INTRODUCTION

Hirschsprung's disease is a condition that affects the large intestine (colon) and causes problems with passing stool. The condition is
present at birth (congenital) as a result of missing nerve cells in the muscles of the baby's colon. Without these nerve cells
stimulating gut muscles to help move contents through the colon, the contents can back up and cause blockages in the bowel.

Signs and symptoms of Hirschsprung's disease vary with the severity of the condition. Usually signs and symptoms appear shortly
after birth, but sometimes they're not apparent until later in life. Typically, the most obvious sign is a newborn's failure to have a
bowel movement within 48 hours after birth. Sometimes occurs in families and might, in some cases, be associated with a genetic
mutation.

Hirschsprung's disease occurs when nerve cells in the colon don't form completely. Nerves in the colon control the muscle
contractions that move food through the bowels. Without the contractions, stool stays in the large intestine. Children who have
Hirschsprung's disease are prone to a serious intestinal infection called enterocolitis. Enterocolitis can be life-threatening and
requires immediate treatment.

Hirschsprung's disease is treated with surgery to bypass the part of the colon that has no nerve (ganglia) cells. The lining of the
diseased part of the colon is stripped away, and normal colon is pulled through the colon from the inside and attached to the anus.
This is usually done using minimally invasive (laparoscopic) methods, operating through the anus. In children who are very ill,
surgery may be done in two steps. First, the abnormal portion of the colon is removed and the top, healthy portion of the colon is
connected to a small hole (ostomy) the surgeon creates in the child's abdomen. Stool then leaves the body through the ostomy into
a bag that attaches to the end of the intestine that protrudes through the hole in the abdomen. This allows time for the lower part
of the colon to heal.

OBJECTIVES:

This case study aims to provide proper knowledge about hirschsprung’s.. The participants will be able to understand appropriate
care and response for the child’s health status and recovery

process.

General Objectives:

At the end of the case study, participants will be able to apply knowledge, skills, attitude in providing therapeutic care for the patient
with hirschsprung’s.

Specific Objectives:

The students will be able to:

Knowledge:

1.Identify and classify the patients condition by knowing different signs and symptoms of hirschsprung’s..

2.Discuss pathophysiology of hirschsprung’s.

3.Learn the procedure of abdomino pull-through surgery ant its management.

Skills:

1. Provide a proper nursing care for the patient in managing the signs and symptoms using the nursing process procedure.

2.Implement nursing care in managing the client's needs.

3. Document correctly patient's condition, nursing interventions and evaluation.

Attitude:

1.Establish rapport with the patient and members of the family.

2. Communicate effectively with the patient.

3. Show outmost confidence in managing patient care.

Defining Nursing Diagnosis Planning Nursing Rationale Evaluation

Characteristics   Intervention
  Constipation related After 8 hours · Determine stool color, · Assists in identifying After 8 hours
to changes in of nursing consistency, frequency, and causative or contributing of nursing
Objective: digestive process. interventions, amoun factors and appropriate interventions,
· Altered bowel sounds the patient intervention. the patient
  will   was able to
   
  demonstrate   demonstrate
changes in · Bowel sounds are generally changes in
· Report of abdominal Rationale: behavior as   increased in constipation. behavior as
pain or cramping
necessitated necessitated
Hirschsprung disease · Auscultate bowel sounds
  by causative   by causative
is a birth defect in
and   and
· V/S are taken as which some nerve · May identify dehydration,
contributing contributing
follow cells are missing in   excessive loss of fluids or aid
factors. factors
the large intestine, in identifying dietary
  so a child's intestine     deficiencies
can't move stool and
Temp – 37.2c   · Monitor intake and output  
becomes
(I&O) with specific attention
Heart Rate – 150bpm blocked.Constipation · Decreases gastric distress
  to food or fluid intake.
occurs when bowel and abdominal distension.
RR: 46 movements become  
less frequent and  
respirations/minute,  
stools become
Weight: 3.2kilograms. · Prevents skin excoriation
difficult to pass. It
· Recommend avoiding gas and breakdown.
  happens most often
forming foods.
due to changes in  
Physical examination diet or routine, or  
shows an alert, active due to inadequate  
neonate with intake of fiber.  
abdominal distension  
and hypoactive bowel   · Assess perianal skin
condition frequently, noting  
sounds, a reducible
  changes or beginning
umbilical hernia, and  
breakdown. Encourage or
bilateral hydroceles. -Nurselab
assist with perineal care  
  after each bowel
 
movement.
abdominal exam at that
point was significant for    
severe distention.  
· Discuss use of stool
  softeners, mild stimulants,  
enemas as
  · Facilitates defecation when
indicated. Monitor constipated is present
abdominal x-ray was effectiveness.
obtained and revealed  
large amount of  
retained   
Collaborative
barium contrast within  
 
normal caliber rectum.
 
   • Consult with dietitian to
provide well balanced diet  
high in fiber and bulk
 
 
 
 
· Fiber resist enzymatic
  digestion and absorbs liquids
in its passage along the
  intestinal tract and thereby
  produces bulk, which acts as
a stimulant for defecation
 
 
 
Full-thickness rectal biopsy
  remains the gold standard
against which all other tests
  are measured since it was
  described by Swenson in
1955. It requires general
underwent a full-thickness anesthesia and may be
rectal biopsy complicated by bleeding,
perforation, and scarring
 
 
 
pull-through surgery is to
  remove the diseased section
  of your child's intestine and
then pull the healthy portion
  of this organ down to the
anu
 
A colostomy is an operation
 
that creates an opening for
  the colon, or large intestine,
through the abdomen. A
scheduled for abdomino- colostomy may be
pull-through surgery. temporary or permanent. It
is usually done after bowel
 
surgery or injury.
 
 
 
 
 
 
 
 
 
 
temporary colostomy and 
 
tube,