Consist of pathophysiology, clinical and physical presentation, management and nursing management.

1. HIRSCHSPRUNG’S DISEASE

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.

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.

6.  https://youtu.be/1pylBKNMcnw
 https://youtu.be/9QjZe6zZpRA

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.

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.

19. Barium enema

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.

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).

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.

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.

46. MANAGEMENT

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