Hirschsprung's Disease - Athigaman

Hirschsprung's Disease - Congenital Mega Colon
Transition Zone
Normal Bowel
Hirschsprung
Disease
Definition: HD is characterized by absence of ganglion

cells in the distal bowel and extending proximally for
varying distances with dilatation and hyper trophy of
the proximal colon with abrupt or gradual transition to
narrow distal afflicted colon.
 1886 - Hirschsprung gave detailed description of
mega colon History
 Ehrenpreis – colonic malfunction due to
imbalanced autonomic enervation
 Wade and Ryle – Lumbar Sympathectomy
 Ross – Segmental Resection of the dilated bowel
 1949 - Swenson – Colostomy and total recto
sigmoidectomy
 1960 – Duhamel – Partial bypassing with side to
side Anastomosis using clamps
 Soave Mucosal Resection with low anal
Anastomosis
 1958 - Rehbein – Low anterior resection with
Anastomosis 3 to 4 cms of muco cutaneous line
with dilatation
 Prem Puri – Indian – Authority on Histology
Hirschsprung's Disease - Dept. of Paed. Surgery, Madurai Medical College. Slide No: 2
Enteric Nervous System
Intrinsic and Extrinsic
Parasympathetic
Vagus Nerve
Enhances peristaltic Activity
Auerbach’s Inter Myentric Plexus
Sympathetic
Splanchnic Nerves ending in
Plexuses
Coeliac, Lumbar, Hypo gastric
Sub Mucosal Meissner’s Plexus

Slide No:
Hirschsprung's Disease - Dept. of Paed. Surgery, Madurai Medical College. 3
Development of Enteric Nervous System
Source
Caudal Hind brain
Enteric Neural Crest
Posterior part of Rhombomere 8
Adjacent to Somites 4 and 5
Development of Enteric Nervous System
Migration
Posterior rhombomeres migrate
through Ventero lateral pathway and
anterior path of somites – eg. GIT
Anterior rhombomeres migrate
through dorso lateral pathway and
pharyngeal arches – eg. Thyroid
Migration to bowel is at Vagal,
Truncal and Sacral regions
Chronology of Development of
Enteric Nervous System
Migration
5th month – Mid gut
6.5 month – Caecum
8th month – Migration complete
Arrest of caudal migration leads to
HD
Genetics of Enteric Nervous System
Neurotrophic factors
GDNF – Glial cell line derived
Neuronotropic factor
Transforming growth factor β
(TGF - β)
Laminin – extra cellular matrix
present in basal lamina of smooth
muscle, presence of which may
inhibit migration by formation of
neurons
Wardenburg
 Pigmentation abnormalities Syndrome
 White fore lock, eye brows, hetero chromia irides
 Cranial and Spinal Nerve abnormalities
 Inner ear degeneration causing sensori neural
deafness
 Bowel dysfunction
 Hyper, Hypoganglionosis, aganglionosis
 Facial abnormalities
 Membranous bones of face and palate
 Chromosome 2q and identified as PAXm3
 Chromosome 3
Genes Genetics of HD
C RET Proto Oncogene (Receptor
Tyrosine Kinase – Super family) –
Chromosome 10
Endothelin 3 (EDN 3)
 Lethal spotted - Chromosome 20
Endothelin B – Receptor - (EDNRB)
 Piebald Lethal Dominant Mega colon
(DOM) - Chromosome 13
MASH 1 gene – Catecholaminergic
Deletion Chromosome 2, 10, 13
Partial Trisomy 11, 12
Trisomy 21
Genetics of Hirschsprung Disease
Increased sibling history (4%)
Unbalanced sex ratio (4:1)
Associations
Down’s
MEN 2
Wardenburg Syndrome
Smith Lemli Opitz Syndrome
Chromosomal deletions or
rearrangements in 10 or 13
Slide No: 10
Hirschsprung's Disease - Dept. of Paed. Surgery, Madurai Medical College.
Mucosa Micro Anatomy of Bowel
Epithelium– Columnar & goblet
Lamina Propria
Sub Mucosa
Muscularis Mucosa
Muscular L. or Muscularis
Propria
Inner Circular muscle
Inter muscular space
Outer Longitudinal Muscle layer
Serosa
Micro Anatomy – Intrinsic Nerves
 Sub Mucosal plexus
 Meissner’s
 Sub mucosal plexus lying below muscularis mucosa
 Predominantly adrenergic
 Henle’s plexus
 Close to the surface of the circular muscle
 Stach’s plexus – Nervous plexus lying on
the mucosal surface of inner circular
muscular layer
 Synonyms: Plexus submucosus extremus
 Plexus externus extremus
 Interstitial
cells of Cajal which form a mono
layer of specialized epithelial network over
the Stach’s plexus
 Auer Bach's Plexus – in the inter muscular
space - Parasympathetic
Normal
Neurological
Innervation
Of Bowel
33
 Slow wave paces governing rhythmic
contractions Colonic motility
 Caecum and Ascending colon
 Purpose is mixing, stasis and absorption
 Whereas pace making signals spread ante
grade in stomach and small intestine the here it
is retro grade peristaltic contractions (anti
peristalsis)
 Slow waves arise in the Interstitial cells of Cajal
 Slow waves are ante grade in the distal colon
 Mass movement occurring at long intervals

