Congenital Development

disorder of esophagus,
stomach, duodenum,
abdomen wall, anus and
rectum

Tutorial pra-bedah
Formation of the
Primitive Gut

Embryo folding in sagital plane (3 weeks old)

Ektoderm

Endoderm
Mesoderm
Formation of the
Primitive Gut
Formation of the
Primitive Gut
Formation of the
Primitive Gut

It devide 3 part as below which based on

vascular supply
Forgut (A. coeliac trunk)– pharinx,
esofagus_respiratory divertivul, stomach, cranial
part of duodenum
Midgut (A. Sup. mesenteric)– caudal part of
duodenum (+liver, gall bladder, pancreas), small
intestine and part of large intestine
Hindgut (A. Inf. mesenteric)– large intestine,
rectum and upper part of anal canal
ESOPHAGUS
 DEVELOMENT OF
ESOPHAGUS

Foregut starts from

the Oral cavity and
terminates at the
level of Ampulla of
Vater (the point
where common
bile duct opens
into Duodenum)
DEVELOMENT OF
ESOPHAGUS
During the 4th wk, a small laringotracheal diverticulum appears
in the ventral wall of Pharynx.
A ‘Tracheoesophageal Septum’ gradually separates the ventral
Respiratory diverticulum from the dorsal part of foregut.
As a result, the Pharynx is divided into;
– a ventral portion the “respiratory primordium”, – a dorsal
portion, the “esophagus”
DEVELOMENT OF
ESOPHAGUS
Due to the growth and
descent of the heart an
lungs,esophagus elongates
The lumen then will be
obliterated due to
proliferation of epithelium
 recanalization

Fail to recanalization?
Congenital malformations of
Esophagus
Atresia of Esophagus & Esophageal Fistula
Congenital malformations of
Esophagus
Atresia of Esophagus & Esophageal Fistula
Clinical manifestation
• Atresia of Esophagus prevents the normal passage of amniotic
fluid into the intestinal tract leading to the accumulation of
excess fluid in the amniotic sac  Polyhydroamnios in
pregnancy
•cannot swallow and drools copious amounts of saliva.
•Aspiration of saliva or milk  RDS, coughing after breast
feeding
•Gastric secretion reflux can harm the lung
•Possible stomach rupture  air from the
trachea can pass down the distal fistula
Congenital malformations of
Esophagus
Atresia of Esophagus & Esophageal Fistula
Work up
•Prenatal ultrasonography - gastric bubble,
polyhydramnios, and VACTERL (vertebral
defects, anorectal malformations,
cardiovascular defects, tracheoesophageal
defects, renal anomalies, and limb deformities)
anomalies
•Radiography
•If there is no prenatal screening, search other
deformities.
malformations of
Esophagus
Congenital
malformations of
Esophagus
Initial management.
•ABCDE
•Uprigth position, Prevent aspiration.
•The Replogle tube or a similar large-bore tube should be in
position in the upper pouch and continously aspirated
•Don't cry. To prevent perforation of the stomach.
•Intravenous 10% dextrose and 0.18% saline will maintain
fluid and eletolyte balance and prevent hypoglycemia.
•A vitamin K analogue should be administered routinely
before operation.
•Preventing heat loss
Take notes
Complete absence of gas in the GI tract = No distal
tracheoesophageal fistula (TEF); however, distal
fistulae simply occluded by mucous plugs have
been rarely reported.

early single-stage primary repair vs

Delayed. repair depend on distance
between the ends Esophagus.
Congenital malformations of
Esophagus
Esophageal Stenosis – due to incoomplete recanalization of
esophagus
Congenital
malformations of
Esophagus
Short Espohagus & congenital hiatal hernia
The espohagus fails to elongate so it is very short and may
be associated with thoracic stomach.
Development of the
stomach
Arround the middle of the 4th week
Develops from the distal part of the forgat.
It was initially a simple tube  dilated & rotated.
Anterior border (lesser curvature) posterior
border (greater curvature)

Rotated anaterior surface to the left;

posterior surface to the right
Congenital malformations of
stomach
Congenital hypertrophic pyloric stenosis – circular muscles
in the pyloric region are hypertrophy.
Congenital malformations of
stomach
Congenital hypertrophic pyloric stenosis
causes :unknown but as evidence based multifactorial
(environmental factors and hereditary factor)
Clinical manifestation :
•Born normally, after few weeks progressively vomiting. From
non projectile  projectile vomit
•Emesis should not be bilious and more acidic (no pancreatic
enzyme). May become brown or coffee color due to blood
secondary to gastritis.
•A firm, non tender, and mobile hard pylorus that is 1-2 cm in
diameter, described as an "olive,”
•In late progress, doctor may also observe gastric peristalsis
•Dehydration, hypokalemia, hypocloremia, metabolic alkalosis
Congenital malformations of
stomach
Congenital hypertrophic pyloric stenosis
causes : multifactorial (environmental factors and hereditary
factor)
Clinical manifestation :
Ultrasonography  thickened pyloric muscle
malformations of
stomach

Congenital hypertrophic pyloric stenosis

Infantile hypertrophic pyloric stenosis (IHPS) may
be described as a medical emergency or a medical
urgency based on how early in the course the
patient presents.

Immediate treatment requires correction of fluid

loss, electrolytes, and acid-base imbalance.

