Hirschsprung disease

1. Management and Surgical
procedures of HD
Dr Sasidhar
Moderator: Dr Babu Rao

2. Anatomy of anal canal

4. The three loops of the external anal sphincter. Continence
depends on the preservation of at least one of the three. Some
subcutaneous muscle fibers encircle the anus; some attach to the
perianal skin anteriorly at C.

5. Diagram of the extrinsic muscles of the surgical anal canal. (1) Coccyx; (2) pubis; (3)
levator ani muscle; (4) puborectalis muscle; (5) deep external sphincter; (6) superfi
cial external sphincter; (7) subcutaneous external sphincter; (8) anococcygeal
ligament; (9) anal verge; (10) rectum.

6. The interior of the anal canal showing the rectal columns, anal valves, and anal
sinuses (crypts). They form the pectinate line
The pectinate line is the most important landmark in the anal canal. It marks the transition
between the visceral area above and the somatic area below. The arterial supply, the
venous and lymphatic drainage, the nerve supply, and the character of the lining all change
at or very near the pectinate line

8. Management and surgical procedures
of HD

9. Surgical interventions – Historical Perspective
Drs. Swenson,
Neuhauser
(a radiologist)
and Pickett Boston
1948 Recognized an area of spasm in the rectum or
Rectosigmoid that defined the site of obstruction in
patients with congenital megacolon using a barium
enema and fluoroscopy
Swenson & Bill 1948 First successful operative procedure.
The operation was undertaken based on careful
clinical observations and thoughtful deduction
ignoring the controversy at the time regarding the
influence of bowel innervation and
the presence or absence of ganglion cells in this
disorder
State (Minneapolis,
Minnesota)
1952 Described low anterior resection
Sandegard 1953 Reported the first successful operation in a patient with
total colonic aganglionosis (TCA) by performing a total
colectomy and an ileoanal anastomosis
Bernard Duhamel
(St Denis, France.)
1956 Described the retrorectal transanal pull-though
procedure.

10. Surgical interventions – Historical Perspective
Rehbein
(Bremen, Germany)
1958 Low anterior resection taking the anastomosis
down to 3–4 cm above the pectinate line
Grob
(Zurich, Switzerland)
1959 Used a different location for the posterior incision. He
made the incision 2.0–2.5 cm above the pectinate line.
Pagès in Paris &
Duhamel
1960 Rectal incision 1.5 cm above the pectinate line to avoid
incontinence and constipation
Soave of Genoa, Italy 1963 Described the endorectal pull-through procedure
Boley
(New York).
1964 Modified the procedure by performing a primary
anastomosis at the time of the pull-through procedure
Martin 1968 Described a modification of the Duhamel procedure
utilizing a side-to-side anastomosis between normally
innervated small bowel and aganglionic colon upto the
level of splenic flexure for TCA

11. Surgical interventions – Historical Perspective
Kimura 1981 used an aganglionic right colon patch.
Boley 1984 Left colon as patch
So et al 1981 First to report a one-stage pull-through procedure in
neonates with HD without a preliminary colostomy
Ziegler 1987 described the concept of myotomy/myectomy of
aganglionic bowel for patients with near total
aganglionosis (NTAG) with less than 40 cm of normally
innervated small bowel
Georgeson et al 1995 Described a laparoscopically assisted Soave endorectal
pull-through procedure avoiding an open laparotomy
De la Torre-
Mondregon &
Ortega-Salgado of
Mexico
1998 First to perform a one-stage totally transanal pull-
through procedure

12. Surgeries available for Hirschsprungs
Disease
• Rectal Biopsy –submucosal , full-thickness
• Colostomy , Ileostomy
• Definitive surgeries
-Swenson’s procedure
-Duhamel’s procedure
-Soave’s procedure
-Rehbein, State – outdated

13. Minimal Access Approaches
-LAPAROSCOPIC PULL-THROUGH
-TRANSANAL (PERINEAL) PULL-THROUGH
-Ultra short - Sphincterotomy
-Short - Extended myomectomy
-Total colonic aganglionosis
-Martin Duhamel
-Kimura Stringel
-Extended myotomy / Myomectomy

14. Rectal suction biopsy
• Less than 4 years of age
• May be performed in ward or clinic without anaesthesia
• Painless if taken 2.5 cm above anal verge in neonate and
3.5 cm in older child
• Preoperative care
– Gentle bowel washout with 10ml/kg of warm saline
– Vitamin k in neonates
• Original suction biopsy instrument was devised by Helen
Noblett in 1969
• Variants: Solo-RBT, rbi2
• Blunt ended tube with a 3mm side hole 2 cm from the
tip
• Lithotomy position for infants
• Left lateral knees bent position for older children

15. • Lubricated instrument inserted into the
anus and side hole positioned at 3 cm from
anal verge
• This is the minimum distance and avoids the
normal hypoganglionic zone and diagnostic
confusion
• Side hole should face posterior wall or
lateral wall of rectum
• Suction applied by withdrawing the syringe
attached to the suction biopsy instrument to
3-5ml (~150cmH2O)
• After 2-3 seconds the knife is triggered