and occupying only a part of the colon –
Movement of propulsion
 Mass movement can be withheld
temporarily by Voluntary Muscles
Colonic motility in Hirschsprung
 Normal Muscle behaviour is attributed to
the neural reflux
 Normal Colonic motility:
 Contraction of Circular and relaxation of
Longitudinal Muscle proximally
 Contraction of Longitudinal and relaxation of
Circular Muscle distally
 Patho Physiology would include
 Defective function of segmental intestinal
motility
 Lack of propagation of peristaltic waves
 Abnormal or absent opening reflux in the
Internal Anal Sphincter
 Reservoir function (cf: stomach)
 Slower and more continuous
 Concentration rather than dilution
 Emptying is abrupt and complete
 Accommodation and receptive relaxation is
capricious
 Closure of anal canal Continence
 Internal sphincter – Involuntary
 Thickening of visceral circular muscle
 Maintains tone constantly
 Major determinant of continence at rest
 External sphincter – Voluntary

 Pubo rectalis, Subcutaneous External, Superficial
External, Deep Internal
 Contraction is predominantly voluntary
 Muscle mass complex
 Sensory
 Initiation by excitation of sensations
 Ano rectal receptors are triggered by mechanical
stimulation
 Distension is a better stimulus than chemical
 First: Urge to defaecate
 Second: Reflex relaxation of Sphincters
 Third: Mass Propulsion
 Gastro colic reflex is initiated by entry of food into the
duodenum
 Voluntary actions


Closure of airway
Descent of diaphragm
Defaecation
 Contraction of abdominal muscles
 Relaxation of the striated anal muscles
 Involuntary actions
 Peristaltic contractions – Mass
 Relaxation of internal sphincter
 Acting on nerves
 AcetylCholine Chemical
 Substance P
 Acting on muscle transmitters
ATP

Vaso active Intestinal Peptide (VIP)

in ENS
 Secretion, Motility, Blood Flow, Activates NO
 Nitric
Oxide
 Tachykinins
 Opioids – Met-Enkephalin, Leu Enkephalin
 Pancreatic Poly Peptide (PPP)
 Somatostatin
 Neurotensin
 Non Adrenergic, Non Cholinergic Inhibitory
Neurones (NANC)
Chemical transmitters in ENS - NANC
Non Adrenergic and Non
Cholinergic Peptidergic Inhibitory
System (NANC)
Controlled predominantly by intra
mural Cholinergics
In the rectum also stimulated by Para
sympathetics
Explains failure of pharmacological
interventions
Discerned by Electron Microscopy
Chemical transmitters in ENS - NO
NO is lipophilic and similar to
Endothelium derived Relaxing
Factor (EDRF)
NO is produced by NANC
NO is synthesised by NO
Synthase
This requires Nicotianmide
Adenine Di nucleotide
Phosphate Diaphosphorase
(NADPH) and Calcium Slide No: 20
Hirschsprung's Disease - Dept. of Paed. Surgery, Madurai Medical College.
Chemical transmitters in ENS - NO
 NADPH diaphospharase is similar in
staining to NO and can be assessed
 NOS & NADPH is used to assess NO
activity which is markedly decreased in
HD
 Enzymatic synthesis is prevented by
 L-NMMA (N Mono Methyl L Arginine)
 L- NNA (N Nitro L Arginine)
 L-NAME (Nitro L Arginine Methyl Esther )
 Absence of three types of ganglion cells
 Cholinergic neurons
 Inter Neurons
 NANC Neurons
 Cholinergic and adrenergic fibres proliferate in number and
size
 Absence of NANC Nerves
 Over production of Acetyl Choline
 This leads to Acetyl Cholinesterase excess measured in
 Biopsy
 Serum
 Erythrocytes Histological


Thickening of muscle layer
No peptidergic neurons
Findings in
 Fewer VIP and Substance P Hirschsprung’s


