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HIRSCHSPRUNG’S DISEASE
Hirschsprung disease, congenital
aganglionic megacolon is a developmental
disorder of the enteric nervous system and is
characterized by an absence of ganglion cells
in the distal colon because of a disruption of
normal neural crest cell migration, result in a
functional obstruction.
 Enlargement of the colon, caused by
bowel obstruction that starts at the anus and
progresses upwards.
Ascending colon
 On the right side of the abdomen, 25 cm.
 Cecum to the hepatic flexure.
 Parasympathetic fibers of Vagus nerve (CN X),
 Ileocolic artery and right colic artery, both branches of the
Superior Mesenteric Artery.
Transverse colon
 Part of the colon from the hepatic flexure to the
splenic flexure.
 The proximal two-thirds :-the middle colic artery, branch
of superior mesenteric artery,
 The latter third:- branches of inferior mesenteric artery.
Descending colon
 Splenic flexure to the beginning of the sigmoid colon.
 Store matter that will be emptied into the rectum.
 The left colic artery.
Sigmoid colon
 After the descending colon and before the rectum.
 S-shaped.
 Muscular, and contract to increase the pressure inside
the colon, causing the stool to move into the rectum.
 Sigmoid arteries, a branch of the Inferior Mesenteric
Artery.
 https://youtu.be/1pylBKNMcnw
 https://youtu.be/9QjZe6zZpRA
Aganglionic segment of colon becomes nonfunctional.
Motor disorder in gut .
Absence of the ganglion cells in the myenteric and submucosal
plexus.
Defect in the migration of the neural crest cell.
(precursor of the entric gangolic cells)
Defect in the
migration of
neuroblasts to
the distal
intestine
.
Failure of
neuroblasts to
survive,
proliferate, or
differentiate.
Smooth muscle
cells of
aganglionic
colon are
electrically
inactive Abnormalities in
pacemaker cells
connecting enteric
nerves & intestinal
smooth muscle
 Three neuronalplexus
 The sub mucosal (ie, Meissner) plexus,
 The intermuscular (ie, Auerbach) plexus,
 The smaller mucosal plexus.
Normal motility is primarily under the control of
intrinsic neurons.
Control both contraction and relaxation of smooth
muscle.
Extrinsic control is mainly through the cholinergic
and adrenergic fibers. The cholinergic fibers cause
contraction, and the adrenergic fibers mainly cause
inhibition.
 Aganglionosis begins with the anus, which
is always involved, and continues
proximally for a variable distance.
Myenteric (Auerbach) plexus & submucosal
(Meissner) plexus are absent, reduced
bowel peristalsis & function.
Frequency
1 case per 1500 newborns to 1 case per 7000
newborns.
Sex
 M>F; M:F=4:1.
 However, with long-segment disease, the
incidence increases in females.
Age
 Uncommon in premature infants.
 Approximately 90% of patients with Hirschsprung
disease are diagnosed in the newborn period.
Approximately 20% of infants have one or more
associated abnormality (neurological,
cardiovascular, urological, or gastrointestinal
system).
Hirschsprung disease has been found to be
associated with Down syndrome
 Untreated aganglionic megacolon in infancy may
result in a mortality rate of as much as 80%
In cases of treated Hirschsprung disease, the
mortality rate may be as high as 30% as a result of
enterocolitis.
 Newborn with delayed passage of meconium
 Any child with a history of chronic constipation since birth.
 Ribbon like stool
 Foul smelly stool.
 Bowel obstruction with bilious vomiting,
 Abdominal distention,
 Poor feeding
 Failure to thrive.
 About 10% diarrhea caused by enterocolitis
 Colonic perforation,
 Sepsis
 vomiting.
◦ Ultra short segment: below recto sigmoid junction
◦ Short segment: up to sigmoid colon
◦ Long segment: up to splenic flexure or beyond
◦ Total segment: affects whole colon.
Distendedabdomen
Inability to releaseflatus
An empty rectum on digital rectal examination
Rectalimpaction
Rapid expulsion of feces afterrectal examination
H/o failure to pass meconium within 24-48 hours after birth.
