthy
before having the pull-through operation.
Some doctors may form a colostomy in every child before
doing the pull-through operation.
A colostomy is created by dividing the large intestine at the
diseased section, and then bringing the two ends through
the abdominal wall. This provides the child with a temporary
way to pass stool.
The pull-through operation is usually done between 3 12
months later, but may be longer.
If baby is feeding and growing well, they may be managed
with rectal washouts and rectal dilatations to help them
empty their bowel, until they are older and bigger before
having the pull-through operation.
Colostomy Care
Since you are dealing with a small baby, there may be some
trial and error in handling the ostomy products. You will be
provided with help from a stoma therapist and the nurses on
the ward.
A referral will be made to a community stoma therapist for
Step 2: The healthy
section is attached
Step 1: The diseased
section is removed
Before surgery: The
diseased section is the
part of the intestine that
Healthy large intestine: Nerve
cells are found throughout the
intestine.
HD large intestine: Nerve cells
are missing from the last part of
the intestine
Symptoms & Diagnosis
HD may be considered in a term baby who has not passed
their first stool within 24 48 hours following birth. Other
symptoms include poor feeding, vomiting, abdominal
distension and constipation.
When HD is suspected the following investigations may be
done:
Contrast enema: Contrast solution is injected into the
rectum via a catheter. This enables the anatomy &
capacity of the large colon to be visualised. In HD the
part of the bowel without ganglion cells is usually
narrow compared to the normal bowel.
Rectal Biopsy: This is the most accurate test for HD.
The doctor removes a tiny piece of the intestine and a
pathologist looks at it under a microscope. If the
ganglion cells are missing, HD is the problem.
Operations
HD is treated with surgery called a pull-through
operation. There are three common ways to do a pull-
through operation, and they are called the Swenson, the
Soave, and the Duhamel procedures. Each is done a little
differently, but all involve taking out the part of the intestine
that doesn't work and connecting the healthy part to the
anus.
After pull-through surgery, the child has a working
intestine.
SSH/WI-3033/008 updated 01/06 SSH/WI-3033/008 updated 01/06 SSH/WI-3033/008 updated 01/06