Higher Nor epinephrine content
Abnormal expression of NCAM
Disease
 Lack of Glial Fibrillary acid protein
Histology Of Hirschsprung's Disease
Mucosa
v Sub Mucosa
Thickened Nerve
(Note Absence of Ganglion)
Myentric Plexus
Classification of HD based on segment involved
Ultra short Segment
Short Segment
Recto Sigmoid (Classical)
Long Segment
Total Colonic Aganglionosis
Total Intestinal Aganglionosis
Zuelzer Wilson Syndrome
Neural Pathway
 Eye abnormalities
Abnormalities
 RET expressing gene and
sympathoblasts from somites 5 to 7
contribute to Cervical ganglion as well
as ganglion cells to distal colon
 Bowel
 Interruption of bowel by a vascular
accident as in atresia prevents distal
migration of the neural crest cells
through the Vagus.
Types of Type 1 Type 2 A Type 2 B
MEN Wermer Sipple Froboese
Organs
Syndrome
Para thyroids
Syndrome
Thyroid
Syndrome
Thyroid
Pancreas Para thyroids Adrenals
Pituitary Adrenals Mucosa -
Neuromas
Alimentary
Tracts
Musculo
Tumours Hyperplasia, Phaeochromocy Phaeochromocy
Skeletal
Adenoma, toma toma
Malignancy: Thyroid ( C Cell Thyroid ( C Cell
Gastrinoma Hyperplasia, Hyperplasia,
Insulinoma MTC) MTC)
Hyper Parathyroid Alimentary
Parathyroidism Hyperplasia Tract
ganglioneurom
VIP oma
atosis
Gene Chromosome 11
Lipoma Chromosome Chromosome
Locus 10 Mega
11 colon
(11q13)
(10q11.2) HD
(10q11.2)
Internal
Anal Sphincter
Achalasia Variants
Smooth Cell Abnormalities
Peri Nuclear Vacuolation
Central Core degeneration
Mega Cystitis Micro Colon

Intestinal Hypo Peristaltic
Syndrome (MMIHS)
Generally fatal
Variants
Intestinal Neuronal Dysplasia
 Type A
 Entire sympathetic system is aplastic or hypo
plastic
 Un modulated Para sympathetic leads to
contracted colon
 Type B
 Hypoplasia of sub mucosal and Myenteric Plexus
 Giant ganglion formation
 ACE activity is elevated
Chagas disease – Acquired Mega Colon
 Common in endemic areas
 Caused by Trypnasoma cruzi
 Heart and gut are primarily infected
 In the gut Esophagus and colon are
involved
 Focal inflammatory lesion leads to
pseudo cyst formation in the walls of the
GIT muscle
 Nerves are destroyed by Auto immune /
Neuro toxins
 ELISA
 Duhamel’s procedure
1 in 5000 Incidence of HD
 Male : Female = 4:1
 Male Preponderance decreases with increasing
length
 Sibling risks
 Males 4% and Females 1 %
 Long Segment - Higher transmission risk
 Brothers 24 % and sons 29 %
 Total Colonic Ganglionois – Familial
Incidence 15 to 21 %
 Total Intestinal Ganglionois – Familial
Incidence 50 %
 Delayed passage of Meconium
 Constipation
 Abdominal distension History
 Vomitting
 Rectal examination or wash outs cause
passage of Meconium and relief of
symptoms
 Occasionally Diarrhoea
 Toxic Mega colon
 Adults – 10 cms
 Children – 5 cms
 Gen. Examination:-
 Mental Retardation
 Cretinism
 Mongolism
 Failure to thrive
Neo nates Presentation
Intestinal obstruction
Infancy
Distension and constipation
Child Hood
Comfortable in spite of
massive distension
Faecaloma and constipation
CONSTIPATION IN
CHILDREN
DEFINITION:-
A. Frequency 48 hours
B. Consistency
CHRONIC HABITUAL
CONSTIPATION
 Over attended child consumes little
solid mostly milk
 More retention – Hard scyabalous
mass
 Reluctance to go to toilet – faces not
evacuated
 Straining – Anal fissure
 Intense pain reflex spasm.
IN
CHRONIC HABITUAL
CONSTIPATION
FEATURES CONSTIPATION HIRSCHSPRUNG
Chronology About 6 months Since birth
Abdominal Distension Not `so common Present
Soiling Common Not present – Super
continent
Diarrhoea No Diarrhoea Entero Colitis
Stool in ampulla Common Unusual
Defaecation Painful No pain
Passes stool in Semi standing Normal posture
posture
Obstructive Symptoms Rare Common
Stool retentive behaviour Common Rare
General Condition Good Emaciation
Anal Fissure Present Never
Per Rectal Examination Loaded from anal Empty
verge (refer next slide)
1. Not fully continent - Fill 1. Super Continent - No Soiling
and spill - Soiling present 2. Fissures absent
2. Fissures - Present 3. PR: Rectum empty, Wall
3. PR: Rectum dilated from collapsed, and griping of
anal verge, Wall dilated, finger present ( Faecal
and facaloma present from matter may be evacuated on
anal verge. Peri anal removing the finger ). If the
excoriations seen. HD is short tip of finger may
enter capricious rectum with
faecaloma
INVESTIGATIONS
PlainX rays
Barium Enema
Ano rectal Manometry
Rectal Biopsy
Fullthickness
Suction
Histo chemical studies

