This document discusses omphalocele and gastroschisis, two types of congenital abdominal wall defects. It begins with an outline and introduction defining the conditions. The embryology, risk factors, clinical features, investigations, differential diagnosis, management, complications and prognosis are then reviewed. It notes that advances in prenatal diagnosis and neonatal care have improved outcomes, though management protocols still lack consensus. The local experience is discussed in comparison to current treatment trends internationally.

1. BRIEFLY DISCUSS OMPHALOCELE
AND GASTROCHISIS
PRESENTER: EZEAKU CHIZOWA
OKWUCHUKWU
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2. OUTLINE
• INTRODUCTION
• EMBROLOGY
• AETIOPATHOGENESIS
• RISK FACTORS
• CLINICAL FEATURES
• DIFFRENTIAL DIAGNOSIS
• MANAGEMENT
• COMPLICATION
• PROGNOSIS
• LOCAL EXPERIENCE
• CURRENT TREND
• CONCLUSION
• REFERENCE
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3. INTRODUCTION
• Omphalocele and gastroschisis are the common forms of
presentation of congenital abdominal wall defect.
• Omphalocele is an anterior wall defect at the base of the
umbilical cord, with herniation of the abdominal content.
• Gastroschisis is a defect in the full anterior abdominal wall
through which the abdominal content protrudes into the
amniotic cavity.
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4. 4

5. Embyrology
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6. Embrology
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7. EPIDEMIOLOGY
• Omphalocele:
• Incidence :1:4,000 births
• General stable
• Male : female is 1.5:1.
• Gastrochisis
• Incidence: 1:2,000 births.
• On the increase in Western
world
• Male : female is 1:1.
• Combined incidence is 1:3,500 births
• No racial or geographic predilection
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8. RISK FACTORS
• OMPHALOCELE
• Increased maternal age
• Multiparity
• Family history
• GASTROCHISIS
• Young maternal age
• Substance abuse
• Low parity
• Prematurity
• Low birth weight
secondary to IUGR
• Tobacco use
• Low socioeconomic
status
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9. CLASSIFICATION
• Omphalocele can be classified:
– Location
– Shape
– Size of defect
– Content of the sac
– Associated anomalies
– State of the membrane
• Gastrochisis : (simple or complex)
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10. AETIOPATHOGENESIS
• GASTROCHISIS
– Failure of migration and
fusion of the lateral folds
– Disruption of the right
omphalomesenteric
artery
– Weakness in abdominal
wall
– Rupture of omphalocele
• OMPHALOCELE
– Failure of midgut to
return to the abdomen
by 10-12weeks
– Persistence of the body
stalk beyond 12weeks
– Failure of the lateral
mesodermal fold to
migrate centrally
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11. CLINICAL FEATURES(Gastroschisis)
• Defect (<5cm) on the right side of an intact umbilical cord. No
sac covering.
• Eviscerated bowel loops may be normal, thickened,
edematous or matted with fibrinous cover.
• May be associated hypothermia, dehydration, sepsis,
hypoglycaemia, bowel atresia, IUGR, birth asphyxia, pseudo
obstruction, malabsorption, midgut volvulus, short gut(closing
gastroschisis).
• Genetic abnormalities rarely occur, hirschsprung disease,.
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12. Inflammatory peel mimicking atresia
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13. CLINICAL FEATURES (Omphalocele)
• Defect (2-12cm)- central, epigastrium or hypogastrium with or
without sac(10-20%), with umbilicus at the apex.
• Content varies from intestinal loops only, to liver, spleen,
bladder, ovaries etc.
• Small , underdeveloped abdominal and thoracic cavities
• Associated anomalies(>70%) eg cardiac(14-47%), Beckwith-
Wiedemann syndrome, Pentalogy of Cantrell, Cloacal
exstrophy, bladder exstrophy, trisomies 12,13, 15, 18 and 21
etc
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15. INVESTIGATIONS
• Prenatal diagnosis via Obstetric scan-20wks
GA(polyhydramnios) & Maternal serum Alpha fetoprotein
• AIM: prenatal counseling and tertiary health care delivery
– Gastroschisis: Serial USS and amniocentesis(lung
maturation)
– Omphalocele: USS, Amniocentesis(genetic workup), fetal
ECHO
• Post natal:
– Urgent RBS, SEUcr, Abdominopelvic scan, ECHO, Chest Xray
and Chromosomal analysis
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16. DIFFERENTIAL DIAGNOSIS
• Umbilical hernia
• Prune belly syndrome
• Bladder exstrophy
• Urachal remnant
• Patent omphalomesenteric duct remnant
• Ectopia cordis
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17. Antenatal
• Delivery in a tertiary hospital
• Timing of delivery- preterm
• Mode of delivery
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18. TREATMENT
• Treatment and care of the general state of the baby;
• Specific treatment of the omphalocele and gastroschisis
• Management of associated anomalies.
