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| Abdominal Wall Defects (Congenital) | ||
| Etiology • Abdominal wall defects include omphalocele, gastroschisis, limb-body wall complex and prune-belly syndrome. • Although grouped together, their etiology and pathogenesis may vary. • Omphalocele is associated with other anomalies (50%); many have trisomy 18; neural-tube defects are also common. • Gastroschisis and limb body wall complex is not associated with other anomalies or chromosome abnormalities. • Prune-Belly syndrome is associated with intestinal abnormalities. | ||
| Pathogenesis • Omphalocele is a defect at the umbilicus. • Gastroschisis is a defect lateral to the midline; theories include either ischemia (interruption of the omphalomesenteric artery). • Limb-body wall complex (body stalk anomaly) is a major disruption which may be due to rupture of the amniotic sac with adhesion to the placenta. Ischemia is also invoked. • Prune-belly is theorized to result either from a defect in the "mesoderm" or secondary to abdominal distention., | ||
| Epidemiology • Omphalocele most common; prune belly most abnormal. • Male=female • Associated with fetal loss. | ||
| General Gross Description • The intestine and other portions of the abdominal organs herniate omphalocele, limb-body wall complex and gastroschisis. • The major differentiation is the location of the defect (umbilical or lateral to the midline) and the associated anomalies. •Examples: | ||
| General Microscopic Description • Little microscopic abnormality. •Examples: | ||
| Clinical Correlation • Although frequently fatal in utero, gastroschisis and omphalocele can be repaired. • Morbidity and mortality revolve around associated abnormalities in the bowel and other organs. | ||
| References • Developmental Pathology of the Embryo and Fetus. Dimmick JE, Kalousek DK (eds). Philadelphia: Lippincott, 1992, pp. 527-9. Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window Abdominal Wall Defects (Congenital)
| Synopsis by: Melinda Sanders M.D. (T89000M28100)[613]
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