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Gastroschisis (also called paraomphalocele, laparoschisis or abdominoschisis) is a type of inherited[citation needed] congenital abdominal wall defect in which the intestines and sometimes other organs develop outside the fetal abdomen through an opening in the abdominal wall.
Commonly believed, this defect is the result of obstruction of the omphalomesenteric vessels during development. It is often detected through AFP screening or a detailed fetal ultrasound.
Omphalocele is a similar birth defect, but it involves the umbilical cord, and the organs remain enclosed in visceral peritoneum instead of penetrating through and becoming in direct contact with the amniotic fluid. Also, omphalocele had associated abnormality in around 50% of cases.
At least five hypotheses have been proposed:
(1) Failure of mesoderm to form in the body wall(Duhamel, 1963);
(2) Rupture of the amnion around the umbilical ring with subsequent herniation of bowel(Shaw, 1975);
(3) Abnormal involution of the right umbilical vein leading to weakening of the body wall and gut herniation(deVries, 1980);
(4) Disruption of the right vitelline (yolk sac) artery with subsequent body wall damage and gut herniation(Hoyme et al., 1981);
(5) Abnormal folding of the body wall results in a ventral body wall defect through which the gut herniates(Marcia et al., 2007)
The first hypothesis can’t explain why the mesoderm defect would occur in such specific small area; The second hypothesis can’t explain the low percentage of associated anormality compared with omphalocele; The third hypothesis was criticized due to no vascular supplement of anterior abdominal wall by umbilical vein. The fourth hypothesis was commonly accepted, but the right vitelline artery(right omphalomesenteric artery) did not supply the anterior abdominal wall in this area by recent demostration(Curry et al., 2006). However, more evidence was needed to justify the fifth hypothesis[1].
Gastroschisis as a stand-alone congenital defect is usually inherited in an autosomal recessive manner. It may begin as a sporadic mutation, can be associated with non-genetic congenital disorders, but has also been observed to be autosomal dominant.[2]
Genetic counseling and further genetic testing, such as amniocentesis, may be offered during the pregnancy, as this and other abdominal wall defects are associated with genetic disorders. If there are no additional genetic problems or birth defects, surgery soon after birth can often repair the opening.
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