This document provides an overview of Hirschsprung disease, including its incidence, etiology, pathophysiology, clinical features, diagnosis, management, nursing management, and complications. It describes Hirschsprung disease as a congenital disorder characterized by the absence of ganglion cells in parts of the colon, most commonly in the rectum and sigmoid colon. The document discusses the various levels of Hirschsprung disease, clinical presentation in newborns and older children, diagnostic tests, surgical treatments including colostomy and pull-through procedures, nursing care both pre-and post-operatively, and potential complications.

3. Content
Hirschsprung disease
a. Introduction , Incidence
b. Etiology , Pathophysiology
c. Clinical features, Diagnosis
d. Management, Nursing management
e. Complication

4. Normal colon Hirschsprung disease

5. Hirschsprung disease

6. Introduction
Hirschsprung disease is a congenital disorder which is
characterized by absence of the parasympathetic
ganglionic nerve cells within the muscle wall of the
intestinal tract, usually at the distal ends of the colon,
resulting in functional obstruction with dilation and
hypertrophy of proximal colon.

7. Introduction
The level of hirschsprung's disease include:-
 Short-segment: Missing ganglion cells in the rectum
and sigmoid colon (the last segment of the colon).
 Long-segment: It occurs when nerve cells are missing
from most of the large intestine and is the most severe
type.

8. Incidence
 Kanti children's hospital has served a total of 103
patients in the Surgical ICU of Kanti Children’s
Hospital, from October to December 2018 from which
hirschsprung's disease patients was 2 (October), 4
(November ), 6 (December ).
Source:- https://realmedicinefoundation.org/wp-
content/uploads/2019/09/RMF_Nepal___Kanti_Childr
en_s_Hospital___Q4_2018.pdf

10. Cause contd..
 Congenital defects
 Common among child with down syndrome,
congenital heart disease.

11. Pathophysiology
Absence of parasympathetic ganglion cells in
segment of colon
Lacks peristalsis movement in the affected part and
becomes contracted and narrowed
No passage of faecal matter from narrow part and
accumulation above affected portion

12. Pathophysiology
That lead to
Dilation of the proximal portion just above the
narrowed part and filled up with faecal material and
gases. Severity of Hirschsprung disease is depends on
length of the large intestine affected.

13. Clinical features
Newborn Infants Older children
I. Failure or
delayed
passage of
meconium
II. Reluctance to
breastfeed
III. Bile stained
vomiting
IV. Abdominal
distension
I. Failure to thrive
II. Chronic
constipation
III. Abdominal
distension
IV. Vomiting
I. Anorexia
II. Chronic
constipation
III. Foul smell,
ribbon like stool
IV. Vomiting
V. Abdominal
distension

14. Clinical features
 Abdominal distension

16. X-ray

17. Diagnosis
 Anorectal Manometry: A small balloon is inflated
inside the rectum. Normally, the rectal muscles should
relax. In HD, it reveals absence of relaxation reflex of
internal sphincter. It is useful only for older children. It
records the reflex response of sphincter.

18. Management
Symptomatic management of older children with mild
and chronic disease until the physical status of
children is improved. That includes
 Isotonic enema
 Stool softeners
 High calorie and high protein diet
 Intravenous fluid in severe condition

19. Management
 Neonate with uncomplicated condition: After
confirmation of diagnosis, transverse colostomy is
done in those cases whose aganglionosis extends up to
recto- sigmoid junction.

20. Management
Baby with complicated condition:
 Emergency laparotomy or colostomy will be needed.
 In case of enterocolitis; rapid resuscitation,
intravenous fluids therapy, blood or fresh frozen
plasma transfusion, antibiotics and colostomy are
come under an emergency procedure.

21. Definitive treatment
The objective of definitive treatment is to removal
of the aganglionic or non functioning dilated
segment of the bowel, followed by anastomosis
and improved functioning of internal rectal
sphincter.

22. Definitive surgery
 Swenson's operation = It is the type of surgery in which
Aganglionic segment is resected down to the sigmoid
colon and rectum and an anastomosis is performed
between the normal colon and the low rectum.

24. Surgery
 Soave's operation= The operation based on removing
the mucosa and sub mucosa of the rectum and pulling
ganglionic intestine through the Aganglionic muscular
cuff. By remaining within the muscular cuff of the
Aganglionic segment, important sensory fibers' and
the integrity of the internal sphincter are preserved.

25. Nursing Management
Assessment
 Ask about the onset of constipation with caregiver
 Assess the character and odour of stools
 Assess the frequency of bowel movements
 Assess the presence of poor feeding habits, anorexia,
and irritability.

26. Nursing Diagnosis
 Constipation related to decreased bowel motility.
 Fear (in the older child) related to impending surgery.
 Risk for impaired skin integrity related to irritation
from the colostomy.
 Acute pain related to the surgical procedure.
 Deficient knowledge of caregivers related to
understanding of postoperative care of the colostomy.

27. Nursing Intervention
 When performing routine colostomy care, give careful
attention to the area around the colostomy;
 Record and report redness, irritation, and rashy
appearances of the skin around the stoma;
 The caregivers should demonstrate their
understanding by caring for the colostomy under the
supervision of nursing personnel several days before
discharge.

28. Preoperative management
 Assessment of child's health condition.
 Written consent from parents.
 Physical preparations: Emptying the with repeated
saline enemas and use systemic antibiotic and colon
irrigations.
 Monitoring vital sign and intake/output record.
 Regular measurement of abdominal girth.
 Provide preoperative medications.

29. Post operative management
 Maintain airway patency, prevent from aspiration,
measure abdominal girth
 Assess wound site and drainage tube if present for
bleeding, leakage
 Prevent contamination from diaper; change diaper
below dressing frequently
 Encourage frequent coughing and deep breathing
exercise.

30. Complication
 Enterocolitis (main cause of death),
 Hydroureter or Hydronephrosis,
 Leaking of anastomosis and pelvic abscess,
 Intussusceptions,
 Abdominal distension,
 Bleeding, Shock.

31. Intussusception
It is a form of bowel obstruction in which one
segment of intestine telescopes inside of another.

33. Reference of Hirschsprung disease
 Uprety K, Child Health Nursing, fourth Edition (2071 Bhadra),
Tara Books and Stationery, Chhetrapati, Kathmandu, pg 163- 166
 Shrestha T. Essential Child Health Nursing. first Edition
2015,August. Medhavi Publication; Jamal, Kathmandu Page
no.281-286
 WilsonD, Rodgers CC, Hockenberry M, Wongs Essential of
pediatric Nursing 10 th edition, ELSEVIER, page no 1371-1373
 Adhikari T, Essential of Pediatric Nursing, first 2014 edition,
vidyarthi pustak bhadar, bhotahity, Kathmandu, page no163-166
 https://www.cincinnatichildrens.org/health/h/hirschsprung