Investigations - Plain X ray - Skiagram
 Abdomen erect PA
 Distended bowel loops showing level of
obstruction approximately
 Multiple fluid levels in late cases
 Excludes perforation which is a rare
complication
 Left lateral Decubitus view with right
side raised is now recommended as a
alternative and is kinder to the baby
 Prone lateral view (10 minutes) with
buttocks elevated
Investigations - Plain X ray –
Differential Diagnosis
 Colonic Atresia
 Barium enema shows complete obstruction
 Distal Ileal Atresia
 Distal micro colon with proximal dilatation with widest
fluid level at obstruction
 Meconium Ileus
 Mottled granular appearance of gas trapped thick
Meconium
 Soap bubble appearance, Neuhasen’s sign
 Meconium plug syndrome
 Left colon syndrome
 Meconium plug and left colon syndrome may have
HD supervening them
 Hypothyroidism
Hypo
Thyroidism
Non
visualisation of
Lower Femoral
epiphysis
 In neonates rectal wash outs and even PR may
be kept to a minimum as the distended proximal
bowel may decompress with distortion of
transition zone leading to false results
 Soft rubber catheter (No Foley’s)
 Barium Sulphate suspension 50 % with warm
Normal Saline ( Water intoxication )
 Buttocks adequately strapped
 Slow injection till the proximal dilated bowel is
demonstrated and then stopped - Fluroscopy
 Rotation of the baby for better views
 Post evacuation films
 24 hours film
Investigations -
Barium enema
Barium Enema:
Swan Neck Sign
Transition zone at recto sigmoid
Total Colonic Ganglionosis
•Question Mark sign
•Blunting of splenic and hepatic
flexures
•Colon may not be
significantly narrowed
•Colon packed with faeces
•Because the proximal
obstruction pushes the
faecal matter passively
into the constricted segment
•Reflux of Barium into
grossly dilated ileum
is diagnostic
Investigation - Anorectal
Manometry
 Combination of pull through and three
point measurement
 Poly Vinyl feeding tubes closed at the
tip with a lateral opening 3 to 5 cms
from the tip are usually used as
pressure receivers
 Another method is pressure
Vectography
Abnormally increased contraction of the upper anal canal with rectal
distension and absence of the relaxation curve in HD
Normal Hirschsprung’s
Investigation
Anorectal Manometry
Ano Rectal Pressure profile (ARPP)
Normal:- Cathether withdrawn