Plain abdominal radiographs may show distended
bowel loops with a paucity of air in the rectum.
Barium enema
Anorectal manometry
Anorectal manometry is a test that measures how well the
rectum and anal sphincter are working.
Rectal biopsy
The specimen must be
obtained at least 1.5 cm
above the dentateline
because aganglionosis
may normally be present
below this level.
Simple suction
rectalbiopsy
Rectal mucosa
and submucosa
are sucked into
the suction
device, and a
self-contained
cylindrical
knife cuts off
the tissue.
Can be easily
performed at the
bedside.
 Chemistry panel: dehydration(diarrhea).
 CBC count: preoperative hematocrit and platelet
count.
 Coagulation studies: clotting disorders are
corrected before surgery.
 Because cardiac malformation (2-5%) and Trisomy
21 (5-15%) are associated with congenital
aganglionosis, cardiac evaluation and genetic
testing may be warranted.
The general goals of medical care are 3-fold
1. To treat the complications of unrecognized or
untreated Hirschsprung disease,
2. To institute temporary measures until definitive
reconstructive surgery can take place,
3. To manage bowel function after reconstructive
surgery.
Management ofcomplications
Reestablishing normal fluid and electrolyte balance,
Preventing bowel over distension,
Managing sepsis.
Intravenous hydration, nasogastric decompression,
administration of intravenous antibiotics remain the
cornerstones of initial medical management.
Colonic lavage, consisting of mechanicalirrigation with a
large-bore rectal tube and large volumes of irrigant, may be
required.
Balanced salt solutions may help prevent electrolyte
imbalances.
Injecting the non relaxing internal sphincter
mechanism with botulinum toxin has been shown to
induce more normal patterns of bowel movements in
postoperative patients with enterocolitis.
Medications
SNo Drug Dosage
Adult Pediatric
1 Ampicillin 1-2 g IV q6h 25 mg/kg IV q6h
2 Gentamicin 5-7 mg/kg/d IV 2.5 mg/kg IV q8h
3 Metronidazole 500 mg PO/IV q6-8h 7.5 mg/kg IV q6h
Toxins
Induce more normal patterns of bowel
movements Inpostoperative patients with
enterocolitis.
Botulinum toxin type A: - Binds to receptor
sites on motor nerve terminals and inhibits
release of acetylcholine, which in turn inhibits
transmission of impulses in neuromuscular
tissue.
Dose: 0.25-1 U IM; may repeat after 3-
4month
1. Diverting colostomy at the time of diagnosis
2. Once the child grows and weighs more than 10
kg, the definitive repair is performed.
In 1949, Swenson described the first consistent
definitive procedure for Hirschsprung disease,
rectosigmoidectomy with coloanal
anastomosis. Since then, other operations have
been described, including the Duhamel and Soave
techniques.
For neonates who are first treated with adiverting
colostomy, the transition zone is identified and the
colostomy is placed proximal to this area. The
presence of ganglion cells at the colostomy site
confirmed by a frozen-section biopsy.
The 3 most commonly performed definitive repairs
are the Swenson, Duhamel, and Soave procedures
(The Pull-through Procedure).
Swensonprocedure
The Swenson procedure was the original pull -
through procedure used to treat Hirschsprung
disease.
The aganglionic segment is resected down to the
sigmoid colon and the remaining rectum, and an
oblique anastomosis is performed between the
normal colon and the low rectum.
Duhamel procedure
The Duhamel procedure was first described
as a modification to the Swenson procedure.
 Key points are that a retrorectal approach is used and a
significant portion of aganglionic rectum is retained
.
 The aganglionic bowel is resected down to the rectum, and
the rectum is over sewn. The proximal bowel is then brought
through the retrorectal space (between the rectum and
sacrum), and an end-to- side anastomosis is performed on
the remaining rectum.
Soave (endorectal) procedure
The Soave procedure was introduced in the 1960s and
consists of removing the mucosa and submucosa of the
rectum and pulling the ganglionic bowel through the
aganglionic muscular cuff of the rectum.
