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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
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
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.
Surgical treatment
1.
2.
Primary closure
Staged closure
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
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 CLOSURE/SILO
SILO (1997)
SILO
SILO
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
On abdominal
wall
Normal
Other
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%