abdominal wall defects dr. catherine b. canete. omphalocoele anterior abdominal wall defect at the...
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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 1:5000
• Large omphalocoele 1:10000
• Male to female ratio 1:1
• 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, Wharton’s jelly and peritoneum
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• 50% have accompanying liver, spleen, testes/ovary
• >50% 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
• 1:20,000-30,000
• Sex ratio 1:1
• 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
• 1:30,000-50,000
• 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 GastroschisisIncidence 1:6,000-10,000 1:20,000-30,000
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 GastroschisisOther Organs Liver often out Rare
Prematurity 10-20% 50-60%
IUGR Less common Common
NEC If sac is ruptured 18%
Associated Anomalies
>50% 10-15%
Treatment Often primary Often staged
Prognosis 20%-70% 70-90%