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Hirschsprung’s Disease is a condition that affects the colon causing an enlargement of the Colon. This condition can also be referred to as aganglionic megacolon.

It is often a result of an obstruction in the bowel because of a lack of ganglionic cells in a section of the bowel. This condition affects newborns who’s intestines lack nerves which prevents normal peristalsis of that part of the intestines

The length of the affected section of the colon can be up to 30 cm.

Recently, studies have considered a possible genetic cause. The RET proto-oncogenes from Chromosome 10 may be a gene involved in this condition.  But this potential genetic cause is still at the center of thsi debate.

Hirschsprung’s disease is rather uncommon and occurs on average of 1 out of every 5000 births. It is 5 times more likely to be seen in boys then girls and more often seen in those children with Down syndrome

Location:

There can be an absence of ganglion cells in the intestines

1.)  Recto-sigmoid –  occurs in 70-75% of cases
2.)  Entire colon –  occurs in 5-8% of cases

Findings

Enlargement of colon –  proximal to affected areas
Narrowing of colon –  distally to affected areas

–  When ganglion cells are absent the colon, as a result, experiences an excess amount of stimulation or contracting of the colon
–  This causes difficulty for feces to pass through the intestines

Symptoms

–  Meconium  [First bowel movement of infant]
–  Constipation
–  Distension of the abdomen
–  Fever
–  Lack of appetitie
–  Vomiting

Normal meconium passage

–  85-90% passage within the first 24 hours of birth
–  8-10% passage within 48 hours

Diagnosis

– Is made by a biopsy of the bowel

Other options

–  X-ray
–  Barium enema
–  Anorectal manometry

Treatment

1.)  Surgical removal of the affected section

2.)  Reanastomosis

–  Use of a colostomy  [is used less often today]
–  Bowel is cut and an opening is made in the abdomen
–  Feces are expressed into a bag

3.) Pull-through procedure

4.) Repairs the colon

–  Often in young children the Pull-through treatment option is used on its own without the use of a colostomy.

6.)  Others

–  Laxatives may be necessary after surgery

–  High fiber diet