18. • Syringe suction is released to neutral pressure
before removing from the patient.
• Instrument is withdrawn and specimen removed
• 3X1mm
• Submucosa- whitish layer
• Procedure repeated at 3.5cm and 4cm above the
anal verge
• 2-4 specimens are collected
• Specimens marked with the level of collection

19. Postoperative care
• A rectal examination should be carried out after
completing the biopsy to exclude active bleeding.
• Observations should be continued for at least 2
hours, to ensure complete hemostasis.
Complications
• Inadequate specimen retrieval. 10 and 20 %
• Perforation.
• Bleeding.
• Pelvic sepsis.

20. • Solo RBT
*Solo-RBT: A New Instrument for Rectal Suction Biopsies in the
Diagnosis of Hirschsprung’s Disease, A. Pini Prato, G. Martucciello, and V.
Jasonni Genova, Italy. Journal of Pediatric Surgery, Vol 36, No 9 (September),
2001: pp 1364-1366

21. • Open rectal biopsy under general anesthesia is required when the
specimen obtained with the RSB instrument is inadequate or the child is
older.
• Preoperative preparation: Rectum irrigated with saline or very dilute
povidone-iodine solution.
• Position: The infant is held in the lithotomy position while an older child
will need to be placed in stirrups in the lithotomy position.
• Prophylactic antibiotics are given.
Open rectal biopsy
Procedure
■ The anal orifice is digitally dilated. It
is held open either with a Parks’
retractor (or similar self-retaining
retractor) or by an assistant holding
two small Langenbeck’s retractors.

22. • The superior aspect of the
dentate line is identified and
marked with a polyglactin
suture (3/0), which is used for
traction.
• Two additional polyglactin
sutures are placed on the
posterior wall of the rectum at
1 and 2 cm proximal to the
dentate line.
• Retain the needle on the most
cephalad of these sutures, as
it can be used to begin the
closure of the defect after the
biopsy is obtained.

23. • hold the middle suture.
• Using sharp curved scissors,
a full-thickness incision is
made along the lower half of
the rectal wall, between the
dentate line and the middle
suture.
• The rectal defect is closed in
a single, full thickness
running or interrupted layer
with an absorbable suture
(e.g. 4/0 polyglactin (Vicryl)

24. • Hemostasis may be
achieved with bipolar
diathermy or, more
usually, by suturing the
defect with a running
locking suture from
above.
• Complications
– Hemorrhage
– Infection

25. New born with
delayed passage of
meconium
Enterocolitis
Older infants/children
with constipation
(+or-)Rectal biopsy
(suction/full thickness)
Urgent decompression by
rectal irrigation +/_
colostomy
Fluid resuscitation
NG aspiration
Antibiotics
Plain X ray
Contrast enema
Primary pull through
Leveling colostomy
R. Transverse colostomy
Three stage procedure
Two stage procedure
Frozen section facility
Anorectal
manometry
for RAIR
(screening)

26. Three stage procedure
• Advantages
– Colostomy can be made even if transition is not evident & frozen
section facility is not available
– Anal anastomosis is well protected by colostomy
– The risk of the error of opening the colostomy in an aganglionic area is
much reduced
• Disadvantages
– Multiple admissions & operations
– Molibilisation of colostomy is needed in cases with long segment HD
– Distal segment may undergo disuse atrophy if left long or child lost for
follow-up
• May not function properly after colostomy closure

27. Two stage procedure
• Initial colostomy is performed at the junction
of the ganglionic & aganglionic colon (Leveling
colostomy)
• Second stage – pull-through done after taking
down the stoma & using the proximal end for
pull-through
– 6 – 12 months age

28. Levelling Colostomy
Indications
• Severe enterocolitis
• Markedly dilated proximal colon due to delayed
diagnosis
Advantages
• Allows determination of aganglionic level- facilitating
subsequent pull- through
• Allows the proximal bowel to grow
• Colostomy closed during pull-through thus avoiding a
3rd operation
• Max. amount of colon for absorption
• Assures colostomy is in functioning bowel
• Avoids risk of second pull through.

29. Leveling colostomy –
Disadvantages
• No benefits of protective colostomy
• Length of colon mobilized and removed may
be longer
Operation technique
• Colostomy performed at the level of normally
innervated ganglion cells as ascertained on
frozen section i.e., just proximal to the
transition zone.

30. Leveling colostomy – operation technique
• Preoperative preparation
– Rectal washouts
– Broad-spectrum, intravenous antibiotics just prior to incision
– No formal bowel preperation is required or effective
• Incision
– Oblique incision in left lower quadrant
– If level of aganglionosis is not readily apparent, incision can be
extended transversely across the midline.

