The embryologic origin of ventral body wall defects.
Ventral body wall defects include ectopia cordis, bladder exstrophy, and the abdominal wall malformations gastroschisis and omphalocele. The etiology of ectopia cordis, gastroschisis, and bladder exstrophy is not known, but they may be linked to abnormalities in the lateral body wall folds responsible for closing the thoracic, abdominal, and pelvic portions of the ventral body wall. These folds form in the fourth week (postfertilization) of development as a combination of the parietal layer of lateral plate mesoderm and overlying ectoderm and must move ventrally to meet in the midline. There are differential rates of cell proliferation in the folds and asymmetries in their movement that may be involved in teratogenic effects of toxic factors. Also, the fusion process between the folds is complex, involving cell-to-cell adhesion, cell migration, and cell reorganization and all of these phenomena may be targets for disruption, leading to malformations. In this regard, closure of the ventral body wall is likened to neural tube closure and involves similar processes. It also encompasses a similar time frame during development, such that most neural tube and ventral body wall defects have their origins during the fourth week of development. Omphalocele is a separate entity whose etiology is known. This defect is attributed to a failure of gut loops to return to the body cavity after their normal physiological herniation into the umbilical cord from the 6th to 10th week of development. Thus, the origin of this defect is completely different from that of the ventral body wall malformations.
Sadler
Thomas W
TW
Department of Pediatrics, Division of Medical Genetics, University of Utah Health Sciences Center, Salt Lake City, Utah, USA. tsadler@3riversdbs.net
D034861:Abdominal Wall / Q000002:abnormalities* / Q000196:embryology*; D001746:Bladder Exstrophy / Q000196:embryology / Q000453:epidemiology / Q000503:physiopathology; D002657:Child Development / Q000502:physiology; D054083:Ectopia Cordis / Q000196:embryology / Q000453:epidemiology / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D020139:Gastroschisis / Q000196:embryology* / Q000453:epidemiology / Q000503:physiopathology; D006554:Hernia, Umbilical / Q000196:embryology / Q000453:epidemiology / Q000503:physiopathology; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D008297:Male