A COLLECTIVE LOOK AT

HIRSCHSPRUNGS DISEASE,
AND ITS NUTRITIONAL
IMPLICATION.
Caitlin R. Lesko

Sodexo Dietetic Intern

Senior Living Concentration

June 5,2016

OUTLINE
The Large Intestine
Normal Gut Function
What is Hirschsprungs Disease
Medical Nutrition Therapy
Study Summary & Review
Summary

THE LARGE INTESTINE

The adult large intestine is roughly 5 feet in
length.
Absorbs water and sodium form material that
has not been digested.
Made up of 3 sections: Cecum, Colon, and
Rectum.
Rid the body of waste products.
Bacteria in gut is necessary for normal function.

NORMAL GUT FUNCTION

Peristalsis: series of muscle contractions.
Enteric Nervous System: the system of neurons
that control the function of the gastrointestinal
system.
Ganglion cells & nerve fibers line the muscle wall
of the GI tract.

PERISTALSIS
The series of wave like muscle contractions
responsible for moving food through the digestive
tract.
What if peristalsis is disrupted?
Can lead to malabsorption
What leads to lack of motility
diet
obstructions
nerve damage

HIRSCHSPRUNGS DISEASE
Occurs when the ganglion cells of the Auerbachs
plexus are missing in sections or all of the large
intestine.
Also known as congenital mega colon.
Discovered by Harald Hirschsprung, a Danish
pediatrician in 1886.

EPIDEMIOLOGY
Congenital disorder
Occurs statistically in roughly 1 in every 5,000
births.
More common in males than females.
More prevalent children with other congenital
disorders.
As a fetus Ganglion cells begin to develop in the
mouth and follow the gastrointestinal tract to the
anus.
Hirschsprungs disease is usually detected early
in infancy or childhood.

SIGNS & SYMPTOMS

90% of infants fail to have a BM in the first 24
hours of life.
Poor appetite, abdominal distention,
constipation, pain, and poor weight gain.
Delayed growth & development.
Enterocolitis

DIAGNOSTIC PROCESS &

PROCEDURES
Rectal Biopsy
Removal of tissues from the rectum to test for
ganglion cells.
2 types of tests
Rectal Suction Biopsy
Rectal Punch Biopsy

RECTAL PUNCH BIOPSY

The removal of a small section of tissue, either

that directly pierces the tissues or enters the skin
via a small incision to removal a cylindrical
biopsy.

RECTAL SUCTION BIOPSY:

The physician will place a thin flexible tube into

the rectum and remove a few tiny pieces of tissue.

DIAGNOSIS
After the rectal biopsy, the patient will have a
Barium Enema.
Shows the location of the affected sections of
colon.

ANORECTAL MANOMETRY.
The next step in the diagnostic process is an
Anorectal Manometry test .
Test is done to test the sphincter muscles and
over all muscle movement.

TREATMENT
Surgical intervention is the only treatment.
Colostomy/ Ileostomy
Pull through procedure, 6-12 months post ostomy.

Swenson

Duhamel
Soave

SURGICAL TREATMENT
CONTINUED.

SWENSON
Orvar Swenson, 1948
The pull-through procedure repairs the colon by
connecting the functioning portion of the bowel to
the anus.
Leaves a small portion of the diseased bowel.

DUHAMEL

1956

Excludes the rectum rather than removing it.

Becoming more popular, roughly 62% of pediatric

surgeons prefer Duhamels technique in treating
Hirschsprungs disease.

SOAVE

Involvies removing the mucosa and submucosa of

the rectum and placing the pull-through bowel
within a cuff of aganglionic muscle.
The original description of the procedure left the
pull-through colon hanging out through the anus
with a subsequent operation several weeks later
creating the final anastomosis.

MEDICAL NUTRITION THERAPY

Each patient needs must be assessed on an
individuals basis.
Most patients will be NPO post surgery, this may
require alternate forms of nutrition to avoid
malnutrition, combat weight loss, and support
growth & healing.

UPON DIET ADVANCEMENT

Those who have an ostomy will follow ostomy
guides.
Clear liquids to full liquid, to soft/low residue,
then finally house/ regular diet.
Constipation & fiber.

LOW FIBER FOODS

Milk & yogurt.
Tender beef, poultry, fish,
Refined grains, white bread, pasta, rice, and
cereal.
Fruits & vegetable without the skin, seeds, or
pulp.

Foods to avoid:
yogurt with added fruit, tough meats, dried
beans, whole wheat grains, high fiber cereals, raw
vegetables, dried fruit & fruit skins.

ALTERNATE NUTRITION
Enteral nutrition is the preferred method.
Indications include: prevention of gut atrophy,
supplemental feedings to help patient meet
nutritional needs.
Must monitor for signs of intolerance.
Refeeding syndrome.
No specific formula, but many benefit from an
elemental for semi-elemental.
Hydrolyzed formulas maybe used for patients
with intolerances, easier to digest.

PARENTERAL NUTRITION
Indications, the gut cant be utilized
The gut cant be utilized, NPO for 5 days or
greater.
Formulas made up of Dextrose, Amino Acids,
Lipids, trace electrolytes, and trace minerals.
Adjusted to patients needs & life style.

Implications, include risk of infection, gut

motility.

IMMUNOHISTOCHEMISTRY VERSUS
ACETYLCHOLINESTERASE HISTOCHEMISTRY IN THE
EVALUATION OF SUCTION RECTAL BIOPSIES FOR
HIRSCHSPRUNG DISEASE

2008 comparative study from the Journal of Pediatric

Development Pathology.
Examined the evaluation of rectal biopsy tissues samples.
The study had multiple physicians reviewed samples of
calretinin immunohistochemistry and acetylcholinesterase
sections from suction rectal biopsies from 14 Hirschsprungs
disease positive subjects and 17 negative Hirschsprungs
disease subjects.
Calretinin immunohistochemistry provided no
misdiagnoses.
Acetylcholinesterase stained sections method resulted in 2
misdiagnosis.
Raj P. Kapur, Robyn C. Reed, Laura S. Finn, Kathleen Patterson, Judy Johanson, and Joe C. Rutledge (2009) Calretinin
Immunohistochemistry versus Acetylcholinesterase Histochemistry in the Evaluation of Suction Rectal Biopsies for Hirschsprung
Disease. Pediatric and Developmental Pathology: January 2009, Vol. 12, No. 1, pp. 6-15.

LINK BETWEEN HIRSCHSPRUNGS

DISEASE & DOWN SYNDROME
2013 study from the International Journal for
Pediatric Surgery.
16,497 HD patients, and 29,418 Down syndrome
patients from a systematic literate based study.
Overall occurrence of Down syndrome among the
positive Hurschprungs disease population to be
7.32%.
Overall rate of Hurschsprungs disease among
positive Down syndrome population to be 2.62%.

CLINICIANS VS. LAY PUBLIC

Issues: age, transitional feeding, right vs.
wrong.
Cincinnati Children's Hospital Colorectal Center.

Other challenges include social stigma.

Seattle Children's Hospital

SUMMARY

Due to advances in surgical procedures,

continued gastrointestinal research, and positive
outcomes post-surgical procedure its a
manageable diagnosis.

THANK YOU,

Questions??

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