ABDOMINAL WALL DEFECTS

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

7. 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

8. 50% have accompanying liver, spleen, testes/ovary • >50% have associated defects • Location: • Epigastric • Central • Hypogastric • Cord attachment is on the sac

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

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

12. 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

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

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

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

18. 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

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

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

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

23. Skin Flaps

24. Primary Closure

29. Staged Closure

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

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

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

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

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

41. Omphalocoele Gastroschisis

42. Omphalocoele Gastroschisis