31. Leveling Colostomy
In general, a 1 × 0.5-cm biopsy specimen is
taken and interrupted silk or polyglactin
sutures are placed to close the biopsy site

32. Leveling colostomy -
Postoperative care
• The stoma usually begins to function within 24
hours, and feeding can begin shortly
thereafter.
• It is occasionally helpful to perform
intermittent dilatations of the proximal
ostomy to prevent narrowing of the opening
and allow the dilated proximal colon to return
to normal size.

33. Surgical procedures
• Rectosigmoidectomy (Swenson and Bill -1948)
• Retrorectal –transanal approach (Duhamel -1956)
• Endorectal procedure (Soave -1964).
• Laparoscopic assisted transanal endorectal pull-through
(LATEP) – Georgeson et al(1990’s)
• Transanal endorectal pull-through (TERPT) - De la Torre and
Ortega-Salgado and Langer et al in the late 1990s.
• The basic principle of all the procedures is to bring the
ganglionic bowel down to the anus

34. Schematic illustrations of several operative pull-through
techniques for the treatment of Hirschsprung disease.

35. Preoperative assessment during definitive surgery in staged
surgery
• History & physical examination
• Records of operative findings, procedures done, HPE reports
• X-ray abdomen – to identify any fecaloma
• Distal cologram- to outline the adequacy of the distal bowel for pull-
through without the need to mobilize the colostomy. (if initially right
Transverse colostomy )

36. Preoperative preparation for pull-through
• First generation cephalosporin or ampicillin + gentamycin or
amikacin + metronidazole started 24hrs prior to surgery
• Mechanical bowel preparation
• Twice daily rectal washes with NS starting at least a week or two
prior to surgery/ Distal colostomy washouts
• Low residue diet for 3days prior to surgery
• Gut irrrigation using polyethylene glycol solution (70ml/kg) on the
day prior to surgery
• On the morning of the surgery a rectal flatus tube is inserted to
ensure complete evacuation of colon.

37. SWENSON’S PULL HROUGH
Surgical principle is to remove the
diseased portion of the bowel that is a
aganglionic distal rectum &
anastomosing normal colon to lower
rectum to maintain the continuity to
allow normal defecation
Orvar Swenson, MD, 1909-2012
The first ever pull through procedure described for treating
HD & published by Swenson & Bill in 1948

38. Swenson’s Pull-through
• Initially anastomosis used to be
completed at the anorectum (2cm from
dentate line)
High incidence of enterocolitis (early
16%, late 27%).
• Modified by
– Resecting virtually whole of the
posterior rectal wall (very top aspect
of IAS)
– Making an oblique anastomosis
– leaving 1.5 to 2cm anteriorly,
– 1 to 1.5cm on both sides &
– 0.5 to 1cm posteriorly from dentate
line

39. Swenson’s Pull-through
• Position
– Should provide simultaneous
exposure of the perineum &
abdomen
– Lithotomy position
– Pelvis is allowed to drop back
over the lower end of the table &
legs are strapped over the
sandbags/ stirrups.
– Foley catheter is inserted into the
bladder

40. Swenson’s Pull-through
• Anal dilatation – slow & gentle anal dilatation
• Incision
– Oblique /Modified hockey stick incision in LLQ incorporating the
colostomy.
• Colostomy mobilised
• Denis Browne retractor applied
• Urinary bladder is lifted forward out of abdomen by stay sutures
• Sigmoid colon is mobilised by dividing sigmoid vessels & retaining
the marginal vessels
• It may be necessary to mobilise the splenic flexure to obtain
adequate length.

41. Swenson’s Pull-through
• Bowel is divided at the rectosigmoid
junction & removed
• Peritoneum is divided around its lateral &
anterior reflection from the rectum
exposing the muscle coat of rectum
• Dissection extends around the rectum,
keeping very close to the bowel wall.
– to prevent damage to the pelvic
splanchnic innervation.

42. Swenson’s Pull-through
• Dissection should commence from side to
side & in an anterior to a posterior
direction with the rectum being gradually
dissected out in a circumferential manner.
• Dissection is done extensively posteriorly
& on both sides but less anteriorly

43. Swenson’s Pull-through
The mobilized rectum is intussuscepted through the anus by passing a
curved clamp or a Babcock forceps

44. Swenson’s Pull-through
• An incision is made anteriorly through the rectal wall about 2 cm
from the dentate line, extending halfway through the rectal
circumference (9 to 3o’clock)
• A clamp is inserted through this incision to grasp multiple sutures
placed through the cut end of the proximal colon

45. Swenson’s Pull-through
• The normal colon is pulled through the pelvis and out through the incision in
the everted rectum.
• An outer layer of interrupted 4-0 absorbable sutures is placed through the
cut muscular edge of the rectum and the muscular wall of the pull-through
colon.