shows a constant rise in pressure
with the maximum pressure
plateau at the sphincter
Normal:- Length of high pressure
zone ranges for 3 to 7 cms
Anorectal Manometry
Ano Rectal Pressure profile (ARPP) in HD
 Elevated tone with increased ARPPP or normal
values
 Absence of internal sphincter relaxation is
pathognomic of HD – Demonstrated by
distending the rectum with saline
 Multi segmental, in coordinated, irregular mass
contractions
 The absence of irregular contractions on
withdrawal – Break off point - helps in mapping
the extent of HD
Investigations - Histological
Methods
Haematoxylin Eosin
Enzyme histo chemical staining
 Rapid Acetyl cholinesterase reaction
(AchE)
 Lactic Dehydrogenase reaction (LDH)
 Succinic Dehydrogenase reaction (SDH)
 Alpha Naphthyl Esterase (ANE)
Findings
Absence of ganglion cells in the sub
mucosa and Myentric plexus
Increase in Acetyl Choline Esterase
activity (AchE) in the Para
sympathetic Nerve fibres of lamina
propria, Mucosa, Muscularis
Mucosa and circular muscle
Investigations
Immuno Histo chemical studies
Neuronal markers
 Neuron Specific Enolase  Tyrosine hydroxylase
(NSE) Dopamine Beta hydroxylase
 Protein Gene product (PGP)  Vaso active Intestinal poly
 Neuro Peptide Y (NPY) peptide (VIP)
 Neuro Filament Protein (NFP) Peptide HI ( (PHI)
 S 100 Proteins  Substance P (SP)
 Neuronal Micro Tubule  Enkephalin
Associated Protein (MAP)  Gastrin releasing peptide
 Synaptophysin Protein (GRP)
 Glial Fibrillary Acidic Protein  Calcitonin gene related
 Chromogranins peptide (CGRP)
 Galanin
Neuronal Markers for ENS
NADPH Diaphophorase
Neural Cell Adhesion
Molecule (NCAM)
Nerve Growth Factor
Receptor (NGF)
Investigations – Electron
Microscopy
 Confirms Microscopical features
 Histopathology of aganglionic intestine is
not exclusively confined to nervous
tissue
 HD includes also disorders in connective
tissue components evidenced by
increased deposition of collagen
 Pathology may not be entirely due to
Neural crest cells but may also include
micro environmental abnormalities
intrinsic to the colonic wall
 Full thickness
 While this may give adequate tissue to the
pathologist the risk of perforation of colon does
exist
 Fibrosis may hinder future pull through surgeries
 Mucosal Biopsy
 Noblett’s
Biopsy
forceps with three suction cups to take
biopsy at three levels
 First biopsy level is 3 cms above dentate line
 (At the dentate line the columnar mucosa gets
converted to squamous, & the ganglion cells would be
absent normally)
 The second biopsy would represent the
transition zone
 The Third biopsy should represent the proximal
dilated normal colon
 Conventional
 Aims at removal of diseased segment partially or
in Toto in three staged procedures
 Colostomy, Definitive procedure, Colostomy
closure
Single staged procedures
Duhamel, Swenson, Soave
Two stage procedures – Transition
zone colostomy
POOP
 Singlestage
 No LaprotomyTreatment
 Endo anal
Perineal One Stage Operative Pull Through - Department of Paediatric Surgery Slide No: 56
First Stage in conventional treatment of HD
Colostomy
Second Stage in conventional treatment of HD
Swenson’s Duhamel’s
Recto Sigmoidectomy Retro Rectal Pull Through
Excision of afflicted
segment in Toto or
partially by
Laprotomy
Soave’s
Endo Rectal Pull Through
Third Stage in conventional treatment of HD
Colostomy
Closure
Transition zone colostomy
 Advantages:
 Number of Surgery reduced to two
 Maximum amount of colon for absorption
 Assures colostomy is in normal functioning colon
 Pelvic Colostomy is preferable to Transverse
colostomy with its due morbidity
 Disadvantages
 Does away with the benefits of protective colostomy
during the definitive procedure
 Length of colon mobilized and removed may be
longer as it includes the colostomy and hence the
pull through would include a longer length of gap to
be bridged
Surgical procedures available for HD
 Biopsy – Rectal, Mucosal
 Colostomy, Ileostomy
 Swenson’s
 Duhamel
 Soave
 POOP
 Rehbein, State – Largely given up
 Ultra short: Sphincterotomy
 Short: Extended Myomectomy
 Total Colonic Ganglionosis
 Martin Duhamel's – extended side to side
 Kimura Stringel
 Extended Myotomy and Myomectomy
Pre operative preparation
 Rectal irrigation twice a day for 3 days or longer
 Rectal irrigation fluid:-
 Antibiotics and Metronidazole – Cipro, Neomycin,
 Phosphatic Saline Enema
 Always saline is used as dilutant
 Evacuation Enema is alternated
 Oral Ante grade Enemas - Peglyte
 Liquid diet for 3 days
 Injection Vitamin K for three days
 Now TPN is being recommended with Nil oral
 Oral antibiotics
 Short pre operative
 Usually it is started three days pre operatively
 IV Antibiotics – One hour before Surgery
 Ryle’s tube from previous night
 Electrolyte estimations before surgery
All procedures are preceded by full
rectal manual dilatation which in
the author’s view off sets the most
common post operative
complication in the long term –
Constipation which is claimed to
be due to:-
Anal Sphincter Achalasia
 Functional
 Myogenic
Swenson’s
Recto Sigmoidectomy
 Operative steps
Access to abdomen and pelvis by lithotomy and appropriate
draping - Trendelenburg position with arms in hyper abduction
Catheterisation with Foley’s, Infant feeding tubes
If colostomy is transitional it is undone and the proximal end
closed with purse string and the surgery proceeds
•Incision:
Hockey stick, Left Para Median, Pffannenstiel
After opening the abdomen the bladder is kept out of the
field by retractors, (Denise Brown self retracting), and stay
Ureter
Diagnosis is confirmed. Ureter, vas, Gonadal

