Omphalocele

OMPHALOCELEPerceptor : DR. AMRAN SINAGA, SP.BAUTHOR: Ida Ayu Ratna W, S.Ked (G1A214018)

BackgroundOmphalocele prevalence 2.5 of 10.000 birth.It is usually together with other congenital disorder (hearth, neural tube defect)Treatment: conservative long time, high risk infection, the result of this treatment make us worried.Operative treatment need reintervention

Anatomy

-Foregut: pharinx, esophagus, gaster, duodenum 2/3 proximal.-Midgut: 1/3 distal duodenum, jejunum, ileum, caecum, apendix, kolon ascenden, 2/3 proximal kolon transversum.-Hindgut: 1/3 distal dari kolon transversum, kolon descenden, sigmoid, rectum, bagian atas dari canalis ani. 3DEFINITIONOmphalocele from Greek Omphalos and celeomphalos umbilicus cele herniaSo it is a central defect of abdominal wall at the umbilicus ring area / umbilical ring herniation of the abdominal organs from the abdominal cavity still covered by a bag or membrane consisting of two layers (amnion and peritoneum with rare layers Whartons jelly)

The appearence

EtiologySeveral risk factors or the factors that contributed to a omphalocele occurrence are:Infection,Drug use and smoking in pregnant women,Folic acid deficiency,Hypoxia,Genetic disordersPolyhydramnios.

30% to 70%, including chromosomal abnormalities (trisomy 18, 21),congenital heart defects,Beckwith-Wiedemann syndromePentalogi Cantrell (defect abd wall, diaphragma, heart ectopic)Prune Belly syndrome

Prune belly syndromeDiagnosePrenatal Diagnose USGPostnatal Diagnose clinical appearanceThere is a central defect in the abdominal wall of the umbilical cordThe diameter starts from 4 cm until 12 cmHerniation of abdominal organs contain both solid and hollowStill covered by a membrane or bag and looked at the height of the umbilical cord inserts into pockets.

MANAGEMENTPRENATALPOSTNATALConservativeOperative

Prenatal ManagementOmphalocele diagnosed during prenatal, informed consent should be performed to the parents about the state of the fetus, the mothers risk, and prognosis.

Postnatal ManagementPlace the baby in a warm room for aseptic and prevent fluid loss, hypothermia and infection.Place the baby as comfortable as possible and gently to avoid baby crying and swallowing water.Assess presence / absence of respiratory distress.

Attach NGTAttach catheterAttach an intravenous linePerform monitoring and stabilitation temperature, acid-base status, fluid and electrolyteDefect was closed with a sterile-saline or povidone-iodineEvaluation of other congenital abnormalities

Operatif ManagementPrimary ClosureStaged Closureskin flapsilo

PROGNOSISSurvive rate of omphalocele in infants is influenced by the following :PrematurityOmphalocele sizeThe anomalies in other organs

Which one omphalocele ?AB

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