This product discuss Hirschsprung’s disease in pediatrics included [pathology, presentation, diagnostic tools, surgical procedures complications and management. Also discuss the recent concepts of management of post operative complications.

1. Post Hirschsprung’s surgery complications
Diagnostic approach and treatment strategies
Dr. Mohammad Daboos
Lecturer of pediatric surgery
Al-Azhar University

2. Introduction
The pathophysiology of HSD is poorly understood. It has been long recognized
that the obstructive symptoms in HSD are secondary to the abnormal motility of
the narrow distal segment
There is still no clear explanation for the cause of this spastic or tonically
contracted segment of bowel.
Normal intestinal motility requires coordinated interaction of the enteric
nervous system (ENS), smooth muscle cells (SMCs), ICCs and platelet-derived
growth factor receptor α+-cells(PDGFRα+),NO, ……
So, several abnormalities have been described to explain the basis for motility
dysfunction in the contracted bowel in HSD (Puri 2009, 2011).

3. Introduction
-The diagnosis of HSD is usually based on clinical history,
imaging, and confirmed by histological examination of
rectal biopsy (Jakobson-Setton et al. 2015).
-Over 90% of affected patients fail to pass meconium in
the first 24 h of life.
-Some patients present later in childhood, or even during
adulthood, with chronic constipation, failure to thrive,
and abdominal distension

4. Barium Enema
typical case of HSD demonstrate
flow of barium from the un-dilated
rectum through a cone shaped
transitional zone into dilated colon.
But, In total colonic aganglionosis
(TCA), the contrast enema is not
pathognomonic and may not
provide a definitive diagnosis. (Puri
2009, 2011).

5. Anorectal Manometry
Revealed that on rectal distension, with an
increment of air, there is complete absence of
internal sphincter relaxation. ( absence Ano
rectal Reflex).

6. Rectal biopsy
A suction rectal biopsy
should involve sampling a
segment of rectal wall 2 cm
proximal to the dentate
line along the posterior
wall of the rectum,
classical finding of absence
of ganglion with presence
of hypertrophied nerve
plexus are diagnostic.
The gold standard in diagnosis of HSD
is the examination of rectal biopsy
specimens.

7. New tools
immunohistochemical staining (IHC) techniques for the detection
of acetylcholinesterase (AChE) activity in the rectal suction biopsy
(SRB) has resulted in a reliable and simple method for the
diagnosis of HSD (de Arruda Lourencao et al. 2013)
Calretinin , a calcium-binding protein that functions as a calcium
sensor/modulator, expressed in submucosal and myenteric
ganglion cells and mucosal nerve fibers has been described as an
adjunctive or primary diagnostic test in HSD.

8. Historical review of surgical treatment
Since the clinical presentations by Harald Hirschsprung in Berlin in 1886, the condition
that bears his name had a rich history.
In 1948, Drs. Swenson, Neuhauser (a radiologist) and Pickett in Boston using a barium
enema and fluoroscopy, recognized that an area of spasm in the rectum or recto-
sigmoid that defined the site of obstruction
on the barium studies and the narrow distal rectum Swenson perform a colo-anal
anastomosis above the dentate line to preserve continence. This was a historic
landmark event, the first successful operative procedure for HSD.
Recognizing that the barium enema was not always diagnostic, particularly in the
neonate, in 1959, Swenson et al. described the full-thickness rectal biopsy to obtain
material for a tissue diagnosis.

9. Historical review of surgical treatment
In 1956, Bernard Duhamel
described the retrorectal
transanal pull-though
procedure for the treatment
of HSD. This concept was
developed to preserve the
pelvic nerves and left the a
ganglionic rectum in place.
A variety of clamps and
subsequently stapling devices
were employed to divide the
colorectal spur comprising the
posterior wall of the a
ganglionic rectal stump and the
anterior wall of the normally
innervated pull-through
segment (Martin, Ikeda, Soper,
Miller and Steichen et al.)
In 1963, Soave of Genoa,
Italy, described the
endorectal pull-through
procedure bringing the
innervated bowel down
to the perineum through
a muscular sleeve of the
aganglionic rectum.
In 1981, So and
colleagues were the
first to report a one-
stage pull-through
procedure in neonates
with HSD without a
preliminary colostomy.

