MANAGEMENT OF CONGENITAL

LOWER GASTRO INTESTINAL

TRACT DISORDER :
EXPERIENCE IN RURAL REGION

dr. Hanafi Gunawan Macan, Sp.B

Department of Surgery Demang Sepulau Raya General Hospital
Central Lampung
 Introduction
 Congenital malformations involving the gastrointestinal
tract (GIT) can be broadly divided into upper and lower gut
abnormalities.
 Upper pathology involves the foregut tubes, which are
proximal to the ligament of Treitz: esophagus, stomach,
duodenum, pancreas and hepatobiliary tract.
 Lower GIT anomalies include the mid and hindgut
structures: the jejunum and ileum, the colon and anorectal
malformations.

(Congenital Anomalies of the Gastrointestinal Tract, Verma 2020)

 Congenital anomalies can further be classified based
on whether the defect is structural or functional.
 Structural anomalies result from either defective
embryogenesis or intrauterine complications, such as
ischemia.
 Functional defects have normal anatomy but disrupted
flow of GIT contents. In most cases, structural defects
adversely impact functional capability.

(Congenital Anomalies of the Gastrointestinal Tract, Verma 2020)

Embryologic derivates of Gastro Intestinal Tract

Anatomic relation Embryonic Blood supply Viscera

source

Upper gastro- Proximal to ligament Foregut Celiac axis Esophagus, Stomach,

intestinal tract of Treitz Duodenum, Biliary ducts,
Liver , Pancreas

Jejunum, Ileum, Caecum,

Superior Ascending colon, Proximal
Midgut Mesenteric 2/3 transverse colon
Artery
Lower gastro- Distal to ligament of
intestinal tract Treitz
Distal 1/3 transverse colon,
Inferior Descending colon, Sigmoid
Hindgut Mesenteric colon, Rectum, Anal canal
Artery

(Congenital Anomalies of the Gastrointestinal Tract, Verma 2020)

 CASE 1
TUBULAR COLONIC
DUPLICATION
Introduction

 Alimentary tract duplications are relatively rare congenital

anomalies, that may be found any where along the
gastrointestinal tract from the mouth to anus.
 Asymptomatic duplications can be discovered incidentally.
 Most duplication are benign condition.
 The majority of patients (80%) present prior to 2 years of
age.
 However, duplications may be found at any age from fetus
to the geriatric patient.

(Ashcraft’s Pediatric Surgery 2010)

(Wu et al, 2018)
 Of the two types, the vast majority are cystic, whereas a small
minority are tubular.
 The most common location is the jejunum/ileum  34%
 Colonic duplication account for approximately 15 % of all
duplications, most occur in the caecum and cystic.
 Tubular colon duplication can also be found and will vary in length
and complexity.
 The rarest case of tubular duplication was affecting several segments
of colon or entire colon.

(Muntadhar et al, Mal J Med Health Sci, 2020)

(Ashcraft’s Pediatric Surgery 2010)
Case Report

 A 5-year-old boy came with complaints of chronic

constipation and abdominal pain.
 Four years earlier, the patient had been treated at several
hospitals with the same complaint. An ultrasound and
abdominal X-Ray were performed  there was an
enlarged caliber of the colon with contain of fecal material.
 The patient has been treated conservatively, but the result
wasn’t good.
Abdominal X-Ray (2015)
 Because the complaints did not disappear, the patient came
to our hospital, we analyzed for the possibility of
Hirschsprung's disease with enterocolitis.
 Patient treated with surgery : Laparotomy was performed
 there was a colonic tubular duplication, then the
reconstruction of the colon was performed with good
results.
Durante Op
Colonic tubular duplication

(Ashcraft’s Pediatric Surgery 2010)

Video Durante Op
 CASE 2
HIRSCHPRUNG’S DISEASE
Introduction

 Hirschsprung disease is the commonest cause of functional

large gut obstruction in children.
 Hirschsprung disease is a developmental disorder of the
enteric nervous system that is characterized by the absence
of ganglion cells in the myenteric and submucosal plexuses
of the distal intestine, which results in lack of peristalsis
and functional intestinal obstruction.

(Langer, 2013)
(Das, 2017)
 The Rectosigmoid form is the classic, common variety
consist of 75–80% .
 Long segment colonic Hirschsprung disease consist of 10-
15%.
 Total colonic aganglionosis with ileal involvement up to 50
cm proximal to the ileocecal junction, consist of 5-7% 
uncommon.

(Langer, 2013)
(Das, 2017)
 The treatment of Hirschsprung disease is primarily surgical, and
involves resection of the aganglionic bowel and reconstruction.
The most common operations currently done are the Swenson,
Soave and Duhamel procedures.
 Today, the two common procedures preferred worldwide are
‘single’ staged – Duhamel and Transanal endorectal pull through
(TAERPT).
 TAERPT is generally reserved for rectosigmoid Hirschprung
disease and avoids a laparotomy.

(Langer, 2013)
(Das, 2017)
Case Report

 A 2-week-old baby boy came with complaints of flatulence

and difficulty defecating. At the beginning of birth there
was a history of delay in passing meconium.
 The orogastric tube was inserted and wash out was
performed with warm normal saline to evacuate the faeces.
 We suggest the suspicious of Hirschprung Disease, then we
performed a Colon in loop examination.
Abdominal X-Ray
Colon in Loop
 The patient was diagnosed with Hirschsprung disease short
segment type.
 A single-stage operative procedure was performed using
the Trans anal endorectal pullthrough (TAERPT) technique
with good result.
 CASE 3
ANORECTAL MALFORMATION
WITH
INTRA ABDOMINAL TUMOR
Introduction

 Anorectal atresia occurs in one of every 4000 to 5000 newborns

and is slightly more common in males.
 The most frequent defect in male patients is anorectal atresia with a
rectourethral fistula.
 In female most frequent  anorectal atresia with a rectovestibular
fistula
 Imperforate anus without a fistula is a rather unusual defect 
occurs about 5%.

(Ashcraft’s Pediatric Surgery 2010)

Classification of Anorectal Malformation

(Ashcraft’s Pediatric Surgery 2010)

Algorithm of Anorectal Maformation

(Ashcraft’s Pediatric Surgery 2010)

Case Report

 A newborn baby girl came with complaints of an enlarged

of the stomach/abdomen, and she has no anus. The patient
was born in a midwife spontaneously and then referred to
the general hospital.
 On examination, there was a cystic mass in the abdomen,
and there was no anus in perineum.
 Plain abdominal X-Ray and knee chest position X-Ray
were performed.
Abdominal X-Ray and
Knee-Chest Position
 The patient was diagnosed with intra-abdominal tumor
with high level of anorectal malformation without fistula.
 We performed a laparotomy + excision of the tumor mass,
and create end colostomy, where the cystic mass almost
filled the lower abdominal cavity. The tumor mass
originates from the colon.
 Histopathology results showed Embryonal
Rhabdomyosarcoma.
Post Op and Histopatology
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