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HIRSCHSPRUN

GS DISEASE
Kim Eligio, RN
Hirschsprungs Disease
Motor disorder of the colon, which is caused by the
absence of enteric neurons (ganglia) in the
myenteric and submucosal layer of the colon. The
resulting aganglionic segment of the colon fails to
relax, causing a functional obstruction.
Also called as Anganglionic Megacolon
1 per 5000 live births
4x Male > Female
Children with HD usually diagnosed at first 2 years
of age
Etiology
During fetal development, neuroblasts that
originated from the neuronal crest cells failed
to migrate to the colon between 8th to 12th
weeks
Accelerated cellular destruction in the
intestine can also be a cause
PATHOPHYSIOLOGY
OF HIRSCHSPRUNGS
Peristalsis takes place in normal colon

Intestinal contents move down (peristalsis)

Partial obstruction at the aganglionic segment of colon


(no movement)

Normal colon becomes distended and walls markedly thickened


because of muscle hypertrophy

Constipation Vomiting Enterocolitis


fecal content *Cx: Toxic Megacolon
sit in the bowel Perforation
growth of
bacteria
Clinical Findings
Unable to pass first meconium for the first 48 hours
Nearly 50% of newborns that has delayed passage
of meconium has Hirschsprungs disease
Obstipation unable to pass gas
Constipation
Abdominal distension
Repeated vomiting
Failure to thrive
Tight anal sphincter meconium plug syndrome
Squirt sign on digital examination
Diagnosis
Abdominal X-Ray
o dilated normal colon (ganglionic segment),
narrowed segment of the aganglionic colon
Diagnosis

Contrast Enema (Barium)


o performed unprepped or without stool
cleanout
o reveals presence of transition zone; from
normally innervated to non-innervated bowel
o transition zone looks like funnel shape;
narrowing is present
Diagnosis

Rectal Manometry
o shows failure to demonstrate reflex
relaxation of the internal anal sphincter in
response to inflation of a rectal balloon.
Diagnosis

Rectal Suction Biopsy


o definitive diagnostic test
o adequate colon tissue sample is examined
o absence of ganglion cells/nerves confirm the
diagnosis of Hirschsprungs disease
Goal of Management:
1. To treat the symptoms of untreated
Hirschsprung disease
2. To perform temporary measures to prevent
complications until reconstructive surgery is
performed
3. Manage postoperative bowel functions
Medical Management:
NPO
Fluid and electrolyte management IVF
Minimize bowel distension
NG decompression
Colonic lavage by mechanical irrigation with
saline 3-4 times daily
IV antibiotics if suspected enterocolitis
Surgical Management
Diverting Colostomy
o Temporarily placed until pt grows to10 kg.
o Generally followed by pull-through procedure
o Performed for pt who present with sepsis due to
enterocolitis, massive distension of ganglionic
bowel prohibiting pull-through procedure
o Transition zone is identified and colostomy is
placed proximal to this area
Surgical Management
Pull-through Procedure
o Aims at surgical resection of aganglionic
segment and anastomosing two normal
ganglionic ends.
o Excellent result in 90%
a) Swenson
b) Duhamel
c) Soave
d) Rehbein
e) Boleys
Surgical Management
Endorectal Pull-through
o Aganglionic segment of the colon is removed
and the normal innervated colon is anastomosed
to the rectum.
Surgical Management
Transanal Endorectal Pull-through
o Laparoscopic procedure latest approach
o Performed safely in infants
o One-stage surgery
Post Surgical Care:
Monitor VS
IVF replacement at maintainance with TPN
o Intermittent fluid boluses may be required in the
first 48hr to maintain adequate urine output and to
treat hypotension and hypoperfusion
o May require more if there is capillary leak from
extensive bowel manipulation
Gastric drainage/decompression Repogle tube on low
continuous suction
o May require volume replacement if drainage more
than 10ml/kg in 12hr shift
Post Surgical Care:
Routine colonic irrigation/lavage
Prophylactic antibiotics to prevent enterocolitis
Feeding initiated after 24-48 hours postop or after
passing flatus/stool, start small volume feeds then
advance slowly as tolerated to prevent NEC
Monitor for signs of complications:
Recurrent enterocolitis
Pain Management
Skin care pt may have frequent loose stools postop
Stoma care if pt has colostomy
Parent Education
Rectal Irrigation
o Manual removal/irrigation of feces by
instilling NS through the rectum, and
aspirating feces
Parent Education
Anal Dilation
o Prevent anal strictures from forming because
of the scar tissue around the anus
o When strictures happen, passing bowel
movement can be difficult
Parent Education
Watch out for:
o Fever
o Abdominal distension
o Vomiting or diarrhea
o Rectal bleeding
o Lethargy
THANK YOU!!!