HIRSCHSPRUNG DISEASE

MEGACOLON
AGANGLIONIC
RIBON LIKE STOOL
HIRSCHSPRUNG DISEASE
A disease caused by the congenital absence of
parasympathetic nerve ganglion cells in the distal
bowel. As a result portion of the bowel is
unable to transmit regular peristaltic waves, which are
coordinated with the proximal portion of the bowel.
When a stool reaches the diseased area it is not
transmitted on down the colon, but it accumulates in
the segment just proximal to this area, forming a
functional obstruction. The bowel above the obstructed
portion eventually becomes hypertrophied in its
attempts to transmit the school.

HIRSCHSPRUNG DISEASE
MEGA COLON
Aganglionic
- ganglion cell @ large intestine
FUNCTION: peristalsis/movement of
food.
CAUSE: unknown/congenital




ASSESSMENT
Assess for failure to pass meconium in newborn.
Assess for symptoms of bilous vomiting.
Evaluate for signs of intestinal obstruction.
Evaluate for sign of constipation and abdominal
distention.
Assess for foul odor of breath and stool.
Note that in older child symptoms of
constipation, offensive odor and ribbon like stool.
DIAGNOSIS TEST
Rectal biopsy- a procedure used to
remove a piece of rectal tissue for
examination.
- ganglion cell
MANAGEMENT
In mild cases, treatment is enemas, stool
softener and low residue diet.
NPO
COLOSTOMY
SURGERY ABD. PERINEAL PULL
THROUGH.
LIGATION + ANASTOMOSIS
OBJECTIVE
1. TO BE ABLE TO DISCUSS THE TOPIC
CLEARLY.
2. TO BA ABLE TO DEFINE THE MEDICAL
WORDS.


Colostomy- is an operation that create an opening
of the colon or large intestine through abdomen.
The edge of the colon are then stitched to the skin
of the abdominal wall to form an opening called a
stomata. Stool drains from the stomata into a bag
or pouche, attached to the abdomen.
Surgical abdomen perineal pull through- a surgey to remove the
portion of the colon that has no nerve cell and pulling the healthy
portion of the colon to the rectum
IMPLEMENTATION
A. Prior to diagnosis, observe carefully for all
gastrointestinal manifestation of the disease and
report them accurately.
B.Prior to the colostomy procedure
1. Cleanse bowel
2. stool softeners
3.colonic irrigation saline
C. Prepare parents for the procedure.
1. Clarify the surgical technique.
2.Describe the stomata.
3. prepare for care of the child with a colostomy.
4. Give parents the oppurtunity to express their
feelings about the procedure.
D. Postoperative care
1. Maintain optimal nutrition.
2. Closely observe stools for reestablishment of
normal elimination pattern.
3. Maintain skin care of colostomy and anal
areas.