Reporter:

Andrea O’Dell
 Alsocalled HSCR and congenital
intestinal aganglionosis
 Congenital absence of the
parasympathetic nerve ganglion
cells in the mesenteric plexus of the
distal bowel
 Areaproximal to the aganglionic
portion becomes hypertrophied and
greatly dilated
 Abdominal distention
 intermittent
 progressively increasing
 Anorexia

 Malnutrition

 bowel obstruction with diarrhea

 dehydration and electrolyte imbalance
 Newborn period
 Failure to pass meconium within 24 to 48
hours after birth
 Reluctance to ingest fluids
 Bile stained vomiting
 Abdominal distention
 Infancy
 Failure to thrive
 Constipation
 Emesis
 Abdominal pain or distention
 Episodes of diarrhea
 Ominous signs that often signify the presence
of enterocolitis:
 Explosive, watery diarrhea
 Fever
 Severe prostration
 Childhood

 Constipation
 Ribbonlike, foul-smelling stools
 Abdominal distention
 Visible peristalsis
 Fecal masses easily palpable
 Child usually poorly nourished and anemic
 Three nerve plexus innervate the intestines
 Submucosal (Meissner) plexus
 Myenteric (Auerbach) plexus
 Smaller mucosal plexus

 The submucosal and myenteric plexus are

absent in HSCR
 Intestinal smooth muscle contraction and
relaxation controlled by enteric ganglia
 Control of the intestinal smooth muscle becomes
overwhelmingly extrinsic
 Activity of extrinsic neural afferents are 2-3 higher

 Causes increased smooth muscles tone

 Loss of intrinsic relaxing impulses means increase

in muscle tone is unopposed
 Leads to:

 Imbalance in smooth muscle contractility

 Uncoordinated peristalsis and a functional obstruction
 Incidence of HSCR is approximately one in
5,000 live births worldwide
 Persons of Northern European origin: 1.5 in
10,000
 African Americans: 2.1 in 10,000
 Asians: 2.8 in 10,000
 8686 birth defect cases reported by PGH between
2001 and 2010.
 582 (6.7%) of these were HSCR
 Rectal biopsy
 Small piece of tissue is removed from the rectum
and examined under a microscope
 Best test to diagnose or rule out HSRC
 Abdominal x-ray
 May show intestinal obstruction
 Anorectal Manometry
 Normal, rectal muscle will relax
 If not, HRSC is suspected
 Lower GI series
 X-ray exam of the large intestine
 Child’s large intestine is filled with barium or
another contrast material
 Can show changes in the bowel and help
diagnose obstructions
 HSCR is surgically correctable, majority of patients
live productive, satisfying lives.
 Physical growth and development generally
approximate normal.
 Intellectual function is mostly good.

 Most patients (93%) achieve acceptable anorectal

function
 Long-term functional results are excellent in some,
good in the majority, and poor in approximately 15–
30%.
 Functional results depend on the length of
aganglionosis, procedure performed, surgical
complications, social circumstances, family support,
and associated anomalies, among other factors.
 Psychological problems may be magnified in those with
poor support systems.
 Ethical issues do arise in cases of very long aganglionic
segments, leading to intestinal failure.
 Ethical issues may be pertinent in certain disabling
associated anomalies.
 Genetic counselling (if not handled correctly) also has
the potential for giving rise to ethical issues, but should
not be handled in isolation.
 Constipation related to reduced bowel function
 Outcome Evaluation: Child has a daily bowel
movement through either a colostomy or by enema.
Before surgery, the child may be prescribed daily
enemas to achieve bowel movement. The fluids
used for enemas must be normal saline (0.9% NaCl)
and not tap water
 Nursing Intervention: An antibiotic solution or saline
may be prescribed for daily infusion into the distal
bowel to reduce the possibility of infection
 Imbalanced nutrition, less than body requirements,
related to reduced bowel function
 Outcome evaluation: Child ingests a low-residue diet;
weight follows a percentile curve on a growth
 Nursing Intervention: Pre-op, older children may be poor
in health from poor food intake over a long period of
time. If so, they may be returned home on a minimal
residue diet, stool softeners, vitamins supplements, and
perhaps daily enemas until their condition improves.
 Help parents to make out a reminder sheet for the stool
softener so it is given daily.
 Post-op, after the removal of the aganglonic
portion and anastomosis of the colon at the 2nd
step of the repair, infants will return w/
nasogastric tube in place attached to low
suction, and iv infusion, and probably an
indwelling urinary (Foley) catheter.
 Assess bowel sounds and observe for
abdominal distention
 The child is introduced gradually to fluids, a soft
diet, a minimal residue diet, and finally, a
normal diet for age.
 Acute pain related to the continuity of body
tissues
 Outcome evaluation: The child complains less about
pain at the surgical site; vital signs are within normal
limits
 Nursing Intervention:
 Assess and record the location and duration of pain as
an effective basis for further intervention.
 Nursing Intervention (cont’d):
 Give a fun position that can prevent muscle spasms
that cause pain
 Observe V/S q2 for pain
 Administer appropriate analgesics that serves as an
inhibitor to stimuli so that pain is reduced/lost.
 Disturbed
sleep pattern related to
postoperative wound pain
 Outcome evaluation: Child sleeps soundly for 7-8
hours and seems cheerful.
 Nursing Intervention:
 Assess sleep patterns to determine further
interventions.
 Create a pleasant environment.
 Encourage the parents to help promote lots of rest
and enough sleep for the child.
 Risk
for compromised family coping related to
chronic illness in child
 Outcome evaluation: Parents state that they are able
to cope with the level of stress present from their
child’s condition
 Nursing Intervention: Most parents feel tremendous
relief after the second-stage of surgery is complete.
Caution parents that the child may still remain a
“Fussy eater” for a few months. Help parents to
diminish the importance of meals gradually
 Treatment

 temporary colostomy
 Pre-op

 clear liquids
 bowel prep
 enemas/laxatives
 antibiotic therapy - decrease normal bowel flora
 Concerns depend on the child’s age and type of
treatment.
 During neonatal period, the main objectives are:

 help parents adjust to a congenital defect in their

child
 foster infant-parent bonding
 prepare them for the medical/surgical
intervention
 assist them in colostomy care after discharge
 Depends on the age and clinical condition.
 A child who is malnourished may not be able
to withstand surgery until the physical status
improves
 This often involves symptomatic treatment w/
enemas; low-fiber, high-calorie, and high-
protein diet; and in severe situation, the use
of total parenteral nutrition (TPN)
 Painmanagement
 Hydration

 Stoma assessment

 Bowel elimination

 Teaching
References:

1. Maternal-Child Nursing Care, Susan L. Ward and Shelton M. Hisley, F. A. Davis

Company, 2009.
2. Maternal-Child Nursing Care, Second Edition, Susan L. Ward, et. al., F. A. Davis
Company, 2015.
3. Paediatric Surgery: A Comprehensive Text for Africa. Emmanuel Ameh, et al, Global
Health, 2010.
4. Pediatric Hirschsprung Disease, Holly L. Neville, Medscape, Aug 9, 2016.
5. Occurrence of Birth Defects at the Philippine General Hospital 2001 – 2010. David C.
Padilla, et.al., Acta Med Philippines Oct 2011, 45(4), p 20-29.
6. Hirschsprung Disease, National Institute of Diabetes, and Digestive and Kidney
Disease, https://www.niddk.nih.gov/health-information/digestive-
diseases/hirschsprung-disease
7. Hirschsprung Disease Overview, Melissa A. Parisi, Gene Reviews, National Center for
Biotechnology Information, October 1, 2015.