OMPHALOCELE AND

GASTROSHISIS

PEMBIMBING
Dr.Muntadhar SpB.SpBA
Dr Dian Adi SpBA

OMPHALOCELE
Omphalocele abad 16 (Ambrose Pare)
Operasi pertama berhasil 1802 (Hey)
Gastroschisis 1894 (Taruffi)
Operasi pertama berhasil 1873 (Visick)

EXOMPHALOS
(OMPHALOCELE)

CONGENITAL Anterior abdominal wall defect at the base of the umbilical cord with herniation of the
abdominal contents
Exomphalos and gastroschisis are two different congenital anomalies
Overall incidence is approximately 2-4: 10,000 live births

PATHOPHYSIOLOGY
Failure of the midgut to return to abdomen by the 10th
week of gestation

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%

ASSOCIATED ANOMALIES
40% have chromosomal abnormalities (Trisomy 13, 18,
21, Turners and Klinefelter synd)
60-70% infants have associated malformations
Cardiovascular, genitourinary, CNS
Beckwith-Wiedman syndrome
Pentalogy of Cantrell

Prognosis based on associated anomalies

MANAJEMEN DAN
RESUSITASI NEONATUS
Evaluasi kardiak (auscultasi, periksa tekanan darah
pada ektremitas, periksa nadi)
IVFD adekuat dan resusitasi cairan
Vital sign (jangan sampai hipotermi)
Dressing dengan silver sulfadia

OMPHALOCELE

Conservative treatment

1.
2.

Reduction by squeezing the sac or placement of a silo for

sequential tightening and staged closure
Children with giant omphaloceles or concomitant problems
that make them poor anesthetic risks may be treated with
topical application of Betadine ointment or silver sulfadiazine
to the intact sac. This allows secondary eschar formation and
eventual epidermal ingrowth. Residual abdominal wall hernias
are then repaired at 1 year of age.

Surgical treatment
1.
2.

Primary closure
Staged closure

Pertimbangan pilihan tindakan operasi

Ukuran defek
Usia gestasional
Anomali yang berasosiasi

PRIMARY CLOSURE

OPERATIVE MANAGEMENT

STAGED CLOSURE

POST OPERATIF
Feeding bila NGT nonbilus/ minimal
peristaltik (+)
AB diberikan selam 48 jam

PASCA OPERASI
Gastroesofageal reflux
Insufisiensi pulmonar
Infeksi paru berulang
Asma
Failure to thrive gastrostomy

GASTROSCHISIS
Congenital defect of the anterior abdominal wall just
lateral to the umbilicus

Pada gastroschisis terjadi 1 : 4000 kelahiran

USG :
Pada minggu ke 20 kehamilan, dengan adanya
peningkatan AFP

PATHOPHYSIOLOGY
Rapid dissolution of the right umbilical
vein after the standard period of
organogenesis leaves an area of relative
weakness in the mesenchyme through
which bowel or abdominal viscera can
herniate and eventually rupture.

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

MANAGEMENT
ABC
Heat Management
Sterile wrap or sterile bowel bag
Radiant warmer
Fluid Management
Nutrition
NPO and TPN
Gastric Distention
OG/NG tube
Infection Control
Ampicillin and Gentamicin
Associated Defects
Closure of the defect

Surgical Management
Primary Closure
Staged Closure

Staged repair using silo pouch

STAGED CLOSURE/SILO

SILO (1997)

SILO

SILO

PRIMARY CLOSURE VS. STAGED

CLOSURE PROBLEMS
Primary Closure:
abdominal
compartment
syndrome dengan
resiko gagal ginjal
dan trauma pada
intestinal
Staged Closure:
Infeksi, yang lebih
lama sembuh

Omphalocele Gastroschisis
Incidence

1:6,000-10,000

Covering
Sac
Size of
Defect
Cord
Location
Bowel

Present

1:20,00030,000
Absent

Small or large

Small

Onto the sac

On abdominal
wall

Normal

Other

Liver often out

Edematous,
matted
Rare

Omphalocele Gastroschisis
Prematurity 10-20%
If sac is
NEC

50-60%

Associated
Anomalies

10-15% most
Intestinal
atresia

Prognosis

ruptured
>50%

20%-70%

18%

70-90%