HIRSCHSPRUNG’S DISEASE of the internal sphincter by inserting

a balloon catheter into the rectum

HIRSCHSPRUNG’S DISEASE is also and measuring the pressure exerted
known as congenital aganglionic against it.
megacolon, so Hirschsprung’s is a disease NURSING DIAGNOSIS:
that is present since birth in which a  Imbalanced nutrition, less than the
ganglion, or cluster of nerves is missing, body requirements, related to
which ultimately leads to a blocked colon, reduced bowel function
causing it to enlarge.  Constipation related to reduced
PATHOPHYSIOLOGY bowel function
 Risk for compromised family coping
Due to the absence of ganglionic cells related to chronic illness in child
 Deficient knowledge of caregivers
There is lack of peristalsis in the affected portion
related to understanding of
Functional Obstruction
postoperative care of colostomy
 Fear (in older children) related to
Accumulation of gas & feces proximal to the defect impending surgery
 Acute pain related to the surgical
Enlargement of the colon occurs and called MEGACOLON
procedure
 Risk for skin integrity related to
ASSESSMENT:
irritation from the colostomy
Symptoms of aganglionic megacolon  Impaired oral and nasal mucous
do not become apparent until 6-12 months membranes related to NPO and
age. irritation from NG Tube
NURSING INTERVENTIONS:
 Thin and undernourished
Preoperative Phase
 Large distended abdomen
Informed Consent
 Hx of not having a bowel movement
Preoperative Assessment:
more than once a weak
 Physical Assessment
 Ribbonlike or watery stools
 Pre-surgical Screening Test
 Vomiting
 Health Factors
 Diarrhea (enterocolitis)
Physiological Preparations:
 Delayed passage of meconium
DIAGNOSTIC TOOLS:  Managing nutrition and Fluids
 Laboratory Studies  Managing Infections – Colorectal
 Rectal Biopsy Irrigation
 Rectal Examination Psychosocial Preparation:
 Plain Abdominal Radiography ‘  Explain the disease properly to the
 Contrast Barium Enema parents
 Anorectal Manometry – a technique
that tests the strength or innervation
Diet: 2. Decompression
 Patient should have nothing by  Nasogastric Tube
mouth before the operation  Normal Saline Rectal Irrigations
Post-Operative Phase 3. Diet
 Regular colostomy care to be done  Breast milk
following aseptic guidelines SURGICAL MANAGEMENT
 IV fluids are given to maintain Surgical options vary according to:
adequate hydration and electrolyte  Patient’s age
balance  Mental status
 To prevent contamination of the  Ability to perform activities of
wound with urine of baby, diaper daily living
must be pinned below the dressing  Length of aganglionic segment
or urethral catheterization can be  Degree of colonic dilation
done.  Presence of enterocolitis
 Explain to significant others about Leveling Colostomy
procedure of skin care, frequency of Leveling Colostomy, which is
care, signs of complication. colostomy at the level of normal bowel; a
Follow-Up: staged procedure with placement of a
Further Inpatient Care leveled colostomy followed by a pull through
 If a diverting colostomy is created in procedure.
a newborn, he/she must remain in Single-Stage Pull-Through Procedure
the hospital until the colostomy is The single-stage pull-through
functioning and feeding goals are procedure may be performed with
obtained. Feeding are usually laparoscopic, open, or transnasal
initiated 24-48 hours after the techniques. This procedure can be
surgery. performed at the time of diagnosis or after
 After definitive pull-through the newborn has had rectal irrigation at
procedure is performed, the patient home and has passed the physiologic nadir.
is hospitalized until full feeding are PHARMACOLOGIC THERAPY
possible and evidence of the return 1) Ampicillin
of bowel functions is obtained. 2) Gentamicin
 Colorectal Irrigation may still be 3) Metronidazole
needed.
Further Outpatient:
 Patients should be monitored for
normal bowel habits. Reporters:
MEDICAL MANAGEMENT SARIGALA, Putri Aisharoqayya B.
1. Initial Therapy SAUMAY, Norjannah
 Intravenous Hydration SIGAYAN, Fais D.
 Withholding of enteral intake