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Omphalocele vs gastroschisis

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Paediatrics rotation MBBS Student Year 4 Fiji School of Medicine
CWM Hospital Fiji

Published in: Health & Medicine
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Omphalocele vs gastroschisis

  1. 1. ABDOMINAL WALL DEFECTS OMPHALOCELE VS GASTROSCHISIS Rusila Tikoitoga MBBS 4 2016
  2. 2. OBJECTIVES • Background • Epidemiology • Etiology • Pathophysiology • Clinical Features • Diagnosis • Management • Prognosis
  3. 3. History • 1634 - Ambroise Paré (French barber surgeon) first described Omphalocele. • Derived from Latin word “Omphalos” meaning prominence or navel. • 1733 – James Calder (Scottish neonatal surgeon) first described Gastroschisis. • Derived from the Greek word “Gaster”(Gastro) meaning belly and “schisis” meaning to tear or split from "
  4. 4. Epidemiology  Gastroschisis  Incidence - 4 per 10,000  M:F is 1:1 • 10-15% association with congenital anomalies such as CHD(VSD), cleft palate and intestinal atresia • 40% are premature/SGA  Omphalocele  Incidence - 3 per 5,000  M:F is 1.5:1  >70% association with congenital anomalies such Bowel atresia, Imperforated anus, Trisomies 13, 18, 21, Beckwith-Wiedemann Syndrome & Pentalogy of Cantrell
  5. 5. Etiology • Gastroschisis o Congenital abdominal wall defect towards the right side of the umbilicus and protruded bowel is not covered by a membrane. o Failure of migration and fusion of the lateral folds of the embryonic disc on the 3rd-4th week of gestation. o Disruption of the right omphalomesenteric artery as midgut returns to abdomen by the 10th week causing ischemia of the abdominal wall and weakness then herniation. o Rupture of omphalocele • Omphalocele o Congenital abdominal wall defect with protrusion of abdominal viscera contained within a parietal peritoneum and amniotic membranous sac with Wharton’s jelly. o Due to failure of the midgut to return to abdomen by the 10th week of gestation during midgut rotation.
  6. 6. Risk Factors Omphalocele • Increased maternal age • Twins • High gravida • Consecutive children Gastroschisis • Young maternal age • Low gravida • Prematurity • Low birth-weight secondary to IUGR
  7. 7. OMPHALOCELE GASTROSCHISIS
  8. 8. OMPHALOCELE GASTROSCHISIS
  9. 9. Embryology of GIT
  10. 10. Clinical Features OMPHALOCELE • central defect of the abdominal wall beneath the umbilical ring. • Defect may be 2-12 cm (Small-<5cm)(Large>8cm) • Always covered by sac • Sac is made of amnion, Wharton’s jelly and peritoneum • The umbilical cord inserts directly into the sac in an apical or lateral position. • Small contains intestinal loops only. Large may involve liver, spleen and bladder, testes/ovary • >50% have associated anomalies GASTROSCHISIS • Defect to the right of an intact umbilical cord allowing extrusion of abdominal content • Umbilical cord arises from normal place in abdominal wall • Opening <=5 cm • No covering sac (never has a sac ) • Evisceration usually only contains intestinal loops • Bowels often thickened, matted and edematous • 10-15% have associated anomalies • 40% are premature/SGA
  11. 11. Diagnosis • Alpha-feto-protein-synthesized in fetal liver and excreted by fetal kidneys and crosses placenta by 12 weeks. • Elevated maternal AFP - neural tube defects, abdominal wall defects, duodenal or esophageal atresia • 40% false positive rate • Fetal ultrasound after 14 weeks gestation is the confirmatory test.
  12. 12. Prenatal Ultrasound • 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 Gastroschisis
  13. 13. Prenatal Ultrasound • Umbilical cord insertion is typically midline on the mass • Located centrally • Contents are intestinal loops and maybe liver, spleen and gonads. Omphalocele
  14. 14. Management Perinatal Management • Maternal Screening  Fetal Ultrasound = positive findings  Alpha-feto-protein elevated = 90% Omphalocele 10% Gastroschisis • Prenatal counselling
  15. 15.  Pre-operative Management • ABC • Heat Management – Sterile wrap or sterile bowel bag – Radiant warmer • Fluid Management – IV bolus 20 ml/kg LR/NS – D10¼NS 2-3 maintenance rate • Nutrition – TPN (central venous line ) • Abdominal Distention – OG/NG tube – urinary catheter • Infection Control Broad-spectrum antibiotics - Ampicillin and Gentamycin • Closure of the Defect
  16. 16. Omphalocele • Conservative 1. Large omphalocele (10-12cm) apply topical application - Betadine ointment or silver sulfadiazine to the intact sac. 2. Secondary eschar formation and granulation. 3. Healing lasts for 12 months then repaired as ventral hernia. o Primary Closure  Small defects (<4cm)  excision of the sac and closure of the fascia and skin over the abdominal contents o Mesh patch  Medium defects (6-8cm)
  17. 17. • Post operative care o NICU o Ventilation o Feeding: – Minimal volume o 48 hrs Antibiotics o Hernia dealt with at 1 yr old
  18. 18. Gastroschisis • Primary closure o If bowel easily reduced • Staged closure o Silo fashioning: Sac excised Silo sewn to rectus fascia/full thickness
  19. 19. • Post operative care o NICU o Feeding delayed for weeks o Oral stimulation/sucking reflex o Broad spectrum antibiotics
  20. 20. Long Term Outcomes Omphalocele • Small - recover well • Large: – Gastro-oesophageal reflux - 43% – Majority improve over time – 20% pulmonary insufficiency – Respiratory Infections – Asthma – Feeding difficulties; • 60% with giant omphalocele • May need gastrostomy for feeding – Failure to thrive Gastroschisis • Generally excellent if no atresia • NEC: – 18.5% of neonates more with formula – Bowel loss - short gut syndrome • Cryptorchidism: – 15-30% – Due either being outside/prematurity – Replacement and orchidopexy by 1 yr • 60% have psychosocial stress if umbilicus sacrificed
  21. 21. Summary
  22. 22. References: • Up to Date • Medscape • O.P Ghai E.pediatrics • Rudolph’s pediatrics

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