Definitive  pyloromyotomy
Development of the
Duodenum
Around the middle of the 4th week
Begins to develop from the endoderm of the caudal part of
the foregut and the cranial part of midgut
Development of the
Duodenum
Blood supply of the duodenum
Development of the
Duodenum
• By the end of the embryonic period, most of the ventral
mesoduodenum has disappeared.
• The lumen then become obliterated because of it’s
proliferation of its epithelial cells.  recanalization (3rd
Month)
• Incomplete recanalization  duodenal stenosis
• Failure of recanalization  duodenal atresia  total
obstruction.
Congenital
malformations of
duodenal
Duodenal stenosis & Duodenal atresia
Cause: unknown, associated with genetic defects. (trisomy
21; Down Syndrome)

Clinical manifestation:
Upper intestinal obstruction commence  Sustained
vomiting (bilious or non-bilious)
Vomiting is associated with variable dehydration, changes in
serum electrolytes, and weight loss.
Risk aspiration!
Distension, distal bowel collapse.
Congenital
malformations of
duodenal
Duodenal stenosis & Duodenal atresia
Antenatal sonographic features of high intestinal obstruction
& polyhidramnios
Congenital
malformations of
duodenal
Duodenal stenosis & Duodenal atresia
Barium contrast
Administered sometimes via an orogastric or nasogastric
tube under fluoroscopy to evaluate the esophagus, stomach,
and duodenum  It is then removed by nasogastric tube
Serum electrolytes
If duodenal atresia is diagnosed early, electrolyte and fluid
balance should be normal. Duodenal stenosis & Duodenal
atresia
Congenital development
of duodenal
Duodenal stenosis & Duodenal atresia
Treatment :
ABCDE
Decompression  NGT
IV Fluid, electrolyte correction

Definitive:
Duodenodoudenostomy
development of
abdomen wall
development of
abdomen wall
 development of
abdomen wall

6th week, normal,

midgut herniation
into umbilical cord

10th week,
Peritoni midgut
um comeback to
abdomial
cavity
Congenital development
of abdomen wall
Omphalocele
Failure of the midgut to return to abdomen (10th week of
gestation). Herniated midgut  intestine or omentum
covered by peritoneal
 Congenital development
of abdomen wall
Gastroschisis

Lateral fold in
transversal
plane

Etilogy?
Mostly on the
right side
Congenital development
of abdomen wall
Congenital development
of abdomen wall
Congenital development
of abdomen wall
Congenital development
of abdomen wall
Omphalocele & gastroshisis
Diagnosis
Prenatal ultrasound
Lab test, increasing maternal serum AFP .
Congenital development
of abdomen wall
Omphalocele & gastroshisis
Treatment
•ABC
•Heat management by sterile wrap or bowel bag, radiant warmer
•Fluid management.
•Abdominal distention? NGT
•Antibiotics
•Surgery- place organ back + repair defect.
• -primary closure or staged closure with silo?
•Introduction of enteral feeding post Operative is often delayed for
weeks while awaiting return of bowel function.
Congenital development
of abdomen wall
Umbilical Hernia
Defect in linea alba, subcutaneous tissue and skin covering
the protruding bowel.
Congenital development
of abdomen wall
Umbilical Hernia
Clinical findings
Bulge in belly button area. More protruding when baby cries.
Usually can be gently pushed

Treatment
Many Umbilical hernias close spontaneously by ages 2-4.
If not  surgical repair.
Congenital development
of anus and rectum
Umbilical Hernia
Clinical findings
Bulge in belly button area. More protruding when baby cries.
Usually can be gently pushed

Treatment
Many Umbilical hernias close spontaneously by ages 2-4.
If not  surgical repair.
development of anus
and rectum
The hindgut develops into distal transverse colon,
descending colon, Rectum and proximal anus which
received blood supply from Inferior Mesentric Artery
During sixth week of gestation, the distal end of the hindgut
(the cloaca) is divided into Urogenital Sinus and Rectum
Hindgut is endoderm. Whereas, the distal canal is deriver
from ectoderm which received blood supply from Internal
Pudendal Artery.
Transisional site  Linea Dentata
Congenital anomalies of
rectum and anus
Congenital anomalies of
rectum and anus
Congenital anomalies of
rectum and anus
The etiology of such malformations remains unclear and is
likely multifactorial. There are however reasons to believe
there is a genetic components.

Clinical manifestation will depends on it’s classification.

Severe manifestation will occurs in the non-fistula group.
Low GIT obstruction symtopms.
Search other abnormalities.
VACTERL.
(Vertbral, Anarectal, Cardiac, Tracheo, Esophageal, Renal,
and Limb
Congenital anomalies of
rectum and anus
Congenital anomalies of
rectum and anus
Congenital anomalies of
rectum and anus
Initial management
ABC, IV fluid, Decompression
If a genitourinary fistula is suspected  broad-spectrum
antibiotics
If malformation complexity (have fistula & >1cm from
perineal skin) or associated comorbidity  colostomy
Colostomy closure: Once the wound has completely healed
and postoperative dilations have achieved their goal.
primary pull-through without colostomy perineal fistulas
(or no fistulas) and close (< 1 cm) rectal pouches usually
using posterior sagittal approach
Reference
Pena A, Levitt MA. Anorectal Malformations. In .Grosfeld
JL,O’Neill JA, Fonkalsrud EW, Coran AG. Pediatric Surgery.6th
ed. Mosby Elsevier Inc. 2006.
Pena A, Levitt MA. Imperforate Anus and Cloacal Malformations.
In Holcomb GW, Murphy JP. Ashcraft’s Pediatric Surgery. 5 th ed.
Elsevier Inc. 2010.
Sadler TW. Langman’s Medical Embryology. 11thed. Lippincott
Williams and Wilkins Inc. 2011.
Ashcraft, Keith W., George W Holcomb, J. Patrick Murphy, and
Daniel J Ostlie. Ashcraft's Pediatric Surgery. 5th ed.
Philadelphia: Saunders/Elsevier, 2010.
Wilkinson AW. Congenital anomalies of the anus and rectum.
Arch Dis Child. 2012;47(256):960–969.
doi:10.1136/adc.47.256.960
Thank you