(HIRSCHSPRUNG’S

DISEASE)

Congenital
Megacolon

Prepared by: ALANGADI, nashibah M.

What is Hirschsprung’s
Disease?
 Is the most common cause of lower intestinal obstruction in
neonates.
 A rare disorder of the bowels, most commonly the large
bowel (colon).
 Is named after Harald Hirschsprung, a pediatrician at Queen
Louise Children´s Hospital in Copenhagen, Denmark, who
described 2 cases of children with megacolon in 1887.
What are the symptoms of
Hirschsprung’s disease?
 Should be considered in any child who has history of constipation dating to newborn period

 90% of cases diagnosed in newborn period

 Most common presentation in newborns: delayed stool passage within first 48 hrs of life

 Constipation, abdominal distension, poor feeding and vomiting

 Constipation followed by explosive diarrhea, failure to thrive

 in older children, large fecal mass palpable in left lower quadrant. rectum is empty

 stools: small pellets, ribbon-like, fluid consistency

Hirschsprungs disease’ xray
How is Hirschsprung’s disease
diagnosed?
 Abdominal x-rays: air fluid levels in colon and distended loops of intestine.
 Should not be done with clinical enterocolitis: may cause perforation.
 Rectal biopsy: gold standard

 Can be performed at bedside without general anesthesia.

 Biopsy taken at 2 cm, 3 cm, 5 cm above dentate line.

 Diagnostic accuracy: 99.7%

 Most common problem is inadequate specimen (insufficient amount of

(submucosa).
Embryology and Etiology

 Neuroenteric cells migrate from neural crest to

upper end of alimentary tract and proceed in distal
direction

 12th week : migration to distal colon – first into

myenteric (Auerbach’s plexus) then into
submucosal plexus

 Embryologic defect
Contrast Enema showing Aganglionic segment with the
transition zone
 Pathology
 Neonatal period: intestine is normal

 Distal intestine: absence of ganglion cells in the

submucosal (Meissner’s) plexus and myenteric
(Auerbach’s) plexus

 Marked increase in nerve fibers which extend into the

submucosa (seen with acetylcholinesterase stain)

 Aganglionosis extends to rectosigmoid region in 80%

of cases

 Rectal exam: normal anal tone followed by explosive

discharge of feces and gas
 Treatment
 Decompression: nasogastric tube, rectal
tubes
 perform surgery after diagnosis established
or perform temporary colostomy until infant
is 6-12 mos old
 3 basic surgical approaches:

a) Swenson
b) Duhamel
c) Soave
Plain X-ray showing dilated loops of bowel
with absence of gas in the rectum (BACK).
 Management
 Acute I.O. : if the patient presents with acute intestinal obstruction in the early
life the management will be
 resuscitation ,
 NGT , NPO
 IVF ,
 Antibiotics ,
 Rectal tube,irrigations .
 The initial treatment requires performing a "leveling" colostomy in the most distal colon
with ganglion cells present. This requires exploration with multiple seromuscular
biopsies of the colon wall to determine the exact extend of the aganglionosis. The
colostomy is placed above the transition zone. Placement of the colostomy in an area of
aganglionosis will lead to persistent obstruction
 When the patient becomes stable, then the definitive treatment will be planned.
 Chronic constipation :
 laxative
 saline enema.
 Work up to establish the diagnosis
 then the definitive treatment will be planned
 Pathophysiology

EMBRYOLOGY: Pathophysiology
The neuroenteric ganglion cells
migrate from the neural crest to the
upper end of the alimentary tract and
then follow the vagal fibers caudally
delay or arrest in this migration
results in the neural crest cells failing
to reach the distal bowel
 Complications

 Early complications: anastomotic

strictures (15%), wound infections
(11%), anastomotic leaks (7%)

 Late complications: chronic

constipation, enterocolitis,
encoporesis

 Good prognosis: more than 90% of

children achieve normal bowel
movement
Hirschsprung figure.
What is the outlook for children with
Hirschsprung disease?
The outlook for children with Hirschsprung
disease is good, with the majority growing up
to live normal lives working and raising a
family. They may however experience long-
term problems with constipation or bowel
control. Some children have ongoing
problems and need more operations.
 CUES NURSING OBJECTIVES INTERVENTIO RATIONAL EVALUATIO
NS N
DIAGNOSIS E
Subjective: Risk for After the a) Evaluate a)Identifying After the
Unable to constipation nursing medication/drug the effects of nursing
pass stool, related to: the intervention the usage and note medications to intervention
Abdominal client/SO will interactions or the client can the client or
narrowing of
pain. be able side effect. minimize its SO was able to
the colon. verbalize b) Check for adverse :verbalize
Objective: understanding presence of effects. understanding
straining of etiology and fecal impaction b) Identify any of etiology and
with appropriate as indicated. presence of appropriate
defecation,Di interventions/s c) Assist with obstruction. interventions/s
stended olutions for medical workup c) olutions for
abdomen; individual for Collaborating individual
abdominal situation; identification of with the situation;
tenderness minimize other possible medical team minimize
with or discomport. causative can detect the discomport.
without factors. root of
palpable problem.
muscle
resistance,
dry, hard,
formed stool.
 NURSING CARE PLAN
CUES NURSING OBJECTIVE INTERVENTIO RATIONALE EVALUATI
DIAGNOSIS S NS ON
a) Teach px and b) Washing After the
Risk for After the caregiver the s/s of between nursing
infection nursing infection, and when to
procedures reduces intervention
report these to the
related to: intervention physician or nurse. the risk of the patient will
post surgical the patient transmitting be able to
procedure was able to b) Wash hands and pathogens from remain free of
teach other caregivers one area of the infection, as
remain free to wash hands before body to another. evidenced by
contact with the px, and
of infection, between procedures
normal VS and
as evidenced with the px. c) Bladder absence of S/S
infection more of infections.
by normal c)Demonstrate and related to over
VS and allow return distended bladder
demonstration of all
absence of high-risk procedures
resulting fron
S/S of infrequent
that px or caregiver
will do after discharge, catheteriarion than
infections. such as dressing to use of clean
changes, peripheral or versus sterile
central IV site care, technique.
peritoneal dialysis, self-
catherization (may use
clean technique).
 NURSING CARE PLAN
CUES NURSING OBJECTIV INTERVENTIO RATIONAL EVALUATIO
DIAGNOSIS NS N
ES E
Subjective: Acute pain After the a) Assess for a)Attention to After the
Patient complains associated signs may
related to: nursing signs and help the nurse in nursing
of pain.
abdominal intervention symptoms evaluating pain.
intervention
Objective: distension the patient relating to pain. b) This influences the patient
Appetite changes; b) Assess the the perceptions of
irritation, was able to will be able
self-focused; px anticipation the effectiveness of
Guarding surgical use for pain relief. the treatment to use
modality and their
behavior, response. pharmacologi c)Evaluatte the eagerness to engage
pharmacologi
protecting body cal and patient’s in further treatments. cal and
part, facial mask
of pain;
nonpharmaco response to c)It is essential to
nonpharmaco
Autonomic logical pain- pain and assist pxs express as logical pain-
responses(e.g., relief management factually as possible
relief
the effect of pain
diaphoresis, an strategies; strategies. relief measures. strategies;
alteration in BP,
HR, pupillary patient patient
dilation; displays displays
alteration in RR; improvement improvement
pallor; nausea)
in mood, in mood,
coping. coping.
BACK
TRIVIA

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