vessels are identified and safe guarded. Arterial
arcade is studied to plan ligation of vessels
Marginal Artery (Drummond Artery) is preserved.
Marker stitch is applied at proposed site of Anastomosis well above
transition zone for identification
(Long strand for anti mesenteric and short strand for mesenteric border)
This besides ensuring adequate resection would also prevent rotation
If facilities are available a frozen section biopsy is done to confirm if
ganglion is present in the segment to be brought down
Peritoneum is mobilized on the lateral side of colon by division of the
peritoneum picked up by straight artery forceps. Ureter, vas, Gonadal
vessels are identified and safe guarded. Mobilisation of the peritoneum of
the descending colon in the Para rectal space area is done adequately
and can extend up to the splenic flexure. A similar procedure is done on
the medial side. The two peritoneal incisions are joined anteriorally, at
the level of peritoneal reflection.
The peritoneum of the Meso colon is dissected exposing the arterial
arcade. The arteries supplying the HD afflicted colon are ligated and
divided. The art. arcade supplying the colon to be preserved is left alone.
The art. which hinder mobilisation to the site of Anastomosis are ligated
without jeopardizing the blood supply.
Ureter
Sacrum
First, the retro rectal space has to be mobilised and this is the easiest.
Ligation of the superior Haemorrhoidal Artery and vein may aid the
reduction of blood loss. Care is taken to stick close to the bowel as the
pre sacral plexus of veins can cause torrential haemorrhage. The
mobilisation is done close to the bowel to avoid injury to adjacent pelvic
Nerves
Pack
The lateral sides are then mobilised. The Middle Rectal vessels may
come in the way and may require ligation. Dissection is kept close to the
bowel muscular wall and all vessels are electro coagulated under direct
vision. Usually the bleeding during this dissection stops with packing
and patient waiting as shown in the diagram.
External
Iliac Ureter
Vessels
Vas
Gonadal
Vessels
The anterior dissection is done in the very end. Bladder is retracted

anteriorally and the rectum posteriorally, and this opens up the Recto
Vesical pouch. Dissection proceeds close to the bowel wall. Ureter, Vas
are guarded. The extent of dissection is confirmed by one of the double
gloved finger on the ano cutaneous junction on the exterior and the end
of the dissection on the interior. Only the skin should be felt in between.
Anterior dissection can be stopped short by about 1 cm.
Surgery then proceeds to the perineum. This can be left to the assistant
to expedite the procedure. The rectum is intussuscepted by Allis forceps
applied sequentially and spirally with minimal traction. Usually this
should be effortless if the mobilisation is adequate.
Intussuception of the bowel proceeds proximally still the bowel is everted
for about 5 cms.
The bowel has been everted for 5 cms and it is painted with
Betadine.
The dentate line is identified and incision is made on the anterior wall 1 cm from
the anal verge proximally extending half way through the circumference. (If the
proximal bowel is too large and cumbersome to be pulled through the small
opening it may be divided at the marker stitches in the abdomen and the proximal
segment pulled through, but this would entail opening the contaminated bowel in
the abdomen. We prefer to open the bowel in the perineum and opening in the
abdomen is only resorted to when it is not possible.) Stay stitches at the sides of
this incision help in delineating the tissues during the Anastomosis.
Dentate line
The bowel which is inside the everted, intussuscepted colon is mobilized through
the incision and pulled out through the ant. incision. The pulled through bowel is
cut (Any bleeding from the cut edges is coagulated and this augurs that the
mobilised segment has its blood supply intact), and the proximal segment pulled
again until the marker stitches are visualized. Torsion is ruled out by proper
alignment of the marker stitches. The Sero muscular layer of suturing is done
between the inner side of the everted cuff and the outer side of the pulled through
colon just .5 cms above the proposed site of section.
The ant. incision of
the outer layer of the
everted rectum is cut
through all around.
Already the sero
muscular layer of the
Perineal Anastomosis
has been completed
at this stage. We
usually prefer a 2
layer interrupted,
suturing technique
with silk. Vicryl is the
now recommended
material. Stay
stitches at 12, 3, 6,
and 9 ‘O’clock
position may help in
proper alignment
especially when there
is bowel wall
disparity.
The two cut edges of the everted Ano Rectal cuff and the pulled through
bowel are sutured with through and through interrupted stitches all
around with Vicryl or silk as in the diagram.
Disparity may be met with because of the size of the proximal dilated
bowel. This is corrected during the suturing if minimal and also by
making the cut in the Ano rectum oblique. The Anastomosis is
completed meticulously and then returned to the abdomen.
Laparoscopic
 Younger patient’s are not catheterised
 Crede’s Manoeuvre is used
 Three or four Trocar sites
3 m.m. or 5 m.m. 30 degree scopes
 Scope placed just below liver edge and the right
of the falciform ligament
 Supra pubic Trocar to provide pelvic retraction
and hold the colon
 Other two Trocars are placed lateral to the rectus
at level of umbilicus
 Leak Complications
 Constipation
 Bowel Control
 Soiling
 Entero Colitis
 Rectal Stricture
 Fistulae
 Association of Down’s has totally
unsatisfactory results
Enterocolitis
 Faecal stasis and mechanical dilatation
 Infectious aetiology
 Loss of mucosal defense mechanism
 Increased prostaglandin activity
 Alteration in Mucin content
 Alterations in Neuro Endocrine Cell
population
Duhamel’s Procedure
Retro rectal and trans anal pull
through
Advantages:
No anterior dissection
Chances of lesser injury to Posterior vesical plexus
Hence theoretically the incontinence and impotence should be lesser
Relatively safer procedure
Disadvantages:
The anterior rectum is left behind
Morbidity of the clamps in the post operative period if used
Residual pouch irrespective of level of Ana. could lead to problems
Stages:
Stages: First: Closure of rectum.
Second: Preparation of retro rectal space
Third: Endo anal incision
Fourth: Retro Rectal pull through
Duhamel’s Procedure
Retro rectal and trans anal pull
through
The initial steps of draping, skin incision, mobilisation of sigmoid, and

