Is a condition that affects the colon causing an enlargement of the colon.
Can also be referred to as aganglionic megacolon.
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 a possible genetic cause may have been studied.
The RET proto-oncogenes from Chromosome 10 may be a gene involved in this condition.
But this is still a debated genetic cause.
This disease is rather uncommon and occurs on average of 1 out of every 5000 births
5 times more likely in boys then girls and more often seen in those children with Down syndrome
Location:
Absence of ganglion cells in the intestines
Recto-sigmoid - occurs in 70-75% of cases
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
- Pull-through procedure
. repairs the colon
Often in young children the Pull-through treatment option is used on its own without the use of a colostomy.
Laxatives may be necessary after surgery
High fiber diet