Hirschsprung’s Disease - Athigaman

Hirschsprung's Disease - Congenital Mega Colon

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.

Normal Bowel

Transition Zone

HirschsprungDisease

Hirschsprung's Disease - Dept. of Paed. Surgery, Madurai Medical College. Slide No: 2

1886 - Hirschsprung gave detailed description of mega colon

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

History


Intrinsic and ExtrinsicParasympathetic

Vagus NerveEnhances peristaltic ActivityAuerbach’s Inter Myentric Plexus

SympatheticSplanchnic Nerves ending in Plexuses

Coeliac, Lumbar, Hypo gastricSub Mucosal Meissner’s Plexus

Enteric Nervous System


SourceCaudal Hind brainEnteric Neural CrestPosterior part of Rhombomere 8Adjacent to Somites 4 and 5

Development of Enteric Nervous System


MigrationPosterior 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

Development of Enteric Nervous System


Migration5th month – Mid gut6.5 month – Caecum8th month – Migration complete

Arrest of caudal migration leads to HD

Chronology of Development of Enteric Nervous System


Neurotrophic factorsGDNF – Glial cell line derived

Neuronotropic factorTransforming growth factor

(TGF - )Laminin – extra cellular matrix

present in basal lamina of smooth muscle, presence of which may inhibit migration by formation of neurons

Genetics of Enteric Nervous System


WardenburgSyndrome Pigmentation abnormalities

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


GenesC 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 13MASH 1 gene – CatecholaminergicDeletion Chromosome 2, 10, 13Partial Trisomy 11, 12Trisomy 21

Genetics of HD


Increased sibling history (4%)Unbalanced sex ratio (4:1)Associations

Down’sMEN 2Wardenburg SyndromeSmith Lemli Opitz SyndromeChromosomal deletions or

rearrangements in 10 or 13

Genetics of Hirschsprung Disease


MucosaEpithelium – Columnar & gobletLamina Propria

Sub MucosaMuscularis Mucosa

Muscular L. or Muscularis PropriaInner Circular muscleInter muscular spaceOuter Longitudinal Muscle layer

Serosa

Micro Anatomy of Bowel


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

Micro Anatomy – Intrinsic Nerves

33

NormalNeurological

InnervationOf Bowel


Colonic motilitySlow wave paces governing rhythmic

contractions 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 HirschsprungNormal Muscle behaviour is attributed to

the neural refluxNormal 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

capriciousClosure of anal canal

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

Continence


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 Contraction of abdominal muscles Relaxation of the striated anal muscles

Involuntary actions Peristaltic contractions – Mass Relaxation of internal sphincter

Defaecation


Acting on nerves Acetyl Choline Substance P

Acting on muscle ATP Vaso active Intestinal Peptide (VIP)

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)

Chemicaltransmitters

in ENS


Chemical transmitters in ENS - NANCNon Adrenergic and Non

Cholinergic Peptidergic Inhibitory System (NANC)Controlled predominantly by intra

mural CholinergicsIn the rectum also stimulated by Para

sympatheticsExplains failure of pharmacological

interventionsDiscerned by Electron Microscopy


Chemical transmitters in ENS - NONO is lipophilic and similar to

Endothelium derived Relaxing Factor (EDRF)

NO is produced by NANCNO is synthesised by NO

SynthaseThis requires Nicotianmide

Adenine Di nucleotide Phosphate Diaphosphorase (NADPH) and Calcium


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 byL-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

Thickening of muscle layer No peptidergic neurons Fewer VIP and Substance P Higher Nor epinephrine content Abnormal expression of NCAM Lack of Glial Fibrillary acid protein

Histological Findings inHirschsprung’sDisease

Histology Of Hirschsprung's Disease

v

Mucosa

Sub Mucosa

Myentric Plexus

Thickened Nerve (Note Absence of Ganglion)


Ultra short SegmentShort SegmentRecto Sigmoid (Classical)Long SegmentTotal Colonic AganglionosisTotal Intestinal Aganglionosis

Zuelzer Wilson Syndrome

Classification of HD based on segment involved


Eye abnormalitiesRET expressing gene and sympathoblasts

from somites 5 to 7 contribute to Cervical ganglion as well as ganglion cells to distal colon

BowelInterruption of bowel by a vascular

accident as in atresia prevents distal migration of the neural crest cells through the Vagus.