The original operation did not include a formal anastomosis,
relying on scar tissue formation between the pull-through
segment and the surrounding aganglionic bowel. The
procedure has since been modified by Boley to include a
primary anastomosis at the anus.
Anorectal myomectomy
 For children (and occasionally adults) with ultra
short-segment Hirschsprung disease, removing a
strip of posterior midline rectal wall is an alternative
surgical option.
 The procedure removes a 1-cm wide strip of extra
mucosal rectal wall beginning immediately proximal
to the dentate line and extending to the normal
ganglionic rectum proximally.
 The mucosa and submucosa are preserved
and closed.
Postoperative enterocolitis with the Swenson
procedure,
Constipation following the Duhamel repair,
Diarrhea and incontinence with the Soave pull- through
procedure.
Anastomosis leakage and stricture formation (5- 15%),
Intestinal obstruction(5%),
Pelvic abscess(5%),
Wound infection(10%),
Wound dehiscence and incompleteresection
requiring re-operation (5%).
Stomal complications, such as prolapse or stricture.
Rectovesical fistulas
Mechanical obstruction
Persistent aganglionosis
Motility disorders
 Internal sphincter achalasia may result in persistent
obstruction. This can be treated with internal
sphincterotomy, intrasphincteric botulinum toxin, or
nitroglycerin paste. Most cases will resolve by the age of
5 years.
Functional megacolon may be present due to stool-
holding behavior.
Incontinence may be the result of abnormal sphincter
function, decreased sensation, or overflow
incontinence secondary to constipation.
 As expected, patients with associated
trisomy 21tend to have poorer clinical outcomes.
 In general, more than 90% of patients with
Hirschsprung disease have satisfactory
outcomes
 Although many patients may have
disturbances of bowel function for several years
before developing normal continence.
MANAGEMENT
History
Family history
General
Feeding habits (poor)
Fussiness
Irritability
Distended abdomen
Signs of under nutrition (pallor, muscle weakness, fatigue)
Bowel habits of the neonate or child
Frequency of the bowel movement
Character of stool
Onset of constipation (ribbon like, foul smelly)
Medical history
Clinical presentation
 Fluid electrolyte imbalance
 Altered bowel pattern
 Potential to develop complications related to the
disease condition & surgery
 Altered nutritional pattern
 Altered self body image related to the colostomy.
 Knowledge deficit of the parent related to the
disease condition
 Feeding modifications related to GI surgery
 Abdominal distension related to obstruction
Preoperative preparation
 Informed consent
Consent to be taken from the parents of the infant or
the legal guardians
Nutritional status:-
 A child who is malnourished may not be able
towithstand the surgery until the physical status
improves.
 Low-fiber, high-calories & high protein diet is given
 In severe cases of malnutrition, total parental
nutrition be given
Psychological preparation
Explain the disease properly to the parents
in the working of the bowel
Demonstrate pictorially the surgical procedure
 Older child is to be prepared emotionally for a
colostomy
 Because the colostomy represents a change in the body
function, the nurse should investigate the care giver’s previous
knowledge of the procedure
 Family members may have misconceptions regarding the
colostomy.
 Temporary colostomy also indicates that a future surgery will
be done to close it. So the capabilities of the parent to afford it
at that time need to be seen.
Bowel preparation
 No need to prepare bowel in newborn as the gut
is sterile
 Patient is kept on NG to administration of systemic
antibiotics & rectal irrigation
 The rule of 8-6-4-2 is followed that is nothing solid
8hr before surgery, no formula 6hr before surgery, no
breast feeding 4hr before surgery & nothing at all 2hr
before surgery.
Diet
The patient should have nothing by mouth
before the operation.
Institute tube feeding or formula/breast milk once
bowel function resumes.
High-fiber diets and diets containing fresh fruits
and vegetables may optimize postoperative
bowel function in certain patients.
Ongoing management
Because progressive distension of the
abdomen is a serious sign, abdominal
circumference is measured with a paper tape
measure at the largest diameter, usually at the
level of the umbilicus.
