Dikki Drajat Kusmayadi, dr.

, SpBA(K)
No. Telp : +62 82130524364
E-mail : dikdra@gmail.com

• 1989 FK UNPAD Bandung

• 2000 PPDS Ilmu Bedah FK UNPAD Bandung
• 2003 Sub-Spesialis Bedah Anak FK UNPAD Bandung

• Kepala Program Studi Bedah Anak

Departemen / KSM Ilmu Bedah, RSUP. Dr. Hasan Sadikin / FK
UNPAD, Bandung
 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
 DIAGNOSIS
 Clinical symptoms
 Contrast
enema
Failure to pass meconeum Cone -shaped
Constipation
Abdominal distension Funnel-shaped
Vomiting Transitional zone
Failure to thrive, malnutrition,
enterocolitis

 Anorectal manometry
Physical Examination :
Visible and palpable transverse colon
Explosive passage of stool in response to
a DRE
Palpable fecal impaction (older child)

 Histopathologic findings
 Histopathologic examination

H&E staining,
IHC staining : AChE

vs

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

20x 100x

200x
CURRENT DIAGNOSIS

??
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
 Positive
S100 ∅ > 40 µ
nerve fiber hypertrophy
20 x 100 x
Negative Calretinin Vs Positive Calretinin

20 x

20x

100x
Negative Calretinin Vs Positive Calretinin

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
 MANAGEMENT

Surgical Management

- Stoma - Pullthrough procedure

 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
CURRENT MANAGEMENT

??
 Trans Anal Endorectal PT
Louis de La Torre
Long mucosectomy, up to ganglionated bowel w/o opening
the abdomen or laparoscopic surgery

less pain, shorter hospital

stay, and a better cosmetic
result than open surgery.
 MIS : Laparoscopic assisted PT

Keith
Georgeson
 Trans-Anal Full-thickness
Swenson like PT

 Prone position
 Hiding Dentate line
 Circumferential incision
 Full thickness dissection
 Resection aganglionic bowel
 Coloanal Anastomosis