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19. General state of the baby
• Airway, Breathing and Circulation
• Positioning and heat management (radiant warmer, sterile
wrap)
• Examination and care of exposed bowel
• Judicious fluid management
• Naso gastric decompression and urethral catheterization
• Nutrition (parenteral vs Enteral)
• Broad spectrum antibiotics
• Assisted ventilation
• Examination to rule out associated anomalies
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20. SPECIFIC TREATMENT(GASTROSCHISIS)
• Primary closure
– Monitoring of intrabdominal pressure as a guide.
• Application of silo and delayed primary closure
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22. Management of associated Atresia
• Done early or late(4-6weeks)
– Primary resection and anastomosis
– Creation of stoma and later repair
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23. Specific treatment(OMPHALOCELE)
• Aim
• Closure of omphalocele minor
– Primary closure
– Delayed primary closure
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24. Simulating an umbilicus
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26. SPECIFIC TREATMENT(OMPHALOCELE)
• Closure of omphalocele major
– Primary closure
– Staged abdominal wall closure
– Secondary abdominal wall closure
• (native body wall vs prosthetic material (Gor-Tex®, Surgisis®, Permacol™ )
– Application of silo vs(bogota bags, IV solution bag)
• Non irritant, non porous, transparent and non adhesive
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28. Staged abdominal wall closure
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29. Staged abdominal wall closure
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30. Use of bipedicled flank flaps
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31. Use of bipedicled flank flaps
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32. Conservative management
• Agents used include
• silver sulfadiazine, povidone iodine solution, silver impregnanted dressings,
neomycin and polymixin/ bacitracin ointment
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33. Conservative management
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34. POST OPERATIVE CARE
• Monitor for signs of intrabdominal compartment syndrome
and respiratory embarrasement.
• NG decompression and parenteral nutrition till passage of
“starvation stool”
• Role of prokinetics
• Early oral stimulation
• Assisted ventilation
• Broad spectrum antibiotics
• Patient education
• Regular followup
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35. COMPLICATIONS
• Sepsis
• Gastroesophageal reflux disease
• Necrotising enterocolitis
• Intestinal atresias
• Short gut syndrome
• Enterocutaneous fistulas
• Renal vein thrombosis
• Renal failure
• Respiratory failure
• Complications of total parenteral nutrition
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37. PROGNOSIS
• Long term morbidity for gastroschisis is related to
prematurity ,inflammatory bowel changes and short gut .
• Omphalocele is related to severity of associated anomalies
and size of thoracic cavity.
• Mortality of omphalocele relative to gastroschisis is 8 :1.
• Irreversible pulmonary hypertension / right heart failure is the
usual terminal condition.
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38. LOCAL EXPERIENCES VS CURRENT TREND
• Definitive repairs is a common trend locally,
• However advances in resuscitative measures such as
extracorporeal membrane oxygenation is lacking.
• Large omphalocele can be managed by tissues expanders.
• Bowel transplantation in cases of intestinal failure associated
liver disease is yet to be done in our region.
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40. CONCLUSION
• Omphalocele and gastroschisis are congenital abdominal wall
defects.
• Early intrauterine diagnosis, advances in neonatal care and
specialized surgical technique has help in improvement of
management.
• However there is presently no general consensus on
management protocol.
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41. REFERENCES
• Kokila L, etal: Congenital abdominal wall defect. Paediatric
surgery: A comprehensive text for Africa, Vol 1, 2011: pg 349-
351
• Saleem Islam: Congenital abdominal wall defect. Infections of
Liver. SRB manual of surgery, 4th edition, 2013: pg 660-669
• Shah R, etal. Gastroschsis and intestinal atresia. J Pediatric
Surg 1991;26; 788-90
• Vachharajani AJ, etal. Outcomes in neonatal gastrochisis, An
institutional experience. Am J Perinatol , 2007;24:461-5
• emedicine.medscape.com/article/Pediatric Omphalocele and
Gastroschisis
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