My two-year-old has been having severe constipation since the first few weeks of life. Initially, her doctor brushed it aside and told me that is was probably related to her diet. She is currently on a high fibre diet, takes lots of fruits and vegetables and I always ensure that she is well hydrated.
The constipation has not resolved. She only passes stool after I have given her some laxatives (usually once or twice a week).
It sometimes gets so bad that I have to take her to hospital for enemas. Recently, her doctor referred me to a paediatric surgeon for review.
After several tests, he told me that my daughter has a condition known as Hirschsprung’s disease. He says that it is a congenital condition and she will need surgery to resolve the constipation. I am very afraid of subjecting my child to surgery.
Can’t it be managed conservatively? He even informed me that she might have to have part of her intestine stitched onto her abdominal wall as part of the procedure. This does not sit well with me.
Why would he want to be so drastic in his approach? This is constipation - it is not a life-threatening condition.
Should I seek a second opinion?
Constipation is one of the most common complaints in children. However, it is important to understand that constipation is not a disease in itself. It is a symptom of an underlying problem.
In most children, it is usually due to nutritional deficits and poor toilet habits.
In some cases, it can be due to hormonal imbalances in the child (e.g. underactive thyroid).
Sometimes, a child can be born with a defect in either the digestive system or the anal opening—all of which can manifest with constipation.
Usually, in these cases, the onset of the constipation is in the first few weeks of life.
Diet usually does not play a role in these cases because most of these children are still on an exclusive breast milk diet when their symptoms begin.
Hirschsprung’s disease and anal/rectal malformations are examples of congenital defects that may present with constipation.
A little anatomy
In order to understand what exactly Hirschsprung’s disease is, you need to know a little bit about the anatomy and function of your child’s digestive system.
The digestive system is made up the mouth, food pipe, stomach and intestines (small and large). Once you ingest food, it goes into the stomach where it is mixed with digestive juices and passed on to the small intestines for absorption of nutrients.
The large intestines are the final part of the digestive system and this is where stool is formed.
The large intestines are made up of several layers. The inner most layer is known as the mucosa and it is very similar to the lining of the inside of your mouth.
It also has special muscle layers which allow it to propel contents from one end of the intestine to the other (and eventually expel it out of the body). The intestines have nerves which enable the muscles to function properly.
In Hirschsprung’s disease, a section of the large intestine lacks proper nerve supply. This means that the intestine cannot propel its contents from one section to the other. Consequently, the stool being formed in the large intestine piles up in one area resulting in constipation.
Diagnosis of Hirschsprung’s disease
Usually the doctor will need to take specimens (biopsies) from the lower part of the large intestines (in a segment known as the rectum). Usually, this is a relatively painless simple procedure that can be done in the doctor’s office. Your child does not need to get general anaesthesia (be put to sleep) for a biopsy.
Check for other congenital defects
Sometimes, Hirschsprung’s disease can be accompanied by other congenital defects— such as those of the heart, limbs, backbone etc. Ensure that your doctor gives your child a thorough check up with these in mind.
Surgery for constipation?
If your child has Hirschsprung’s disease, the surgery will be to remove the ‘defective’ segment of intestine. There are various methods of doing this and these are tailored to your child’s anatomy.
You mentioned that the surgeon said that your child’s intestines might be attached to her abdominal wall. This is medically known as a colostomy or ileostomy— depending on which part of the intestines is attached to the abdominal wall.
In cases of Hirschsprung’s disease surgery, this is usually a temporary measure which is reversed after a few months. (In some children, a colostomy is not needed).
Will she ‘outgrow’ Hirschsprung’s disease?
No, she will not. The sections of the intestines which are not normal will not ‘transform’ and become functional as the child grows older. In fact, with most children, the symptoms become worse with time.
Should you seek a second opinion?
If you are not comfortable with the diagnosis, by all means do. However, it is important to be objective when going to the next doctor. Do not move from doctor to doctor trying to find one whose diagnosis is ‘acceptable’ to you.
You are trying to get to the bottom of your daughter’s problem —remember, sometimes the answers you seek may be difficult to accept.