46. First the anterior muscular anastomosis is completed.
Incision on the rectum is extended 1-1.5cm from dentate line on both sides
& 0.5 – 1cm posteriorly
Through the diagonal excision of the rectum, the internal sphincter is
preserved to maintain fecal continence & partial sphincterectomy is done
in posterior direction to reduce the risk of post-op HAEC
With traction on the three o'clock and six o'clock sutures, the left posterior
quadrant of the muscular anastomosis is done.
In a similar manner, the right posterior quadrant of the anastomosis is
completed

47. Swenson’s Pull-through
• When the outer layer (muscular) anastomosis is completed, the end of the
pulled-through colon is incised a few millimeters from the muscular suture
line.
• The mucosa of the cuff of the everted rectum is anastomosed to the mucosa
of the pulled-through colon ( interrupted 4-0 absorbable sutures).
• When anastomosis is completed, the sutures are cut, allowing the
anastomosis to retract within the anus.

48. Post op care
• keep the infant on total parenteral nutrition
for 7 days postoperatively and then gradually
start oral feeds.
• The urethral catheter is removed after 3 days.
• Antibiotics are discontinued after 5 days.
• Rectal examination is performed 2 weeks later
during an outpatient visit.

49. Experience with Swenson’s procedure
• Mortality
• The mortality after Swenson’s operation is reported to be 0–
5.8%
• Postoperative mortality is considerably increased in patients
in whom anastomotic leaks occur
• Significantly higher in babies with Down’s synd
• Bowel control
• Bowel control increases with age
• A followup of 5 years is required for complete evaluation
• Best predictors of abnormal bowel habits by sherman et al
are – temporary soiling after discharge and rectal stricture.

50. Experience with Swenson’s procedure
• constipation is the most common late
complication
• Soiling
• Temporary soiling after discharge was the only factor which
influenced the occurrence of soiling at follow-up
• Influence of Trisomy 21 on Bowel Control
• Studies show 10-15% of association
• More than 3/4th of patients with Down’s synd had
completely unsatisfactory outcome.
• Attainment of normal post op defecation is dependent on –
intensity of bowel training, social back ground, intelligence
of child and motivation to be socially clean which are lacking
in these children.

51. Persistent Bowel Symptoms due to Associated Intestinal Neuronal Dysplasia
– HD is associated with intestinal neuronal dysplasia (IND) in
about 25% of patients
– Patients have constipation, enterocolitis or soiling.
• Rectal stricture
– 9.5% after swenson’s procedure, 4.3% required operation, higher
incidence is seen in patients operated before 4 months of age.
• Fistulae were rare
• Other rare complications are – intestinal obstruction,
urinary incontinence, sexual dysfnction
• Enterocolitis remains the most serious complication.

52. DUHAMEL’S PROCEDURE
Principle of Duhamel’s technique is to exclude the rectum
instead of removing it

53. Duhamel operation
• Bernard Duhamel first described his operation for Hirschsprung‘s
disease in 1956.
• The procedure consisted of a retrorectal dissection, whereby a
significant portion of the aganglionic rectum was preserved and
anastomosed to ganglionated proximal colon.
• The advantages of this procedure included
– Ease of performance,
– Reduction of anastomotic leaks and strictures,
– Retention of anal sensory receptors, and
– Preservation of the nervi erigentes.

54. Duhamel operation
• Operative principles
– Minimal pelvic dissection
– Retrorectal approach for the pulled-through colon to anal
opening
– Partial disruption of internal anal sphincter posteriorly
– Preservation of the anterior wall of the rectum & its nerve
supply
– Elimination of colo-rectal septum with wide side-to-side
anastomosis (stapled/crushed) between the anterior aganglionic
& pulled down ganglionic colon

55. Modifications
• In 1956 1st description - Anal transverse incision
was made at the ano-cutaneous junction- high
anal incontinence & prolapse
• In 1959 - Grob modification- post. anal wall
incision 2-2.5cm above anocutaneous junction- to
preserve IAS – high incidence constipation &
fecalomas
• In 1960,Duhamel-Pages modification - Anal
incision 1-1.5cm above pectinate- preserves 1/3rd
of IAS- less soiling & less constipation.

57. Modifications
• Centered around the elimination of the
common wall of the rectal pouch spur
• 1964 Zachary & Lister - O shaped clamp
• 1966 Talbert - Suturing & stapling device
• 1987 Steichen - EEA
• Martin & Caudill – End-to-side anastomosis

59. Duhamel procedure.
• Operation generally done when the child is 6-
12 months age & wt 10kg
• Prior to surgery rectal examination should be
done to ensure rectum is empty
• Child placed in lithotomy position with proper
padding
• Child cleaned, draped & catheterized
• Incision – muscle cutting hockey stick incision
is given

60. Duhamel procedure.
• Colostomy mobilised & dismanteled, transected
• Alternatively the colostomy site is removed with an automatic
stapling device.
• Proximal colon closed with a running silk suture
• 4 stages
– Mobilisation of upper colon & closure of the rectum
– Preparation of retrorectal space
– Endoanal incision
– Retrorectal pull-through procedure

61. Duhamel procedure -Mobilisation of colon
• Proximal mobilisation is done- it can be
brought easily to below the pubic symphysis
– May sometimes need division of left colic artery/IMA at
aortic root
– Marginal artery of Drumond should be preserved so that
colon viability is based on the left branch of middle colic
artery

62. Duhamel procedure – Closure of rectum
• Peritoneum over the mesosigmoid
is incised both laterally & medially,
joined anteriorly in the colo-vesical
pouch
• Both the ureters are identified
• Colon mobilised distally upto the
level of pelvic peritoneum
• The rectum is transected just above
the pelvic peritoneum (and closed).

