Transcript Document 7256033

Hirschsprung’s disease,
the past and the present
Dr.Dávidovics Sándor
Petz A.County Teaching Hospital
Department of Paediatric Surgery
Győr
Hirschsprung’s disease
 One
in 5000 live births
 Male-to-female ratio of 4:1
 In 90 % are mature newborns
 In 1-6 % are familial
 In 10%-30% are associated anomalies
Hirschsprung’s disease
 To
rectosigmoid in 75%
 Short segment (rectum)
 Ultra short (part of the rectum)
 Long segment
 Total aganglionosis (large intestine,
sometimes a part of small intestine) up to
10%
 Segmental
Hirschsprung’s disease
Embriology
 Migration
of neuroblasts on the 5-7 g.week
 On the 12. g.week they reach the rectum
 Myenteric plexus
 Submucous plexus
 Ganglions
 The process of maturation continues after
the birth
Hirschsprung’s disease
 Innervation
 adrenergic
 cholinergic
 Nonadrenergic and noncholinergic
-pepdidergic
-nitrergic
-nerve-supporting cells
-interstitial cells in Cajal
Hirschsprung’s disease
Histopathology



Absence of ganglionic cells
Hypertrophic cholinergic nerve trunks
Limited number of adrenergic fiber
Hirschsprung’s disease
Clinical symptoms
 The
disease can considered to be
incomplete intestinal obstruction
 The lenght of the aganglionic segment is
variable
 The symptoms are variable too
 The symtoms appears in different ages
Hirschsprung’s disease
Symptoms in newborn age
 Fail
to pass meconium (in 24 hours of life)
 Abdominal distension, but the abdomen is
palpable
 Vomiting
 The rectal tube can’t be put easily
 After irrigation the signs and symptoms
return again in a few days
Hirschsprung’s disease
Symptoms in newborn age(enterocolitis)







Life-threatening condition
Diarrhea: it can be an early sign
Toxic megacolon
Abdominal distension
Bile-stained vomiting
Fiver and signs of dehydration
Rectal tube:explosive expulsion of gas and foulsmelling stools
Hirschsprung’s disease
Symptoms in infants
 Constipation
 Meteorism
 Palpable
faecaloma
 Sometimes putrescent diarrhea
 Ulceration, bleeding
 Hypoproteinaemia, anaemia
 Electrolyt disorders
Hirschsprung’s disease
Symptoms in childhood
Gracile limbs
 Dilated drumlike belly
 Long history of constipation
 Defecation in 7-10 days
 Multiple fecal masses
 The stimulus of defecation is missing
 Rectum is empty and narrow

Hirschsprung’s disease
Hirschsprung’s disease
Examinations
 Plain
abdominal radiographs
 Contrast enema
 Anorectal manometry
 Rectal biopsy,histopathology
Hirschsprung’s disease


Plain X ray: air/fluid levels,free air
Contrast enema:
-Narrow distal segment
-Funnel-shaped transition zone
-Dilation of proximal colon
-After 24-48 hours the contrast material
is in the bowel
-Mucosal irregularity (enterocolitis)
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Anorectal manometry
 Anorectal
pressure is elevated
 No relaxation of the internal sphincter
 It can be put through safely in newborn
age as well
Hirschsprung’s disease
Hirschsprung’s disease
Biopsy and Histology
 Full-thickness
strip-biopsy
 Suction biopsy
Hirschsprung’s disease
Hirschsprung’s disease
Nitrergic innervation pattern
(light microscopy, x100)
Normal colon
Hirschsprung’s disease
Nitrergic innervation pattern
(light microscopy, x100)
Hirschsprung's disease
Total intestinal aganglionosis
Hirschsprung’s disease
Treatment:
 Decompression:
introduce a rectal tube
and irrigation
 Colostomy
 Definitive procedures
 Closing of the stoma
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Treatment
Transanal Endorectal Pull-Trough
 It can be performed safely in infant as well
 Generally one-stage surgery
 No abdominal phase
 The anastomosis is happening in a „safe”
place at the pectinate line
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Hirschsprung’s disease
Long-term outcome
 If started in time, under adequate
treatment is good.
Summary
Past






Operation in 2 or 3
steps
Pull-through on the
afunctional bowel
Strip-biopsy
Stoma-wearing
Nursing,dilatations
Higher possibility of
complcations
Present

Operation in 1 step
- Pull-through the
functional gut
 Suction biopsy
 No stoma
- No dilatation
 Smaller possibility of
complications
Summary



Past
Long hospitalisation
High input
/operations,medicines/
 Free stoma tools
 Travelling support
 Long-term financial
support of goverment
 Harder social integration
/kidergarten,school/

Present


Short hospitalisation
Small input



No stoma
Less controll examination
The support can be
extinguished earlier
Easy social integration

Thank you for your
attention !