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Agung P

11.023
Abdominal wall defect
- Omphalocele
- Gastroschisis
Omphalocele
birth defect in which the infant's
intestine or other abdominal organs
stick out of the belly button (navel). In
babies with an omphalocele, the
intestines are covered only by a thin
layer of tissue and can be easily seen.
Pathophysiology

Failure of the midgut to return to


abdomen by the 10th week of
gestation
Minggu 6
Minggu 10
Minggu 11
Back to normal from
herniasi
Clinical Findings
central defect of the abdominal wall beneath the umbilical ring.
Defect may vary from 2-10 cm

Always covered by sac


The sac may be intact or ruptured
Sac is composed of amnion, Whartons jelly and peritoneum
The umbilical cord inserts directly into the sac in an apical or occasionally
lateral position.

Sac contains intestinal loops, liver, spleen and bladder ,


testes/ovary
>50% have associated defects
Prognosis depends on theses associated anomalies
Mortality is approximately 40%
Omphalocele
Incidence has remained constant
Increased risk with advanced maternal age
Probable genetic predisposition
Associated syndromes and anomalies (45-55%):
- gastrointestinal
- cardiac
- trisomy 13, 18, 21
- OEIS complex (omphalocele, bladder extrophy,
imperforate anus, spinal defects
- Beckwith-Wiedemann
- pentalogy of Cantrell
- cleft palate
- pulmonary hypoplasia
May be associated with maternal use of valproic acid
Gastroschisis
No membrane covering
Abdominal wall defect typically 2-4cm
diameter
Lateral to the right side of the umbilical
cord
Usually contains midgut and stomach
Thickened, atretic, and possibly ischemic
bowel
Associated with malrotation
Embryology of Gastroschisis

Failure of vascularization of the


abdominal wall due to abnormal
involution of the right umbilical vein or
a vascular accident of
omphalomesenteric artery causes
abdominal wall weakness and
subsequent rupture
Rupture of a small omphalocele with
absorption of the sac and growth of a
skin bridge between the abdominal wall
defect and umbilical cord
Clinical Findings
Defect to the right of an intact umbilical cord allowing extrusion of

abdominal content

Umbilical cord arises from normal place in abdominal wall

Opening 5 cm

No covering sac (never has a sac )

Evisceration usually only contains intestinal loops

Bowels often thickened, matted and edematous

Infants have better prognosis than those with an omphalocele

(Mortality is approximately 10% )

10-15% have associated anomalies (intestinal atresia)

40% are premature/SGA


Diagnosis
AFP synthesized in fetal liver and excreted
by fetal kidneys and crosses placenta by
12 weeks
Elevated maternal MSAFP in neural tube
defects, abdominal wall defects, duodenal
or esophageal atresia
40% false positive rate
Fetal ultrasound after 14 weeks gestation
Amniocentesis and fetal echocardiography
Treatment
NGT to low intermittent suction
Use of bowel bags, saran wrap
Conservation of body heat and fluid
losses
Antibiotics
Careful positioning to avoid kinking
of mesenteric vessels
1.5 times maintenance fluids with
isotonic fluids
Surgical Management
Operative repair within 2-4 hours of
birth
Primary closure for smaller defects
Delayed primary closure for large
defects
Avoid compromised ventilation and
abdominal compartment syndrome
Use of silo with sequential reduction of
abdominal contents
Later fascial closure
Staged Closure