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Gastroschisis and Omphalocele

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Gastroschisis and Omphalocele

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    2. Gastroschisis and Omphalocele

    3. Gastroschisis Defect in abdominal wall, usually to right of intact umbilical cord Results in herniaton of free bowel loops into amniotic fluid No sac covering 1/10,000 live births

    4. Gastroschisis bowel shortened thick, inflammatory peel 25% association w/ intestinal atresias Malrotation, meckel’s diverticulum, low birth weight also assoc

    5. Etiology Unknown cause, many theories Failure of abdominal wall to form properly Vascular disruption at abd wall In utero disruption of omphalocele

    6. Prenatal diagnosis

    7. Prenatal management Vaginal delivery OK Prognosis good d/t low associated anomalies Immediate Surgical Repair Delays d/t institutional transfers costly

    8. Treatment Initially IVF, gastric decompression, Broad spectrum antibiotics, coverage of bowel Intraop - ? midgut volvulus, intestinal perforation 75% of cases, reduction is successful Monitor intraabdominal pressures

    10. Surgical repair Resection of infarcted bowel Primary anastamoses should not be performed Small bowel atresias can be left alone, repaired later

    11. Outcome Overall 85% survival complications Short gut syndrome Developmental delay Necrotizing Enterocolitis Cholestatic liver disease from prolonged TPN

    12. Omphalocele Central abdominal defect into which abdominal viscera can herniate Covered by sac

    13. Omphalocele Umbilical cord inserts onto the membrane Large defects can extend from umbilicus to costal margin – contain liver, small and large bowel

    14. Omphalocele 1/4000 live births Failure of abdominal wall to fuse at umbilical ring Unlike gastroschisis, bowel is not significantly injured, normal motility and function

    15. Associated anomalies 40% have chromosomal abnormalities (Trisomy 13, 18, 21, Turner’s and Klinefelter synd) 60-70% infants have associated malformations Cardiovascular, genitourinary, CNS Beckwith-Wiedman syndrome Pentalogy of Cantrell Prognosis based on associated anomalies

    16. Prenatal Diagnosis

    17. Prenatal management Due to hi co-incidence of malformations Karyotype Fetal US – CNS, cardiac, craniofacial abnormalities Mortality and Morbidity directly related to associated congenital abnormalities Consideration of termination of pregnancy May need C-section d/t risk of dystocia

    18. Initial postnatal management Cardiac evaluation (echo) Exam for other abnormalities Emergent Surgical repair not necessary as membrane protects viscera If too unstable for anesthetic – nonoperative management Topical agent – provides bacteriostatic eschar, then progressively epithelializes

    19. Operative Managment

    20. Operative management Abdominal pressures monitored Larger defects silo closure w/ progressive reduction Skin closure over defect, later ventral hernia repair

    21. Outcome Overal mortality 35% Survival > 90% if free of cardiac, chrom abnls Bowel function normal Most Morbidity and Mortality d/t associated abnormalities

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