10. Historical review of surgical treatment
Georgeson et al. described a laparoscopically assisted Soave endorectal pull-through procedure
avoiding an open laparotomy . He adapted this to a primary procedure in 1999.
In 1998, de la Torre and Ortega-Salgado of Mexico were the first to perform a one-stage totally trans
anal pull-through procedure.
Results with the transanal endorectal pull-through were favorable when compared to the open
procedure.
Since then, the transanal operation has been used extensively in the neonatal period by three
multicenter studies in Europe, North America and Egypt
References:-
M. E. Höllwarth and J. L. Grosfeld. Hirschsprung’s Disease: A Historical Perspective – 1691–2018 , Hirschsprung’s
Disease and Allied Disorders Fourth Edition. Prem Puri,etal Springer Nature 2019 ch (1) p 8-10

11. There are remains much to be learned.
Why some
patients with
HSD do poorly
following
operation.?
the proper
management of
many patients
with variants of
HSD needs to be
more clearly
elucidated.!!!!
Continuing studies
of the ENS and the
molecular genetics
of these conditions
may shed further
light on these
issues and provide
a better
understanding of
the choice of
management in the
future.
Recent studies
have
transplanted
human enteric
neuralprogenitor
s into the mouse
colon and shown
engraftment.
Currently,
researchers are
working to develop
novel stem cell
therapies, whereby
stem cells could be
transplanted into
the aganglionic
segment of bowel
to replace the
missing ENS
References:-
Stamp LA, Young HM. Recent advances in regenerative medicine to treat enteric neuropathies: use of human cells.Neurogastroenterol Motil. 2017;29
https://doi.org/10.1111/nmo.12993

13. Bowel preparation
what are recommendations????

14. Bowel preparation
Preoperative
decompression is
usually performed by
bowel irrigation with
warm saline and anal
dilatation for 6–7 days
before surgery.
For severe and
persistent
constipation or
distension, the bowel
preparation may be
longer.
Oral intake is limited
to clear liquids within
14–24 hours before
surgery. A nasogastric
tube is used for
decompression and
better exposure just
at the beginning of
the operation(1-2)
References:-
1-Oancea M, Vatra L, Kadar A, Copaescu C. Laparoscopic approach for rectosigmoidian resection in children. Chirurgia (Bucur). 2014;109(1):117–
22.
2-Liem NT, Hau BD. Primary laparoscopic endorectal colon pull-through for Hirschsprung’s disease: early results of 61 cases. Asian J Surg.
2006;29(3):173–5.

15. Patient Selection Criteria ????
Staged procedure (colostomy then definitive
surgery)
One stage open procedures
Laparoscopic Assisted procedures
Trans-anal pull through

16. Patient selection
-Hirschsprung’s disease-
associated enterocolitis
(HAEC), malnutrition, huge
fecalith, or severe dilation of
the proximal bowel, surgery
should be staged and a
stoma, and an intraoperative
leveling biopsies should be
performed at first.
-Most patients are eligible to
undergo laparoscopic pull-
through, such as patients
with long-segment HSD, total
colonic aganglionosis (TCA),
reoperation, stoma, or allied
HSD disorders.
-The totally transanal
endorectal pull-through
procedure is more popular in
short-segment HSD. (jie-
xiong etal, 2019)
Jie-xiong Feng, Ting Li, and Ning Li. Laparoscopically Assisted Pull-Through Operation for Hirschsprung’s Disease. Hirschsprung’s Disease and Allied
Disorders ,Fourth Edition. Prem Puri,etal Springer Nature 2019 ch (24) p 358-59

18. Principles of surgery
The basic principle in all procedures:-
-Excision of Aganglionic segment.
-Bring the ganglionic bowel down to the anal canal.

19. How do you
determine the level
of ganglionosis ?

20. Principles of surgery

21. Total colonic Aganglionosis how to
be managed?

22. Post HSD operations complications

23. The advantages with this system are that all possible adverse events are included

24. Early complications
-Wound Infection
-Anastomosis Leakage (minor, major leakage)
-Anastomosis twisting
-Cuff Abscess
-Pelvic Abscess
-Dehiscence/Retraction of the Pull Through
-Fistula (enterocutaneous, rectourethral, or
rectovaginal)
-Soiling and perineal excoriation

25. What are the risk factors for early complications?

26. Management of early complication
Cuff Abscess
Pelvic Abscess  Drainage
Anastomosis Leakage
Anastomosis twisting Diversion and redo
Dehiscence/Retraction of the Pull Through
Fistula
r

27. Late complication post HSD surgery

28. constipation/obstructive symptoms
Causes
Its incidence varies widely and may change with time, but rates of approximately 8–30%are
reported, generally unrelated to the type of operation

30. constipation/obstructive symptoms
Work up

31. constipation/obstructive symptoms
Meticulous
Clinical
Examination
Contrast
Enema
Rectal serial
Biopsies.
Anorectal
Manometry
Colonic
Transit Time
studies

33. constipation/obstructive symptoms
Management Strategies???