The point of measurement is marked with pen to
ensure its reliability
Postoperative management:-
The infant is given nothing by mouth & have NG tube .
Intake & output, including NG tube lose & stool are
measured.
To prevent contamination of the wound with urine of the baby,
the diaper must be pinned below the dressing or urethral
catheterization can be done.
IV fluids are given to maintain adequate hydration &
electrolyte balance
An abdominal assessment, including monitoring of return of
bowel sounds & passage of stool, will indicate when oral
feeding can be started.
Postoperative management :-
 Regular colostomy care to be done following aseptic guidelines.
 Ongoing education of the older child & care givers regarding
ostomy care begins with preparation for their discharge.
 The nurse should explain the function of each piece of equipment,
stressing features that permit the child to be safely moved & handled,
such as length of tubing, use of arm boards & IV site & tape to secure
NG tube to the nose.
 Parents should be encouraged & assisted in holding &comforting their
child.
 Involve the child & the parents in the dressing change, as this will
increase their confidence & will also help in the acceptance of the
ostomy by the child & parents
Postoperative management:-
Explain them about the procedure of skin care & change
of appliance & the frequency of care, control of order, sign
of complications (ribbon like stool, excessive diarrhea,
bleeding, prolapsed, failure to pass flatus or stool)
If enterostomal therapist is available, then refer the
parents to him for expert assistance in planning home
care
Sometimes families require financial assistance &
additional psychological support. So refer the parents to
social worker
A referral to home health care agency for home nursing
visits can meet the need of additional supervision of care,
reinforcement of child & parent’s education & support.
Activity
Limit physical activity for about 6 weeks to allow the wound to heal
properly (applies more to older children).
Follow-up
Further Inpatient Care
If a diverting colostomy is created in a newborn, he or she must
remain in the hospital until the ostomy is functioning and feeding
goals are obtained. Feedings are usually initiated 24-48 hours after
the creation of the colostomy.
After the definitive pull-through procedure is performed, the patient is
hospitalized until full feedings are possible and evidence of the return
of bowel function is obtained.
Patients are to take nothing by mouth, with intravenous fluid
hydration until they pass flatus or have a bowel movement. Once
this occurs, clear liquids may be started, and the diet may be
advanced until feeding goals are obtained. Intravenous antibiotics
are also continued until evidence of proper bowel function is
observed.
Further Outpatient Care
 After a definitive pull-through procedure is
performed, normal growth and development
should ensue.
 Patients should be monitored for normal bowel habits.
Patients with no other underlying disorders and no
postoperative complications should develop normal
bowel habits. However, such habits may not develop
until the patient is older.
 Nursing Intervention for Outpatient
Rehabilitation in Pediatric Patients with
Hirschsprung Disease after Colectomy
Hui Wang et al. Eur J Pediatr Surg. 2015 Oct.
 Objective: The present study was designed to
evaluate the effectiveness of a nursing intervention
program for outpatient rehabilitation of children with
Hirschsprung-associated enterocolitis (HAEC) after
radical colectomy for Hirschsprung disease (HD).
 Methods: This prospective, randomized control trial
enrolled 85 pediatric patients with HAEC after colectomy.
They were randomly divided into the control and
intervention groups. All the patients were followed up for
6 to 12 months after initial HAEC treatment.
 Results: The intervention group had a lower
enterocolitis recurrence rate than the control group, with
a better ability to control defecation and their parents
had better rehabilitation nursing knowledge (p < 0.05).
The degree of parents' satisfaction regarding the
outcome was significantly better in the intervention
group than that in the control group (p < 0.05)
 Conclusion: A systemic rehabilitation nursing
program could decrease enterocolitis recurrence,
improve the recovery of anorectal functions, and
enhance the quality of life in the pediatric patients
after radical colectomy.