63. Duhamel procedure-
preparation of retrorectal space
 Opening of the mesorectum provides
access to the retrorectal space
 The blunt dissection is carried out with
index finger/ long curverd forceps with a
small sponge/ long kelly’s clamp
 The dissection is carried down to the pelvic
floor so that the assistant’s finger can be
felt when inserted no further than 1 –
1.5cm into the anus

64. Duhamel procedure-Endoanal incision
– A semicircular incision (posterior 1800 ) is made on the rectal
wall between silk stay sutures placed at 3, 6 & 9 O’ clock
positions
– The incision should be 0.5 – 1.0 cm proximal to the dentate line
• .

65. Retrorectal pull-through
• The kelly’s clamp/long curved
forceps with sponge is passed
from abdomen through the
retrorectal space
• The sponge is grasped with
another long curved forceps
through the incision
• Using sponge as a guide, forceps
is drawn in a retrograde direction
into the abdomen

66. Duhamel procedure.
• The proximal colon is grasped with the
forceps and drawn downward into the
retrorectal space to the level of
endoanal incision
• Care should be taken that bowel is not
twisted, not in tension, mesentry to be
directed posteriorly in sagittal plane.

68. Duhamel procedure.
• Anastomosis technique
– Two long kocher clamps are inserted with one blade in the
rectum & other in the drawn colon
– The bases of the clamps are held apart so that Their end
points meet in an inverted V at the apex of the rectal
pouch
– The position of the clamps is checked by direct abdominal
palpation

69. Duhamel procedure.
• The walls of the colon & rectum deprived from circulation necrotize & the
clamps with necrotic tissue slides out of the anus (after 4 – 10 days)
• By this way a large enterotomy is carried out with a longitudinal
anastomosis between the drawn colon & native rectum
• Disadvantages –
– The protrusion of the handles of the clamps through the anus-
inconvenient to the child.
– Incomplete division of the colo-rectal septum
– Occurrence of stenosis

70. Alternative method
• The proximal end of the rectal stump
is kept open
• A longitudinal colotomy is made on
the pulled down colon opposite the
open rectal stump
• GIA linear cutter is inserted through
the anus with one blade in the
rectum & other in pulled down colon.

71. Alternative method
• Care to be taken that the entire length is
included and the tip of the stapler is
protruding well beyond the rectal stump &
colostomy in abdomen
• The stapler is then fired to complete the
longitudinal anastomosis & division of the
common wall.
• In case the entire common wall is not
divided, the same needs to be completed by
another firing with the stapler now applied
from the abdominal end in prograde
fashion.

72. Duhamel procedure.
• The open proximal end of the rectal stump is anastomosed to the
colostomy on the pulled down colon in end-to-side fashion…
Martin’s modification
• The anastomosis is extraperitonealized after placing a small pelvic
drain of CRD
• Peritoneum on both the medial & lateral aspects of pulled down
colon is repaired
• Abdominal wound closed in layers
• Rectal pack of paraffin gauze is applied, removed after 24hrs

73. Complications and results
• Anastomotic leak – 2.2%
• Necrosis – 0.09%
• Stricture – 0.7%
• Mortality rate – 1.6%
• Majority of post op deaths are related to
enterocolitis (incidence – 5-26%)
• Incontinence – 0-20%
• Constipation and fecal impaction due to larger
reservoir – 5-8%
• Lower UTI, ? Partial detrussor denervation

74. • Primary vs staged Duhamel’s
• Limited studies are available.
• Laparoscopic Duhamel’s procedure
– Laparoscopic techniques have mainly been described
for Swenson’s procedure and less frequently for
Duhamel’s.
– Bowel biopsies are taken laparoscopically to
determine the extent of aganglionosis before ablation.
– Further steps – laparoscopic mobilization, dissection
and closure of rectal stump and laparoscopically
controlled pull through.

