Hirschsprung disease ; avoiding complications

1. Hirschsprung’s disease; Avoiding
complications
Sameh Shehata

2. Topics to be covered
constipation
Biopsy
Technical details.
EC
Minimum
age?
incontinence
Imaging.

3. Sources
Personal
experience
25 Yrs.
National
ExperiencesInternational
literature

4. Journal of Pediatric Surgery, Vol 39, No
3 (March), 2004: pp 345-351

5. HOW TO AVOID IMAGING
PITFALLS?

6. Rectal Balloon!

7. The value of lateral view

8. Too crowded!
Immediate 24 hr delayed

9. Proper technique CE
• Stop bowel prep. 12 hours.
• Avoid inflating balloon.
• AP and Lateral .
• 24 hours delayed film.

10. What we ae looking for ?
N
D
Nm

11. HOW TO AVOID PITFALLS
DURING BIOPSY?

12. Error traps and culture of safety in
Hirschsprung disease
L. De La Torre a , ∗, L.A. Wehrli b

13. Error in mucosal biopsy

14. Error in seromuscular biopsy

15. Intraoperative FT

16. MINIMUM AGE FOR ONE
STAGE?
1

17. Performing single-stage transanal endorectal pull-
through in the nonneonatal period may be more
appropriate than the neonatal period.

18. Operating in neonates
higher rates of:
• perianal excoriation,
• anastomotic strictures
• leakage,
• postoperative enterocolitis,
• Incontinence.

19. Preoperative anal dil.

20. Conclusion; anal dilatation
• NO reduced HAEC or postoperative
obstructive symptoms
• it may decrease the difficulties of
surgeries, so the operative time is shorten.

21. HOW MUCH ANAL STRETCH ?

22. Excessive traction Minimal Traction

23. No Retractor – No Stitches

24. HOW MUCH ANAL MUCOSA
WE KEEP?

25. APSA HD interest group 2019
T, L ,L

26. L. Error traps and culture of safety in Hirschsprung
disease De La Torre a , ∗, L.A. Wehrli b

27. JPS 2017

28. Poor outcome!

29. Proper technique

30. • What happens in the first 10
minutes of the operation
can affect the whole life of
the patient !
10 minutes

31. HOW TO AVOID PROBLEMS
WITH SOAVE CUFF?

33. Soave Cuff

34. IN SOAVE PT , WHAT IS THE
LENGTH OF SEROMUSCULAR
CUFF?

35. Soave cuff
• Short cuff.
• How short is short?
• Don’t incise.
• Avoid inverted cuff.
6 cm
4 cm
2 cm

36. WHAT IS YOUR PREFERRED
TECHNIQUE?

37. Transanal Swenson Or Soave ?

38. Transanal Swenson Or Soave ?
Similar
• overall hospital stay
• bowel function
Swenson
• less blood loss,
• shorter operation
time,

39. HOW TO AVOID PROBLEMS
WITH DUHAMEL SPUR ?

40. Spur after Duhamel

43. Avoid persistent spur
Double firing from above and below

44. HOW TO AVOID TWIST OF THE
PULLED COLON?

45. Keep a clamp on the bowel
To avoid twist

46. WHAT TO TO DO WITH DILATED
SEGMENT AND SIGMOID LOOP?

47. • Stretch sphincter .
• Ectatic colon .
• Sensitive anal
mucosa.

49. Barium Enema
PreOP PostOP

50. WHY REMOVE dilated
COLON?
• Ectatic .
• Hypomotility.
• Constipation .22-40% PO
• Pseudo-incontinence.

51. HOW TO AVOID ISCHEMIA OF
PULLED COLON?

52. Part to be resected
dissection on the wall

53. Part to be mobilized
keep marginal arcade

54. Discrepancy during
anastomosis

55. Discrepancy !
ColonAnus

56. Discrepancy !

57. Discrepancy !

59. Rectal tube PO?

60. DO YOU USE RECTAL TUBE
PO?

61. JPS 2020
Rectal tube can reduce
• abdominal distension
• HAEC
• HAEC recurrence

62. 25 %
Anastomotic Stricture.
Duhamel spur
Long Soave cuff
Twisted colon
PO enterocolitis

63. 25 %
Fever
Abdominal distension
Diarrhea
Toxic
Shock
PO enterocolitis

64. post operative
• Enterocolitis. 25% - 37%
• Obstructive symptoms 8%–30%

65. Causes PO enterocolitis
Avoidable
Anatomical
• Anastomotic Stricture.
• Duhamel spur
• Long Soave cuff
• Twisted colon
Functional
• Missed segment.
• Dilated atonic segment
Unavoidable !

66. HOW TO MINIMIZE POST OP
EC?

67. Meta analysis JPS 2018

68. Effect on EC
• Routine postoperative dilatations no effect
on stricture incidence
• Routine postoperative rectal irrigations possible
reduced HAEC incidence).
• Posterior myotomy/myectomy : Not
recommended 25% incontinence!

69. • Botulinum toxin injection : short-term
response in 77.3% long-term
response in 43.0%
• Probiotic prophylaxis no reduction in
HAEC

70. CONCLUSION AND SUMMARY

71. Avoid too
much
stretch.
Avoid direct
sphincter
injury.
Keep anal
mucosa.
Avoid soiling/Incontinence
Motor Sensory

72. Minimize postoperative
constipation
• Remove dilated ganglionic segment.
• Remove sigmoid extraloop.

73. Technical details
Not before
one month.
Preop
dilatation.
Short cuff
(Soave)
Divide the Spur
(Duhamel)
Stick to the wall
(Swenson)
Vascular colon
(Arcade)
Post OP
Rectal tube.

75. Swenson : stick to the wall!

76. Soave : Inside the wall!

77. Duhamel: Behind the wall!

78. Hirschsprung’s disease
Normal sphincter
Normal sensory
Training.
Normal
Continence

80. Thank You
CONTACT
• Sameh Shehata
• +201001449045
• drsamehs@yahoo.com