Introduction toHD
Anatomy physiology related to H
D
Incidence
Pathophysiology of HD
Sign & symptoms ofHD
Diagnosis & differential diagnosis
Management
Medical
Surgical
Nursing
Hirschprung's  Disease (HD) Lecture.pptx

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Hirschprung's Disease (HD) Lecture.pptx

  • 2. Hirschsprung disease, congenital aganglionic megacolon is a developmental disorder of the enteric nervous system and is characterized by an absence of ganglion cells in the distal colon because of a disruption of normal neural crest cell migration, result in a functional obstruction.  Enlargement of the colon, caused by bowel obstruction that starts at the anus and progresses upwards.
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  • 4. Ascending colon  On the right side of the abdomen, 25 cm.  Cecum to the hepatic flexure.  Parasympathetic fibers of Vagus nerve (CN X),  Ileocolic artery and right colic artery, both branches of the Superior Mesenteric Artery. Transverse colon  Part of the colon from the hepatic flexure to the splenic flexure.  The proximal two-thirds :-the middle colic artery, branch of superior mesenteric artery,  The latter third:- branches of inferior mesenteric artery.
  • 5. Descending colon  Splenic flexure to the beginning of the sigmoid colon.  Store matter that will be emptied into the rectum.  The left colic artery. Sigmoid colon  After the descending colon and before the rectum.  S-shaped.  Muscular, and contract to increase the pressure inside the colon, causing the stool to move into the rectum.  Sigmoid arteries, a branch of the Inferior Mesenteric Artery.
  • 7. Aganglionic segment of colon becomes nonfunctional. Motor disorder in gut . Absence of the ganglion cells in the myenteric and submucosal plexus. Defect in the migration of the neural crest cell. (precursor of the entric gangolic cells)
  • 8. Defect in the migration of neuroblasts to the distal intestine . Failure of neuroblasts to survive, proliferate, or differentiate. Smooth muscle cells of aganglionic colon are electrically inactive Abnormalities in pacemaker cells connecting enteric nerves & intestinal smooth muscle
  • 9.  Three neuronalplexus  The sub mucosal (ie, Meissner) plexus,  The intermuscular (ie, Auerbach) plexus,  The smaller mucosal plexus. Normal motility is primarily under the control of intrinsic neurons. Control both contraction and relaxation of smooth muscle. Extrinsic control is mainly through the cholinergic and adrenergic fibers. The cholinergic fibers cause contraction, and the adrenergic fibers mainly cause inhibition.
  • 10.  Aganglionosis begins with the anus, which is always involved, and continues proximally for a variable distance. Myenteric (Auerbach) plexus & submucosal (Meissner) plexus are absent, reduced bowel peristalsis & function.
  • 11.
  • 12. Frequency 1 case per 1500 newborns to 1 case per 7000 newborns.
  • 13. Sex  M>F; M:F=4:1.  However, with long-segment disease, the incidence increases in females. Age  Uncommon in premature infants.  Approximately 90% of patients with Hirschsprung disease are diagnosed in the newborn period.
  • 14. Approximately 20% of infants have one or more associated abnormality (neurological, cardiovascular, urological, or gastrointestinal system). Hirschsprung disease has been found to be associated with Down syndrome  Untreated aganglionic megacolon in infancy may result in a mortality rate of as much as 80% In cases of treated Hirschsprung disease, the mortality rate may be as high as 30% as a result of enterocolitis.
  • 15.  Newborn with delayed passage of meconium  Any child with a history of chronic constipation since birth.  Ribbon like stool  Foul smelly stool.  Bowel obstruction with bilious vomiting,  Abdominal distention,  Poor feeding  Failure to thrive.  About 10% diarrhea caused by enterocolitis  Colonic perforation,  Sepsis  vomiting.
  • 16. ◦ Ultra short segment: below recto sigmoid junction ◦ Short segment: up to sigmoid colon ◦ Long segment: up to splenic flexure or beyond ◦ Total segment: affects whole colon.
  • 17. Distendedabdomen Inability to releaseflatus An empty rectum on digital rectal examination Rectalimpaction Rapid expulsion of feces afterrectal examination
  • 18. H/o failure to pass meconium within 24-48 hours after birth. Plain abdominal radiographs may show distended bowel loops with a paucity of air in the rectum.
