Gastroschisis and Omphalocele

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Gastroschisis and Omphalocele
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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

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Gastroschisis

• bowel shortened
• thick, inflammatory peel
• 25 association w/ intestinal atresias
• Malrotation, meckels diverticulum, low birth
  weight also assoc

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Etiology

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

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Prenatal diagnosis
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Prenatal management

• Vaginal delivery OK
• Prognosis good d/t low associated anomalies
• Immediate Surgical Repair
• Delays d/t institutional transfers costly

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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

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Surgical repair

• Resection of infarcted bowel
• Primary anastamoses should not be performed
• Small bowel atresias can be left alone, repaired
  later

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Outcome

• Overall 85 survival
• complications
• Short gut syndrome
• Developmental delay
• Necrotizing Enterocolitis
• Cholestatic liver disease from prolonged TPN

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Omphalocele

• Central abdominal defect into which abdominal
  viscera can herniate
• Covered by sac

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Omphalocele

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

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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

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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
• Prognosis based on associated anomalies

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Prenatal Diagnosis
Abd wall
sac
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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

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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

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Operative Managment
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Operative management

• Abdominal pressures monitored
• Larger defects
• silo closure w/ progressive reduction
• Skin closure over defect, later ventral hernia
  repair

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Outcome

• Overal mortality 35
• Survival gt 90 if free of cardiac, chrom abnls
• Bowel function normal
• Most Morbidity and Mortality d/t associated
  abnormalities