Oomphalocoele

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Definition

-  Closed umbilical base defect in abdominal wall of newborns

 

Features

-  Visceral hernaition w parietal peritoneum often w malrotation

-  2.5:10,000 births

-  Associated w chromosome abnormalities

-  No comorbidites low mortality, 30% mortality w comorbidity

 

Associated S/S

-  Chromosomal anomalies 40-60%

-  Trisomy 13, Trisomy 18, Trisomy 21

-  Turners, Klinefelters

-  Cardiac

-  Teratology of Falot

-  Coarcatation of aorta

-  VSD (25%)

-  Beckwith-Weidemann Syndrome

-  Gastronintetinal

-  Midgut volvulus

-  Malrotation

-  Meckels Diverticulum

-  Imperforate anus

-  Colonic Agenesis

-  Neurologicai anomaly

-  Renal anomaly

-  Pentology of Cantrell

-  Bladder Extrophy

-  Herniation of abdominal contents

-  Intestine

-  Liver

-  Gall bladder

-  Stomach

-  Bladder

-  Pancreas

-  Spleen

 

Ix

-  Prenatal Ultrasound (fetal morphology scan)

-  Alpha fetoprotein: elevated maternal serum or amniotic fluid

 

DDx

-  Gastroschesis: US

 

Mx

-  Warm fluid impermeable dressing over defect

-  Orogastric tube: decompression

-  TPN

-  Surgical repair 1st day of life