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  • 21. Anorectal manometry Anorectal manometry is a test that measures how well the rectum and anal sphincter are working. Rectal biopsy The specimen must be obtained at least 1.5 cm above the dentateline because aganglionosis may normally be present below this level.
  • 22. Simple suction rectalbiopsy Rectal mucosa and submucosa are sucked into the suction device, and a self-contained cylindrical knife cuts off the tissue. Can be easily performed at the bedside.
  • 23.  Chemistry panel: dehydration(diarrhea).  CBC count: preoperative hematocrit and platelet count.  Coagulation studies: clotting disorders are corrected before surgery.  Because cardiac malformation (2-5%) and Trisomy 21 (5-15%) are associated with congenital aganglionosis, cardiac evaluation and genetic testing may be warranted.
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  • 25. The general goals of medical care are 3-fold 1. To treat the complications of unrecognized or untreated Hirschsprung disease, 2. To institute temporary measures until definitive reconstructive surgery can take place, 3. To manage bowel function after reconstructive surgery.
  • 26. Management ofcomplications Reestablishing normal fluid and electrolyte balance, Preventing bowel over distension, Managing sepsis. Intravenous hydration, nasogastric decompression, administration of intravenous antibiotics remain the cornerstones of initial medical management. Colonic lavage, consisting of mechanicalirrigation with a large-bore rectal tube and large volumes of irrigant, may be required. Balanced salt solutions may help prevent electrolyte imbalances.
  • 27. Injecting the non relaxing internal sphincter mechanism with botulinum toxin has been shown to induce more normal patterns of bowel movements in postoperative patients with enterocolitis.
  • 28. Medications SNo Drug Dosage Adult Pediatric 1 Ampicillin 1-2 g IV q6h 25 mg/kg IV q6h 2 Gentamicin 5-7 mg/kg/d IV 2.5 mg/kg IV q8h 3 Metronidazole 500 mg PO/IV q6-8h 7.5 mg/kg IV q6h
  • 29. Toxins Induce more normal patterns of bowel movements Inpostoperative patients with enterocolitis. Botulinum toxin type A: - Binds to receptor sites on motor nerve terminals and inhibits release of acetylcholine, which in turn inhibits transmission of impulses in neuromuscular tissue. Dose: 0.25-1 U IM; may repeat after 3- 4month
  • 30. 1. Diverting colostomy at the time of diagnosis 2. Once the child grows and weighs more than 10 kg, the definitive repair is performed. In 1949, Swenson described the first consistent definitive procedure for Hirschsprung disease, rectosigmoidectomy with coloanal anastomosis. Since then, other operations have been described, including the Duhamel and Soave techniques.
  • 31. For neonates who are first treated with adiverting colostomy, the transition zone is identified and the colostomy is placed proximal to this area. The presence of ganglion cells at the colostomy site confirmed by a frozen-section biopsy. The 3 most commonly performed definitive repairs are the Swenson, Duhamel, and Soave procedures (The Pull-through Procedure).
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  • 37. Swensonprocedure The Swenson procedure was the original pull - through procedure used to treat Hirschsprung disease. The aganglionic segment is resected down to the sigmoid colon and the remaining rectum, and an oblique anastomosis is performed between the normal colon and the low rectum.
  • 38.
  • 39. Duhamel procedure The Duhamel procedure was first described as a modification to the Swenson procedure.  Key points are that a retrorectal approach is used and a significant portion of aganglionic rectum is retained .  The aganglionic bowel is resected down to the rectum, and the rectum is over sewn. The proximal bowel is then brought through the retrorectal space (between the rectum and sacrum), and an end-to- side anastomosis is performed on the remaining rectum.
  • 40. Soave (endorectal) procedure The Soave procedure was introduced in the 1960s and consists of removing the mucosa and submucosa of the rectum and pulling the ganglionic bowel through the aganglionic muscular cuff of the rectum. The original operation did not include a formal anastomosis, relying on scar tissue formation between the pull-through segment and the surrounding aganglionic bowel. The procedure has since been modified by Boley to include a primary anastomosis at the anus.
