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- 2. Background: Gastroschisis and omphalocele are among the most frequently encountered congenital anomalies in pediatric surgery. Combined
- 3. Background: Many babies have correctable lesions and simply require routine pediatric care. For others, the abdominal
- 4. Pathophysiology: Embryology the human embryo initially is disc-shaped and composed of 2 cell layers. It acquires
- 5. Pathophysiology: Embryology By the sixth week, rapid growth of the midgut causes a physiologic hernia of
- 6. Pathogenesis of omphalocele and gastroschisis Abdominal wall defects occur as a result of failure of the
- 7. Pathogenesis of Omphalocele In babies with omphalocele failure of central fusion at the umbilical ring by
- 8. Baby with an omphalocele.
- 9. Baby with a ruptured omphalocele.
- 10. Pathogenesis of gastroschisis Possible explanations of the embryology of abdominal wall defect in gastroschisis include the
- 11. Baby with an umbilical cord hernia.
- 12. Frequency: In the US: Combined incidence of omphalocele and gastroschisis is 1 in 2000 births. Incidence
- 13. Mortality/Morbidity: Over the past 30 years, the survival rate of babies with gastroschisis and omphalocele has
- 14. Mortality/Morbidity: Long-term morbidity from gastroschisis is related to intestinal dysfunction and wound problems. Short gut syndrome
- 15. Mortality/Morbidity: Management of babies with short gut syndrome also has improved significantly as a result of
- 16. Mortality/Morbidity: Poor healing of the abdominal wound usually results in a ventral hernia, which may require
- 17. Mortality/Morbidity: Even with successful repair, which usually requires a synthetic patch, and good clinical outcome, the
- 18. CLINICAL Physical: Omphalocele In babies with omphaloceles, the size of the abdominal wall defect ranges from
- 19. CLINICAL Babies with the Beckwith-Wiedemann syndrome (ie, macroglossia, gigantism) have large, rounded facial features, hypoglycemia from
- 20. Beckwith-Wiedemann syndrome
- 21. Baby with pentalogy of Cantrell
- 22. CLINICAL- Gastroschisis The defect is fairly uniform in size and location; a 5-cm vertical opening to
- 23. Baby with gastroschisis and associated intestinal atresia.
- 24. baby with gastroschisis and colon atresia.
- 25. Causes: Factors associated with high-risk pregnancies, such as maternal illness and infection, drug use, smoking, and
- 26. DIFFERENTIALS Other Problems to be Considered: In babies with omphalocele, a 35-80% incidence of other clinical
- 27. WORKUP Lab Studies: Maternal serum alpha-fetoprotein Prenatal diagnosis of abdominal wall defects can be made by
- 28. WORKUP Imaging Studies: Fetal sonography may detect a genetic abnormality, with identification of a structural marker
- 29. TREATMENT Medical Care: Intestinal inflammation Intestinal inflammation may occur with either gastroschisis or ruptured omphalocele. The
- 30. TREATMENT Intact omphalocele Usually, neonates with intact omphalocele are in no distress, unless associated pulmonary hypoplasia
- 31. TREATMENT The omphalocele should be supported to avoid excessive traction to the mesentery. Give prophylactic antibiotics
- 32. TREATMENT Gastroschisis Respiratory distress in neonates with gastroschisis may respond to gastric decompression, although endotracheal intubation
- 33. TREATMENT-Surgical Care: Omphalocele Ambroise Pare, the 17th-century French surgeon, accurately described omphalocele and the dire consequences
- 34. TREATMENT-Surgical Care: Healing may be hastened by surgically mobilizing skin flaps sufficient to cover the omphalocele
- 35. TREATMENT-Surgical Care Gastroschisis In 1969, Allen and Wrenn adapted Schuster’s technique for treatment of gastroschisis. Silastic
- 36. TREATMENT-Surgical Care In addition, tight closure of the abdominal cavity impedes venous return to the heart,
- 37. Consultations: Neonatologists and pediatric surgeons usually care for babies with these anomalies. Consult with cardiology, pulmonology,
- 38. Diet: Babies with omphalocele usually do not require special formulas; their intestines are typically normal, with
- 39. Activity: A child with a repaired giant omphalocele has an epigastric liver. In this location, the
- 40. FOLLOW-UP Further Inpatient Care: Omphalocele Babies with omphalocele usually have rapid return of intestinal function after
- 41. FOLLOW-UP Gastroschisis Even if primary closure of the abdominal wall defect is obtained, a period of
- 42. Silo closure of a baby with gastroschisis.
- 43. FOLLOW-UP Further Outpatient Care: After hospital discharge, babies require close follow-up care to assess growth and
- 44. FOLLOW-UP Transfer: The best way to treat the exposed intestines of a baby with gastroschisis who
- 45. The patient’s condition improved dramatically once closure of the abdominal cavity was achieved. Again, the author
- 46. Prognosis: Omphalocele Prognosis is dependent upon the severity of the associated problems. Babies with omphalocele are
- 47. Prognosis Gastroschisis Prognosis is dependent mainly upon severity of associated problems, including prematurity, intestinal atresia, short
- 48. Patient Education: Instruct parents regarding the significance of bilious (green) vomiting, since these babies may develop
- 49. Special Concerns: Prenatal care and planning With increased availability of sonography, prenatal diagnosis is more frequent.
- 50. Comparison of Gastroschisis and Omphalocele
- 51. Comparison of Gastroschisis and Omphalocele
- 52. Comparison of Gastroschisis and Omphalocele
- 53. Completed reduction of the bowel contained within the silo; the silo is about to be removed
- 54. Baby with a giant omphalocele.
- 55. Same patient as in slide 54. Closure of the giant omphalocele using a synthetic patch.
- 56. Same patient as in slides 54-55. Tightening the abdominal wall closure
- 57. Same patient as in slides 54-56. Flank flaps were used to close the giant omphalocele in
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