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2021

Abdominal Wall Defects


Omphalocele vs Gastroschisis
Pembimbing :
dr. Melvin Pascamotan Togatorop, SpB
MOHD SHAHMIN BIN MAT GHANI
Omphalocele Gastroschisis
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Omphalocele Gastroschisis
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Epidemiologi
Gastroskisis Omphalocele
 Insiden – 1:3000-8000  Insiden – 1 : 4000-7000
 L: P is 1:1  L:P is 1.5:1
 Ibu usia muda (<20 tahun)  Ibu usia lanjut
 Merokok  Lahir cukup bulan
 Bayi premature  >70% dikaitkan dengan kelainan
 BBLR kongenital seperti Bowel atresia,
• 10-15% dikaitkan dengan kelainan Imperforated anus,
kongenital seperti CHD (VSD), CLEFT  Trisomies 13, 18, 21,
PALATE dan malrotasi

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Etiologi
Gastroskisis Omphalocele
o Kelainan vaskularisasi o Dikarenakan kegagalan midgut
o Arteri omphalomesenterica untuk masuk kedalam rongga
dextra abdomen pada minggu ke-10 gestasi
o Vena umbilicalis dextra
o Kelainan dinding abdomen
o Distrupsi dari artery kongenital dengan protusi selaput
omphalomesenterika kanan pada visera abdomen kedalam peritoneum
kejadian midgut masuk ke abdomen parietal dan kantong membrane
di usia minggu ke-10 gestasional amnion yang terisi Wharton’s jelly
yang menyebabkan iskemik dinding
abdomen dan akhirnya menimbulkan
rupture dinding abdomen.
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Faktor Resiko
Gastroskisis Omphalocele

• Young maternal age • Increased maternal age


• Low gravida • Twins
• Prematurity • High gravida
• Low birth-weight secondary to • Consecutive children
IUGR
• Smoker
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Lokasi dan Size


Gastroskisis Omphalocele

• Right lateral to umbilicus • Epigastric


• Mid-abdominal
• 2-5cm • Hypogastric
• Vertical • 4-10cm
• Small and large intestine • > 10cm
• Rarely liver • Giant omphalocele
Omphalocele vs Gastroschisis • Liver may be in the sac (30-50%) 9
Gejala Klinis - Omphalocele
• Defek sentral pada dinding abdomen dibawah cincin
umbilicus.
• Defek berukuran 4-12 cm (Small-<5cm)
(Large>8cm)
• Selalu dibungkus dengan kantong /Sac
• Sac terbentuk dari amnion, Wharton’s jelly dan
peritoneum
• The umbilical cord inserts directly into the sac in an
apical or lateral position.
• Pada ukuran yang kecil mungkin mengandungi
intestinal loop only, tapi pada ukuran besar tidak
menolak kemungkinan juga diisi oleh liver,
spleen dan bladder, testes/ovary
• >50% mempunyai anomali lain

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Gejala Klinis - Gastroschisis
• Defek selalunya di sebelah kanan tali
pusat yang intak
• Tali pusat biasanya muncul dari dinding
abdominal yang normal
• Saiz bukaan <=5 cm
• Tidak ada kantong / Sac
• Penonjolan biasanya hanya mengandungi
intestinal loop
• Usus biasanya menebal, kusut dan
bengkak
• 10-15% disertai dengan anomaly lain
• 40% are premature/ BBLR

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Diagnosis
• Alpha-Feto-Protein- di sintesis di dalam fetal liver dan di ekresi oleh fetal
kidney dan melewati placenta pada minggu ke-12
• Peningkatan maternal AFP - Neural Tube Defects, Abdominal Wall
Defects, Duodenal Or Esophageal Atresia
• 40% false positive rate
• Fetal ultrasound  setelah 14 minggu gestasi untuk tes konfirmasi

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Prenatal Ultrasound -
Gatroschisis
• Normal umbilical cord insertion site
• Small bowel loops seen in the
amniotic cavity
• No covering membrane over the
loops of bowel
• Can include stomach and large
bowel
• Majority occur to the right of the
umbilical cord

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Prenatal Ultrasound -
Omphalocele

• Umbilical cord insertion is


typically midline on the mass
• Located centrally
• Contents are intestinal loops and
maybe liver, spleen and gonads.

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Management pra-bedah
• ABC
Tatalaksana • Heat Management
– Sterile wrap or sterile bowel bag
– Radiant warmer
Perinatal Management • Management cairan
– IV bolus 20 ml/kg LR/NS
– D10¼NS 2-3 maintenance rate
• Skrining maternal
• Nutrisi
 Peningkatan Alpha-feto-protein = – TPN (central venous line )
90% Omphalocele 10% Gastroschisis • Distensi Abdomen
 Fetal Ultrasound  temuan pasti – NGT
• Edukasi dan konseling prenatal – Kateter Urin
• Infeksi
• Broad-spectrum antibiotics -
Ampicillin and Gentamycin
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• Tutup defek
Heat loss

• Large surface area exposed


• Radiant warmer
• Cover defect with non-adherent dressing
 Warm saline gauze
• Quickly loses warmth and may promote cooling of not
constantly changed
 Bowel bag
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Timing of Surgery
Gastroskisis Omphalocele

• Emergent / urgent • Not Emergent/ urgent


• Evaluasi preoperative terutama • Evaluasi preoperative terutama
pada dalam identifikasi
• Volume status • Kelainan kngenital
• CVS dan Renal
• Keseimbangan Elektrolic/
• Kelainan Kromosomal
Asam-Basa
Omphalocele vs Gastroschisis
• Giant omphalocele 19
• Urgent
Omphalocele

• Terapi Konservatif
1. . Omphalocele yang besar (10-
12cm) dikasi terapi topical  •o Primary Closure
Betadine ointment atau silver
sulfadiazine pada kantong  Small defects (<4cm)
yang intak.  excision of the sac and closure of the
2. Secondary eschar formation and fascia and skin over the abdominal
granulation. contents
3. Penyembuhan selama 12 bulan
dan nanti di reparasi sebagai •o Mesh patch
hernia ventralis  Medium defects (6-8cm)
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Staged Closure

• Creation of a Silo
• Abdominal organs reduced
slowly over days

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o NICU
o Ventilation
o
Post operative care o
Feeding:– Minimal volume
48 hrs Antibiotics
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o Hernia dealt with at 1 yr old
Gastroschisis

• Primary closure
o If bowel easily reduced
• Staged closure
o Silo fashioning:
 Sac excised
 Silo sewn to rectus fascia/full
thickness

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o NICU
o Feeding delayed for weeks
Post operative care o Oral stimulation/sucking reflex
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o Broad spectrum antibiotics 24
Outcome jangka panjang

Omphalocele Gastroschisis
• Kecil  Sembuh • Generally excellent if no atresia
• Besar  • NEC:
– Gastro-oesophageal reflux - 43% – 18.5% of neonates more with formula
– Majority improve over time – Bowel loss - short gut syndrome
– 20% pulmonary insufficiency • Cryptorchidism:
– Respiratory Infections 15-30%
– Asthma – Due either being outside/prematurity
– Feeding difficulties; – Replacement and orchidopexy by 1 yr
• 60% with giant omphalocele • 60% have psychosocial stress if umbilicus
• May need gastrostomy for feeding sacrificed
– Failure to thrive
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Ringkasan

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THANK YOU!
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