75. Postulated advantages
– Visualization
– Atraumatic dissection
– Less post op pain
– Faster recovery
Duhamel’s procedure for re-do pull through is
Indicated in patients with
– Recurrent fecoloma formation
– Recurrent episodes of enterocolitis
– Retained aganglionosis
– Segmental bowel dysfunction with bowel dilatation

76. SOAVE’S (BOLEY SCOT)
ENDORECTAL PULL-THROUGH
The operation based on removing the mucosa and submucosa 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.
Avoids injury to the pelvic nerves

77. Indications
• Hirschsprung’s disease
• Multiple Juvenile polyposis
• Ulcerative colitis
• Familial poliposis.
History
• 1955 Roumaldi proposed it at Roman society of surgery
• 1957 Soave used it for ARM with fistula & in 1961 he
performed this tech for HD.
• 1964 Boley modification- Primary anastomosis

78. • Coran & Weintraub modification – eversion of
submucosal-mucosal tube onto the perineum to
facilitate anastomosis
• Rintala & Lindahl- Transanal approach combined
with an open laparotomy for mobilization of
aganglionic segment
• Georgeson- Lap assisted mobilization of
aganglionic segment
• Torre-Mondragon & Ortega-Salgado– entire
dissection & mobilisation via transanal route

79. •In Original Soave procedure the pulled through colon is left hanging
beyond the anal verge.
•A•fter a period (~ 2 wks) to allow adherence of the bowel to the
anal tissues,the protruding segment was resected
•No anastomosis.
•Boley (1964)- performed a primary anastomosis at the anus.
Endorectal Pull-through

80. Endorectal Pull-through
Preparation
• Upto 1980’s - >5 months & weight > 8 kg
• If soave’s procedure is performed with in 3 months of life, chronic
inflammatory process of rectum (because of chronic proctitis leading to
tenaceous adhesions on submucosal layer) are generally avoided.
• Procedure does’t require any protective colostomy
• Aim- achieve radical treatment with out contaminating the operative field
• Colostomy is indicated if – acute enterocolitis/ intestinal obstruction
• Colostomy is done in the most distal part of the ganglionic colon 
leveling colostomy.
• In order to achieve this it is necessary to perform multiple seromuscular
biopsies intra operatively.

81. • Preparation starts from one week before the
procedure to reduce complications
• Rectal probing should be repeated and
alternated with evacuating enemas.
• Perioperative IV antibiotic prophylaxis is
started 1 hour before surgery.

82. Soave’s – operative tech
• General anesthesia
• Catheter placement
• Performing wide dilatation of the anal canal
using two fore fingers – essential for sucessful
pull through procedure.
• Supine position with buttocks lying at the
extreme edges of operating table and legs
hanging freely wrapped in drapes and fixed to
prevent slipping of patient pelvis.

83. Laparotomy
• Paramedian left incision/ pfannensteil incision
• In patients with level ileostomy for total colonic
aganglionosis, a xiphopubic median incision is required in
order to perform an endorectal ileal pull-through
procedure.
• All mesocolic adhesions to the left parietal peritoneum are
dissected up to the splenic flexure (classic form of HSCR).
• Before starting endorectal dissection, it is essential to
perform multiple seromuscular biopsies of the rectum and
colon in order to evaluate the length of the aganglionic and
associated hypoganglionic segments

84. • Seperation of seromuscular from mucosal
layer of rectum
• Most technically difficult and peculiar step
• mepivacaina 2% with epinephrine 1:100,000
(10 μg) in 10 ml of normal saline solution is
injected between the layers in order to
facilitate initial seperation

85. Endorectal Pull-through
• The endorectal dissection is started
approximately 2 cm below the peritoneal
reflection
• The seromuscular layer incised with
either sharp dissection or cautery.
• Once the submucosal layer is reached the
seromuscular layer is divided
circumferentially using blunt dissection
with hemostat/Kitner dissector.

86. Endorectal Pull-through
• After the plane is established, dissection is continued distally.
– Once muscular cuff begins to develop, traction sutures placed in
muscle in each quadrant.
• Communicating vessels coagulated.
• Dissection is carried down within 0.5cm (in neonates) – 1cm( older
chidren) from dentate line

87. Endorectal Pull-through –Perineal dissection
• Narrow retractors placed at anal-mucocutaneous junction
• Kelly clamp is inserted into the rectum
• The mucosal-submucosal tube is then everted onto the
perineum.

88. Endorectal Pull-through –Perineal dissection
• The mucosal-submucosal tube is
incised on the anterior half
0.5cm – 1cm from the dentate
line

89. Endorectal Pull-through –Perineal dissection
 A Kelly clamp is inserted into this
opening for grasping the two
previously placed traction sutures.
 Great care is taken not to twist

90. Endorectal Pull-through –Perineal dissection
 Anterior ½ of the ganglionic colon
is incised & anastomosed to the
anterior half of the mucosal-
submucosal tube with interrupted
absorbable sutures
 One ¼ of the remaining
 Final ¼
 invert the neorectum into correct
position

91. Endorectal Pull-through
• The pulled through colon is attached with seromuscular bites
to the muscular cuff to prevent prolapse.
• No drain is placed

92. PRIMARY PULL THROUGH
IN NEONATES
All the surgical procedures described have been performed as
primary/single stage with good & comparable outcome

