ABDOMINAL WALL DEFECTS

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ABDOMINAL WALL DEFECTS

• Dr. Catherine B. Canete

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OMPHALOCOELE

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

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Incidence

• Small omphalocoele 15000
• Large omphalocoele 110000
• Male to female ratio 11
• Pacific Islanders have low risk for omphalocoele

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Pathophysiology

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

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Clinical Findings

• Covered clinical defect of the umbilical ring
• Defect may vary from 2-10 cm
• Sac is composed of amnion, Whartons jelly and
  peritoneum

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• 50 have accompanying liver, spleen, testes/ovary
• gt50 have associated defects
• Location
• Epigastric
• Central
• Hypogastric
• Cord attachment is on the sac

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GASTROSCHISIS

• Defect of the anterior abdominal wall just
  lateral to the umbilicus

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Incidence

• 120,000-30,000
• Sex ratio 11
• 10-15 have associated anomalies
• 40 are premature/SGA

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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

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

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Clinical Findings

• Defect to the right of an intact umbilical cord
  allowing extrusion of abdominal content
• Opening ? 5 cm
• No covering sac

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

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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

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

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

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

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Skin Flaps
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Primary Closure
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Staged Closure
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UMBILICAL HERNIA

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

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PRUNE BELLY SYNDROME

• Thin, flaccid abdominal wall
• Dilation of bladder, ureter and renal collecting
  system
• 130,000-50,000
• 95 are male

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BLADDER EXTROPHY

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

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PENTALOGY OF CANTRELL

• Omphalocoele
• Anterior diaphragmatic hernia
• Sternal cleft
• Ectopia Cordis
• Intracardiac defect

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BECKWITH-WIEDEMANN SYNDROME

• Macrosomia
• Macroglossia
• Organomegaly
• Abdominal wall defects
• Embryonal tumors

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Omphalocoele Gastroschisis

Incidence 16,000-10,000 120,000-30,000
Delivery Vaginal or CS CS
Covering Sac Present Absent
Size of Defect Small or large Small
Cord Location Onto the sac On abdominal wall
Bowel Normal Edematous, matted
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Omphalocoele Gastroschisis

Other Organs Liver often out Rare
Prematurity 10-20 50-60
IUGR Less common Common
NEC If sac is ruptured 18
Associated Anomalies gt50 10-15
Treatment Often primary Often staged
Prognosis 20-70 70-90