Neural PathwayAbnormalities

Types of MEN Type 1

Wermer Syndrome

Type 2 A

Sipple Syndrome

Type 2 B

Froboese Syndrome

Organs Para thyroids

Pancreas

Pituitary

Thyroid

Para thyroids

Adrenals

Thyroid

Adrenals

Mucosa - Neuromas

Alimentary Tracts

Musculo Skeletal

Tumours Hyperplasia, Adenoma, Malignancy:

Gastrinoma

Insulinoma

Hyper Parathyroidism

VIP oma

Lipoma

Phaeochromocytoma

Thyroid ( C Cell Hyperplasia, MTC)

Parathyroid Hyperplasia

Phaeochromocytoma

Thyroid ( C Cell Hyperplasia, MTC)

Alimentary Tract ganglioneuromatosis

Mega colon

HD

Gene Locus Chromosome 11

(11q13)

Chromosome 10

(10q11.2)

Chromosome 11

(10q11.2)


VariantsInternal Anal Sphincter

AchalasiaSmooth Cell Abnormalities

Peri Nuclear VacuolationCentral Core degenerationMega Cystitis Micro Colon

Intestinal Hypo Peristaltic Syndrome (MMIHS)

Generally fatal


VariantsIntestinal 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


Common in endemic areasCaused by Trypnasoma cruziHeart and gut are primarily infected In the gut Esophagus and colon are

involvedFocal inflammatory lesion leads to

pseudo cyst formation in the walls of the GIT muscle

Nerves are destroyed by Auto immune / Neuro toxins

ELISADuhamel’s procedure

Chagas disease – Acquired Mega Colon


1 in 5000Male : 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 %

Incidence of HD


HistoryDelayed passage of MeconiumConstipationAbdominal distension VomittingRectal examination or wash outs cause

passage of Meconium and relief of symptoms

Occasionally DiarrhoeaToxic Mega colon

Adults – 10 cms Children – 5 cms

Gen. Examination:-Mental RetardationCretinismMongolismFailure to thrive


PresentationNeo nates

Intestinal obstructionInfancy

Distension and constipationChild Hood

Comfortable in spite of massive distension

Faecaloma and constipation

CONSTIPATION IN CHILDRENDEFINITION:-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 fissureIntense 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 posture

Normal posture

Obstructive Symptoms Rare Common

Stool retentive behaviour Common Rare

General Condition Good Emaciation

Anal Fissure Present Never

Per Rectal Examination Loaded from anal verge

Empty

(refer next slide)

1. Super Continent - No Soiling

2. Fissures absent

3. PR: Rectum empty, Wall collapsed, and griping of finger present ( Faecal matter may be evacuated on removing the finger ). If the HD is short tip of finger may enter capricious rectum with faecaloma

1. Not fully continent - Fill and spill - Soiling present

2. Fissures - Present

3. PR: Rectum dilated from anal verge, Wall dilated, and facaloma present from anal verge. Peri anal excoriations seen.

INVESTIGATIONSPlain X raysBarium EnemaAno rectal ManometryRectal Biopsy

Full thicknessSuction

Histo chemical studies


Investigations - Plain X ray - SkiagramAbdomen erect PA

Distended bowel loops showing level of obstruction approximately

Multiple fluid levels in late casesExcludes perforation which is a rare

complicationLeft 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 SignTransition zone at recto sigmoid

Total Colonic Ganglionosis•Question Mark sign•Blunting of splenic and hepatic flexures•Colon may not besignificantly narrowed•Colon packed with faeces•Because the proximalobstruction pushes thefaecal matter passivelyinto the constricted segment•Reflux of Barium intogrossly dilated ileumis 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


InvestigationAnorectal 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 ManometryAno 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

MethodsHaematoxylin EosinEnzyme histo chemical staining

Rapid Acetyl cholinesterase reaction (AchE)

Lactic Dehydrogenase reaction (LDH)Succinic Dehydrogenase reaction (SDH)Alpha Naphthyl Esterase (ANE)


Investigations - HistologicalFindings

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


InvestigationsImmuno Histo chemical studies

Neuronal markers Neuron Specific Enolase

(NSE) Protein Gene product (PGP) Neuro Peptide Y (NPY) Neuro Filament Protein (NFP) S 100 Proteins Neuronal Micro Tubule