retro rectal mobilisation are similar to Swenson, but that the arterial
supply to the Ano rectum to be left behind is preserved. (Please refer to
Slide: 65 to 72). First and second stage have already been discussed in
Swenson’s
Opening of the meso rectum provides access to the retro cellular space.
The dissection is kept close to the rectum fully preserving the sacro
genito pubic laminae which guards the pre sacral plexus of veins. A long
curved forceps with a small sponge is pushed into this space to abet on
the posterior wall of the anal canal
The sigmoid colon and descending colon has been mobilized, and the arterial supply of the
segment to be removed, ligated and divided. Proximal clamp marks the site to be brought
down & distal clamp the recto sigmoid (as in Hartmann’s close to the peritoneal reflection).
The sigmoid colon in between can be divided and surgery proceeded with. The proximal
colon is closed as a layer or with purse string leaving the ends of the suture long as a stay
which assists in the pull through.
A semi circular incision is made in the posterior half of the anal canal one
centimetre from the anal margin, exposing the sponge. The two angles
are marked by two stay sutures.
An Allis/Long Haemostat, is
locked on to the gauze and pulled
into the abdomen (similar to the
rail road technique) through the
retro rectal space created. In the
abdomen the beaks are opened
and locked on to the proximal
bowel to be brought out. Pulling
the Allis delivers the proximal
bowel through the opening in the
posterior wall of Anus which has
already been created.
The vitality of the pulled out bowel is checked and then divided flush to
the opening in the anal canal. The trimmed edge of the pulled through
bowel is sutured to the opening in the anus with interrupted silk
HD
Duhame
l’s
procedu
re
The suturing is completed all around and the septum in between the
pulled through bowel and excluded rectum marked with stay stitches.
HD
Duhame
l’s
procedu
re
Here we see the septum well delineated between the excluded rectum
anteriorally and the pulled through bowel posteriorally. The retractor and
catheter are seen in the anterior part of the excluded rectum.
Two Kocher’s clamps are inserted with one blade in the excluded rectum
and the other in the pulled through portion of the colon. The bases of the
clamps are held apart so that they lie at the lateral angles of the colonic
suture. Their points meet in an inverted V at the apex of the Rectal pouch
at the highest possible level.
The position of the clamps is checked by the asst. by direct intra
abdominal palpation and visualisation. The opening in the stump of the
Excluded rectum is closed in the abdomen by silk in 2 layers, forming the
pouch.
The Kocher’s forceps are locked depriving the septum in between of its blood
supply and allowing it to necrotize, effectively forming an Anastomosis. The two
Kocher’s are tied with gauze and allowed to protrude through the anus and
allowed to fall of naturally.
HD
Duhame
l’s
procedu
re
While the Kocher’s clamps serve admirably, they cause morbidity to the
patient who has the instrument locked in the anus and the legs tied
together for about a week. The Endo Linear stapler with cutter forms an
excellent alternative. This forms the Anastomosis and divides the
septum in between immediately completing the procedure on the table.
The only off set would be the recurring price.
Linear Stapler
with cutter
The Linear stapler shown with two layers of clips which are
stapled on either side of the groove in the centre. The central
groove is then cut with the device effectively completing the
procedure expeditiously.
HD
Duhame
l’s
procedu
re
Excised
Colon
 Modification’s of Duhamel’s:-
 1956 Duhamel - Ano Cutaneous Junction
 High incidence of anal incontinence and prolapse due to complete division of the internal
sphincter
 1959 Grob - 2 to 2.5 cms above the muco cutaneous junction
 High incidence of constipation with formation of faecaloma
 1960 Duhamel’s modification of Duhamel - 1 cms above the anal margin
1956 - Duhamel 1959 - Grob 1960 - Duhamel

