Omphalocele vs gastroschisis

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

1. ABDOMINAL WALL DEFECTS OMPHALOCELE VS GASTROSCHISIS Rusila Tikoitoga MBBS 4 2016

2. OBJECTIVES Background Epidemiology Etiology Pathophysiology Clinical Features Diagnosis Management Prognosis

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. 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. Etiology Gastroschisis Congenital abdominal wall defect towards the right side of the umbilicus and protruded bowel is not covered by a membrane. Failure of migration and fusion of the lateral folds of the embryonic disc on the 3 rd -4 th week of gestation. Disruption of the right omphalomesenteric artery as midgut returns to abdomen by the 10 th week causing ischemia of the abdominal wall and weakness then herniation. Rupture of omphalocele Omphalocele Congenital abdominal wall defect with protrusion of abdominal viscera contained within a parietal peritoneum and amniotic membranous sac with Wharton’s jelly. Due to failure of the midgut to return to abdomen by the 10 th week of gestation during midgut rotation.

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. OMPHALOCELE GASTROSCHISIS

8. OMPHALOCELE GASTROSCHISIS

9. Embryology of GIT

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. 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. 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. 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. Management Perinatal Management Maternal Screening Fetal Ultrasound = positive findings Alpha-feto-protein elevated = 90% Omphalocele 10% Gastroschisis Prenatal counselling

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. Omphalocele Conservative Large omphalocele (10-12cm) apply topical application - Betadine ointment or silver sulfadiazine to the intact sac. Secondary eschar formation and granulation. Healing lasts for 12 months then repaired as ventral hernia. Primary Closure Small defects (<4cm) excision of the sac and closure of the fascia and skin over the abdominal contents M esh patch Medium defects (6-8cm)

17. Post operative care NICU Ventilation Feeding: Minimal volume 48 hrs Antibiotics Hernia dealt with at 1 yr old

18. Gastroschisis Primary closure If bowel easily reduced Staged closure Silo fashioning: Sac excised Silo sewn to rectus fascia/full thickness

19. Post operative care NICU Feeding delayed for weeks Oral stimulation/sucking reflex Broad spectrum antibiotics

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. Summary

22. References: Up to Date Medscape O.P Ghai E.pediatrics Rudolph’s pediatrics