Associated Protein (MAP) Synaptophysin Protein Glial Fibrillary Acidic Protein Chromogranins

Tyrosine hydroxylase Dopamine Beta hydroxylase

Vaso active Intestinal poly peptide (VIP)

Peptide HI ( (PHI) Substance P (SP) Enkephalin Gastrin releasing peptide (GRP) Calcitonin gene related peptide

(CGRP) Galanin


Investigations - HistologicalNeuronal Markers for ENS

NADPH DiaphophoraseNeural Cell Adhesion

Molecule (NCAM)Nerve Growth Factor

Receptor (NGF)


Investigations – Electron Microscopy

Confirms Microscopical featuresHistopathology 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 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

Biopsy

Perineal One Stage Operative Pull Through - Department of Paediatric Surgery

Slide No: 56

Conventional Aims at removal of diseased segment partially or

in Toto in three staged procedures Colostomy, Definitive procedure, Colostomy

closureSingle staged procedures

Duhamel, Swenson, SoaveTwo stage procedures – Transition

zone colostomyPOOP

Single stage No Laprotomy Endo anal

Treatment

Colostomy

First Stage in conventional treatment of HD

Excision of afflicted segment in Toto or

partially by Laprotomy

Duhamel’sRetro Rectal Pull Through

Swenson’sRecto Sigmoidectomy

Soave’sEndo Rectal Pull Through

Second Stage in conventional treatment of HD

Colostomy Closure

Third Stage in conventional treatment of HD


Transition zone colostomyAdvantages:

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 morbidityDisadvantages

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

FunctionalMyogenic

Swenson’sRecto Sigmoidectomy

Operative steps

Access to abdomen and pelvis by lithotomy and appropriate draping - Trendelenburg position with arms in hyper abductionCatheterisation with Foley’s, Infant feeding tubesIf colostomy is transitional it is undone and the proximal end closed with purse string and the surgery proceeds

•Incision:Hockey stick, Left Para Median, PffannenstielAfter opening the abdomen the bladder is kept out of the field by retractors, (Denise Brown self retracting), and stay sutures

Ureter

Diagnosis is confirmed. Ureter, vas, Gonadal vessels are identified and safe guarded. Arterial arcade is studied to plan ligation of vesselsMarginal 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 rotationIf 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.

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

Sacrum

Ureter

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.

Pack

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.

Ureter

Vas

ExternalIliacVessels

Gonadal Vessels

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.


LaparoscopicYounger patient’s are not catheterisedCrede’s Manoeuvre is usedThree 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


ComplicationsLeakConstipation Bowel Control

SoilingEntero ColitisRectal StrictureFistulaeAssociation of Down’s has totally

unsatisfactory results


EnterocolitisFaecal stasis and mechanical dilatation Infectious aetiologyLoss of mucosal defense mechanism Increased prostaglandin activityAlteration in Mucin contentAlterations in Neuro Endocrine Cell

population

Duhamel’s ProcedureRetro rectal and trans anal pull

through

Advantages:No anterior dissection

Chances of lesser injury to Posterior vesical plexusHence 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 usedResidual pouch irrespective of level of Ana. could lead to problems

Stages:Stages: First: Closure of rectum.

Second: Preparation of retro rectal spaceThird: Endo anal incision

Fourth: Retro Rectal pull through

Duhamel’s ProcedureRetro 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

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The suturing is completed all around and the septum in between the pulled through bowel and excluded rectum marked with stay stitches.

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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.

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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.

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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

1987 – Steichen - EEA instrument 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

Modifications of Duhamel’s

Soave’s ProcedureExtra Mucosal endo rectal pull throughOperative steps

Soave’s ProcedureExtra 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.

Mucosal layer pulled through

Full thickness bowel pulled through

Marker stitch

Everted anal mucosa

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.


POOPPrimaryOne stageOrthogradePerineal Pull Through

Mucosa

Sero Muscular LayerDilated normal bowel

NarrowedAffectedBowel

Levator Ani

Plane of dissection in POOP

Peritoneum

•Total Colonic Aganglionosis•Appendicectomy•Ileostomy

ExtendedMartinDuhamelOperation

ExtendedMartinDuhamelOperation(Side to sideAnastomosisCompleted)

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

Documents

Hirschsprung’s Disease - Athigaman