93. Primary Versus Staged Pull-through
• Principle
– Colonic dilatation can be quickly controlled by rectal washouts -
Calibre of the pull-through bowel is near normal-allows accurate
anastomosis-minimizes leakage & cuff infection
• Advantages of primary pull-through in neonates
– Specific stoma complications are avoided
– No. of admissions & hospital stay reduced
– Anal function and the anorectal reflex are reestablished as early
as possible.
• If attempted in older children hugely dilated colon segment (normal
ganglionic segment)needs to be resected

94. Selection criteria for Primary Pull-through
Parameter Primary Staged
Presentation Early Delayed presentation (very
dilated bowel)
Disease invovlement Rectosigmoid Long segment, TCA
Enterocolitis Absent/resolved Active/severe
Rectal washes Effective decompression No decompression
General condition Stable Deteriorating
Local expertise Frozen section histology Not available

95. LAPAROSCOPIC PULL-
THROUGH
Avoidance of a painful abdominal incision,
Rapid return of bowel function,
Shorter postoperative recovery,
Improved cosmetic appearance.

96. • In the early 1990s, Georgeson et al described a minimal access approach,
consisting of a
– Laparoscopic biopsy to identify the transition zone,
– Laparoscopic mobilization of the rectum below the peritoneal
reflection, and
– Short endorectal mucosal dissection from below.
– The anastomosis was done from below after prolapsing and excising
the rectum.

97. • Multiple reports documented a short time in the hospital, and early
results were equivalent to those reported for the open procedures.
• Subsequently, laparoscopic approaches have been described for the
Duhamel and Swenson operations, with excellent short-term results
reported

98. Laparoscopic Endorectal Technique
• With the patient positioned transversely at the end of the operating table, CO2
insufflation of the peritoneal cavity was performed using a Varess needle
inserted through the abdominal wall.
• Three 5mm (3mm) trocars were inserted as shown
• Additional midline suprapubic trochar site is useful to provide pelvic retraction &
hold the colon in traction during dissection of pelvis

99. Laparoscopic Endorectal Technique
• Dissection and leveling of the
aganglionic segment are performed as
in the open technique.
• Blood vessels may be ligated with
surgical clips or cautery for smaller
vessels .
• Once mobilization is complete, the
surgeon moves to the perineum, where
a trans anal dissection and anastomosis
are performed

100. TRANSANAL ENDORECTAL PULL
THROUGH

101. Transanal Endorectal Pull-through
• Selection criterion
– Neonates, infants (proximal bowel not much
dilated)
– A barium enema study must suggest the diagnosis
of HD & also the level of aganglionsis
– Confirmed diagnosis of HD
– HD limited to rectosigmoid or short segment

102. Transanal Endorectal Pull-through
• Advantages of one-stage anal procedures
– Avoidance of multiple laparotomies with its associated complications
– Avoidance of colostomy with its associated complications
– Reduced operating time
– Less blood loss
– No pelvic structure damage
– Decreased need of analgesics
– No external scars & Improved cosmetic appearance
– Single hospital admission
– Short hospital stay
– Lower hospital costs
– High degree of parental acceptance

103. Transanal endorectal pull through -
procedure
Preparation
– diagnosis is confirmed by rectal biopsy
– Prior to surgery, the colon must be decompressed and
enterocolitis, if present, controlled
– Nutritional status must also be evaluated and optimized.
– In an older child with severe enterocolitis or massive colonic
distension, a defunctioning stoma should be considered
– routine preoperative mechanical bowel preparation is
unnecessary
– Mechanical
– irrigation of the bowel can be accomplished with equal
effectiveness from below once the child has undergone
anesthesia.
– Intravenous prophylactic broad spectrum antibiotics are used

104. Transanal endorectal pull through -
procedure
• General anesthesia
• Lithotomy position/ prone jackknife position
• Urinary catheterization is optional

105. Submucosal Dissection
• anal retractor or retraction sutures are placed
• submucosal injection of a dilute epinephrine
solution or air
• rectal mucosa is circumferentially incised using
cautery approximately 3–5 mm from the dentate
line
• fine sutures are placed in the proximal cut edge
of the mucosal cuff, and traction is applied while
the endorectal submucosal dissection is carried
proximally

106. Mobilization of the Rectum
• When the submucosal dissection has been
completed, the rectal muscle is divided
circumferentially
• Dissection then continues proximally, dividing all
vessels
• When the peritoneal reflection is reached, the
sigmoid is then mobilized in the same fashion and
the rectum and sigmoid are delivered through
the anus

107. • Anastomosis
• colonic dissection is completed when the
transition zone is reached
• colon is divided at least 2 cm above the most
distal normal biopsy to prevent the possibility of
a transition zone pull-through
• rectal muscular cuff is then split longitudinally
• standard Soave-Boley anastomosis is then
performed.