  • 41. Anorectal myomectomy  For children (and occasionally adults) with ultra short-segment Hirschsprung disease, removing a strip of posterior midline rectal wall is an alternative surgical option.  The procedure removes a 1-cm wide strip of extra mucosal rectal wall beginning immediately proximal to the dentate line and extending to the normal ganglionic rectum proximally.  The mucosa and submucosa are preserved and closed.
  • 42. Postoperative enterocolitis with the Swenson procedure, Constipation following the Duhamel repair, Diarrhea and incontinence with the Soave pull- through procedure. Anastomosis leakage and stricture formation (5- 15%), Intestinal obstruction(5%), Pelvic abscess(5%), Wound infection(10%), Wound dehiscence and incompleteresection requiring re-operation (5%).
  • 43. Stomal complications, such as prolapse or stricture. Rectovesical fistulas Mechanical obstruction Persistent aganglionosis Motility disorders  Internal sphincter achalasia may result in persistent obstruction. This can be treated with internal sphincterotomy, intrasphincteric botulinum toxin, or nitroglycerin paste. Most cases will resolve by the age of 5 years.
  • 44. Functional megacolon may be present due to stool- holding behavior. Incontinence may be the result of abnormal sphincter function, decreased sensation, or overflow incontinence secondary to constipation.
  • 45.  As expected, patients with associated trisomy 21tend to have poorer clinical outcomes.  In general, more than 90% of patients with Hirschsprung disease have satisfactory outcomes  Although many patients may have disturbances of bowel function for several years before developing normal continence.
  • 47. History Family history General Feeding habits (poor) Fussiness Irritability Distended abdomen Signs of under nutrition (pallor, muscle weakness, fatigue) Bowel habits of the neonate or child Frequency of the bowel movement Character of stool Onset of constipation (ribbon like, foul smelly) Medical history Clinical presentation
  • 48.  Fluid electrolyte imbalance  Altered bowel pattern  Potential to develop complications related to the disease condition & surgery  Altered nutritional pattern  Altered self body image related to the colostomy.  Knowledge deficit of the parent related to the disease condition  Feeding modifications related to GI surgery  Abdominal distension related to obstruction
  • 49. Preoperative preparation  Informed consent Consent to be taken from the parents of the infant or the legal guardians Nutritional status:-  A child who is malnourished may not be able towithstand the surgery until the physical status improves.  Low-fiber, high-calories & high protein diet is given  In severe cases of malnutrition, total parental nutrition be given
  • 50. Psychological preparation Explain the disease properly to the parents in the working of the bowel Demonstrate pictorially the surgical procedure  Older child is to be prepared emotionally for a colostomy  Because the colostomy represents a change in the body function, the nurse should investigate the care giver’s previous knowledge of the procedure  Family members may have misconceptions regarding the colostomy.  Temporary colostomy also indicates that a future surgery will be done to close it. So the capabilities of the parent to afford it at that time need to be seen.
  • 51. Bowel preparation  No need to prepare bowel in newborn as the gut is sterile  Patient is kept on NG to administration of systemic antibiotics & rectal irrigation  The rule of 8-6-4-2 is followed that is nothing solid 8hr before surgery, no formula 6hr before surgery, no breast feeding 4hr before surgery & nothing at all 2hr before surgery.
  • 52. Diet The patient should have nothing by mouth before the operation. Institute tube feeding or formula/breast milk once bowel function resumes. High-fiber diets and diets containing fresh fruits and vegetables may optimize postoperative bowel function in certain patients.
  • 53. Ongoing management Because progressive distension of the abdomen is a serious sign, abdominal circumference is measured with a paper tape measure at the largest diameter, usually at the level of the umbilicus. The point of measurement is marked with pen to ensure its reliability
  • 54. Postoperative management:- The infant is given nothing by mouth & have NG tube . Intake & output, including NG tube lose & stool are measured. To prevent contamination of the wound with urine of the baby, the diaper must be pinned below the dressing or urethral catheterization can be done. IV fluids are given to maintain adequate hydration & electrolyte balance An abdominal assessment, including monitoring of return of bowel sounds & passage of stool, will indicate when oral feeding can be started.
