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ABDOMINAL WALL DEFECTS

ABDOMINAL WALL DEFECTS. Dr. Catherine B. Canete. OMPHALOCOELE. Anterior abdominal wall defect at the base of the umbilical cord with herniation of the umbilical contents. Incidence. Small omphalocoele 1:5000 Large omphalocoele 1:10000 Male to female ratio 1:1

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ABDOMINAL WALL DEFECTS

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  1. ABDOMINAL WALL DEFECTS Dr. Catherine B. Canete

  2. OMPHALOCOELE • Anterior abdominal wall defect at the base of the umbilical cord with herniation of the umbilical contents

  3. Incidence • Small omphalocoele 1:5000 • Large omphalocoele 1:10000 • Male to female ratio 1:1 • Pacific Islanders have low risk for omphalocoele

  4. Pathophysiology • Failure of the midgut to return to abdomen by the 10th week of gestation

  5. Clinical Findings • Covered clinical defect of the umbilical ring • Defect may vary from 2-10 cm • Sac is composed of amnion, Wharton’s jelly and peritoneum

  6. 50% have accompanying liver, spleen, testes/ovary • >50% have associated defects • Location: • Epigastric • Central • Hypogastric • Cord attachment is on the sac

  7. GASTROSCHISIS • Defect of the anterior abdominal wall just lateral to the umbilicus

  8. Incidence • 1:20,000-30,000 • Sex ratio 1:1 • 10-15% have associated anomalies • 40% are premature/SGA

  9. Pathophysiology • Abnormal involution of right umbilical vein • Rupture of a small omphalocoele • Failure of migration and fusion of the lateral folds of the embryonic disc on the 3rd-4th week of gestation

  10. Lateral folds • Form • Lateral abdominal wall • Future umbilical ring

  11. Clinical Findings • Defect to the right of an intact umbilical cord allowing extrusion of abdominal content • Opening  5 cm • No covering sac

  12. Bowels often thickened, matted and edematous • 10-15% with intestinal atresia

  13. 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

  14. Nutrition • NPO and TPN • Gastric Distention • OG/NG tube • Infection Control • Ampicillin and Gentamicin • Associated Defects

  15. Conservative treatment • Reduction by squeezing the sac • Painting sac with escharotic agent • 0.25% Silver nitrate • 0.25% Merbromin (Mercurochrome)

  16. Surgical Management • Skin Flaps • Primary Closure • Staged Closure • Staged repair using silo pouch

  17. Skin Flaps

  18. Primary Closure

  19. Staged Closure

  20. UMBILICAL HERNIA • Defect in linea alba, subcutaneous tissue and skin covering the protruding bowel • Frequent in premature infants

  21. PRUNE BELLY SYNDROME • Thin, flaccid abdominal wall • Dilation of bladder, ureter and renal collecting system • 1:30,000-50,000 • 95% are male

  22. BLADDER EXTROPHY • Defective enfolding of caudal folds • 3.3 in 100,000 births • Associated with prolapsed vagina or rectum, epispadias, bifid clitoris or penis

  23. PENTALOGY OF CANTRELL • Omphalocoele • Anterior diaphragmatic hernia • Sternal cleft • Ectopia Cordis • Intracardiac defect

  24. BECKWITH-WIEDEMANN SYNDROME • Macrosomia • Macroglossia • Organomegaly • Abdominal wall defects • Embryonal tumors

  25. Omphalocoele Gastroschisis

  26. Omphalocoele Gastroschisis

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