CURRENT DIAGNOSIS AND

MANAGEMENT
OF HIRSCHSPRUNG ‘S DISEASE

Dikki Drajat Kusmayadi

Div. Of Pediatric Surgery
Dr. Hasan Sadikin Hospital -
Faculty of medicine Padjadjaran
University
 Introduction
HD : absent of myenteric and
submucosal ganglion
Prevalence in Asia : 1,4 per 5000 live-
birth
Hasan Sadikin Hospital Bandung : 60
cases per year
RSCM Jakarta :20-40 cases per year
Epidemiology

Prevalence in Asia : 1,4 per 5000 live-

birth
RSHS-Bandung : 60 cases/year
RSCM Jakarta :
 DIAGNOSIS
 Clinical symptoms  Contrast
enema

 Anorectal
manometry  Histopathologic
findings
 DIAGNOSIS
Contrast Enema
 DIAGNOSIS
Histopathologic examination
H&E staining, AChE

Imunohistochemistry
S100
Calretinin
H&E staining: Ganglion (-)
Nerve fiber hypertrophy

20x 100x

200x
IMUNOHISTOCHEMISTRY STAINING

S100
Calretinin :
• It can be applied on parafin embedded tissue
• Simple staining patern
• Simple interpretation : Negative and positive
• Cost- effective
IHC S100 : Positif, >40 mikrometer

20 x 100 x

200x
IHC S100 : Positif in lamina propria nerve
fibers >40 microns

20 x 100 x
Negative Calretinin Vs Positive Calretinin

20 x

20x

100x
IMUNOHISTOCHEMISTRY STAINING

The Advantage of Calretinin Staining:

• It can be applied on parafin embedded tissue

• Simple staining patern
• Simple interpretation : Negative and positive
• Cost- effective
 Diagnosis
 Genetics
 MANAGEMENT
 Surgical Management
 Non surgical
 Botox injection
 Internal sphincterotomy for Internal

sphincter achalasia

 Stem Cells therapy

• Hypothesis : it may be possible to use
stem cells to form a “neo ENS” to
normalize function and improve
functional results following surgery
 Surgical Management

Orvar Swenson
Franco Soave Pena & Levitt

Early Late
1948 1980 Present
90’s 90’s

One Stage
Procedure

Bernard Duhamel

Keith Geogeson De La Torre

Fritz Rehbein
MIS
MIS : Laparoscopic assisted PT

Keith
Georgeson
 Trans Anal Endorectal PT
Louis De La Torre:
Mucosectomy up to ganglionated bowel

less pain, shorter hospital stay, and a better

cosmetic result than open surgery
Full-thickness Swenson like PT
 Prone position
 Hiding Dentate line
 Circumferential incision
 Full thickness dissection
 Coloanal Anastomosis