Abdominal wall-defects


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Abdominal wall-defects

  1. 1. ABDOMINAL WALL DEFECTS Presented by Dr: Adam Mohammed August 2012
  2. 2. Introduction• 1634, Ambroise Paré first described an omphalocele.• Calder described a child with gastroschisis.
  3. 3. OMPHALOCOELE• It is a defect in abdominal wall musculature and skin with protrusion of abdominal viscera contained within a membranous sac.
  4. 4. OmphaloceleAbdominal wall defect
  5. 5. Incidence• Small omphalocoele 1:5000• Large omphalocoele 1:10000• Male to female ratio 1:1• Pacific Islanders have low risk for omphalocoele
  6. 6. Pathophysiology• Failure of the midgut to return to abdomen by the 10th week of gestation
  7. 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. 8. • 50% have accompanying liver, spleen, testes/ovary• >50% have associated defects• Location: – Epigastric – Central – Hypogastric• Cord attachment is on the sac
  9. 9. • The sac may rupture in utero in 10-18% or from the delivery process in4%.• The incidence of associated major congenital anomalies in up to 81%.
  10. 10. Defects of cranial fold• congenital heart disease• diaphragmatic hernia• ectopia cordis• sternal cleft,
  11. 11. Defects of caudad fold• imperforate anus• genitourinary malformations• bladder or cloacal exstrophy• colon atresia• sacral and vertebral anomalies, and• meningomyelocele.
  12. 12. GASTROSCHISIS• It is the defect in the abdominal wall was displaced to the right of the umbilicus and eviscerated bowel was not covered by a membrane.
  13. 13. GastroschisisAbdominal wall defect
  14. 14. Incidence• 1:20,000-30,000• Sex ratio 1:1• 10-15% have associated anomalies• 40% are premature/SGA
  15. 15. 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 3 rd- 4th week of gestation
  16. 16. Clinical Findings• Defect to the right of an intact umbilical cord allowing extrusion of abdominal content• Opening ≅ 5 cm• No covering sac
  17. 17. • Bowels often thickened, matted and edematous• 10-15% with intestinal atresia
  18. 18. • Evisceration of the bowel leads to malrotation.• Constriction of the base may cause intestinal stenosis, atresia, and volvulus• Undescended testicles• preterm or small for gestational age (SGA)
  19. 19. Causes• Folic acid deficiency• hypoxia• salicylates
  20. 20. Diagnosis• History : Prenatal U/S• Polyhydramnios• MSAFP• Amniocentesis
  21. 21. 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
  22. 22. • Nutrition – NPO and TPN (central venous line )• Gastric Distention – OG/NG tube – urinary catheter• Infection Control Broad-spectrum antibiotics• Associated Defects
  23. 23. • Conservative treatment – Reduction by squeezing the sac – Painting sac with escharotic agent • 0.25% Silver nitrate • 0.25% Merbromin (Mercurochrome)
  24. 24. • Surgical Management – Skin Flaps – Primary Closure – Staged Closure • Staged repair using silo pouch
  25. 25. Skin Flaps
  26. 26. Primary Closure• In 1967, Schuster technique• A circumferential incision along the skin- omphalocele junction; the omphalocele membrane is left intact• Teflon sheets• DualMesh patch (Gore-Tex)• AlloDerm patch (acellular human dermis)
  27. 27. Primary Closure
  28. 28. Staged ClosureIn 1969, Allen and Wrenn adapted Schusters technique to treat gastroschisisSilo
  29. 29. Staged Closure
  30. 30. UMBILICAL HERNIA• Defect in linea alba, subcutaneous tissue and skin covering the protruding bowel• Frequent in premature infants
  31. 31. PRUNE BELLY SYNDROME• Thin, flaccid abdominal wall• Dilation of bladder, ureter and renal collecting system• 1:30,000-50,000• 95% are male
  32. 32. BLADDER EXTROPHY• Defective enfolding of caudal folds• 3.3 in 100,000 births• Associated with prolapsed vagina or rectum, epispadias, bifid clitoris or penis
  33. 33. PENTALOGY OF CANTRELL• Omphalocoele• Anterior diaphragmatic hernia• Sternal cleft• Ectopia Cordis• Intracardiac defect
  34. 34. BECKWITH-WIEDEMANN SYNDROME • Macrosomia • Macroglossia • Organomegaly • Abdominal wall defects • Embryonal tumors
  35. 35. • Have coarse, rounded facial features• hyperplasia of the pancreatic islet cells with hypoglycemia; visceromegaly• genitourinary abnormalities
  36. 36. Omphalocoele GastroschisisIncidence 1:6,000-10,000 1:20,000-30,000Delivery Vaginal or CS CSCovering Sac Present AbsentSize of Defect Small or large SmallCord Location Onto the sac On abdominal wallBowel Normal Edematous, matted
  37. 37. Omphalocoele GastroschisisOther Organs Liver often out RarePrematurity 10-20% 50-60%IUGR Less common CommonNEC If sac is ruptured 18%Associated >50% 10-15%AnomaliesTreatment Often primary Often stagedPrognosis 20%-70% 70-90%
  38. 38. Baby with an umbilical cord hernia.
  39. 39. Baby with gastroschisis and associated intestinal atresia
  40. 40. Silo closure of a baby with gastroschisis.
  41. 41. Completed reduction of the bowel contained within the silo; the silo isabout to be removed and the abdominal wall closed.
  42. 42. Case A. Baby with a giant omphalocele.
  43. 43. Case A. Closure of the giant omphalocele using a synthetic patch
  44. 44. Case A. Tightening the abdominal wall closure
  45. 45. Case A. Flank flaps were used to close the giant omphalocele in the babywhose patch became infected.
  46. 46. Case A. The flank wounds were skin grafted and closure of the giantomphalocele obtained.
  47. 47. Baby with prune-belly syndrome.
  48. 48. Note the laxity of the abdominal wall in this baby with prune-bellysyndrome
  49. 49. Baby with cloacal exstrophy.
  50. 50. Note the bifid genitalia in this baby with cloacal exstrophy.
  51. 51. In the repair of cloacal exstrophy, the ileum in the middle of the bifidbladder is excised and used to create an ostomy, and the bladder halvesare approximated.
  52. 52. Closure of the bladder exstrophy.
  53. 53. Baby with bladder exstrophy and epispadias; note the appearance of thebladder mucosa, indicating chronic inflammation.
  54. 54. Another view demonstrating the epispadias shown in the previous image.
  55. 55. Baby with isolated epispadias.
  56. 56. Closure of a giant omphalocele with an AlloDerm patch
  57. 57. Two months after implantation: epithelialization of the AlloDerm patch.
  58. 58. Eight months after implantation: epithelization is nearly complete, but ahuge ventral hernia has developed
  59. 59. Baby with an omphalocele.
  60. 60. Complicated gastroschisis.
  61. 61. Following reduction of eviscerated viscera (and lysis of adhesions,tubularization of the viable, mesenteric portion of the proximal jejunum).
  62. 62. THANKS