ila - hirschsprungs disease
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8/13/2019 ILA - Hirschsprungs Disease
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Hirschsprung's Disease - Congenital Mega Colon
Normal Bowel
Transition Zone
Hirschsprung
Disease
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Hirschsprung Disease
The primary defect is the absence of the
intramural ganglion cells of the
submucosal and myenteric plexuses
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Hirschsprung Disease
This absence of normal
parasympathatic innervation prevents
gut peristalsis, leading to functional
constipation.
The proximal colon hypertrophied by
trying to overcome functional
obstruction.
Transitional zone exists between normal
and abnormal aganglionic intestine.
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Hirschprung’s Disease
More than 50 years old since the
discovery of the cause and the
treatment for Hirschprung disease.
Incidence : 1 in 5000 live birth
80 % of patients are males
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Hirschsprung Disease
Aganglionosis is restricted to the rectum
and sigmoid colon in 75% of patients .
extends more
proximally in 15-20% . and affects the entire colon and a
variable length of ileum in 8%. Rarely,
ganglion cells are absent from
most ofthe gastrointestinal tract.
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Ultra short Segment
Short Segment
Recto Sigmoid (Classical)
Long Segment
Total Colonic Aganglionosis
Total Intestinal Aganglionosis
Zuelzer Wilson Syndrome
Classification of HD based on segment involved
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Visible transitional zone
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for Hirschsprung disease. Note the dilated, proximal portion separated
from the constricted distal portion by a transition zone.
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Hirschsprung Disease
Association with inheritance in
chromosome 10 in some patients.
RET- protooncogene Autosomal dominant in totally
agangloinic bowel.
Common in Down syndrome
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Histology Of Hirschsprung's Disease
v
Mucosa
Sub Mucosa
Myentric Plexus
Thickened Nerve(Note Absence of Ganglion)
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Clinical diagnosis
Only 15% are diagnosed in the first
month of life, but two thirds are in the
first 3 months. Cases beyond 5 years of
age usually have ultra- short segment
disease.
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History
Delayed passage of Meconium
Constipation
Abdominal distension
Rectal examination or wash outs causepassage of Meconium and relief of
symptomsOccasionally Diarrhoea
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Clinical diagnosis
. Symptoms within the first week of life
include failure to pass meconium within
48 hours, reluctance to feed, bilious
vomiting, abdominal distention, often
have a worried or frowning appearance.
They may be confused with obstruction
from meconium ileus, ileal atresia.
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Clinical diagnosis
. Explosive liquid stools, fever, and
severe prostration are indicative of
enterocolitis.
Enterocolitis is rare (10%) in the first
month but rises to 33% in the second
and third months. Recall that diarrhea
may be a late sign.
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PresentationNeo nates
Intestinal obstructionInfancy
Distension and constipation
Child Hood
Comfortable in spite of
massive distension
Faecaloma and constipation
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1. Super Continent - No Soiling
2. Fissures absent
3. PR: Rectum empty, Wallcollapsed, and griping of
finger present ( Faecalmatter may be evacuated onremoving the finger ). If theHD is short tip of finger mayenter capricious rectum with
faecaloma
1. Not fully continent - Filland spill - Soiling present
2. Fissures - Present
3. PR: Rectum dilated from
anal verge, Wall dilated,and facaloma present fromanal verge. Peri analexcoriations seen.
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INVESTIGATIONSPlain X raysBarium Enema
Ano rectal ManometryRectal Biopsy
Full thicknessSuctio
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Anorectal Manometry Ano Rectal Pressure profile (ARPP) in HD
Elevated tone with increased ARPPP or normalvalues
Absence of internal sphincter relaxation is
pathognomic of HD –
Demonstrated bydistending the rectum with saline
Multi segmental, in coordinated, irregular masscontractions
The absence of irregular contractions onwithdrawal – Break off point - helps in mappingthe extent of HD
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Investigations - Histological
MethodsHaematoxylin Eosin
Enzyme histo chemical staining
Rapid Acetyl cholinesterase reaction
(AchE)
Lactic Dehydrogenase reaction (LDH)
Succinic Dehydrogenase reaction (SDH)
Alpha Naphthyl Esterase (ANE)
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Investigations - Histological
Findings Absence of ganglion cells in the sub
mucosa and Myentric plexus
Increase in Acetyl Choline Esteraseactivity (AchE) in the Para
sympathetic Nerve fibres of laminapropria, Mucosa, MuscularisMucosa and circular muscle
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propria which, in the absence of ganglion cells, is diagnostic of
Hirschsprung disease.
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Complicatons
Enterocolitis,
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Enterocolitis
Faecal stasis and mechanical dilatation
Infectious aetiology
Loss of mucosal defense mechanism Increased prostaglandin activity
Alteration in Mucin content
Alterations in Neuro Endocrine Cellpopulation
SURGERY
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SURGERY
principle
Excision of aganglionic
segment.Restoration of bowel
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Technique
Swenson
DuhamelSoave
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staging
3 stage
2 stage1 stage
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Approach
Abdomoinoperineal
Laparoscopico-perineal
Purely transanal
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How many
stages?
one
Approach? Transanal
Technique? soave
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Most recent !
One stage
TransanalSoave .
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Surgery
Swenson - proctolectomy
Duhamel --posterior pull-through withside to side anastomosis to
angangolionic rectum
Soave -- pull ganglionated bowel
through sleeve of rectum
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Fi t St i ti l t t t f HD
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Colostomy
First Stage in conventional treatment of HD
S d St i ti l t t t f HD
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Excision of afflicted
segment in Toto or
partially by
Laprotomy
Duhamel s
Retro Rectal Pull ThroughSwenson s
Recto Sigmoidectomy
Soave s
Endo Rectal Pull Through
Second Stage in conventional treatment of HD
Thi d St i ti l t t t f HD
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Colostomy
Closure
Third Stage in conventional treatment of HD
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TAPTOne stage
TRANSANAL
Pull Through
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Mucosa
Sero Muscular LayerDilated normal bowel
Narrowed
Affected
Bowel
Levator Ani
Plane of dissection in POOP
Peritoneum
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The retractor
Placement of the traction
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Placement of the traction
stitches
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Clear Transitional Zone
S t R t d
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Segment Resected
23 cm to 53 cm (38cm)
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