ILA - Hirschsprungs Disease

8/13/2019 ILA - Hirschsprungs Disease

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Hirschsprung's Disease - Congenital Mega Colon

Normal Bowel

Transition Zone

Hirschsprung

Disease



Hirschsprung Disease

The primary defect is the absence of the

intramural ganglion cells of the

submucosal and myenteric plexuses




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.



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




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.



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



Visible transitional zone



for Hirschsprung disease. Note the dilated, proximal portion separated

from the constricted distal portion by a transition zone.




Association with inheritance in

chromosome 10 in some patients.

RET- protooncogene Autosomal dominant in totally

agangloinic bowel.

Common in Down syndrome



Histology Of Hirschsprung's Disease

v

Mucosa

Sub Mucosa

Myentric Plexus

Thickened Nerve(Note Absence of Ganglion)



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.



History

Delayed passage of Meconium

Constipation

Abdominal distension

Rectal examination or wash outs causepassage of Meconium and relief of

symptomsOccasionally Diarrhoea



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.



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.





PresentationNeo nates

Intestinal obstructionInfancy

Distension and constipation

Child Hood

Comfortable in spite of

massive distension

Faecaloma and constipation



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.



INVESTIGATIONSPlain X raysBarium Enema

Ano rectal ManometryRectal Biopsy

Full thicknessSuctio







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





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)



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



propria which, in the absence of ganglion cells, is diagnostic of

Hirschsprung disease.



Complicatons

Enterocolitis,



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



SURGERY

principle

Excision of aganglionic

segment.Restoration of bowel



Technique

Swenson

DuhamelSoave



staging

3 stage

2 stage1 stage



Approach

Abdomoinoperineal

Laparoscopico-perineal

Purely transanal



How many

stages?

one

Approach? Transanal

Technique? soave



Most recent !

One stage

TransanalSoave .



Surgery

Swenson - proctolectomy

Duhamel --posterior pull-through withside to side anastomosis to

angangolionic rectum

Soave -- pull ganglionated bowel

through sleeve of rectum







Fi t St i ti l t t t f HD



Colostomy

First Stage in conventional treatment of HD

S d St i ti l t t t f HD



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



Colostomy

Closure

Third Stage in conventional treatment of HD



TAPTOne stage

TRANSANAL

Pull Through



Mucosa

Sero Muscular LayerDilated normal bowel

Narrowed

Affected

Bowel

Levator Ani

Plane of dissection in POOP

Peritoneum





The retractor

Placement of the traction



Placement of the traction

stitches



Clear Transitional Zone

S t R t d



Segment Resected

23 cm to 53 cm (38cm)



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ILA - Hirschsprungs Disease

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