109. Transanal Endorectal Pull-through
• Contraindications
– Redo pull-through
– Grossly dilated bowel in older chidren
– Without confirmed diagnosis of HD
– Long segment HD/TCA
– Any H/O bleeding disorders

111. Postoperative care
• Oral feeding can be started as soon as child starts to pass stools if there is
no abdominal distension
• Examination of perineal region
– To look for erythema/cellulitis
• Early sign of an anastomotic leak (leak uncommon)
• In cases of ERPT Per rectal examination with cotton tip applicator to
ensure patency of rectal anastomosis befor discharge
• Instructing the parents to apply thick coat of barrier creams
• Educating the parents regarding enterocolitis.
– Preventive measures
• Follow up to late complications

112. • Stooling pattern change in the first 2 yrs after pull-through
– Frequency
• 5-10 bowel movements/day – immidiately after pull-
through
• 1 - 4 bowel movements/day – within 6-12 months
• Normal pattern
– Constipation
• Slowly improves

113. Complications.
• All post-operative complications which are recognised during the 1st
4weeks following the surgery
• Factors which influence
– Extent of aganglionsis
– General condition of the child
– Technical expertise & experience of the surgeon
– Age at the time of operation.
– Whether or not colostomy done
– The administration of prophylactic antibiotics
– Family care
• The late follow-up offers the best oppurtunity to critically evaluate
the efficiancy & results of any particular surgical procedure.

114. Early complications
• Wound infection (4%)
• Wound dehiscence(1%)
• Hemorrhage (<2%)
• Intestinal obstruction (7.5% -
10%)
• Anastomotic leak or disruption
(1-10%)
• Cuff abscess (5%)
• Retraction of bowel(2 -10%)
• Rectal stenosis (10 -20%)
• Enterocolitis (5-42%)
• Minor complications
– Perianal excoriation
– UTI/bladder dysfunction
• Mortality

115. Late complications
• Constipation
• Incontinence (1-39%)
• Enterocolitis
• Urological complications
• Incontinence
• Retention
• Impotence
• Anastomotic
complications/fistula
• Intestinal obstruction
• Moratlity (07%)

116. Enterocolitis (HAEC)
• Can be severe or life threatening
• Clinical features
– Fever,
– Abdominal distention,
– Diarrhea,
– Elevated white blood cell count, and
– Evidence of intestinal edema on abdominal radiograph
• Reported incidence varies in literature (2% - 40%)
(depending on the definition)
• HAEC score may be useful

118. Enterocolitis (HACE)
• Majority of the episodes occur within 2yrs ofter pull-through
• There is no correlation between enterocolitis before & after surgery
• The incidence of enterocolitis directly correlates with mortality
– In a survey by AAP (1196 cases) , 14% developed enterocolitis with a
mortality of 30%
– Swenson’s series of 880 cases death after discharge from enterocolitis
– 1%
• Early recognition with prompt management influence the outcome

119. Enterocolitis
• Swenson & Fisher (1956) advocated rectal tube decompression for
initial management
• Other measures
– Aggressive fluid resusciation
– Bowel rest
– Broad spectrum antibiotics
– Resection with Diversion if peritonitis or clinical worsening
occurs
• Children with repeated bouts of enterocolitis needs to be evaluated
for mechanical obstruction
– Contrast enema, manometry, rectal biopsy

120. Enterocolitis
• Most patients will improve with time
• Patients with persistent enterocolitis despite investigation
– Can be managed temporarily with botulism toxin
– Permanently by internal sphincterotomy ( Polley et al & Marty
et al)/posterior myectomy
• Preventive measures to minimise the risk of HACE
– Chronic administration of metronidazole ( 1-2months) or
probiotic agents
– Prophylactic rectal irrigations(Marty et al)
– Should be advised particularly in those who are thought to be
at higher risk for this complication on the basis of clinical or
histologic grounds.
• It is extremely important that the surgeon educate the family
about the risk of this complication

121. Voiding & sexual dysfunction
• Many of the operations for Hirschsprung's disease have been designed
specifically to avoid pelvic nerve injury.
• Overall, the incidence of impotence and urinary dysfunction is quite low.
• Postoperative enuresis ~9.5%.
– Swenson 10.4%
– Duhamel 14.3%
– Soave 15.3%
• More recent series report no incidence of impotence or urinary problems.

122. Late mortality
• Most common cause is enterocolitis
• Other causes of death
– Intestinal obstruction
– Pneumonia
– Nonrelated medical disorders.
• In most studies the differentiation of early & late deaths is lacking
• No specific pull-through procedure is associated with higher rate of
late deaths

123. Overall quality of life
• Is difficult to assess
• Overall quality of life was described as quite good with ~94%
of children becoming well adjusted members of the society
• Developmental milestones are satisfactory in ~95% cases
• School performanace is satisfactory in ~ 82%
• Clearly children with poor functional outcome have
psychosocial problems

124. Comparison of different procedures

126. Thank you