Congenital Anomaly of

Digestive System
Presenter:
1. Novan Ardiansyah
2. Ismail Harun Ziha
Participan:
1. Kurnia Tejawati
2. Marizca Saras Chitra
3. Nunie Ismi Amri
Preceptor:
dr. Liza, Sp.B

Esophageal Atresia and

Transesophageal Fistula
Type:
A. Isolated EA: 8-10 %
B. EA with TEF between proximal segment of
esophagus and trachea: 1%
C. EA with TEF between distal esophagus and
trachea: 75-85%
D. EA with TEF between both proximal and
distal end of esophagus and trachea: 1-2%
E. TEF without EA (H-type fistula):5-8%

Etiology:
- The esophagus and trachea share a
common embryologic origin
fail to separate:
1.Absent of sonic-hedhog signaling
pathway
2.Thyroid transcription factor one (TTF-1)
3.Fobroblast growth factor (FGF-10)

Clinical presentation:
1.Excessive drooling
2.Chocking or coughing after feeding
3.Abdominal distention: caused by air pass
into stomach
4.Difficult to breathe: caesed by distention
5.Atelectasis
pulmonary disfunction
6.C and D type: regurgitated gastric juice
passes through fistula
chemical
pneumonitis

Diagnosis:
1.Percussion of abdomen: air
2.Inability to pass orogastric tube
3.Radiography: dilated upper pouch,
air in abdomen.
4.Coexisting anomalies: cardiac defect,
skeletal defect, neurological defect,
anorectal decet, other.

Management:
1.Initial: place in an infant warmer,
head elevated 30, sump catheter, iv
antibiotic, warmed electrolyte
solution, search other defect:
cardiac.
2.Esophagoesophagostomy

Hypertrophic Pyloric
Stenosis
Occurs in 1 in 300 live births,
between 3 and 6 weeks of age.
Etiology: has not been determined.
Study have shown: familial link,
Erythromycin in early infancy.

Clinical presentation:
1.Prijectile nonbilious vomiting
2.Intolerant to feed even clear liquid
3.Severe dehydration
4.Jaundice may occur
5.Less flatus
6.Hypochloremic, hypokalemic,
metabolic alkalosis.

Diagnosis:
1.PE: inspection: visible gastric waves.
Palpation: olive in RUQ, if cannot
palpated use USG
2.USG: channel length of over 16 mm
and pyloric thickness over 4 mm.

Management:
1.Fluid resuscitation
2.Fredet-Ramstedt pyloromyotomy

 Incidence: 1 in 2000 to 1 in 5000.

male = female
Clinical presentation:
1.Jejunal and ileal atresia: bilious
vomiting and progressive abdominal
distention.
2.More distal more abdominal
distention.

Diagnosis:
1.Plain abdominal film: 1.Obstructed loop.
2.Duodenal atresia: double bubble. 3.Airfluid level.
2.Barium enema: DD: microcolon, meconium
plug, small left colon syndrome,
Hirschsprungs disease, or meconium ileus.
Management: 1. decompression. 2.
laparotomy

Malrotation and Midgut

Volvulus
During 6th week the mudgut grow
rapid and prolapses into umbilical
cord, and 10th 12th return to abdominal
cavity undergoing 270 degree
counterclockwise. If the rotation is
incomplete, the caecum remains in
the epigastrium, but the bands fixing
the duodenum to the retroperitonium
and caecum continue to form.

This result in (Ladds) bands extending

from the caecum to the lateral
abdominal wall and crossing the
duodenum, which creates the
potential for obstruction. Volvulus
may occur around the mesentery
resulting obstruction of proximal
jejunum and infarction of the midgut.

Clinical presentation:
1.Bilious vomiting
2.Bloody stool
3.Circulatory collapse.
4.Erythema and edema of abdominal
wall due to ischemia which
progresses to shock and death

Diagnosis: abdominal film: air-fluid

level, volvulus: corkscrew shape.
Management:
1.Fluid resuscitation
2.Exploratory laparotomy.

Intestinal Duplication
Most common in ileum
May long and tubular, but usually are
cystic masses.
Clinical presentation:
1.Recurrent abdominal pain
2.Emesis: obstruction
3.Hematochezia: ulceration or ectopic
gastric mucose

 PE: palpable mass

Diagnosis: CT-scan, USG, barium
enema
Management: resection: laparotomy

Meckels Diverticulum
Remnant of portion of the embryonic
omphalomesenteric (vitelline) duct.
It is located on antimesenteric border
of ileum, usually within 60 cm of the
ileocaecal valve.
It can contain heterotopic mucose:
gastric mucose. Also pancreatic acini.
Other: brunners gland, pancreatic
islet, colonic mucose, endometriosis,
hepatobiliary tissue

 Clinical presentation:
1. GI bleeding, maroon-colored stool.
Management:
Surgical: ileostomy

Mesenteric Cyst
Duplication within the mesentery,
but do not contain mucosa or
muscular wall.
Can cause intestinal obstruction and
may present as an abdominal mass.
Diagnosis: USG or CT-scan
Management: surgical excision

Hirschsprungs Disease
Caused by a malformation in pelvic
parasympathetic system which result
in the absence of ganglion cells in
Auerbachs plexus of a segment of
distal colon.

Clinical presentation: functional distal

intestinal obstruction
1.Abdominal distention
2.Failure to pass meconium within 48 hours
3.Bilious emesis
4.Constipation
5.Complication: enterocolitis: abdominal
distention and tenderness, systemic
toxicity: fever, failure to thrive, lethargy.
Dehydrate, leukocytosis.

 Rectal examination: forceful

propulsion of foul-smelling liquid
feces
Diagnosis: rectal biopsy: sample
1,2,3 cm from the dentate line.
Barium enema: dilated colon.

 Management:
1.decompression: NGT, IVFD.
2.Antibiotics
3.Rectal irrigation
4.Surgery: pull-through

Anorectal Malformation