  • 55. Postoperative management :-  Regular colostomy care to be done following aseptic guidelines.  Ongoing education of the older child & care givers regarding ostomy care begins with preparation for their discharge.  The nurse should explain the function of each piece of equipment, stressing features that permit the child to be safely moved & handled, such as length of tubing, use of arm boards & IV site & tape to secure NG tube to the nose.  Parents should be encouraged & assisted in holding &comforting their child.  Involve the child & the parents in the dressing change, as this will increase their confidence & will also help in the acceptance of the ostomy by the child & parents
  • 56. Postoperative management:- Explain them about the procedure of skin care & change of appliance & the frequency of care, control of order, sign of complications (ribbon like stool, excessive diarrhea, bleeding, prolapsed, failure to pass flatus or stool) If enterostomal therapist is available, then refer the parents to him for expert assistance in planning home care Sometimes families require financial assistance & additional psychological support. So refer the parents to social worker A referral to home health care agency for home nursing visits can meet the need of additional supervision of care, reinforcement of child & parent’s education & support.
  • 57. Activity Limit physical activity for about 6 weeks to allow the wound to heal properly (applies more to older children).
  • 58. Follow-up Further Inpatient Care If a diverting colostomy is created in a newborn, he or she must remain in the hospital until the ostomy is functioning and feeding goals are obtained. Feedings are usually initiated 24-48 hours after the creation of the colostomy. After the definitive pull-through procedure is performed, the patient is hospitalized until full feedings are possible and evidence of the return of bowel function is obtained. Patients are to take nothing by mouth, with intravenous fluid hydration until they pass flatus or have a bowel movement. Once this occurs, clear liquids may be started, and the diet may be advanced until feeding goals are obtained. Intravenous antibiotics are also continued until evidence of proper bowel function is observed.
  • 59. Further Outpatient Care  After a definitive pull-through procedure is performed, normal growth and development should ensue.  Patients should be monitored for normal bowel habits. Patients with no other underlying disorders and no postoperative complications should develop normal bowel habits. However, such habits may not develop until the patient is older.
  • 60.  Nursing Intervention for Outpatient Rehabilitation in Pediatric Patients with Hirschsprung Disease after Colectomy Hui Wang et al. Eur J Pediatr Surg. 2015 Oct.  Objective: The present study was designed to evaluate the effectiveness of a nursing intervention program for outpatient rehabilitation of children with Hirschsprung-associated enterocolitis (HAEC) after radical colectomy for Hirschsprung disease (HD).
  • 61.  Methods: This prospective, randomized control trial enrolled 85 pediatric patients with HAEC after colectomy. They were randomly divided into the control and intervention groups. All the patients were followed up for 6 to 12 months after initial HAEC treatment.  Results: The intervention group had a lower enterocolitis recurrence rate than the control group, with a better ability to control defecation and their parents had better rehabilitation nursing knowledge (p < 0.05). The degree of parents' satisfaction regarding the outcome was significantly better in the intervention group than that in the control group (p < 0.05)
  • 62.  Conclusion: A systemic rehabilitation nursing program could decrease enterocolitis recurrence, improve the recovery of anorectal functions, and enhance the quality of life in the pediatric patients after radical colectomy.
  • 63. Introduction toHD Anatomy physiology related to H D Incidence Pathophysiology of HD Sign & symptoms ofHD Diagnosis & differential diagnosis Management Medical Surgical Nursing

Editor's Notes

  1. Interstitial cells of cajal are sp pacemaker cells in stomach, small n large intestine
  2. Shows dilated loops of bowel with air-fluid levels.
  3. Dentate line: which divided upper 2 third and lower third of the anal canal.
  4. Mast cell stabilizing agent
  5. 1956