36. Redo- pullthrough
Timing??
Indications??

37. Redo- pullthrough
Preferred
technique??

38. Mohamed I. El-sawaf, MD, Arnold G. Coran, MD, and Daniel H. Teitelbaum, MD. Reoperative Surgery for Hirschsprung
Disease,. Reoperative Pediatric Surgery, Editor(Steven Teich, md and Donna A. Caniano, md, 2008Ed(1) ch (17), p 300-
301

39. Redo- pullthrough

40. Redo- pullthrough
Outcomes??
Recurrence
after redo??

41. Myetomy / Myectomy
Indications? Technique? Results?

45. Soiling/Incontinence
True incontinence
psaudoincontience
Reported rates of incontinence range widely in various series, with median rates of
20–30% The incidence of soiling is higher for children with total colonic disease or
extensive aganglionosis and in Down syndrome.

46. How to evaluate incontinence??

48. Soiling/Incontinence
Causes of true incontinence?
How do you avoid it?
Causes of psaudoincontience?
How do you avoid it?

49. Investigation for fecal incontinence

50. Neuropathic

52. References:-
1-Rebecca M. Rentea and Charles L. Snyder Early and Late Complications Following Pull-Through Operation for Hirschsprung’s Disease. Hirschsprung’s Disease and Allied Disorders
,Fourth Edition. Prem Puri,etal Springer Nature 2019 ch (26) p 395-
2-Krois.W., ReckC.A., A. Darbari et al., A technique to reconstruct the anal sphincters following iatrogenic stretching related to a pull-through for Hirschsprung disease, Journal of
Pediatric Surgery, https://doi.org/10.1016/j.jpedsurg.2020.12.007

55. Hypomotility
Dilated colon??
Hypermotility
undilated colon??

59. Hirschsprung-Associated Enterocolitis (HAEC)
Causes????
Enterocolitis has a widely variable incidence of between 15% - 40%.
Postoperative enterocolitis may affect one-third of patients, with a
mortality rate varying from 0–40%

60. -Interferes with the normal passage of stool (retained aganglionosis, stricture, twist in the pull-
through, internal anal sphincter achalasia, or tight muscular cuff following the Soave
procedure) can result in stasis and predispose the patient to enterocolitis[1]
-Abnormalities in the intestinal microbiome (dominance of fungi and bacteria predisposing
patients to development of HAEC)
impaired intestinal mucosal barrier function (abnormal goblet cells and mucus) [2]
-Altered systemic immune system [3]
1-Rebecca M. Rentea and Charles L. Snyder Early and Late Complications Following Pull-Through Operation for Hirschsprung’s Disease. Hirschsprung’s Disease and Allied Disorders ,Fourth
Edition. Prem Puri,etal Springer Nature 2019 ch (26) p 397
2-Thiagarajah JR, Yildiz H, Carlson T, Thomas AR, Steiger C, Pieretti A, et al. Altered goblet cell differentiation and surface mucus properties in Hirschsprung disease. PLoS ONE [Internet].
2014 [cited 2018 Jul6];9(6).
3-Gosain A, Barlow-Anacker AJ, Erickson CS, Pierre JF, Heneghan AF, Epstein ML, et al. Impaired cellular immunity in the murine neural crest conditional deletion of endothelin receptor-B
model of Hirschsprung’s disease. PLoS One [Internet]. 2015 [cited 2018 Jul 6];10(6).

61. Hirschsprung-Associated Enterocolitis (HAEC)
Presentation
criteria????

64. Regular
dilations
Rectal
irrigations
Probiotics
Antibiotic
prophylaxis

65. Rectal Wash &
Irrigation
diversion and/or
colectomy
N.P.O
I.V Fluids
Antibiotics
(mpicillin/gentamycin)
&metronidazol

66. -Up to 45% of children with Hirschsprung disease have
unstable detrusor contractions that often resolve after the
abnormal bowel is resected (Boemers et al., 2001).
-Transient urinary retention was noted in up to 7% of children
undergoing surgery for Hirschsprung disease (Ateş et al.,
2007).
-From to 0% to 6% of children had urinary incontinence
postoperatively with long-term followup (Holschneider et al.,
1982; Boemers et al., 2001).
Voiding and Sexual dysfunction

67. Voiding and Sexual dysfunction
Causes????

68. Voiding and Sexual dysfunction