|Abdominal Wall Defects (Congenital)|
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.
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.,
Omphalocele most common; prune belly most abnormal.
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.
|General Microscopic Description|
Little microscopic abnormality.
Developmental Pathology of the Embryo and Fetus. Dimmick JE, Kalousek DK (eds). Philadelphia: Lippincott, 1992, pp. 527-9.
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|Abdominal Wall Defects (Congenital)
||Synopsis by: Melinda Sanders M.D. (T89000M28100)