 The protrusion of the handles of the clamps, occurrence
of stenosis, and the incomplete division of the septum
were circumvented by:-
 1964 – Zachary and Lister’s – O shaped clamp without
handles locked by a screw
 1966 – Talbert – Suturing and Stapling instrument –
Antero lateral portion of Rectal stump sutured to the
colon Modifications of
 1987 – Steichen - EEA instrument
Duhamel’s
 Martin and Caudill – End to side Anastomosis of the
Rectal stump and pulled through colon
 No blind rectal pouch
 Necessitates another Anastomosis
 1968 – Martin – Side to side Anastomosis between
normal Ileum and the aganglionic colon from the anus
to the splenic flexure with the hope that it would
recapture the absorptive properties of the left Colon in
TCA
Soave’s
Procedure
Extra Mucosal endo
rectal pull through
Operative steps
Soave’s Procedure
Extra Mucosal endo rectal pull through
 Indications
 Hirschsprung’s Disease
 Multiple Juvenile Polyposis
 Familial Polyposis
 Ulcerative Colitis
 History
 1955 – Romauldi proposed it
 1961 – Soave used it for ARM with fistula and HD
 1964 – Boley – Primary Anastomosis

 Dilatation precedes the procedure
 Two fore finger and opposite traction
 To facilitate this procedure a few add internal
myomectomy also, though not essential
 As the colon is not opened anywhere it is ideal
for single stage
 Technically easier below three months because
Endo rectal dissection is easier due the
absence of adhesions and inflammation
 Multiple biopsies is mandatory
 If possible Enzymo histo chemical techniques
are used
Procaine Hydrochloride 0.5 is injected between the layers, above the peritoneal
reflection. Longitudinal Sero muscular incision is made on the anterior wall.
Incision is widened with blunt dissection. The borders of the Sero muscular layer
are held apart with Allis.
The mucosal tube is now freed completely all around. It is progressively carried
downward, taking care not to cause any perforation, in the mucosa or Sero
muscular layer. A combination of blunt and sharp dissection is performed as
appropriate. Dissection is easier on the posterior surface than anteriorally. The
dissection is carried proximally to 1 cms above the dentate line. This is checked
by bi digital examination. Bleeding is arrested by electro coagulation and
packing.
Malecot’s catheter is introduced into the lumen of the rectal mucosal cylinder and
a strong ligature tied behind the head in the mucosal tube at the proximal end.
Gentle traction pulls out the mucosal tube and everts it causing intussusception.
The outer mucosal tube is cut 1cm from the Ano cutaneous junction. The inner
tube is pulled till the marker stitches in the Normal bowel are seen, and the bowel
is cut flush at this level.
Everted anal mucosa
Marker stitch
Full thickness bowel pulled through
Mucosal layer pulled through
Initially in Soave, Anastomosis was deferred to a later date as the bleeding in

between the cuffs had to be drained. But this lead to a boggy, infected stump
and the Anastomosis at a later date was difficult and fraught with complications.
As techniques improved the Anastomosis was done in the same sitting with a
sump drain in the plane between the cuffs.
Anastomosis is done in Boley’s modification in one layer or two layers with
interrupted silk or Vicryl, as a primary procedure. On completion of the
Anastomosis it recedes in to the colon. In the abdomen the Sero muscular cuff at
its upper end is sutured to the normal pulled through bowel but for the posterior
side , so as not to injure the mesentery with blood vessels. Opening between the
colon and peritoneum is closed to prevent herniation.
POOP
Primary
One stage
Orthograde
Perineal Pull Through
Plane of dissection in POOP
Mucosa
Dilated normal bowel Sero Muscular Layer
Peritoneum
Narrowed
Levator Ani
Affected
Bowel
•Total Colonic Aganglionosis
•Appendicectomy
•Ileostomy
Extended
Martin
Duhamel
Operation
Extended
Martin
Duhamel
Operation
(Side to side
Anastomosis
Completed)
Staged Kimura Stringel Operation – First Stage:
Ganglionated small intestine is patched to
Aganglionated bowel.
Staged Kimura Stringel Operation – Second Stage:
Right colon mesentery is divided and the composite
intestine that is vascularised by the small bowel
mesentery is prepared for pull through

Hirschsprung's Disease - Athigaman

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Hirschsprung's Disease - Congenital Mega Colon

Definition: HD is characterized by absence of ganglion

Auerbach’s Inter Myentric Plexus

Sub Mucosal Meissner’s Plexus

Posterior part of Rhombomere 8

Adjacent to Somites 4 and 5

 Mass movement occurring at long intervals

 Major determinant of continence at rest

 External sphincter – Voluntary

 Muscle mass complex

Mega Cystitis Micro Colon

Histo chemical studies

Normal:- Cathether withdrawn

 Succinic Dehydrogenase reaction (SDH)

 Alpha Naphthyl Esterase (ANE)

Diagnosis is confirmed. Ureter, vas, Gonadal

The anterior dissection is done in the very end. Bladder is retracted

The initial steps of draping, skin incision, mobilisation of sigmoid, and

1956 - Duhamel 1959 - Grob 1960 - Duhamel

 1964 – Boley – Primary Anastomosis

Full thickness bowel pulled through

Mucosal layer pulled through

Initially in Soave, Anastomosis was deferred to a later date as the bleeding in

Dilated normal bowel Sero Muscular Layer

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