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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 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 • Pacific Islanders have low risk for omphalocoele
Pathophysiology • Failure of the midgut to return to abdomen by the 10th week of gestation
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
50% have accompanying liver, spleen, testes/ovary • >50% have associated defects • Location: • Epigastric • Central • Hypogastric • Cord attachment is on the sac
GASTROSCHISIS • Defect of the anterior abdominal wall just lateral to the umbilicus
Incidence • 1:20,000-30,000 • Sex ratio 1:1 • 10-15% have associated anomalies • 40% are premature/SGA
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
Lateral folds • Form • Lateral abdominal wall • Future umbilical ring
Clinical Findings • Defect to the right of an intact umbilical cord allowing extrusion of abdominal content • Opening 5 cm • No covering sac
Bowels often thickened, matted and edematous • 10-15% with intestinal atresia
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
Nutrition • NPO and TPN • Gastric Distention • OG/NG tube • Infection Control • Ampicillin and Gentamicin • Associated Defects
Conservative treatment • Reduction by squeezing the sac • Painting sac with escharotic agent • 0.25% Silver nitrate • 0.25% Merbromin (Mercurochrome)
Surgical Management • Skin Flaps • Primary Closure • Staged Closure • Staged repair using silo pouch
UMBILICAL HERNIA • Defect in linea alba, subcutaneous tissue and skin covering the protruding bowel • Frequent in premature infants
PRUNE BELLY SYNDROME • Thin, flaccid abdominal wall • Dilation of bladder, ureter and renal collecting system • 1:30,000-50,000 • 95% are male
BLADDER EXTROPHY • Defective enfolding of caudal folds • 3.3 in 100,000 births • Associated with prolapsed vagina or rectum, epispadias, bifid clitoris or penis
PENTALOGY OF CANTRELL • Omphalocoele • Anterior diaphragmatic hernia • Sternal cleft • Ectopia Cordis • Intracardiac defect
BECKWITH-WIEDEMANN SYNDROME • Macrosomia • Macroglossia • Organomegaly • Abdominal wall defects • Embryonal tumors